Bio

Clinical Focus


  • Cardiac Surgery
  • Cardiothoracic Surgery, Pediatric
  • Thoracic Surgery

Academic Appointments


Honors & Awards


  • Lawrence Crowley, MD, Endowed Professorship in Child Health, Stanford (2004)
  • Excellence in Teaching Award in the Department of Surgery, University of California, San Francisco (1992-94)
  • Outstanding Surgical Chief Resident Award, University of California, San Francisco (1986)
  • Outstanding Resident Teaching Award, University of California, San Francisco (1986)
  • Alpha Omega Alpha medical society, University of California, San Francisco (1986)
  • Outstanding Graduating Student in Surgery (Martin J Loeb Award), Tufts Medical School (1978)

Professional Education


  • Medical Education:Tufts University (1978) MA
  • Fellowship:UCSF Medical Center (1988) CA
  • Residency:UCSF Medical Center (1986) CA
  • Board Certification: Thoracic Surgery, American Board of Thoracic Surgery (1990)
  • Internship:UCSF Medical Center (1979) CA

Research & Scholarship

Current Research and Scholarly Interests


Frank L. Hanley, MD, is a professor of cardiothoracic surgery and director of the Children’s Heart Center and also directs the pediatric cardiac surgery programs at three satellite surgical sites—Oakland Children’s Hospital of Central California in Madera and Sutter Memorial Hospital in Sacramento—making the expertise of Lucile Packard Children’s Hospital available to these communities.

His research and clinical work focuses on the development of interventional techniques for fetal and neonatal treatment of congenital heart disease, pulmonary, vascular physiology, and the neurologic impact of open-heart surgery. He developed and pioneered the “unifocalization” procedure, in which a single procedure is used to repair a complex and life-threatening congenital heart defect rather than several staged open-heart surgeries as performed by other surgeons. Currently, Lucile Packard Children’s Hospital is a worldwide referral site for patients requiring these procedures.

Hanley earned his BA from Brown University and his MD from Tufts University School of Medicine. He completed a surgical internship at the University of California, San Francisco (UCSF), where he later became chief resident in both general surgery and cardiothoracic surgery and completed a research fellowship at UCSF’s Cardiovascular Research Institute. Hanley was a tenured professor and chief of the Division of Cardiothoracic Surgery at UCSF and received the Excellence in Teaching Award in the Department of Surgery in 1994. Prior to joining UCSF, he was on the faculty at Children’s Hospital Boston and served as associate professor at Harvard Medical School from 1989 to 1992. He joined the Stanford faculty in 2001 and was appointed the first holder of the Lawrence Crowley, MD, Endowed Professorship in Child Health in December 2004.

Hanley is actively involved in exploring new approaches for the surgical repair of pediatric heart disease and is developing evidence-based guidelines for clinical care. He is a member of many professional societies, including the American Heart Association, the American Association for Thoracic Surgery, the Howard C. Naffziger Surgical Society, and the Society of Thoracic Surgeons. He is a frequently invited guest lecturer, having given more than 75 presentations at regional, national, and international conferences and symposiums, and having published more than 100 peer-reviewed articles and book chapters, and authored or edited three major textbooks.

Clinical Trials


  • Measuring the Amount of Methadone or Morphine in the Blood of Neonates, Infants & Children After Cardiac Surgery. Not Recruiting

    Study Population: Neonates, infants and children from birth to 5 years of age undergoing cardiac surgery with CPB. The use of methadone to provide analgesia may be increasing due to advantages compared to other commonly used opioid analgesic drugs. While efficacy of methadone has been reported in adults, there is a paucity of information in neonates and infants. In the latter population, fentanyl and morphine are most commonly used for opioid analgesia following major surgery, while methadone is frequently used for weaning of opioid dependent and tolerant patients, in spite of the paucity of knowledge of methadone pharmacology in this population. There are several clinical problems associated with fentanyl and morphine, and methadone may offer superior efficacy with fewer side effects than these agents. We propose to study the pharmacokinetics (PK) and pharmacodynamics (PD) of methadone in neonates and infants in the intensive care unit following cardiac surgery.

    Stanford is currently not accepting patients for this trial. For more information, please contact Gregory Hammer, (650) 723 - 7835.

    View full details

Teaching

2014-15 Courses


Publications

All Publications


  • Left Ventricular Retraining and Late Arterial Switch for D-Transposition of the Great Arteries ANNALS OF THORACIC SURGERY Watanabe, N., Mainwaring, R. D., Carrillo, S. A., Lui, G. K., Reddy, V. M., Hanley, F. L. 2015; 99 (5): 1655-1663

    Abstract

    For many decades, patients with d-transposition of the great arteries underwent an atrial switch procedure. Although many of these patients have continued to do well, a subset experience profound right ventricular failure. Some may be candidates for left ventricular (LV) retraining and late arterial switch. The purpose of this study was to review our experience with LV retraining and late arterial switch.This was a retrospective review of 32 patients with d-transposition. Thirty patients underwent a previous atrial switch and subsequently experienced right ventricular failure, whereas 2 presented late (8 months and 6 years) without previous intervention. The median age at the time of enrollment in this program was 15 years. Seven patients proceeded directly to late arterial switch owing to systemic LV pressures. The remaining 25 underwent a pulmonary artery band for LV retraining.Twenty of the 32 (63%) patients enrolled in this program were able to undergo a late arterial switch. There were 2 operative mortalities (10%). Two additional patients survived surgery but died in the early outpatient time period. There has been no late mortality after the arterial switch with a median follow-up of 5 years. Twelve patients underwent one or more pulmonary artery band procedures without evidence of effective LV retraining. There have been 2 early and 3 late (42%) deaths in this subgroup.The outcomes after arterial switch are encouraging and suggest that LV retraining and late arterial switch provide a viable option for this complex group of patients.

    View details for DOI 10.1016/j.athoracsur.2014.12.084

    View details for Web of Science ID 000353877900040

    View details for PubMedID 25817887

  • Fate of Right Ventricle to Pulmonary Artery Conduits After Complete Repair of Pulmonary Atresia and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Patrick, W. L., Punn, R., Palmon, M., Reddy, V. M., Hanley, F. L. 2015; 99 (5): 1685-1691

    Abstract

    Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) requires insertion of a conduit from the right ventricle to the reconstructed pulmonary arteries. Although there is extensive literature on conduit longevity for many forms of congenital heart defects, there is currently limited information for PA/VSD/MAPCAs. It is likely that conduits will have a different longevity in PA/VSD/MAPCAs because pulmonary artery pressures are higher after unifocalization than in other congenital heart defects. The purpose of this study was to evaluate the fate of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs.This was a retrospective review of 103 infants who underwent complete repair of PA/VSD/MAPCAs with insertion of an aortic homograft conduit. The average age at complete repair was 5.1 ± 2.8 months, average conduit size was 13 ± 2 mm, and average conduit z score was a positive 1.6 ± 0.9.Conduit z scores were linearly correlated with increasing homograft conduit size. The average longevity of small homografts (8 to 11 mm) was 15 ± 4 months, medium homografts (12 to 14 mm) was 39 ± 7 months, and large homografts (15 to 17 mm) was 50 ± 14 months. There was a negative correlation between increasing pulmonary artery pressure and conduit longevity.Conduit z scores and conduit size worked synergistically to predict the longevity of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs. Pulmonary artery pressure was an additional modifier of longevity. These data provide a framework for expectations of aortic homograft conduits used in the repair of PA/VSD/MAPCAs.

    View details for DOI 10.1016/j.athoracsur.2014.12.071

    View details for Web of Science ID 000353877900045

    View details for PubMedID 25805573

  • Contemporaneous comparison of the Yasui and Norwood procedures at a single institution. journal of thoracic and cardiovascular surgery Carrillo, S. A., Mainwaring, R. D., Schaffer, J. M., Wright, G., Maeda, K., Hanley, F. L., Reddy, V. M. 2015; 149 (2): 508-513

    Abstract

    It is recognized that there are numerous anatomic variants that result in hypoplastic left heart physiology. One such variant includes critical aortic stenosis or atresia, a hypoplastic aortic arch, and a reasonably well-developed left ventricle due to the presence of a ventricular septal defect. These patients are candidates for 1 of 3 surgical options: (1) a Norwood procedure followed by a single-ventricle pathway; (2) a Norwood procedure followed by a Rastelli procedure (2-stage Yasui); or (3) a single-stage Yasui procedure. Because 2 of the 3 options include a Norwood procedure as the initial step, the purpose of this study was to evaluate the contemporaneous results of the Yasui and Norwood procedures at a single institution.This was a retrospective review of patients who underwent a Yasui or Norwood procedure at Lucile Packard Children's Hospital between 2004 and 2013. Eighteen patients underwent a Yasui, of whom 15 had a single-stage procedure and 3 had a 2-stage procedure. During this time frame, 113 patients underwent a Norwood procedure. Kaplan-Meier survival curves and freedom from reoperation were compared for the 2 procedures.The operative mortality (using the Society of Thoracic Surgeons definition) for the single-stage Yasui was 6.7% compared with 16% for the Norwood procedure (P < .05); survival was 85% versus 62% at 1 year, 85% versus 60% at 3 years, and 85% versus 58% at 5 years, respectively (log-rank P = .06). The average interval to first reoperation was 13.5 ± 3 months versus 4.5 ± 1 months for the Yasui and Norwood procedures, respectively (P < .001).The Yasui procedure had a significantly lower operative mortality compared with the Norwood procedure. Early and midterm survival was also higher in the Yasui group versus the Norwood followed by a single ventricle pathway. These results indicate that the Yasui procedure has significant midterm benefits compared with the Norwood procedure and should be pursued when the anatomy is amenable for this approach.

    View details for DOI 10.1016/j.jtcvs.2014.09.120

    View details for PubMedID 25451485

  • Are preoperative B-type natriuretic peptide levels associated with outcome after pulmonary artery banding and the double switch operation in patients with congenitally corrected transposition of the great arteries: A pilot study JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Char, D. S., Shiboski, S. C., Hanley, F. L., Fineman, J. R. 2014; 148 (5): 2434-2436
  • Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Asija, R., Roth, S. J., Hanley, F. L., Peng, L., Liu, K., Abbott, J., Zhuo, H., Matthay, M. 2014; 148 (4): 1560-1565
  • Tetralogy of Fallot: aorto-pulmonary collaterals and pulmonary arteries have distinctly different transcriptomes PEDIATRIC RESEARCH Ma, X., Barboza, L. A., Siyahian, A., Reinhartz, O., Maeda, K., Reddy, V. M., Hanley, F. L., Riemer, R. K. 2014; 76 (4): 341-346
  • Coronary sinus obstruction after atrioventricular canal defect repair. Congenital heart disease Peng, D. M., Sun, H. Y., Hanley, F. L., Olson, I., Punn, R. 2014; 9 (4): E121-4

    Abstract

    The coronary sinus can become obstructed with any instrumentation at or near the ostium such as in atrioventricular canal defect repairs. This complication may lead to a wide range of consequences including dyspnea, angina, myocardial infarction, and sudden death. The following report illustrates the importance of careful perioperative echocardiographic evaluation of the coronary sinus in procedures that may affect the sinus and its ostium.

    View details for DOI 10.1111/chd.12096

    View details for PubMedID 23682752

  • Preoperative B-type natriuretic peptide levels are associated with outcome after total cavopulmonary connection (Fontan). journal of thoracic and cardiovascular surgery Radman, M., Keller, R. L., Oishi, P., Datar, S. A., Wellnitz, K., Azakie, A., Hanley, F., Char, D., Hsu, J., Amrinovin, R., Adatia, I., Fineman, J. R. 2014; 148 (1): 212-219

    Abstract

    The study objective was to determine the association between preoperative B-type natriuretic peptide levels and outcome after total cavopulmonary connection. Surgical palliation of univentricular cardiac defects requires a series of staged operations, ending in a total cavopulmonary connection. Although outcomes have improved, there remains an unpredictable risk of early total cavopulmonary connection takedown. The prediction of adverse postoperative outcomes is imprecise, despite an extensive preoperative evaluation.We prospectively enrolled 50 patients undergoing total cavopulmonary connection. We collected preoperative clinical data, preoperative plasma B-type natriuretic peptide levels, and postoperative outcomes, including the incidence of an adverse outcome within 1 year of surgery (defined as death, total cavopulmonary connection takedown, or the need for cardiac transplantation).The mean age of patients was 4.7 years (standard deviation, 2.1 years). The median (interquartile range) preoperative B-type natriuretic peptide levels were higher in patients who required total cavopulmonary connection takedown and early postoperative mechanical cardiac support (n = 3; median, 55; interquartile range, 42-121) compared with those with a good outcome (n = 47; median, 11; interquartile range, 5-17) (P < .05). A preoperative B-type natriuretic peptide level of 40 pg/mL or greater was highly associated with the need for total cavopulmonary connection takedown (sensitivity, 100%; specificity, 93%; P < .05), yielding a positive predictive value of 50% and a negative predictive value of 100%. Higher preoperative B-type natriuretic peptide levels also were associated with longer intensive care unit length of stay, longer hospital length of stay, and increased incidence of low cardiac output syndrome (P < .05).Preoperative B-type natriuretic peptide blood levels are uniquely associated with the need for mechanical support early after total cavopulmonary connection and total cavopulmonary connection takedown, and thus may provide important information in addition to the standard preoperative assessment.

    View details for DOI 10.1016/j.jtcvs.2013.08.009

    View details for PubMedID 24079880

  • Surgical repair of anomalous aortic origin of a coronary artery†. European journal of cardio-thoracic surgery Mainwaring, R. D., Reddy, V. M., Reinhartz, O., Petrossian, E., Punn, R., Hanley, F. L. 2014; 46 (1): 20-26

    Abstract

    Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischaemia and sudden death. There is an ongoing controversy over the indications for surgical intervention and the efficacy of that treatment compared with the natural history. The purpose of this study was to evaluate the medium-term results of surgical repair of AAOCA.Seventy-six patients underwent surgical repair of AAOCA at our institution from 1999 to 2013. There were 55 males and 21 females, and the median age at surgery was 15 years. Forty-seven (62%) of the 76 patients had an anomalous right coronary artery, 27 had an anomalous left coronary and 2 had an eccentric single coronary ostia. Forty-one patients had preoperative symptoms of myocardial ischaemia.Surgical repair was accomplished by unroofing of an intramural coronary in 55, reimplantation in 7 and pulmonary artery translocation in 14. There has been no early or late mortality, with a median duration of follow-up of 6 years. One patient presented with severe myocardial ischaemia and subsequently underwent heart transplantation a year following AAOCA surgery. The remaining patients have all remained free of cardiac symptoms.The results of this study demonstrate two major principles. First, surgical repair of AAOCA is quite safe in centres that take care of a significant number of patients with this entity. Secondly, the surgery is highly effective in eliminating symptoms of myocardial ischaemia. The growing amount of data on postoperative patients suggests that surgical repair can prevent the adverse events seen in the untreated 'natural' history. Based on these observations, it is our current recommendation that all teenagers identified with AAOCA should undergo surgical repair.

    View details for DOI 10.1093/ejcts/ezt614

    View details for PubMedID 24431169

  • Incidence and Risk Factors of Complete Atrioventricular Block after Operative Ventricular Septal Defect Repair CONGENITAL HEART DISEASE Siehr, S. L., Hanley, F. L., Reddy, V. M., Miyake, C. Y., Dubin, A. M. 2014; 9 (3): 211-215

    Abstract

    BACKGROUND: Complete atrioventricular block (AVB) is a recognized complication of ventricular septal defect (VSD) repair. The objective of this study was to examine incidence and risk factors. METHODS: This is a single-center, retrospective study. All pediatric patients (age <18 years) who underwent VSD repair between November 2001 and August 2009 with concordant atrioventricular and ventriculoarterial connections were included. Patients were classified as having no AVB or transient AVB, and outcomes of early pacemaker placement (before hospital discharge) or late pacemaker placement (after hospital discharge) were recorded. RESULTS: Eight hundred twenty-eight patients (48.6% female) underwent VSD repair during the study period. Mean age at repair was 14 ± 29.4 months. A total of 64 patients (7.7%) developed surgical AVB. Among those patients who developed AVB, 48 (75%) had transient AVB. Sixteen patients (1.9%) required a pacemaker, 14 early (88%) and 2 late (12%). Patients <4 kg (4.2% vs. 1%, P ≤ .01) and those with inlet VSDs (11.6% vs. 1.4%, P ≤ .01) were more likely to develop surgical AVB. Surgical AVB was not influenced by the presence of chromosomal abnormalities or other congenital heart disease. CONCLUSION: The overall incidence of surgical AVB is consistent with previous reports. Weight <4 kg and presence of an inlet VSD were significant risk factors.

    View details for DOI 10.1111/chd.12110

    View details for Web of Science ID 000337721700012

    View details for PubMedID 23764088

  • Early Complete Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Watanabe, N., Mainwaring, R. D., Reddy, V. M., Palmon, M., Hanley, F. L. 2014; 97 (3): 909-915

    Abstract

    Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex and diverse form of congenital heart defect. Although most patients with PA/VSD/MAPCAs can wait until they are 3 to 6 months of age to undergo surgical reconstruction, there are three specific criteria that merit an earlier repair. These 3 criteria are (1) unremitting heart failure; (2) a ductus to one lung and MAPCAs to the other; and (3) hemitruncus to one lung and MAPCAs to the other. The purpose of this study was to evaluate our surgical experience with early complete repair of PA/VSD/MAPCAs.This was a retrospective review of patients undergoing complete repair of PA/VSD/MAPCAs within the first 60 days of life. Twenty-seven patients were identified in our database (2002 to 2013) who met these criteria. Fifteen had congestive heart failure, 9 had a ductus plus MAPCAs, and 3 had hemitruncus plus MAPCAs. The median age at surgery was 5 weeks.There was no operative mortality in this cohort of 27 patients. Hemodynamics at the conclusion of the complete repair demonstrated an average right ventricular peak systolic pressure of 32 ± 5 mm Hg and an average right ventricle to aortic pressure ratio of 0.36 ± 0.06. The median length of hospital stay was 26 days. There have been 2 subsequent mortalities (7%), with a median follow-up duration of 4 years. Eight of the 27 patients have subsequently undergone conduit replacements at our institution. The hemodynamics at the conclusion of the conduit change were statistically unchanged compared with the hemodynamics after complete repair.The data demonstrate that early complete repair of PA/VSD/MAPCAs can be accomplished with low mortality and excellent postoperative hemodynamics. These early hemodynamic results are maintained at medium-term follow-up. We conclude that early complete repair is an appropriate choice for this highly select subgroup of patients.

    View details for DOI 10.1016/j.athoracsur.2013.10.115

    View details for Web of Science ID 000332408500036

    View details for PubMedID 24480261

  • Home monitoring program reduces interstage mortality after the modified Norwood procedure. journal of thoracic and cardiovascular surgery Siehr, S. L., Norris, J. K., Bushnell, J. A., Ramamoorthy, C., Reddy, V. M., Hanley, F. L., Wright, G. E. 2014; 147 (2): 718-23 e1

    Abstract

    BACKGROUND: From 2002 to 2005, the interstage mortality after a modified Norwood procedure was 7% in our program. An interstage home monitoring program (HMP) was established to identify Norwood procedure patients at increased risk of decompensation and to reduce interstage mortality. METHODS: Results of the first 5 years of the Norwood HMP were reviewed retrospectively. Interstage was defined as the time between Norwood hospital discharge and admission for second stage surgical palliation. In the HMP, families documented oxygen saturation, heart rate, weight, and feedings daily. Nurse practitioners called each family at least weekly, and when issues arose, action plans were determined based on symptom severity. RESULTS: Between October 2005 and October 2010 there were 46 Norwood procedure patients who survived to hospital discharge. All were enrolled in the HMP. Forty-five patients had a Norwood procedure with right ventricle to pulmonary artery conduit, and 1 patient had a modified Blalock-Taussig shunt. Interstage survival was 100%. Nineteen patients (41%) were admitted interstage; 5 patients were admitted twice, 1 patient was admitted 4 times. Seventeen patients (37%) required interstage interventions. Eight patients (17%) required major interventions: conduit stenting, aortic arch balloon angioplasty, emergent shunt, or early Glenn surgery. Minor interventions included supplemental oxygen, blood transfusion, intravenous hydration, diuresis, anti-arrhythmic therapy, or feeding adjustments. CONCLUSIONS: In the first 5 years of the HMP, all infants discharged after a modified Norwood procedure survived the interstage period. The HMP altered clinical management in 37% of patients. Home monitoring of oxygen saturation, heart rate, weight, and feedings, along with comprehensive care coordination, allowed timely interventions and reduced interstage mortality from 7% to 0%.

    View details for DOI 10.1016/j.jtcvs.2013.04.006

    View details for PubMedID 23663957

  • Home monitoring program reduces interstage mortality after the modified Norwood procedure JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Siehr, S. L., Norris, J. K., Bushnell, J. A., Ramamoorthy, C., Reddy, V. M., Hanley, F. L., Wright, G. E. 2014; 147 (2): 718-?
  • Atherosclerosis Causing Recurrent Catastrophic Aortopulmonary Shunt Dehiscence in a Patient with Alagille Syndrome PEDIATRIC CARDIOLOGY May, L., Hanley, F. L., Connolly, A. J., Reddy, S. 2013; 34 (8): 1945-1948

    Abstract

    Alagille syndrome (ALGS) is an autosomal dominant disorder associated with cholestatic liver disease, pulmonary valvar stenosis or atresia, vasculopathy, and renal disease. Although the liver and cardiac manifestations contribute to overall morbidity and mortality during their life span, these patients also carry a burden of important but often underappreciated vascular abnormalities. This report describes a 3 year-old girl with Alagille syndrome, hepatic cholestasis, systemic hypertension, hypercholesterolemia, hypertriglyceridemia, and tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs). She presented for bilateral pulmonary artery plasty and central shunt upsizing. She then experienced three shunt dehiscence episodes, necessitating emergent intervention. Autopsy showed diffuse atherosclerosis and significant atherosclerotic plaque at the site of shunt dehiscence. This is the first reported case of ALGS with TOF/PA/MAPCAs and catastrophic shunt dehiscence due to significant generalized vasculopathy caused by dyslipidemia and atherosclerosis. Dyslipidemia, a known comorbidity in ALGS, is one of few modifiable risk factors that should be screened for and treated, particularly before cardiac surgery.

    View details for DOI 10.1007/s00246-012-0484-4

    View details for Web of Science ID 000327065100039

  • Innominate artery cannulation and antegrade cerebral perfusion for aortic arch reconstruction in infants and children. World journal for pediatric & congenital heart surgery Nasirov, T., Mainwaring, R. D., Reddy, V. M., Sleasman, J., Margetson, T., Hanley, F. L. 2013; 4 (4): 356-361

    Abstract

    Innominate artery cannulation has been widely adopted as a means to perform aortic arch reconstruction with continuous cerebral perfusion in the newborn. Although this technique has been subsequently utilized in infants and children, there is currently no data regarding the safety or efficacy in these older children. The purpose of this study was to review our experience with innominate artery cannulation for aortic arch reconstruction in patients beyond the neonatal period.This was a retrospective review of 42 infants and children who underwent aortic arch reconstruction using the technique of innominate artery cannulation with continuous cerebral perfusion. Of these procedures, 29 (69%) were primary arch reconstructions, while 13 (31%) were aortic arch reoperations. The median age at surgery was 8 months, and 22 patients underwent concomitant intracardiac repairs.There was no operative mortality in this cohort of 42 patients undergoing aortic arch reconstruction. There was also no overt evidence of neurologic injury. Specifically, none of the patients demonstrated a neurologic deficit, clinical seizure, or unexpected delay in regaining a normal state of consciousness. The median duration of antegrade cerebral perfusion was 34 minutes. The median duration of hospital stay was 11 days. No patient required reoperation on the aortic arch with a median of 45-month follow-up.Innominate artery cannulation is a safe and effective technique for aortic arch reconstruction in nonneonates. We conclude that antegrade cerebral perfusion is a useful technique for aortic arch reconstruction in this patient population.

    View details for DOI 10.1177/2150135113497767

    View details for PubMedID 24327627

  • Fetal cardiac intervention: Improved results of fetal cardiac bypass in immature fetuses using the TinyPump device JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sebastian, V. A., Ferro, G., Kagawa, H., Nasirov, T., Maeda, K., Ferrier, W. T., Takatani, S., Riemer, R. K., Hanley, F. L., Reddy, V. M. 2013; 145 (6): 1460-1464

    Abstract

    Fetal cardiac surgery is a potential innovative treatment for certain congenital heart defects that have significant mortality and morbidity in utero or after birth, but it has been limited by placental dysfunction after fetal cardiac bypass. We have used the TinyPump device for fetal cardiac bypass in sheep fetuses at 90 to 110 days gestation.Ten mixed-breed pregnant ewes were used over a period of 6 months, and 10 fetuses were placed on bypass for 30 minutes. Five fetuses with a mean gestational age of 104 ± 4.5 days and mean weight of 1.4 ± 0.4 kg were placed on bypass using the TinyPump device, and 5 fetuses with a mean gestational age of 119 ± 4.5 days and mean weight of 3.4 ± 0.4 kg were placed on bypass using the roller head pump. The fetuses were monitored for up to 3 hours after bypass or until earlier demise.Progressive respiratory and metabolic acidosis developed in all fetuses. The TinyPump group had a lower gestational age and weight compared with the roller head pump group. However, the rate of postbypass deterioration in the TinyPump group, as measured with blood gases, was noted to be significantly slower compared with the roller head pump group.We demonstrate the feasibility of the TinyPump device for fetal cardiac bypass in a fetal sheep model. The TinyPump group showed improved results compared with the roller head group despite more immature fetuses. The TinyPump device seems to be a promising device for future studies of fetal cardiac bypass in immature fetal sheep and in primates.

    View details for DOI 10.1016/j.jtcvs.2012.08.014

    View details for Web of Science ID 000319066300024

  • Surgical management of neonatal atrioventricular septal defect with aortic arch obstruction. Annals of thoracic surgery Shuhaiber, J., Shin, A. Y., Gossett, J. G., Wypij, D., Backer, C. L., Hanley, F. L., Khan, M. S., Fraser, C. D., Jacques, F., Manning, P. B., Van Arsdell, G., Mayer, J. E., Costello, J. M. 2013; 95 (6): 2071-2077

    Abstract

    For neonates with atrioventricular septal defect and aortic arch obstruction including coarctation of the aorta, we sought to determine whether a difference in outcomes exists after a primary neonatal versus staged surgical repair (neonatal arch repair with delayed intracardiac repair).This retrospective cohort study included consecutive neonates with atrioventricular septal defect and aortic arch obstruction who underwent cardiac surgery before 28 days of age at six centers from 1990 to 2009. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared.Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. At baseline echocardiogram, a greater percentage of neonatal repair patients had relative unbalanced ventricular size (56% versus 35%, p = 0.02). There were no other differences in demographic characteristics, cardiac anatomical or functional details, or surgical technique. Those undergoing neonatal repair tended to be more likely to have at least moderate left atrioventricular valve regurgitation early after repair (42% versus 19%, p = 0.05) and to have at least one major in-hospital complication (42% versus 20%, p = 0.06). After the initial cardiac operation, compared with the neonatal repair group, patients undergoing staged repair had greater survival (87% versus 57% at 6 years, log-rank p = 0.02) and freedom from the first unplanned cardiac reoperation (69% versus 45% at 6 years, log-rank p = 0.005).For neonates with atrioventricular septal defect and aortic arch obstruction, when compared with neonatal repair, a staged approach was associated with improved survival and lower morbidity.

    View details for DOI 10.1016/j.athoracsur.2012.11.069

    View details for PubMedID 23415240

  • Aortic Valve Disease in Pulmonary Atresia and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Dimeling, G., Punn, R., Hanley, F. L. 2013; 95 (6): 2122-2125

    Abstract

    Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects.This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1.Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve.This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.

    View details for DOI 10.1016/j.athoracsur.2013.03.070

    View details for Web of Science ID 000319335400049

    View details for PubMedID 23643545

  • Postoperative Respiratory Failure in Children With Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals: A Pilot Study PEDIATRIC CRITICAL CARE MEDICINE Asija, R., Hanley, F. L., Roth, S. J. 2013; 14 (4): 384-389

    Abstract

    : Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs), who undergo unifocalization surgery are at risk for prolonged postoperative respiratory failure. We sought to identify risk factors that are associated with prolonged mechanical ventilation in this population.: We collected preoperative and operative data from medical records and postoperative data prospectively. Mechanical ventilation beyond postoperative day 5, which was greater than the 50th percentile for the subjects enrolled, was considered prolonged. Risk factors were analyzed using multiple logistic regression, independent samples t test, Fisher's exact test, and Kruskal-Wallis test. Enrollment occurred over a 20-month period between May 2009 and January 2011.: Lucile Packard Children's Hospital (Palo Alto, CA).: All patients with TOF/PA/MAPCAs presenting for unifocalization or pulmonary artery revision procedures were eligible, including those with additional structural cardiac abnormalities requiring surgical intervention. We excluded patients with single-ventricle cardiac anatomy and preoperative respiratory failure or infection.: We enrolled 35 consecutive patients undergoing 37 procedures over the study period. One patient was excluded for single-ventricle anatomy. There were 12 cases (32%) of prolonged mechanical ventilation. Delayed sternal closure was the only risk factor associated with prolonged mechanical ventilation (p = 0.01). Age, weight, cardiopulmonary bypass time, 22q11 microdeletion, postoperative fluid balance, bronchospasm, and nonrespiratory infection were not significantly associated with prolonged mechanical ventilation. Respiratory complications occurred in both groups, and patients with pneumonia were more likely to have a prolonged course (p = 0.03). There was no significant association between the type of surgery performed and duration of mechanical ventilation.: Prolonged postoperative respiratory failure in children undergoing surgery for TOF/PA/MAPCAs was independently associated with delayed sternal closure. Respiratory complications occur after unifocalization surgery, and pneumonia is associated with prolonged mechanical ventilation. Our pilot study suggests that clinical features common in this patient population, such as bronchospasm and 22q11 microdeletion, were not associated with more postoperative respiratory failure.

    View details for DOI 10.1097/PCC.0b013e318272062b

    View details for Web of Science ID 000318680000012

  • Surgical Reconstruction of Pulmonary Stenosis With Ventricular Septal Defect and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Punn, R., Reddy, V. M., Hanley, F. L. 2013; 95 (4): 1417-1421

    Abstract

    Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA.This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation.There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (-PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the -PBF group (p<0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the -PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the -PBF group (p<0.005).Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs.

    View details for DOI 10.1016/j.athoracsur.2013.01.007

    View details for Web of Science ID 000317150600039

    View details for PubMedID 23434256

  • Hemodynamic Assessment After Complete Repair of Pulmonary Atresia With Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Peng, L., Kuan, C., Palmon, M., Hanley, F. L. 2013; 95 (4): 1397-1402

    Abstract

    Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex form of congenital heart defect. There are limited data regarding late hemodynamics of patients after repair of PA/VSD/MAPCAs. This study evaluated the hemodynamics of patients who underwent complete repair of PA/VSD/MAPCSs and subsequently returned for a conduit change.This was a retrospective review of 80 children undergoing a right ventricle (RV)-to-pulmonary artery conduit replacement after complete repair of PA/VSD/MAPCAs. All patients underwent preoperative cardiac catheterization to define the cardiac physiology. Patients were an average age of 6.5±1.2 years, and the average interval between complete repair and conduit change was 4.5±1.1 years.The preoperative cardiac catheterization demonstrated an average RV right peak systolic pressure of 70±22 mm Hg and pulmonary artery pressure of 38±14 mm Hg. This pressure gradient of 32 mm Hg reflects the presence of conduit obstruction. After conduit change, the intraoperative RV systolic pressure was 34±8 mm Hg, similar to 36±9 mm Hg at the conclusion of the previous complete repair. The corresponding RV/aortic pressure ratios were 0.36±0.07 and 0.39±0.09, respectively.The data demonstrate that patients who underwent complete repair of PA/VSD/MAPCAs had nearly identical pulmonary artery pressures when they returned for conduit change some 4.5 years later. This finding indicates that the growth and development of the unifocalized pulmonary vascular bed is commensurate with visceral growth. We would hypothesize that complete repair, along with low RV pressures, will confer a long-term survival advantage.

    View details for DOI 10.1016/j.athoracsur.2012.12.066

    View details for Web of Science ID 000317150600036

  • MAJOR AORTOPULMONARY COLLATERAL ARTERY NOMENCLATURE Algaze, C. A., Peng, L. F., Feinstein, J. A., Reddy, V. M., Hanley, F. L., Perry, S. B. ELSEVIER SCIENCE INC. 2013: E543-E543
  • EXTRACARDIAC CONDUIT FONTAN PROCEDURE WITHOUT THE USE OF CARDIOPULMONARY BYPASS: EARLY OUTCOMES Algaze, C. A., Reddy, V. M., Hanley, F. L., Perry, S. B. ELSEVIER SCIENCE INC. 2013: E503-E503
  • Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Monge, M. C., Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. 2013; 145 (2): 476-481

    Abstract

    Peripheral pulmonary artery stenosis is a rare congenital heart defect frequently found in association with Williams and Alagille syndromes. Controversy exists regarding the optimal treatment of peripheral pulmonary artery stenosis, with most centers favoring catheter-based interventions. In contrast, we have preferentially used surgical reconstruction of peripheral pulmonary artery stenosis. The purpose of the present study was to review our experience with surgical reconstruction of peripheral pulmonary artery stenosis.We performed a retrospective review of patients who underwent surgical reconstruction of peripheral pulmonary artery stenosis. A total of 16 patients were identified: 7 had Williams syndrome, 6 had Alagille syndrome, and 3 had no identifiable syndrome. Detailed pulmonary angiography was performed in all patients to define stenoses at the main, branch, lobar, and segmental arterial levels. The mean preoperative right ventricular/left ventricular pressure ratio was 0.88 ± 0.07. The surgical approach was a median sternotomy with cardiopulmonary bypass. All peripheral stenoses were augmented with pulmonary artery homograft tissue. The median age at surgery was 14 months, and concomitant procedures were performed in 9 of the 16 patients.There was 1 operative mortality (6%). The mean right ventricular/left ventricular pressure ratio decreased to 0.40 ± 0.04 postoperatively (P < .005), representing a 55% reduction compared with the preoperative values. The patients were followed up for a median of 5 years. No late mortality occurred and reoperation was not required.The data have demonstrate that this comprehensive surgical approach to the treatment of peripheral pulmonary artery stenosis was associated with low early and no late mortality. Surgical reconstruction of the peripheral pulmonary artery stenosis resulted in a significant decrease in right ventricular pressure. We hypothesize that this reduction in right ventricular pressures will confer a long-term survival advantage for this cohort of patients.

    View details for DOI 10.1016/j.jtcvs.2012.09.102

    View details for Web of Science ID 000313634700030

  • Computational fluid dynamic simulations for determination of ventricular workload in aortic arch obstructions JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Coogan, J. S., Chan, F. P., LaDisa, J. F., Taylor, C. A., Hanley, F. L., Feinstein, J. A. 2013; 145 (2): 489-U535
  • Computational fluid dynamic simulations for determination of ventricular workload in aortic arch obstructions. journal of thoracic and cardiovascular surgery Coogan, J. S., Chan, F. P., LaDisa, J. F., Taylor, C. A., Hanley, F. L., Feinstein, J. A. 2013; 145 (2): 489-495 e1

    Abstract

    The cardiac workload associated with various types of aortic obstruction was determined using computational fluid dynamic simulations.Computed tomography image data were collected from 4 patients with 4 distinct types of aortic arch obstructions and 4 controls. The categorization of arch hypoplasia corresponded to the "A, B, C" nomenclature of arch interruption; a type "D" was added to represent diffuse arch hypoplasia. Measurements of the vessel diameter were compared against the normal measurements to determine the degree of narrowing. Three-dimensional models were created for each patient, and additional models were created for type A and B hypoplasia to represent 25%, 50%, and 75% diameter narrowing. The boundary conditions for the computational simulations were chosen to achieve realistic flow and pressures in the control cases. The simulations were then repeated after changing the boundary conditions to represent a range of cardiac and vascular adaptations. The resulting cardiac workload was compared with the control cases.Of the 4 patients investigated, 1 had aortic coarctation and 3 had aortic hypoplasia. The cardiac workload of the patients with 25% narrowing type A and B hypoplasia was not appreciably different from that of the control. When comparing the different arch obstructions, 75% type A, 50% type B, and 50% type D hypoplasia required a greater workload increase than 75% coarctation.The present study has determined the hemodynamic significance of aortic arch obstruction using computational simulations to calculate the cardiac workload. These results suggest that all types of hypoplasia pose more of a workload challenge than coarctation with an equivalent degree of narrowing.

    View details for DOI 10.1016/j.jtcvs.2012.03.051

    View details for PubMedID 22516390

  • Prevalence of Anomalous Coronary Arteries in Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals. Journal of the American College of Cardiology Muralidaran, A., Mainwaring, R. D., Reddy, V. M., Hanley, F. L. 2013

    View details for PubMedID 23871887

  • Early Outcomes After Extracardiac Conduit Fontan Operation Without Cardiopulmonary Bypass PEDIATRIC CARDIOLOGY McCammond, A. N., Kuo, K., Parikh, V. N., Abdullah, K., Balise, R., Hanley, F. L., Roth, S. J. 2012; 33 (7): 1078-1085

    Abstract

    Cardiopulmonary bypass is associated with a systemic inflammatory response. The authors hypothesized that avoiding cardiopulmonary bypass would lead to improved postoperative outcomes for patients undergoing the extracardiac Fontan operation, the final stage in surgical palliation of univentricular congenital heart defects. A review of the Children's Heart Center Database showed a total of 73 patients who underwent an initial Fontan operation at Lucile Packard Children's Hospital at Stanford between 1 November 2001 and 1 November 2006. These patients were divided into two groups: those who underwent cardiopulmonary bypass (n = 26) and those who avoided cardiopulmonary bypass (n = 47). Preoperative demographics, hemodynamics, and early postoperative outcomes were analyzed. The two groups had comparable preoperative demographic characteristics and hemodynamics except that the average weight of the off-bypass group was greater (17.9 ± 9.1 vs 14.2 ± 2.7 kg; P = 0.01). Intraoperatively, the off-bypass group trended toward a lower rate of Fontan fenestration (4.3 vs 19.2%; P = 0.09), had lower common atrial pressures (4.6 ± 1.4 vs 5.5 ± 1.5 mmHg; P = 0.05), and Fontan pressures (11.9 ± 2.1 vs 14.2 ± 2.4 mmHg; P ? 0.01), and required less blood product (59.1 ± 37.6 vs 91.9 ± 49.4 ml/kg; P ? 0.01). Postoperatively, there were no significant differences in hemodynamic parameters, postoperative colloid requirements, duration of mechanical ventilation, volume or duration of pleural drainage, or duration of cardiovascular intensive care unit or hospital stay. Avoiding cardiopulmonary bypass influenced intraoperative hemodynamics and the incidence of fenestration but did not have a significant impact on the early postoperative outcomes of children undergoing the Fontan procedure.

    View details for DOI 10.1007/s00246-012-0228-5

    View details for Web of Science ID 000308828200011

    View details for PubMedID 22349678

  • Late Outcomes in Patients Undergoing Aortopulmonary Window for Pulmonary Atresia/Stenosis and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Perry, S. B., Peng, L., Hanley, F. L. 2012; 94 (3): 842-849

    Abstract

    Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure.This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy.There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure.The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed.

    View details for DOI 10.1016/j.athoracsur.2012.03.061

    View details for Web of Science ID 000308138900031

    View details for PubMedID 22857982

  • Predictors for use of temporary epicardial pacing wires after pediatric cardiac surgery JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Gupta, P., Jines, P., Gossett, J. M., Maurille, M., Hanley, F. L., Reddy, V. M., Miyake, C. Y., Roth, S. J. 2012; 144 (3): 557-562

    Abstract

    The objectives of this study were (1) to determine the use of temporary epicardial pacing wires to diagnose and treat early postoperative arrhythmias in pediatric cardiac surgical patients and (2) to determine the predictive factors for the need of pacing wires for diagnostic or therapeutic purposes.We collected preoperative, intraoperative, and postoperative data in a prospective, observational format from patients undergoing pediatric cardiac surgery between August 2010 and January 2011 at a single academic children's hospital.A total of 157 patients met the inclusion criteria during the study period. Of these 157 patients, pacing wires were placed in 127 (81%). Pacing wires were used in 25 patients (19.6%) for diagnostic purposes, 26 patients (20.4%) for therapeutic purposes, 15 patients (11.8%) for both diagnostic and therapeutic purposes, and 36 patients (28.3%) for diagnostic or therapeutic purposes. Need for cardioversion in the operating room, presence of 2 or more intracardiac catheters, severely reduced ventricular ejection fraction, and elevated serum lactate level at the time of operating room discharge were found to be independent predictors for the use of pacing wires. The only complication noted in the cohort was a skin infection at a pacing wire insertion site in 1 patient. A permanent pacemaker was required in 8 (6.2%) of all patients with temporary pacing wires.Our data support the use of temporary epicardial pacing wires in approximately 30% of children after congenital heart surgery. We found the need for cardioversion in the operating room, presence of 2 or more intracardiac catheters, severely reduced ventricular ejection fraction, and high serum lactate level at the time of discharge from the operating room to be independent predictors of the use of pacing wires in the early postoperative period.

    View details for DOI 10.1016/j.jtcvs.2011.12.060

    View details for Web of Science ID 000308064200037

    View details for PubMedID 22329984

  • Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. OXFORD UNIV PRESS INC. 2012: 235-241

    Abstract

    Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.

    View details for DOI 10.1093/ejcts/ezr310

    View details for Web of Science ID 000306365600013

    View details for PubMedID 22402453

  • Midterm Results of the Modified Ross/Konno Procedure in Neonates and Infants ANNALS OF THORACIC SURGERY Maeda, K., Rizal, R. E., Lavrsen, M., Malhotra, S. P., Akram, S. A., Davies, R., Suleman, S., Reinhartz, O., Murphy, D. J., Hanley, F. L., Reddy, V. M. 2012; 94 (1): 156-163

    Abstract

    The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients.Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft.Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years.Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants.

    View details for DOI 10.1016/j.athoracsur.2012.03.007

    View details for Web of Science ID 000305801600033

    View details for PubMedID 22626750

  • Pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Maskatia, S. A., Feinstein, J. A., Newman, B., Hanley, F. L., Roth, S. J. 2012; 144 (1): 184-189

    Abstract

    The aims of our study are to describe the incidence, clinical profile, and risk factors for pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries. We hypothesized the following: (1) Pulmonary reperfusion injury is more likely to occur after unifocalization procedures in which a septated circulation is not achieved, (2) pulmonary reperfusion injury is directly related to the severity of stenosis in major aortopulmonary collateral arteries, and (3) pulmonary reperfusion injury leads to longer intubation time and longer hospitalization.Consecutive patients with tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries who underwent unifocalization procedures over a 5-year period were identified in our institutional database. Chest radiographs before the unifocalization procedure, from postoperative days 0 to 4, and from 2 weeks after the unifocalization procedure or at discharge were evaluated by a pediatric radiologist for localized pulmonary edema. Determination of stenosis severity was based on review of preoperative angiograms. Statistical analyses using multivariate repeated-measures analyses were performed with generalized estimating equations.Pulmonary reperfusion injury was present after 42 of 65 (65%) unifocalization procedures. In 36 of 42 cases of reperfusion injury, unilateral injury was present. Risk factors for the development of reperfusion injury included bilateral unifocalization (P = .01) and degree of stenosis (P = .03). We did not identify an association between pulmonary reperfusion injury and time to tracheal extubation or hospital discharge.Pulmonary reperfusion injury is common after the unifocalization procedure for tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries. Severity of stenosis and bilateral unifocalization are associated with the development of reperfusion injury.

    View details for DOI 10.1016/j.jtcvs.2011.12.030

    View details for Web of Science ID 000305412200031

    View details for PubMedID 22244564

  • Surgical Reconstruction of Tracheal Stenosis in Conjunction With Congenital Heart Defects Mainwaring, R. D., Shillingford, M., Davies, R., Koltai, P., Navaratnam, M., Reddy, V. M., Hanley, F. L. ELSEVIER SCIENCE INC. 2012: 1266-1273

    Abstract

    Surgical reconstruction is the primary method of treating airway obstruction in children. Tracheal stenosis is frequently associated with congenital heart defects, which may further complicate the overall management strategy. The purpose of this study was to review our experience with surgical reconstruction of airway obstruction in conjunction with congenital heart defects.This was a retrospective review of our surgical experience with tracheal stenosis from February 2003 to August 2011. Twenty-seven patients were identified in our database. Six patients had isolated, congenital tracheal stenosis, and 21 had tracheal stenosis in association with congenital heart defects. There were two identifiable subgroups. Thirteen patients had airway stenoses identified concurrently with congenital heart defects and underwent combined repair. The second group comprised 8 patients who had previous correction of their congenital heart defects and experienced delayed presentation of tracheal (n = 6) or bronchial (n = 2) obstruction.The median age at surgery was 9 months. There were 2 postoperative deaths, both in children with single ventricle. The median duration of follow-up for the entire cohort of 25 surviving patients was 4 years. None of the patients have required reoperations on the trachea; 5 have had minor reinterventions.The data demonstrate that tracheal obstruction is frequently found in conjunction with congenital heart defects. Nearly one third of our patients had delayed presentation of airway obstruction that was identified subsequent to previous congenital heart defect repair. Tracheal reconstructive techniques were effective regardless of the cause of the airway obstruction.

    View details for DOI 10.1016/j.athoracsur.2011.12.063

    View details for Web of Science ID 000302120200049

    View details for PubMedID 22381444

  • Reconstruction of Pulmonary Artery in a Newborn Using a Porcine Small Intestinal Submucosal Patch ANNALS OF THORACIC SURGERY Chalajour, F., Barboza, L. A., Boni, L., Snyder, R., Hanley, F. L., Reddy, V. M., Riemer, R. K. 2012; 93 (4): 1311-1315

    Abstract

    In this case report, we evaluated cellular structure and the growth potential of a porcine small intestinal submucosal patch used for pulmonary artery augmentation in a 20-day-old newborn with pulmonary atresia. The patch was resected 2 months postoperatively due to apparent abnormal wall thickening and evaluated by histologic and immunohistologic staining.

    View details for DOI 10.1016/j.athoracsur.2011.08.055

    View details for Web of Science ID 000302120200064

    View details for PubMedID 22450088

  • Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot ANNALS OF THORACIC SURGERY Mainwaring, R. D., Pirolli, T., Punn, R., Hanley, F. L. 2012; 93 (2): 677-679

    Abstract

    Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.

    View details for DOI 10.1016/j.athoracsur.2011.09.016

    View details for Web of Science ID 000299540200069

    View details for PubMedID 22269747

  • Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous Connection ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, M., Reinhartz, O., Punn, R., Tacy, T., Hanley, F. L. 2011; 92 (5): 1756-1760

    Abstract

    Pulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects.A retrospective review was performed to identify patients who had surgery for PA/MAPCAs in association with TAPVC. From November 2001 to March 2011, 9 patients presented with this combination of defects. Eight of the 9 patients had heterotaxy with an unbalanced atrioventricular canal and functional single ventricle. The ninth patient had double outlet right ventricle (two ventricles). Timing of surgical intervention was typically predicated on the degree of pulmonary venous obstruction. The median age at surgery was 14 days. All nine patients had surgical correction of TAPVC, unifocalization of MAPCA's into a central confluence, and placement of a shunt.There was one early mortality (< 30 days) and two late mortalities. For the 6 survivors, 5 have subsequently undergone a bidirectional Glenn procedure, and 3 had completion of their Fontan. Two patients are currently at the bidirectional Glenn stage; one is a good candidate for Fontan completion while the other is not suitable. The sixth patient is awaiting further assessment.The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis.

    View details for DOI 10.1016/j.athoracsur.2011.06.020

    View details for Web of Science ID 000296925400045

    View details for PubMedID 21944736

  • Anomalous Aortic Origin of a Coronary Artery: Medium-Term Results After Surgical Repair in 50 Patients ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Reinhartz, O., Petrossian, E., Macdonald, M., Nasirov, T., Miyake, C. Y., Hanley, F. L. 2011; 92 (2): 691-697

    Abstract

    Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischemia and sudden death. Controversies exist regarding the diagnosis, treatment, and long-term recommendations for patients with AAOCA. The purpose of this study is to evaluate the medium-term results of surgical repair for AAOCA.From January 1999 through August 2010, 50 patients underwent surgical repair of AAOCA. The median age at surgery was 14 years (range, 5 days to 47 years). Thirty-one patients had the right coronary originate from the left sinus of Valsalva, 17 had the left coronary originate from the right sinus, and 2 had an eccentric single coronary ostium. Twenty six of the 50 patients had symptoms of myocardial ischemia preoperatively, and 14 patients had associated congenital heart defects. Repair was accomplished by unroofing in 35, reimplantation in 6, and pulmonary artery translocation in 9.There was no operative mortality. The median time of follow-up has been 5.7 years. Two patients were lost to follow-up, and 1 patient required heart transplantation 1 year after AAOCA repair. In the remaining 47 postoperative patients, all have remained free of cardiac symptoms and no one has experienced a sudden death event.The surgical treatment of AAOCA is safe and appears to be highly effective in eliminating ischemic symptoms. These medium-term results are encouraging and suggest that many patients may be able to resume normal activities.

    View details for DOI 10.1016/j.athoracsur.2011.03.127

    View details for Web of Science ID 000293221000050

    View details for PubMedID 21718962

  • Can randomized clinical trials impact the surgical approach for hypoplastic left heart syndrome? World journal for pediatric & congenital heart surgery Tabbutt, S., Goldberg, C., Ohye, R. G., Morell, V. O., Hanley, F. L., Lamberti, J. J., Jacobs, M. L., Jacobs, J. P. 2011; 2 (3): 445-456

    Abstract

    The Eighth International Conference of the Pediatric Cardiac Intensive Care Society was held in Miami, Florida, December 8 to 11, 2010. The program included a session dedicated to the management of hypoplastic left heart syndrome (HLHS), with particular emphasis on the innovations that have led to contemporary schemes of management and the role of clinical trials in the evolution and acceptance of these strategies. An invited panel of experts reviewed the historical evolution of staged surgical reconstruction, the randomized clinical trials that have been undertaken thus far, and the extent to which these have, or have not, influenced individual and institutional approaches to management of HLHS.

    View details for DOI 10.1177/2150135111406942

    View details for PubMedID 23803996

  • Survival after extreme left atrial hypertension and pulmonary hemorrhage in an infant supported with extracorporeal membrane oxygenation for refractory atrial flutter PEDIATRIC CRITICAL CARE MEDICINE Cisco, M. J., Asija, R., Dubin, A. M., Perry, S. B., Hanley, F. L., Roth, S. J. 2011; 12 (3): E149-E152

    Abstract

    We report here the survival of an infant who developed extreme left atrial hypertension and severe pulmonary hemorrhage while supported with extracorporeal membrane oxygenation for refractory atrial flutter. The patient recovered after decompression of the left heart and catheter ablation of the atrioventricular node.Lucile Packard Children's Hospital (Stanford, CA).Chart review.Recovery of lung function is possible despite systemic-level left atrial pressure resulting in pulmonary hemorrhage and complete solidification of lung parenchyma on gross inspection. Resolution of pulmonary hemorrhage despite anticoagulation while on extracorporeal membrane oxygenation can occur after relief of left atrial hypertension.

    View details for DOI 10.1097/PCC.0b013e3181e8b3e5

    View details for Web of Science ID 000290248500008

    View details for PubMedID 20693934

  • PULMONARY REPERFUSION INJURY AFTER THE UNIFOCALIZATION PROCEDURE FOR TETRALOGY OF FALLOT, PULMONARY ATRESIA AND MAJOR AORTOPULMONARY COLLATERAL ARTERIES Maskatia, S., Feinstein, J. A., Newman, B., Hanley, F. L., Roth, S. J. ELSEVIER SCIENCE INC. 2011: E419-E419
  • The hybrid procedure for the borderline left ventricle CARDIOLOGY IN THE YOUNG Davis, C. K., Pastuszko, P., Lamberti, J., Moore, J., Hanley, F., El Said, H. 2011; 21 (1): 26-30

    Abstract

    IntroductionIn patients with varying degrees of left heart hypoplasia, it is often difficult to determine whether the left heart structures are adequate in size to support biventricular circulation. Historically, the decision to pursue a single ventricle or biventricular repair needed to be made early and was often irreversible. The hybrid procedure may be a better initial approach for patients with borderline left ventricles.We describe a series of four patients with various congenital cardiac malformations, all of whom had borderline left ventricles. Based on pre-operative echocardiograms, several scoring systems and left ventricle volumes were used to predict the optimal type of repair. A left ventricular volume of 20 millilitres per square metre was used as the minimum cut-off value for adequacy of biventricular repair.The left ventricular volumes for the patients were 17.1, 23.7, 25.4, and 25.8 millilitres per square metre. In none of the four patients were the calculations unanimous in the recommendation to pursue either type of repair. All patients underwent the hybrid procedure and then eventual single ventricle palliation (two patients) or biventricular repair (two patients). All survived with a mean follow-up of 18 plus or minus 3.9 months.The hybrid procedure may be the best option in patients with a borderline left ventricle. It can serve as a bridge to a more definitive repair when patients are older, larger, and for whom the decision between single ventricle and biventricular repair can be more easily made.

    View details for DOI 10.1017/S1047951110001423

    View details for Web of Science ID 000285981400005

    View details for PubMedID 21070689

  • The hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of the great arteries: Rationale and midterm results JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Malhotra, S. P., Reddy, V. M., Qiu, M., Pirolli, T. J., Barboza, L., Reinhartz, O., Hanley, F. L. 2011; 141 (1): 162-170

    Abstract

    This study was undertaken to assess the risks and benefits of the double-switch operation using a hemi-Mustard atrial switch procedure and the bidirectional Glenn operation for congenitally corrected transposition of the great arteries. To avoid complications associated with the complete Senning and Mustard procedures and to assist right-heart hemodynamics, we favor a modified atrial switch procedure, consisting of a hemi-Mustard procedure to baffle inferior vena caval return to the tricuspid valve in conjunction with a bidirectional Glenn operation.Between January 1994 and September 2009, anatomic repair was achieved in 48 patients. The Rastelli-atrial switch procedure was performed in 25 patients with pulmonary atresia and the arterial-atrial switch procedure was performed in 23 patients. A hemi-Mustard procedure was the atrial switch procedure for 70% (33/48) of anatomic repairs.There was 1 in-hospital death after anatomic repair. There were no late deaths or transplantation. At a median follow-up of 59.2 months, 43 of 47 survivors are in New York Heart Association class I. Bidirectional Glenn operation complications were uncommon (2/33), limited to the perioperative period, and seen in patients less than 4 months of age. Atrial baffle-related reoperations or sinus node dysfunction have not been observed. Tricuspid regurgitation decreased from a mean grade of 2.3 to 1.2 after repair (P = .00002). Right ventricle-pulmonary artery conduit longevity is significantly improved.We describe a 15-year experience with the double-switch operation using a modified atrial switch procedure with favorable midterm results. The risks of the hemi-mustard and bidirectional Glenn operation are minimal and are limited to a well-defined patient subset. The benefits include prolonged conduit life, reduced baffle- and sinus node-related complications, and technical simplicity.

    View details for DOI 10.1016/j.jtcvs.2010.08.063

    View details for Web of Science ID 000285407500029

    View details for PubMedID 21055773

  • Anomalous coronary arteries from the opposite sinus of Valsalva in asymptomatic siblings treated with pulmonary arterial translocation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Obayashi, D. Y., Yeh, J., Silverman, N. H., Hanley, F. L. 2010; 140 (6): 1430-1432

    View details for DOI 10.1016/j.jtcvs.2010.05.012

    View details for Web of Science ID 000284149200036

    View details for PubMedID 21078428

  • Cerebral Oxygen Metabolism During Total Body Flow and Antegrade Cerebral Perfusion at Deep and Moderate Hypothermia ARTIFICIAL ORGANS Sasaki, T., Boni, L., Riemer, R. K., Yeung, J. T., Ramamoorthy, C., Beckman, R., Gisner, C., Shuttleworth, P., Hanley, F. L., Reddy, V. M. 2010; 34 (11): 980-986

    Abstract

    The aim of this study is to evaluate the effect of temperature on cerebral oxygen metabolism at total body flow bypass and antegrade cerebral perfusion (ACP). Neonatal piglets were put on cardiopulmonary bypass (CPB) with the initial flow rate of 200mL/kg/min. After cooling to 18°C (n=6) or 25°C (n=7), flow was reduced to 100mL/kg/min (half-flow, HF) for 15min and ACP was initiated at 40mL/kg/min for 45min. Following rewarming, animals were weaned from bypass and survived for 4h. At baseline, HF, ACP, and 4?h post-CPB, cerebral blood flow (CBF) was measured using fluorescent microspheres. Cerebral oxygen extraction (CEO(2) ) and cerebral metabolic rate of oxygen (CMRO(2) ) were monitored. Regional cranial oxygen saturation (rSO(2) ) was continuously recorded throughout the procedure using near-infrared spectroscopy. At 18°C, CBF trended lower at HF and ACP and matched baseline after CPB. CEO(2) trended lower at HF and ACP, and trended higher after CPB compared with baseline. CMRO(2) at ACP matched that at HF. Cranial rSO(2) was significantly greater at HF and ACP (P<0.001, P<0.001) and matched baseline after CPB. At 25°C, CBF trended lower at HF, rebounded and trended higher at ACP, and matched baseline after CPB. CEO(2) was equal at HF and ACP and trended higher after CPB compared with baseline. CMRO(2) at ACP was greater than that at HF (P=0.001). Cranial rSO(2) was significantly greater at HF (P=0.01), equal at ACP, and lower after CPB (P=0.03). Lactate was significantly higher at all time points (P=0.036, P<0.001, and P<0.001). ACP provided sufficient oxygen to the brain at a total body flow rate of 100mL/kg/min at deep hypothermia. Although ACP provided minimum oxygenation to the brain which met the oxygen requirement, oxygen metabolism was altered during ACP at moderate hypothermia. ACP strategy at moderate hypothermia needs further investigation.

    View details for DOI 10.1111/j.1525-1594.2010.01131.x

    View details for Web of Science ID 000284588300019

    View details for PubMedID 21092040

  • Surgical management of life threatening events caused by intermittent aortic insufficiency in a native valve: case report JOURNAL OF CARDIOTHORACIC SURGERY Martin, M. H., Perry, S. B., Prochazka, J. V., Hanley, F. L., Silverman, N. H. 2010; 5

    Abstract

    We describe a case of a patient admitted with apparent life threatening events characterized by hypotension and bradycardia. The patient was ultimately found to have intermittent severe aortic insufficiency. Upon surgical exploration, abnormalities were discovered in the aortic valve, which had a small left coronary cusp with absence of the nodulus of Arantius. Following surgical repair of the valve, aimed at preventing the small cusp from becoming stuck in the open position, the patient has remained episode free for over one year.

    View details for DOI 10.1186/1749-8090-5-94

    View details for Web of Science ID 000284471500001

    View details for PubMedID 21034457

  • Optimal flow rate for antegrade cerebral perfusion JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sasaki, T., Tsuda, S., Riemer, R. K., Ramamoorthy, C., Reddy, V. M., Hanley, F. L. 2010; 139 (3): 530-535

    Abstract

    Antegrade cerebral perfusion is widely used in neonatal heart surgery, yet commonly used flow rates have never been standardized. The objective of this study was to determine the antegrade cerebral perfusion flow rate that most closely matches standard cardiopulmonary bypass conditions.Nine neonatal piglets underwent deep hypothermic cardiopulmonary bypass at a total body flow of 100 mL/kg/min (baseline). Antegrade cerebral perfusion was conducted via innominate artery cannulation at perfusion rates of 10, 30, and 50 mL/kg/min in random order. Cerebral blood flow was measured using fluorescent microspheres. Regional oxygen saturation and cerebral oxygen extraction were monitored.Cerebral blood flow was as follows: baseline, 60 +/- 17 mL/100 g/min; antegrade cerebral perfusion at 50 mL/kg/min, 56 +/- 17 mL/100 g/min; antegrade cerebral perfusion at 30 mL/kg/min, 36 +/- 9 mL/100 g/min; and antegrade cerebral perfusion at 10 mL/kg/min, 13 +/- 6 mL/100 g/min. At an antegrade cerebral perfusion rate of 50 mL/kg/min, cerebral blood flow matched baseline (P = .87), as did regional oxygen saturation (P = .13). Antegrade cerebral perfusion at 30 mL/kg/min provided approximately 60% of baseline cerebral blood flow (P < .002); however, regional oxygen saturation was equal to baseline (P = .93). Antegrade cerebral perfusion at 10 mL/kg/min provided 20% of baseline cerebral blood flow (P < .001) and a lower regional oxygen saturation than baseline (P = .011). Cerebral oxygen extraction at antegrade cerebral perfusion rates of 30 and 50 mL/kg/min was equal to baseline (P = .53, .48) but greater than baseline (P < .0001) at an antegrade cerebral perfusion rate of 10 mL/kg/min. The distributions of cerebral blood flow and regional oxygen saturation were equal in each brain hemisphere at all antegrade cerebral perfusion rates.Cerebral blood flow increased with antegrade cerebral perfusion rate. At an antegrade cerebral perfusion rate of 50 mL/kg/min, cerebral blood flow was equal to baseline, but regional oxygen saturation and cerebral oxygen extraction trends suggested more oxygenation than baseline. An antegrade cerebral perfusion rate of 30 mL/kg/min provided only 60% of baseline cerebral blood flow, but cerebral oxygen extraction and regional oxygen saturation were equal to baseline. An antegrade cerebral perfusion rate that closely matches standard cardiopulmonary bypass conditions is between 30 and 50 mL/kg/min.

    View details for DOI 10.1016/j.jtcvs.2009.12.005

    View details for Web of Science ID 000274735400002

    View details for PubMedID 20176202

  • Selective Right Ventricular Unloading and Novel Technical Concepts in Ebstein's Anomaly Malhotra, S. P., Petrossian, E., Reddy, V. M., Qiu, M., Maeda, K., Suleman, S., Macdonald, M., Reinhartz, O., Hanley, F. L. ELSEVIER SCIENCE INC. 2009: 1975-1981

    Abstract

    Favorable outcomes in Ebstein's anomaly are predicated on tricuspid valve competence and right ventricular function. Successful valve repair should be aggressively pursued to avoid the morbidity of prosthetic tricuspid valve replacement. We report our experience with valve-sparing intracardiac repair, emphasizing novel concepts and techniques of valve repair supplemented by selective bidirectional Glenn (BDG).Between June 1993 and December 2008, 57 nonneonatal patients underwent Ebstein's anomaly repairs. The median age at operation was 8.1 years. All were symptomatic in New York Heart Association (NYHA) functional class II (n = 38), III (n = 17), or IV (n = 1). Preoperatively, 26 had mild or moderate cyanosis at rest. We used a number of valve reconstructive techniques that differed substantially from those currently described. BDG was performed in 31 patients (55%) who met specific criteria.No early or late deaths occurred. At the initial repair, 3 patients received a prosthetic valve. Four patients required reoperation for severe tricuspid regurgitation. Repeat repairs were successful in 2 patients. At follow-up (range, 3 months to 6 years), all patients were acyanotic and in NYHA class I. Tricuspid regurgitation was mild or less in 49 (86%) and moderate in 6 (11%). Freedom from a prosthesis was 91% (52 of 57).Following a protocol using BDG for ventricular unloading in selected patients with Ebstein's anomaly can achieve a durable valve-sparing repair using the techniques described. Excellent functional midterm outcomes can be obtained with a selective one and a half ventricle approach to Ebstein's anomaly.

    View details for DOI 10.1016/j.athoracsur.2009.07.019

    View details for Web of Science ID 000272029100037

    View details for PubMedID 19932271

  • Deep Brain Hyperthermia While Rewarming from Hypothermic Circulatory Arrest JOURNAL OF CARDIAC SURGERY Amir, G., Ramamoorthy, C., Riemer, R. K., Hanley, F. L., Reddy, V. M. 2009; 24 (5): 606-610

    Abstract

    Neurologic injury is a feared and serious long-term complication of cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). Postoperative hyperthermia was found to enhance postischemic neurologic injury. The use of core temperature as the reference point through CPB assumes parallel changes in brain temperature. We tested the hypothesis that regional and deep brain temperature (DBT) differ during cooling, DHCA, and rewarming.Neonatal piglets (n = 9) were subject to CPB and cooled to rectal temperature (RT) of 18 degrees C, 30 minutes of DHCA were initiated, and subsequently the piglets were rewarmed to RT of 36.5 degrees C and weaned from CPB. Temperature probes were inserted into the DBT targeting the caudate and thalamic nuclei, their position confirmed by pathology. Superficial brain temperature was measured by a temperature probe inserted extradurally. RT, nasopharyngeal (NPT), and tympanic (TT) temperatures were recorded.During cooling the deep brain cooled faster and to lower temperatures compared to RT and TT; NPT reflected DBT accurately. During rewarming DBT was significantly higher than RT and TT. By the end of rewarming the difference between the deep brain and the RT reached statistical significance (30 minutes: 35.1 +/- 0.7 vs. 32.3 +/- 0.7 p < 0.05, respectively, 40 minutes: 37.5 +/- 0.3 vs. 34.7 +/- 0.8 p < 0.05, respectively).Deep brain hyperthermia routinely occurs during the last stages of rewarming following DHCA. DBT is accurately reflected by NPT and is directly correlated with inflow temperature. Therefore, during rewarming inflow temperatures should not exceed 36 degrees C and NPT should be closely monitored.

    View details for DOI 10.1111/j.1540-8191.2009.00883.x

    View details for Web of Science ID 000269540900034

    View details for PubMedID 19740304

  • Outcome Analysis for a Small, Start-up Congenital Heart Surgery Program JOURNAL OF CARDIAC SURGERY Mainwaring, R. D., Reddy, V. M., Reinhartz, O., Lamberti, J. J., Jacobson, J. G., Jimenez, D. L., Hanley, F. L. 2008; 23 (6): 622-626

    Abstract

    There will be a continuing need to start new congenital heart surgery programs to serve communities experiencing significant population growth. However, small congenital heart programs frequently underperform their larger counterparts. This study summarizes the clinical outcome data for the first 42 months of a small, start-up congenital heart surgery program. Clinical outcomes were summarized from the start of the program in September 2003 through March 2007. Risk adjustment analysis was performed using the risk adjustment in congenital heart surgery (Rachs-1) risk adjustment model and Society of Thoracic Surgeons (STS) congenital database. Three hundred eighty-six operations have been performed during this time period, including 47 newborns, 96 infants, 217 children/adolescents, and 26 adults (greater than 18 years). There have been two operative mortalities (0.5%). Assigning these cases to the Rachs-1 categories, there were 64 level I, 188 level II, 90 level III, 12 level IV cases, with 32 "others." The predicted mortality for the 354 categorized cases calculates to be 17.4 (Rachs-1) and 10.2 (STS). The data demonstrate that a start-up program with a relatively modest surgical volume can achieve satisfactory clinical results. This model has relied upon careful case selection and direct, senior-level surgeon involvement through an affiliation with a university-based program. These results suggest that a small congenital heart surgery program can be successfully started if the circumstances are carefully controlled.

    View details for DOI 10.1111/j.1540-8191.2008.00675.x

    View details for Web of Science ID 000260499400007

    View details for PubMedID 19016985

  • Dynamics of human myocardial progenitor cell Populations in the neonatal period ANNALS OF THORACIC SURGERY Amir, G., Ma, X., Reddy, V. M., Hanley, F. L., Reinhartz, O., Ramamoorthy, C., Riemer, R. K. 2008; 86 (4): 1311-1320

    Abstract

    Pluripotent cardiac progenitor cells resident in myocardium offer a potentially promising role in promoting recovery from injury. In pediatric congenital heart disease (CHD) patients, manipulation of resident progenitor cells may provide important new approaches to improving outcomes. Our study goals were to identify and quantitate populations of progenitor cells in human neonatal myocardium during the early postnatal period and determine the proliferative capacity of differentiated cardiac myocytes.Immunologic markers of cell lineage (stage-specific embryonic antigen 4 [SSEA-4], islet cell antigen 1 [Isl1], c-kit, Nkx2.5, sarcoplasmic reticulum calcium-regulated ATPase type 2 [SERCA2]) and proliferation (Ki67) were localized in right ventricular biopsies from 32 CHD patients aged 2 to 93 days.Neonatal myocardium contains progenitor cells and transitional cells expressing progenitor and differentiated myocyte marker proteins. Some cells expressed the pluripotent cell marker c-kit and also coexpressed the myocyte marker SERCA2. Multipotent progenitor cells, identified by the expression of Isl1, were found. Ki67 was expressed in some myocytes and in nonmyocyte cells. A few cells expressing SSEA-4 and Isl1 were observed during the early postnatal period. Cells expressing c-kit, the premyocyte marker Nkx2.5, and Ki67 were found throughout the first postnatal month. A progressive decline in cell density during the first postnatal month was observed for c-kit+ cells (p = 0.0013) and Nkx2.5+ cells (p = 0.0001). The percentage of cells expressing Ki67 declined during the first 3 postnatal months (p = 0.0030).Cells in an incomplete state of cardiomyocyte differentiation continue to reside in the infant heart. However, the relative density of progenitor cells declines during the first postnatal month.

    View details for DOI 10.1016/j.athoracsur.2008.06.058

    View details for Web of Science ID 000259848000036

    View details for PubMedID 18805183

  • Morphological studies of pulmonary arteriovenous shunting in a lamb model of superior cavopulmonary anastomosis PEDIATRIC CARDIOLOGY McMullan, D. M., Reddy, V. M., Gottliebson, W. M., Silverman, N. H., Perry, S. B., Chan, F., Hanley, F. L., Riemer, R. K. 2008; 29 (4): 706-712

    Abstract

    We sought to identify and characterize the abnormal vascular structures responsible for pulmonary arteriovenous shunting following the Glenn cavopulmonary shunt. Superior cavopulmonary shunt is commonly performed as part of the staged pathway to total cavopulmonary shunt to treat univentricular forms of congenital heart disease, however, clinically significant pulmonary arteriovenous malformations develop in some patients after the procedure. The causes of pulmonary arteriovenous malformations and other pulmonary vascular changes that occur after cavopulmonary shunt are not known. Using a juvenile lamb model of superior cavopulmonary anastomosis that reliably produces pulmonary arteriovenous malformations, we performed echocardiography and morphological analyses to determine the anatomic site of shunting and to identify the vascular structures involved. Pulmonary arteriovenous shunting was identified by contrast echocardiography in all surviving animals (n = 40) following superior cavopulmonary anastomosis. Pulmonary vascular corrosion casts revealed abnormal tortuous vessels joining pulmonary arteries and veins in cavopulmonary shunt animals but not control animals. In conclusion, unusual channels that bridged pulmonary arteries and veins were identified. These may represent the vascular structures responsible for arteriovenous shunting following the classic Glenn cavopulmonary shunt. Detailed analysis of these structures may elucidate factors responsible for their development.

    View details for DOI 10.1007/s00246-007-9152-5

    View details for Web of Science ID 000257393800003

    View details for PubMedID 18043856

  • Routine continuous perfusion for aortic arch reconstruction in the neonate. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Malhotra, S. P., Hanley, F. L. 2008: 57-60

    Abstract

    Continuous perfusion has evolved over the past 15 years as a viable cerebral protection strategy for neonatal aortic arch reconstruction. It presents an attractive alternative to deep hypothermic circulatory arrest. However, because of its relatively recent development, a standardized technique for its application is lacking. Here we describe our approach for continuous perfusion for repairs of the aortic arch based on experience with over 700 cases.

    View details for DOI 10.1053/j.pcsu.2007.12.004

    View details for PubMedID 18396226

  • Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Gulati, R., Reddy, V. M., Culbertson, C., Helton, G., Suleman, S., Reinhartz, O., Silverman, N., Hanley, F. L. 2007; 134 (5): 1171-U12

    Abstract

    Patients with a coronary artery arising from the wrong sinus are susceptible to ischemia and sudden death. Risk is higher when the artery courses interarterially--between the pulmonary artery and aorta--has an intramural course, or has an abnormal orifice. In single coronary ostium without intramural course, unroofing and coronary reimplantation are inappropriate, and coronary artery bypass grafting is suboptimal. For this variant, we have devised pulmonary artery translocation.A retrospective review of 18 patients undergoing repair between January 1999 and March 2005 was performed. Mean age was 8.1 years (range 6 weeks-16 years). All anomalous arteries coursed interarterially. Ten patients had a right coronary artery from the left coronary sinus; 8 had a left coronary artery from the right sinus. Eleven had an intramural course, and 4 had a single coronary ostium without an intramural course. Ten (56%) patients had symptoms: chest pain (9/10), syncope (3/10), or dyspnea (2/10). Repair was implemented by unroofing (n = 11), reimplantation (n = 3), or pulmonary artery translocation (1 lateral, 3 anterior). All patients were followed up clinically and echocardiographically.At a mean of 2.2 years (2 weeks-5 years), there was no mortality. Symptoms improved and function remained normal in all but 1 patient. He had sustained multiple infarcts in the anomalous artery's distribution and required transplantation despite repair.Repair is indicated in all patients with coronary insufficiency and in asymptomatic patients with high-risk morphologic abnormalities. We recommend unroofing when an intramural component (or slit-like orifice) is present, reimplantation for separate ostia without an intramural course, and pulmonary artery translocation for single ostium without an intramural course. Coronary artery bypass grafting is thus avoided.

    View details for DOI 10.1016/j.jtcvs.2007.02.051

    View details for Web of Science ID 000250576200011

    View details for PubMedID 17976445

  • Ductus-associated proximal pulmonary artery stenosis in patients with right heart obstruction INTERNATIONAL JOURNAL OF CARDIOLOGY Moon-Grady, A. J., Teltel, D. F., Hanley, F. L., Moore, P. 2007; 114 (1): 41-45

    Abstract

    Proximal pulmonary artery stenosis is a common acquired lesion in infants treated for congenital heart disease. We hypothesized that a large number of stenoses develop at the site of ductal insertion in patients with right ventricular outflow tract obstruction (RVOTO) and that these patients are at risk for developing hypoplasia of the ipsilateral pulmonary artery. The surgical and cardiac catheterization databases at our institution during the years 1988-2000 were searched for all patients under 1 year of age carrying a diagnosis of pulmonary atresia with intact ventricular septum (PA), tetralogy of Fallot (TOF) or pulmonary stenosis (PS), yielding 700 patients (62 PA, 373 TOF, 265 PS). The cardiac catheterization database was also searched for all patients with any diagnosis under 1 year of age found at catheterization to have proximal pulmonary artery stenosis. Proximal pulmonary artery stenosis associated with the ductal insertion site was diagnosed at catheterization in 33 infants (18 with PA, 5 with TOF, 6 with PS, 4 other diagnoses). This represents 29% of patients with PA, 1% with TOF and 2% with PS. Among patients with RVOTO and ductal insertion site-associated stenosis, there was a high prevalence (59%) of associated distal pulmonary arterial hypoplasia, defined as diameter of the stenosed vessel at first distal branch < or = 80% the diameter of the contralateral vessel. Symptomatology failed to identify this lesion; therefore, a high index of suspicion is necessary if proximal pulmonary artery stenosis is to be detected early in these patients.

    View details for DOI 10.1016/j.ijcard.2006.01.005

    View details for Web of Science ID 000243051900008

    View details for PubMedID 16644039

  • Visual light spectroscopy reflects flow-related changes in brain oxygenation during regional low-flow perfusion and deep hypothermic circulatory arrest JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Amir, G., Ramamoorthy, C., Riemer, R. K., Davis, C. R., Hanley, F. L., Reddy, V. M. 2006; 132 (6): 1307-1313

    Abstract

    Regional low-flow perfusion has been used to minimize ischemic brain injury during complex heart surgery in children. However, optimal regional low-flow perfusion remains undetermined. Visible light spectroscopy is a reliable method for continuous determination of capillary oxygen saturation (SgvO2). We used visible light spectroscopy to follow deep and superficial brain SgvO2 during cardiopulmonary bypass, regional low-flow perfusion, and deep hypothermic circulatory arrest.Visible light spectroscopy probes were inserted into the superficial and deep brain of neonatal (3.9-4.5 kg) piglets, targeting the caudate and thalamic nuclei. The piglets were subjected to cardiopulmonary bypass and cooled to a rectal temperature of 18 degrees C using pH stat. Regional low-flow perfusion was initiated through the innominate artery at 18 degrees C, and pump flows were adjusted to 40, 30, 20, and 10 mL/kg/min for 10-minute intervals followed by 30 minutes of deep hypothermic circulatory arrest. Regional low-flow perfusion was reestablished, and flows were increased in a stepwise manner from 10 to 40 mL/kg/min. SgvO2 was continuously monitored. Carotid flow was measured using a flow probe, and cerebral blood flow (milliliters per kilogram body weight per minute) was calculated.There were no significant differences between the deep and superficial brain tissue oxygenation during regional low flow brain perfusion before deep hypothermic circulatory arrest. However, after deep hypothermic circulatory arrest, the superficial brain SgvO2 was lower than the deep brain SgvO2 (24 +/- 12 vs 55.3 +/- 8, P = .05, at flows of 30 mL/kg/min, and 34.2 +/- 17 vs 62.5 + 8, P = .06, at a flow rate of 40 mL/kg/min). During regional low-flow perfusion, SgvO2 was maintained at flows of 30 to 40 mL/kg/min (cerebral blood flows of 15 to 21 mL/kg/min and 19 to 24 mL/kg/min, respectively), but was significantly lower at pump flows of 20 mL/kg/min (cerebral blood flow of 10 to 14 mL/kg/min) and 10 mL/kg/min (cerebral blood flow of 5 to 9 mL/kg/min) compared with the values obtained just before regional low-flow perfusion (pre-deep hypothermic circulatory arrest, 37 +/- 6 vs 65.5 +/- 4.4, P < .05, and 21.6 +/- 3.7 vs 65.5 +/- 4.4, P < .01, respectively; and post-deep hypothermic circulatory arrest, 32 +/- 4.5 vs 65.5 +/- 4.4, P < .05, and 16.6 +/- 4.7 vs 65.5 +/- 4.4, P < .01, respectively).Regional low-flow perfusion at pump flows of 30 to 40 mL/kg/min with resulting cerebral blood flows of 14 to 24 mL/kg/min was adequate in maintaining both deep and superficial brain oxygenation. However, lower pump flows of 20 and 10 mL/kg/min, associated with cerebral blood flow of 9 to 14 mL/kg/min, resulted in significantly reduced SgvO2 values.

    View details for DOI 10.1016/j.jtcvs.2006.04.056

    View details for Web of Science ID 000242626200012

    View details for PubMedID 17140947

  • The extracardiac conduit Fontan operation using minimal approach extracorporeal circulation: Early and midterm outcomes JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Petrossian, E., Reddy, V. M., Collins, K. K., Culbertson, C. B., MacDonald, M. J., Lamberti, J. J., Reinhartz, O., Mainwaring, R. D., Francis, P. D., Malhotra, S. P., Gremmels, D. B., Suleman, S., Hanley, F. L. 2006; 132 (5): 1054-1063

    Abstract

    Our approach to the extracardiac conduit Fontan operation has evolved over time from full-pump, to partial-pump, to completely off-pump. This study is designed to report our overall experience with the extracardiac conduit Fontan operation and to evaluate the evolution in bypass technique on postoperative outcomes.From September 1992 to April 2005, 285 patients, median age 4.5 years (1.4-44 years), median weight 16 kg (9.4-94 kg), underwent a primary extracardiac conduit Fontan procedure. Early and late outcomes were analyzed for the entire cohort and for 2 patient groups depending on whether an oxygenator was used in the bypass circuit (166 patients; 58%) or not (119 patients; 42%).Early failure (including death and takedown) occurred in 7 patients (2.5%). Prevalence of new early postoperative sinus node dysfunction necessitating a permanent pacemaker was 0.4%, and that of new tachyarrhythmias necessitating discharge home on a regimen of antiarrhythmia medications was 2.5%. Ten-year actuarial freedom from Fontan failure, new sinus node dysfunction necessitating a permanent pacemaker, and reoperation for conduit thrombosis or stenosis was 90%, 96%, and 98%, respectively. Fenestration rate was lower (P = .001) in the no-oxygenator group (8%) than in the oxygenator group (25%). Patients in the no-oxygenator group had lower intraoperative Fontan pressure (12.0 +/- 2.3 vs 13.5 +/- 2.4 mm Hg, P < .001), common atrial pressure (4.6 +/- 1.8 vs 5.3 +/- 1.8 mm Hg, P = .003), and transpulmonary gradient (7.5 +/- 2.1 vs 8.3 +/- 2.2 mm Hg, P = .013) than did the oxygenator group.The extracardiac conduit Fontan operation coupled with minimal use of extracorporeal circulation is associated with favorable intraoperative hemodynamics, low fenestration rate, minimal risk of thrombosis or stenosis, and minimal early and late rhythm disturbance.

    View details for DOI 10.1016/j.jtcvs.2006.05.066

    View details for Web of Science ID 000241497500009

    View details for PubMedID 17059923

  • Esophageal saturation during antegrade cerebral perfusion: a preliminary report using visible light spectroscopy PEDIATRIC ANESTHESIA Heninger, C., Ramamoorthy, C., Amir, G., Kamra, K., Reddy, V. M., Hanley, F. L., Brock-Utne, J. G. 2006; 16 (11): 1133-1137

    Abstract

    Visible light spectroscopy (VLS) is newer technology that measures real-time tissue oxygenation. It has been validated in detecting mucosal ischemia in adults. During complex neonatal heart surgery, antegrade cerebral perfusion (ACP) maintains cerebral saturation. Whether ACP maintains peripheral tissue perfusion in humans is not known.Five patients undergoing neonatal open heart surgery with hypothermic cardiopulmonary bypass (CPB) were studied using a VLS esophageal probe in addition to bilateral near infrared cerebral oximetry. Three of five patients required ACP for arch repair, while two patients did not. VLS and cerebral saturation data were collected and analyzed in 5 min intervals prior to CPB, during CPB, and during ACP.In the two patients undergoing heart surgery with routine hypothermic CPB, both cerebral and esophageal saturations were maintained. However in all three neonates requiring ACP, although cerebral saturations did not decrease, esophageal saturation fell below the ischemic threshold (35%). Following establishment of normal CPB, esophageal saturation returned to baseline.Antegrade cerebral perfusion maintains cerebral oxygen delivery, however, it does not adequately perfuse the esophagus in neonates. This could have clinical implications.

    View details for DOI 10.1111/j.1460-9592.2006.01965.x

    View details for Web of Science ID 000241245400004

    View details for PubMedID 17040301

  • Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: Two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies EUROPEAN JOURNAL OF ECHOCARDIOGRAPHY Greenway, S. C., Bradley, T. J., Caldarone, C. A., Silverman, N. H., Hanley, F. L., Smallhorn, J. F. 2006; 7 (5): 379-382

    Abstract

    This report describes two infants with an aortopulmonary window in association with anomalous origin of the right coronary artery from the pulmonary artery. In both cases the diagnosis was made pre-operatively by transthoracic echocardiography, with the initial clue being extensive collateral flow within the myocardium. In each case there was surgical confirmation of the echocardiographic findings. These two cases demonstrate that coronary artery evaluation should be an integral part of every new echocardiographic evaluation, particularly in the setting of conotruncal anomalies.

    View details for DOI 10.1016/j.euje.2005.10.010

    View details for Web of Science ID 000242966600007

    View details for PubMedID 16356774

  • Coarctation of the aorta in the right aortic arch with left aberrant innominate artery PEDIATRIC CARDIOLOGY Bein, S., Saba, Z., Patel, H., Reinhartz, O., Hanley, F. L. 2006; 27 (5): 621-623

    Abstract

    Right aortic arch with an aberrant left innominate artery is a rare combination. We report a unique case of coarctation of the aorta in a patient with right aortic arch and aberrant left innominate artery. The diverticulum of Kommerell gave rise to the left innominate artery and a left ligamentum arteriosum, completing a vascular ring. This unusual case raises interesting points regarding the development of coarctation in the context of a right-sided arch and the approach to management of these patients.

    View details for DOI 10.1007/s00246-006-1327-y

    View details for Web of Science ID 000240913400017

    View details for PubMedID 16933062

  • Unifocalization of major aortopulmonary collaterals in single-ventricle patients ANNALS OF THORACIC SURGERY Reinhartz, O., Reddy, V. M., Petrossian, E., Suleman, S., Mainwaring, R. D., Rosenthal, D. N., Feinstein, J. A., Gulati, R., Hanley, F. L. 2006; 82 (3): 934-939

    Abstract

    Unifocalization of major aortopulmonary collateral arteries (MAPCAs) in pulmonary atresia with ventricular septal defect and intracardiac repair has become the standard of care. However, there are no reports addressing unifocalization of MAPCAs in single-ventricle patients. It is unknown whether their pulmonary vascular bed can be reconstructed and low enough pulmonary vascular resistance achieved to allow for superior or total cavopulmonary connections.We reviewed data on all patients with functional single ventricles and unifocalization procedures of MAPCAs. From 1997 to 2005, 14 consecutive children with various single-ventricle anatomies were operated on.Patients had a median of three surgical procedures (range, 1 to 5). Two patients had absent, all others diminutive central pulmonary arteries, with an average of 3.5 +/- 1.2 MAPCAs. Seven patients (50%) had bidirectional Glenn procedures, and 3 of these had Fontan procedures. Median postoperative pulmonary artery pressures measured 12.5 mm Hg (Glenn) and 14 mm Hg (Fontan), respectively. Six patients are alive today (46%), with 1 patient lost to follow-up. Three patients died early and 3 late after initial unifocalization to shunts. One other patient survived unifocalization, but was not considered a candidate for a Glenn procedure and died after high-risk two-ventricle repair. Another patient with right-ventricle-dependent coronary circulation died of sepsis late after Glenn.In selected patients with functional single ventricles and MAPCAs, the pulmonary vascular bed can be reconstructed sufficiently to allow for cavopulmonary connections. Venous flow to the pulmonary vasculature decreases cardiac volume load and is likely to increase life expectancy and quality of life for these patients.

    View details for DOI 10.1016/j.athoracsur.2006.03.063

    View details for Web of Science ID 000239996300025

    View details for PubMedID 16928512

  • Homograft valved right ventricle to pulmonary artery conduit as a modification of the Norwood procedure CIRCULATION Reinhartz, O., Reddy, V. M., Petrossian, E., Macdonald, M., Lamberti, J. J., Roth, S. J., Wright, G. E., Perry, S. B., Suleman, S., Hanley, F. L. 2006; 114: I594-I599

    Abstract

    The use of a right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure has been proposed to increase postoperative hemodynamic stability. A valve within the conduit should further decrease RV volume load. We report our clinical experience with this modification.From February 2002 through August 2005, we performed 88 consecutive Norwood procedures using RV-PA conduits. We used composite valved conduits made from cryopreserved homograft and polytetrafluoroethylene (PTFE) in 66 cases (54 pulmonary, 12 aortic homografts), other valved conduits in 14, and unvalved PTFE in 8 cases. Hospital survival was 88.6% overall and increased to 93.1% after the initial year. Early interventions were required in 18 patients (16 for cyanosis). Prestage II cardiac catheterization was performed at a mean age of 126 days. Mean Qp/Qs was 1, with mean aortic saturation 71%, mean O2 extraction 24%, and mean right ventricular end-diastolic pressure 9 mm Hg. Patient weight, use of an aortic homograft valve in the conduit, stage I palliation within the first year of our experience, and low O2 extraction and high transpulmonary gradient prestage II were risk factors for overall death. Early interventions were more frequent in aortic valve conduits compared with all other conduits.The valved RV-PA conduit was associated with low early mortality after the Norwood procedure. The majority of these patients had normal cardiac output and well-maintained RV function. There may be a higher risk for early conduit interventions and death when aortic valve homografts are used in the RV-PA conduit.

    View details for DOI 10.1161/CIRCULATIONAHA.105.001438

    View details for Web of Science ID 000238688200097

    View details for PubMedID 16820644

  • MAPCAs, bronchials, monkeys, and men EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY Hanley, F. L. 2006; 29 (5): 643-644

    View details for DOI 10.1016/j.ejcts.2006.02.031

    View details for Web of Science ID 000237923200001

    View details for PubMedID 16626963

  • Profile of serum S-100 beta levels during maturation in fetal and neonatal sheep AMERICAN JOURNAL OF PERINATOLOGY Parry, A. J., McMullan, D. M., Yelich, S., Hanley, F. L. 2006; 23 (3): 153-157

    Abstract

    Serum levels of the protein S-100beta are dependent on three factors: rate of production, permeability of the blood-brain barrier, and rate of clearance. In the developing fetus and neonate all of these factors change at different rates. This study was performed to determine how serum S-100 levels varied during fetal and early postnatal life. Blood samples were obtained from 41 fetal and neonatal lambs. The blood was separated in a centrifuge and the serum drawn off and assayed for S-100beta using a commercially available radioimunoassay kit. S-100beta did not appear in the blood until halfway through pregnancy. Thereafter, levels steadily increased until 1 month after birth. Following this, S-100beta levels decreased progressively until by 1 year of age, they had reached a plateau. S-100beta levels change significantly with normal fetal and neonatal maturation. Valid interpretation of other data from subjects of similar developmental stage must take into consideration this physiological variation.

    View details for DOI 10.1055/s-2006-931914

    View details for Web of Science ID 000236848700002

    View details for PubMedID 16586228

  • Management of systemic venous anomalies in the pediatric cardiovascular surgical patient. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Gandy, K., Hanley, F. 2006: 63-74

    Abstract

    Systemic venous anomalies are rare and heterogeneous entities. Although these anomalies are rare in the general population, they occur more frequently in the subpopulation with congenital heart disease. In and of themselves, most of these lesions have no physiologic significance. However, in the setting of congenital heart disease these lesions may significantly alter surgical treatment. This review is dedicated to these lesions.

    View details for PubMedID 16638550

  • Effect of surgical case volume on outcome after the Norwood procedure JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY MacDonald, M. J., Reinhartz, O., Hanley, F. L. 2005; 130 (6): 1731-1731

    View details for DOI 10.1016/j.jtcvs.2005.06.028

    View details for Web of Science ID 000233896300046

    View details for PubMedID 16308032

  • Neonatal brain protection and deep hypothermic circulatory arrest: pathophysiology of ischemic neuronal injury and protective strategies ANNALS OF THORACIC SURGERY Amir, G., Ramamoorthy, C., Riemer, K., Reddy, V. M., Hanley, F. L. 2005; 80 (5): 1955-1964

    Abstract

    Deep hypothermic circulatory arrest (DHCA) has been used for the past 50 years in the surgical repair of complex congenital cardiac malformations and operations involving the aortic arch; it enables the surgeon to achieve precise anatomical reconstructions by creating a bloodless operative field. Nevertheless, DHCA has been associated with immediate and late neurodevelopmental morbidities. This review provides an overview of the pathophysiology of neonatal hypoxic brain injury after DHCA, focusing on cellular mechanisms of necrosis, apoptosis, and glutamate excitotoxicity. Techniques and strategies in neonatal brain protection include hypothermia, acid base blood gas management during cooling, and pharmacologic interventions such as the use of volatile anesthetics. Surgical techniques consist of intermittent cerebral perfusion during periods of circulatory arrest and continuous regional brain perfusion.

    View details for DOI 10.1016/j.athoracsur.2004.12.040

    View details for Web of Science ID 000232970500075

    View details for PubMedID 16242503

  • Religion, politics... deep hypothermic circulatory arrest JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Hanley, F. L. 2005; 130 (5): 1236-1241

    View details for DOI 10.1016/j.itcvs.2005.07.047

    View details for Web of Science ID 000233120100001

    View details for PubMedID 16256773

  • Homograft valved right ventricle to pulmonary artery conduit as a modification of the norwood procedure Reinhartz, O., Reddy, V. M., MacDonald, M. J., Lamberti, J. J., Petrossian, E., Perry, S. B., Suleman, S., Hanley, F. L. LIPPINCOTT WILLIAMS & WILKINS. 2005: U577-U577
  • Management of major aortopulmonary collateral arteries Hanley, F. L., Reinhartz, O., Suleman, S., Gremmels, D. B., Perry, S. B., Feinstein, J. A., Reddy, V. M. LIPPINCOTT WILLIAMS & WILKINS. 2005: U576-U576
  • Isolated unilateral absence of right proximal pulmonary artery: Surgical repair and follow-up ANNALS OF THORACIC SURGERY Welch, K., Hanley, F., Johnston, T., Cailes, C., Shah, M. J. 2005; 79 (4): 1399-1402

    Abstract

    The isolated unilateral absence of a proximal pulmonary artery is a rare congenital lesion with a diverse clinical presentation. If the connecting ductus arteriosus closes after birth, the ipsilateral pulmonary artery will lose its source of blood supply, resulting in hypoplasia or obliteration of intrapulmonary vessels. Despite a seemingly benign early clinical course, a significant number of untreated patients will develop pulmonary hypertension, hemoptysis, and recurrent respiratory infections. Early detection and surgical repair provides restoration of physiologic pulmonary circulation, regression of pulmonary hypertension, and the potential for normal distal pulmonary vascular development.

    View details for DOI 10.1016/j.athoracsur.2003.10.037

    View details for Web of Science ID 000228091100052

    View details for PubMedID 15797092

  • Preliminary results of fetal cardiac bypass in nonhuman primates JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Ikai, A., Riemer, R. K., Ramamoorthy, C., Malhatra, S., Cassorla, L., Amir, G., Hanley, F. L., Reddy, V. M. 2005; 129 (1): 175-181

    Abstract

    Fetal cardiac surgery has potential benefits for treatment of some congenital heart defects. However, placental dysfunction as a result of fetal bypass, fetal stress, and fetal exposure to external milieu needs to be overcome to optimize the outcomes of fetal cardiac bypass. In this study we evaluated the technical feasibility of cardiac bypass in the nonhuman primate fetus and the efficacy of different anesthetic approaches.Twelve baboon fetuses, average gestation 146 +/- 8 days and weight 696 +/- 184 g, were used. Three fetuses were excluded from the study because of nuchal cord presentations. The animals were separated into two anesthesia groups: isoflurane (n = 6) and fentanyl and midazolam (n = 3). A miniature roller pump circuit without oxygenator was used for fetal bypass for 30 minutes. No blood transfusion was performed. Fetal blood gas samples were collected before bypass, during bypass, and at 15 and 60 minutes after bypass.All fetuses in the isoflurane group were successfully placed on the cardiac bypass circuit. However, 2 animals in the fentanyl and midazolam group were not placed on the bypass circuit because of sustained elevation in maternal uterine tone. All maternal baboons survived. Of the 6 fetuses in the isoflurane group, 5 survived for 60 minutes; however, placental function continued to deteriorate after bypass (Pa o 2 33 +/- 3 mm Hg before bypass, 23 +/- 6 mm Hg 15 minutes after, and 18 +/- 9 mm Hg 60 minutes after).The technical feasibility of cardiac bypass in nonhuman primate fetuses weighing less than 1000 g was confirmed. Isoflurane anesthesia appears to be superior to fentanyl and midazolam anesthesia for fetal cardiac surgery because of adequate uterine relaxation.

    View details for DOI 10.1016/j.jtcvs.2004.09.003

    View details for Web of Science ID 000226216600024

    View details for PubMedID 15632840

  • Pulmonary arteriovenous shunting in the normal fetal lung JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McMullan, D. M., Hanley, F. L., Cohen, G. A., Portman, M. A., Riemer, R. K. 2004; 44 (7): 1497-1500

    Abstract

    We hypothesized that pulmonary arteriovenous shunting (PAVS) is normally present in fetal lungs and that cavopulmonary anastomosis-induced PAVS may represent a return to an earlier morphologic stage of development.The surgical superior cavopulmonary anastomosis is performed as part of the staged Fontan pathway to treat univentricular forms of congenital heart disease; PAVS is a known sequela after superior cavopulmonary anastomosis and may have important clinical consequences. Although the etiology and true morphology of the structures responsible for PAVS are unknown, a leading theory is that PAVS is caused by absence of normal hepatic venous drainage to the pulmonary circulation.To determine whether normal fetal lungs demonstrate PAVS, we performed contrast echocardiograms on 13 fetal lambs, 8 neonatal lambs, 4 juvenile lambs, and 4 adult sheep using a blended mixture of saline and blood injected directly into the proximal pulmonary artery.Pulmonary arteriovenous shunting was detected by direct epicardial echocardiography in all fetal lambs (n = 13) and neonatal animals studied at one and three days of life (n = 4) and in two of four animals studied at six to nine days of life. Pulmonary arteriovenous shunting was not present in animals studied at four weeks of life (n = 2) and in adult sheep (n = 5).These studies demonstrate that PAVS is normally present in late gestation fetal and early neonatal lambs but then disappears during the later neonatal period. Furthermore, these findings suggest that PAVS associated with cavopulmonary anastomosis or other processes affecting the developing pulmonary circulation may represent a return to an earlier morphologic stage of development.

    View details for DOI 10.1016/j.jacc.2004.06.064

    View details for Web of Science ID 000224225600025

    View details for PubMedID 15464334

  • Pulmonary expression of the hepatocyte growth factor receptor c-Met shifts from medial to intimal layer after cavopulmonary anastomosis JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Ikai, A., Riemer, R. K., Ma, X. Y., Reinhartz, O., Hanley, F. L., Reddy, V. M. 2004; 127 (5): 1442-1449

    Abstract

    Pulmonary arteriovenous malformations occur in up to 60% of patients after cavopulmonary anastomosis. We compared the effects of cavopulmonary anastomosis and pulmonary artery banding on lung gene expression in an ovine model to study the abnormal pulmonary vascular remodeling after the exclusion of inferior vena caval blood independent of reduced pulmonary blood flow. We previously demonstrated by contrast echocardiography that pulmonary arteriovenous malformations develop by 8 weeks after cavopulmonary anastomosis but not after pulmonary artery banding. Hepatocyte growth factor, a pleiotropic factor with morphogenic, mitogenic, and angiogenic activities, signals via its specific receptor c-Met to induce the antiapoptotic factor Bcl-2. In this study, we examined pulmonary artery expression of these factors and their potential role in pulmonary artery remodeling after cavopulmonary anastomosis and pulmonary artery banding.Eighteen lambs aged 35 to 45 days were placed into 3 groups: cavopulmonary anastomosis, pulmonary artery banding, and control (n = 6/group). In the cavopulmonary anastomosis group, the superior vena cava was anastomosed to the right pulmonary artery in an end-to-end fashion. In the pulmonary artery banding group, the left pulmonary artery was banded to reduce blood flow to 20% of control. The control group had a simple right pulmonary artery clamp for 30 minutes. Lung was harvested for Western blot, reverse transcriptase-polymerase chain reaction, and immunostaining at 2 weeks (n = 3/group) and 5 weeks (n = 3/group) after surgery.The expression of c-Met mRNA after cavopulmonary anastomosis was increased by twofold compared with the control or pulmonary artery banding group. The total lung expression of c-Met by Western blot was also up regulated at 2 weeks (P <.05). However, total lung expression of hepatocyte growth factor and Bcl-2 by Western and reverse transcriptase-polymerase chain reaction was not different from the control and pulmonary artery banding groups at both 2 and 5 weeks after surgery. Immunohistochemical analysis revealed that c-Met expression was localized to the intimal layer of the pulmonary artery in the cavopulmonary anastomosis, while its expression in the control and pulmonary artery banding lungs was localized to the medial layer. Localization of Bcl-2 on the intimal layer in lambs with cavopulmonary anastomosis followed the same pattern as c-Met.After cavopulmonary anastomosis, pulmonary artery expression of the hepatocyte growth factor receptor c-Met and one of its downstream effectors, Bcl-2, had increased in the intimal layer and decreased in the medial layer. Because the hepatocyte growth factor signaling promotes increased endothelial cell survival, it may have a role in pulmonary artery remodeling following cavopulmonary anastomosis. In addition, the change of c-Met expression in the medial layer after cavopulmonary anastomosis suggests a possible mechanism for the smooth muscle cell alteration related to abnormal angiogenesis.

    View details for DOI 10.1016/j.jtcvs.2003.09.009

    View details for Web of Science ID 000221134600031

    View details for PubMedID 15116006

  • A method for selectively limiting lumen diameter in corrosion casting MICROVASCULAR RESEARCH McMullan, D. M., Hanley, F. L., Riemer, R. K. 2004; 67 (3): 215-217

    Abstract

    Corrosion casting is a technique frequently used to evaluate the form and spatial relationship of three dimensional biological structures, such as vascular networks, in vitro [Scann. Microsc. 5 (1991) 1097; Schraufnagel, D.E. The lung microstructure. In: Motta, P.M., Murakami, T. Fujita, H. eds. Scanning Electron Microscopy of Vascular Casts: Methods and Applications. Boston: Kluwer Academic Publishers; 1992:123-137]. However, because corrosion casts tend to reproduce the complexity of surrounding vascular structures as well as the structures of interest, the use of this technique in highly complex vascular systems may obscure important changes such as A-V shunts because they may get lost among the myriad of capillaries. We developed a novel modification for creating vascular corrosion casts by utilizing polystyrene microspheres to selectively embolize normal capillary networks and thereby reduce the overall complexity of the cast. his technique may aid investigators in the evaluation of a variety of vascular beds and is useful in demonstrating non-capillary arteriovenous communications.

    View details for Web of Science ID 000221443700002

    View details for PubMedID 15121445

  • Electrical resychronization of failing right ventricle - Response CIRCULATION Dubin, A. M., Feinstein, J. A., Van Hare, G. F., Rosenthal, D. N., Reddy, V. M., Hanley, F. L. 2004; 109 (2): E5-E5
  • Effect of enalaprilat on postoperative hypertension after surgical repair of coarctation of the aorta. Pediatric critical care medicine Rouine-Rapp, K., Mello, D. M., Hanley, F. L., Mohan Reddy, V., Soifer, S. 2003; 4 (3): 327-332

    Abstract

    Hypertension in pediatric patients after surgical repair of coarctation of the aorta can be difficult to control and may lead to morbidity. The renin-angiotensin system mediates at least part of this hypertension. Enalaprilat, the only intravenous angiotensin-converting enzyme inhibitor, is used to treat hypertension in pediatric patients in other settings. However, its effect on postoperative hypertension during the early postoperative period in patients undergoing surgical repair of coarctation of the aorta is unknown.Prospective, randomized, double-blind study.Operating room and the pediatric intensive care unit.Fourteen consecutive pediatric patients between the ages of 1 and 18 yrs scheduled to undergo surgical repair of coarctation of the aorta.Patients were randomized to receive enalaprilat or saline placebo. Infusions were begun intraoperatively within 15 mins of aortic repair and repeated every 6 hrs.Plasma renin activity was measured at baseline and on postoperative day 1. Blood pressure was determined at 30 mins and at 2, 4, and 6 hrs after infusion and scored relative to the preoperative blood pressure. The blood pressure in the enalaprilat group was consistently lower at 30 mins, 2 hrs, and 4 hrs after infusion (p <.05), but not at 6 hrs. Plasma renin activity was significantly lower in the placebo group on postoperative day 1. Length of stay in the pediatric intensive care unit trended shorter in the treated group.Conclusions are limited by a small cohort. Angiotensin-converting enzyme inhibitor therapy resulted in improved blood pressure control after coarctation repair. Further improvement of blood pressure control may be achievable by use of a larger dose of enalaprilat or a 4-hr enalaprilat-dosing interval.

    View details for PubMedID 12831415

  • Induced fibrillation is equally effective as crystalloid cardioplegia in the protection of fetal myocardial function JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Malhotra, S. P., Thelitz, S., Riemer, R. K., Reddy, V. M., Suleman, S., Hanley, F. L. 2003; 125 (6): 1276-1282

    Abstract

    Fetal cardiac intervention represents a potential advance in the treatment of congenital cardiac lesions that increase in complexity during development. Prenatal repair of a primary defect might prevent pathologic blood-flow patterns that can result in hypoplasia of a cardiac chamber or great vessel. However, strategies to optimize fetal myocardial protection have not been studied. A biventricular working fetal heart preparation was used to evaluate the cardioprotective properties of induced fibrillation and crystalloid cardioplegia.Hearts from 16 fetal lambs at 115 to 125 days' gestation were harvested and perfused with Krebs-Henseleit solution. The descending aorta was ligated distal to the ductal insertion and the branch pulmonary arteries were ligated to simulate the parallel circulation of the fetus. Hearts were arrested with normothermic fibrillation (n = 8) or hypothermic crystalloid cardioplegia (n = 8) before reperfusion with Krebs-Henseleit solution. Baseline and postarrest myocardial function measurements were obtained from analysis of pressure-dimension relationships.Fibrillatory and cardioplegic arrest were equally effective at preserving postarrest systolic function (left ventricle, 70% +/- 5% vs 68% +/- 15%, P =.52; right ventricle, 68% +/- 4.5% vs 65% +/- 4.5%, P =.26) and preventing increased diastolic stiffness (left ventricle, 32% +/- 5.3% vs 38% +/- 11%, P =.24; right ventricle, 25% +/- 3.3% vs 27% +/- 2.1%, P =.46). Myocardial water content was unchanged in hearts arrested with fibrillation and cardioplegia (84% +/- 1.5% vs 83.7% +/- 0.9%, P =.71).Normothermic fibrillation and hypothermic crystalloid cardioplegia provide equal protection of the fetal myocardium. In the setting of diminished fetal myocardial reserve and because of the limited ability to manipulate the surrounding temperature in the fetus, normothermic fibrillation may be preferable for in utero repairs of selected congenital heart defects.

    View details for DOI 10.1016/S0022-5223(02)73245-5

    View details for Web of Science ID 000183864700012

    View details for PubMedID 12830044

  • Fetal myocardial protection is markedly improved by reduced cardioplegic calcium content ANNALS OF THORACIC SURGERY Malhotra, S. P., Thelitz, S., Riemer, R. K., Reddy, V. M., Suleman, S., Hanley, F. L. 2003; 75 (6): 1937-1941

    Abstract

    Fetal cardiac surgery holds a clear therapeutic benefit in the treatment of lesions that increase in complexity due to pathologic blood flow patterns during development. Fetal and neonatal myocardial physiology differ substantially, particularly in the regulation of myocardial calcium concentration. To examine issues of calcium homeostasis and fetal myocardial protection, a novel isolated biventricular working fetal heart preparation was developed.Hearts from 20 fetal lambs, 115 to 125 days gestation, were harvested and perfused with standard Krebs-Henseleit (K-H) solution. The descending aorta was ligated distal to the ductal insertion and the branch pulmonary arteries were ligated to mimic fetal cardiovascular physiology. Hearts were arrested for 30 minutes with normocalcemic (n = 8), hypocalcemic (n = 6), or hypercalcemic (n = 6) cold crystalloid cardioplegia before reperfusion with K-H solution.Compared with normocalcemic cardioplegia, hypocalcemic cardioplegia improved preservation of left ventricular (LV) systolic function (88% +/- 2.2% vs 64% +/- 15% recovery of end-systolic elastance, p = 0.02), diastolic function (12% +/- 21% vs 38% +/- 11% increase in end-diastolic stiffness, p = 0.04), and myocardial contractility (97% +/- 9.6% vs 75.2% +/- 13% recovery of preload recruitable stroke work [PRSW], p = 0.04). In contrast, the fetal myocardium was sensitive to hypercalcemic arrest with poor preservation of LV systolic function (37.5% +/- 8.4% recovery of elastance), diastolic function (86% +/- 21% increased stiffness), and overall contractility (32% +/- 13% recovery of PRSW). Myocardial water content was reduced in hearts arrested with hypocalcemic cardioplegia (79% +/- 1.8% vs 83.7% +/- 0.9%, p = 0.0006).This study demonstrates the sensitivity of the fetal myocardium to cardioplegic calcium concentration. Hypocalcemic cardioplegia provides superior preservation of systolic, diastolic, and contractile function of the fetal myocardium.

    View details for Web of Science ID 000183311400054

    View details for PubMedID 12822639

  • Electrical resynchronization - A novel therapy for the failing right ventricle CIRCULATION Dubin, A. M., Feinstein, J. A., Reddy, V. M., Hanley, F. L., Van Hare, G. F., Rosenthal, D. N. 2003; 107 (18): 2287-2289

    Abstract

    Many patients with congenital heart disease develop right ventricular (RV) failure due to anatomy and prior therapy. RV problems may include right bundle-branch block (RBBB), volume loading, and chamber enlargement. Because the failing RV may have regional dyskinesis, we hypothesized that resynchronization therapy might augment its performance.We studied 7 patients with RV dysfunction and RBBB, using a predefined pacing protocol. QRS duration, cardiac index (CI), and RV dP/dt were measured in 4 different pacing states. Atrioventricular pacing improved CI and RV dP/dtmax and decreased QRS duration as compared with atrial pacing or sinus rhythm.Atrioventricular pacing in patients with RBBB and RV dysfunction augments RV and systemic performance. RV resynchronization is a promising novel therapy for patients with RV failure.

    View details for DOI 10.1161/01.CIR.0000070930.33499.9F

    View details for Web of Science ID 000182807000014

    View details for PubMedID 12732607

  • Reliability of intraoperative contrast transesophageal echocardography for detecting interatrial communications in patients with other congenital cardiovascular malformations AMERICAN JOURNAL OF CARDIOLOGY Cassorla, L., Miller-Hance, W. C., Rouine-Rapp, K., Reddy, V. M., Hanley, F. L., Silverman, N. H. 2003; 91 (8): 1027-1031
  • Robotic-assisted endoscopic thoracic aortic anastomosis in juvenile lambs HEART SURGERY FORUM Malhotra, S. P., Le, D., Thelitz, S., Hanley, F. L., Riemer, R. K., Suleman, S., Reddy, V. M. 2003; 6 (1): 38-42
  • The evolving management of aortic arch obstruction with ventricular septal defect. Outcomes of two-stage, circulatory arrest, and low-flow cerebral perfusion approaches Malhotra, S. P., Suleman, S., Hanley, F. L., Reddy, V. M. LIPPINCOTT WILLIAMS & WILKINS. 2002: 395-395
  • Intermediate term follow-up of the end-to-side aortic anastomosis for coarctation of the aorta ANNALS OF THORACIC SURGERY Younoszai, A. K., Reddy, V. M., Hanley, F. L., Brook, M. M. 2002; 74 (5): 1631-1634

    Abstract

    Classic techniques for repairing coarctation of the aorta, especially in neonates, have a significant incidence of recurrent obstruction. By connecting the descending aorta to the proximal aortic arch, the end-to-side aortic anastomosis isolates hypoplastic distal arch and encroaching ductal tissue from the anastomotic site.Follow-up data were available for 88 patients (54 male) who underwent an end-to-side aortic anastomosis from November 1992 until November 1999. The median postoperative follow-up was 1.9 years (range, 0.1 to 6.3 years). Fifty-four patients were corrected as neonates. Thirty-four patients were operated on out of the neonatal period (> 1 month of age). A systolic blood pressure gradient > or = 20 mm Hg and a Doppler flow velocity > or = 2.5 ms across the area of repair were considered a recurrent obstruction.No patients in the pediatric group had a recurrent obstruction. In the neonatal group, 3 patients (5.5%) had recurrent obstruction. Of those, 2 patients had a reintervention performed; one reintervention was a balloon angioplasty and the other one was a reoperation. Kaplan-Meier analysis of the neonatal group revealed a 95.8% freedom from reintervention at 1 and 2 years.The end-to-side aortic anastomosis is an effective repair for coarctation of the aorta. Even when performed in the neonatal period, recurrence of coarctation is rare.

    View details for Web of Science ID 000179262300044

    View details for PubMedID 12440621

  • The role of oxidative stress in the development of pulmonary arteriovenous malformations after cavopulmonary anastomosis JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Malhotra, S. P., Reddy, V. M., Thelitz, S., He, Y. P., McMullan, D. M., Hanley, F. L., Riemer, R. K. 2002; 124 (3): 479-485

    Abstract

    Cavopulmonary anastomosis is used for palliation of cyanotic heart disease. Clinically significant pulmonary arteriovenous malformations occur in up to 25% of patients after surgical intervention. Cavopulmonary anastomosis creates several modifications to pulmonary physiology that may contribute to the development of pulmonary arteriovenous malformations, including reduced pulmonary blood flow and the exclusion of inferior vena caval effluent.By comparing the expression of angiogenic and stress-related proteins after cavopulmonary anastomosis and pulmonary artery banding, we sought to determine which genes were upregulated independent of reduced pulmonary blood flow.Lambs aged 35 to 45 days were placed into 1 of 3 groups: cavopulmonary anastomosis (n = 6), pulmonary artery banding (n = 6), and sham control (n = 6) animals. In our model pulmonary arteriovenous malformations are detectable by means of bubble-contrast echocardiography 8 weeks after cavopulmonary anastomosis. Lung tissue was harvested for Western blotting at 2 and 5 weeks after surgery.Cavopulmonary anastomosis and pulmonary artery banding both increased angiogenic gene expression, but only cavopulmonary anastomosis induced the expression of endothelial stress-related genes. Vascular endothelial growth factor was upregulated 2.5-fold after both cavopulmonary anastomosis (P =.002) and pulmonary artery banding (P =.007). Only cavopulmonary anastomosis upregulated 2 stress-related genes, HO1 and GLUT1, 2.7-fold (P =.002) and 3.8-fold (P =.03), respectively. Hypoxia-inducible factor was upregulated 4-fold (P =.003) after cavopulmonary anastomosis. Pulmonary artery banding failed to induce the increased expression of any of these proteins.Reduced pulmonary blood flow induces a pulmonary angiogenic response but not an endothelial stress response. These results suggest that oxidative stress is more relevant to the formation of pulmonary arteriovenous malformations than angiogenic signaling alone because pulmonary artery banding does not result in pulmonary arteriovenous malformations. Oxidative stress of the pulmonary endothelium resulting from cavopulmonary anastomosis may predispose the affected vasculature to arteriovenous shunting.

    View details for DOI 10.1067/mtc.2002.120346

    View details for Web of Science ID 000177840600009

    View details for PubMedID 12202863

  • Surgical creation of aortopulmonary window in selected patients with pulmonary atresia with poorly developed aortopulmonary collaterals and hypoplastic pulmonary arteries JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Rodefeld, M. D., Reddy, V. M., Thompson, L. D., Suleman, S., Moore, P. C., Teitel, D. F., Hanley, F. L. 2002; 123 (6): 1147-1154

    Abstract

    The morphologic characteristics of the pulmonary circulation vary widely in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Although we favor single-stage unifocalization and complete repair as the procedure of choice, a subgroup of patients who meet specific criteria have been treated with initial surgical creation of an aortopulmonary window.Eighteen patients who were considered unsuitable candidates for single-stage repair underwent surgical creation of an aortopulmonary window. Selection criteria included the following: (1) presence of centrally confluent true pulmonary arteries 1.0 to 2.5 mm in diameter, with a well-developed peripheral arborization pattern; (2) multiple small aortopulmonary collateral vessels, most of which communicated with the true pulmonary arterial system; and (3) the presence of marked cyanosis.There were no early deaths, and the 2 late deaths were both unrelated to the procedure. Follow-up angiography (n = 17) demonstrated good growth of true pulmonary arteries in 15 of 17 cases (88%). Mean pulmonary artery diameter increased from 1.76 mm to 3.45 mm. Subsequent operations have been performed in 15 of 18 cases (83%). Intracardiac repair with or without additional unifocalization was achieved in 8 of these 15 (53%). Seven patients (47%) have undergone staged unifocalization as the next procedure; of these, 3 were able to have intracardiac repair. Thus 11 of the 15 patients who have undergone second operations (73%) have had complete intracardiac repair.The initial surgical creation of an aortopulmonary window in carefully selected patients can increase the size of the true pulmonary arteries, making these patients better candidates for eventual intracardiac repair. The procedure should be avoided in patients with pulmonary overcirculation, a predominance of isolated supply collaterals, or true pulmonary arteries larger than 2.5 mm in diameter, and it is not applicable without a true pulmonary artery central confluence.

    View details for DOI 10.1067/mtc.2002.121685

    View details for Web of Science ID 000176384000020

    View details for PubMedID 12063462

  • Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex AMERICAN JOURNAL OF CARDIOLOGY Moon-Grady, A. J., Tacy, T. A., Brook, M. M., Hanley, F. L., Silverman, N. H. 2002; 89 (11): 1280-1285

    Abstract

    We describe clinical and echocardiographic features of tetralogy of Fallot with absent pulmonary valve complex (TOF/APVC) and hypothesized that outcome might be related to pulmonary artery enlargement or severity of illness. We examined the clinical records of all 23 patients evaluated at our institution before death or surgical correction of TOF/APVC between 1990 and 2000. Echocardiograms for 16 patients (including 5 fetuses) were also reviewed, and measurements of the semilunar valves and pulmonary arteries were obtained and compared with patient's aortic annulus size and with established normal subjects. Actuarial survival was 15 of 23 patients (68%) at 4 years. Four fetuses were hydropic and none survived; 7 patients were ventilator dependent at operation and only 3 survived. No difference was noted in pulmonary artery diameters in survivors versus nonsurvivors. Pulmonary valve annulus size was larger in nonsurvivors (103 +/- 25% vs 71 +/- 24% of normal, p = 0.03); however, when fetal examinations were excluded, this difference did not persist. Thus, only hydrops and ventilator dependence at diagnosis predicted mortality. There was no correlation between postnatal measurements of pulmonary arteries and outcome. Larger pulmonary annulus size in hydropic fetuses and poor survival among patients diagnosed in utero suggests that the pathophysiology in TOF/APVC is not due entirely to the aneurysmal dilation of the pulmonary arteries but may be related to right-sided cardiac dysfunction.

    View details for Web of Science ID 000175985300008

    View details for PubMedID 12031728

  • Cavopulmonary anastomosis induces pulmonary expression of the angiotensin II receptor family Malhotra, S. P., Reddy, V. M., Thelitz, S., He, Y. P., Hanley, F. L., Suleman, S., Riemer, R. K. MOSBY-ELSEVIER. 2002: 655-660

    Abstract

    Cavopulmonary anastomosis is used for palliation of cyanotic cardiac lesions. Postoperative development of pulmonary arteriovenous malformations can be significant in 10% to 25% of patients. To study the basis for formation of arteriovenous malformations, we developed an ovine model that reliably induces their development 8 weeks after cavopulmonary anastomosis. Previously, we found that cavopulmonary anastomosis inhibits the expression of pulmonary angiotensin-converting enzyme and suppresses angiotensin II production.This study examines the role of the angiotensin II receptors, type 1 and type 2, in this setting of pulmonary vascular remodeling.Lambs, aged 40 to 50 days, underwent cavopulmonary anastomosis. In age-matched control animals, a sham operation was performed. Messenger RNA and protein expression in lung specimens was measured at successive time points after cavopulmonary anastomosis or sham operations (n = 3 at each time point).Angiotensin type 1 mRNA was maximally upregulated 2-fold at 5 weeks after cavopulmonary anastomosis (P =.006). Expression of angiotensin type 1 protein was increased at least 2-fold at 2, 5, and 15 weeks after cavopulmonary anastomosis (P =.005). Cavopulmonary anastomosis also increased angiotensin type 2 mRNA and protein expression at least 2-fold at 2 and 5 weeks (P =.02) after surgical intervention. At 15 weeks, expression of angiotensin type 2 mRNA and protein was unchanged from that seen in control animals. Immunolocalization in pulmonary tissue sections 2 weeks after cavopulmonary anastomosis revealed markedly enhanced staining of angiotensin II receptor type 1 in vascular smooth muscle and angiotensin II receptor type 2 in the endothelium of pulmonary arteries.Rapid elevation in the expression of the type 1 and 2 angiotensin II receptors in the affected pulmonary vasculature after cavopulmonary anastomosis suggests their involvement in the pathologic vascular remodeling that occurs after cavopulmonary anastomosis.

    View details for DOI 10.1067/mtc.2002.119699

    View details for Web of Science ID 000175400100008

    View details for PubMedID 11986592

  • Management of neonatal bronchovenous fistula after cardiopulmonary bypass ANNALS OF THORACIC SURGERY Reinhartz, O., DeSilva, A. M., Hanley, F. L. 2002; 73 (4): 1320-1322

    Abstract

    Bronchovenous fistula is occasionally encountered after traumatic lung injury or, in neonates, due to ventilation injuries with high ventilatory pressures. We report a case of massive air embolism associated with a bronchopulmonary venous communication in an infant post-repair of truncus arteriosus. Selective ventilation of the opposite lung for 3 days sealed the fistula.

    View details for Web of Science ID 000174807400080

    View details for PubMedID 11996288

  • Neonatal truncus arteriosus repair: surgical techniques and clinical management. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Rodefeld, M. D., Hanley, F. L. 2002; 5: 212-217

    Abstract

    Truncus arteriosus is now ideally repaired in the neonatal period with low morbidity and mortality. Published reports have documented mortality rates in the range of 4% to 5% with mean age at repair as low as 11 days. The physiologic basis for improved outcomes with earlier repair is the avoidance of damaging sequelae of pulmonary overcirculation and heart failure. Data show that baseline mean pulmonary artery pressure is lower in infants undergoing earlier repair. Improved operative outcomes also have been achieved with aggressive truncal valve repair versus replacement in the presence of truncal valve dysfunction, right ventricular outflow tract reconstructive techniques that are patient anatomy-specific, and use of regional perfusion techniques for repair of associated interrupted aortic arch. In addition, a heightened awareness of anomalies of coronary artery ostial location, number, angle of takeoff, and degree of patency can result in avoidance of inadvertent injury to the artery and associated myocardial insult.

    View details for PubMedID 11994881

  • Arch reconstruction without circulatory arrest: current clinical applications and results of therapy. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual MacDonald, M. J., Hanley, F. L., Reddy, V. M. 2002; 5: 95-103

    Abstract

    Laboratory and clinical data have confirmed the deleterious effects of deep hypothermic circulatory arrest. Long-term data and rigorous neuropsychiatric data are currently sparse, but that which are available show adverse outcomes following circulatory arrest, and therefore support the use of continuous perfusion techniques. There are no available long-term data on continuous perfusion techniques with respect to neurologic outcomes, but using these techniques the incidence of postoperative seizures or other neurologic events is rare. Currently available bypass systems and microsurgical techniques have allowed continuous flow and regional perfusion to become practical alternatives. Several innovative techniques for avoiding circulatory arrest during neonatal aortic arch reconstruction for univentricular and biventricular hearts are described. It would appear prudent and desirable to provide continuous perfusion now that long-term survival after repair of even the most complex cardiac anomalies including single ventricle defects is commonplace.

    View details for PubMedID 11994869

  • Robotic-assisted endoscopic thoracic aortic anastomosis in juvenile lambs. heart surgery forum Malhotra, S. P., Le, D., Thelitz, S., Hanley, F. L., Riemer, R. K., Suleman, S., Reddy, V. M. 2002; 6 (1): 38-42

    Abstract

    Advances in robotic technology have enabled a wider range of applications for minimally invasive techniques in cardiac surgery, including mitral valve repair and coronary artery bypass grafting. With increased technical sophistication, robotic-assisted techniques can be developed for the endoscopic repair of certain congenital cardiac lesions.The purpose of this study was to assess the feasibility of closed chest thoracic aortic anastomosis in a juvenile ovine model.Lambs, aged 45 to 55 days, underwent surgery that was performed using the da Vinci robotic surgical system. Using 3 ports, the surgeon dissected the descending thoracic aorta and mobilized it free from attachments, using single-lung ventilation and CO2 insufflation. Snares were introduced through 2 stab wounds for aortic occlusion proximally and distally. In 4 lambs, the aorta was completely transected and reanastomosed using interrupted nitinol sutures. One lamb underwent longitudinal aortotomy, and patch aortoplasty was performed with the placement of a Gore-Tex patch. Snares were released and the animals were recovered once hemodynamically stable. Animals were sacrificed at 6 to 12 hours after surgery and the descending aorta was harvested. Burst-pressure testing was performed on the anastomoses.All 5 lambs survived the procedure with stabilization of hemodynamic parameters following surgery. The mean aortic clamp time was 47 +/- 17 minutes, and the anastomosis was completed in 26 +/- 5 minutes. The mean burst pressure was 163 +/- 9 mm Hg.Endoscopic thoracic aortic anastomosis can be performed safely and with adequate exposure in a juvenile large-animal model using computer-assisted surgical techniques. With further refinements, these approaches could be applied to the repair of congenital anomalies of the aorta, including interrupted aortic arch and aortic coarctation.

    View details for PubMedID 12611730

  • Pulmonary artery translocation: A surgical option for complex anomalous coronary artery anatomy ANNALS OF THORACIC SURGERY Rodefeld, M. D., Culbertson, C. B., Rosenfeld, H. M., Hanley, F. L., Thompson, L. D. 2001; 72 (6): 2150-2152

    Abstract

    Rather than perform a difficult and potentially high risk coronary reimplantation in a patient with an aberrant right coronary artery coursing between the aorta and pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary hilum to create additional space between the aortic and pulmonic trunks.

    View details for Web of Science ID 000172584500084

    View details for PubMedID 11789826

  • Hemidiaphragmatic paralysis increases postoperative morbidity after a modified Fontan operation Amin, Z., McElhinney, D. B., Strawn, J. K., Kugler, J. D., Duncan, K. F., Reddy, V. M., Petrossian, E., Hanley, F. L. MOSBY-ELSEVIER. 2001: 856-862

    Abstract

    After a Fontan procedure, forward pulmonary blood flow is augmented during inspiration because of negative intrathoracic pressure. Total pulmonary blood flow is higher during inspiration. With hemidiaphragmatic paralysis, inspiratory augmentation of pulmonary flow is lost or diminished. The objective of this study was to compare early postoperative morbidity after the modified Fontan operation in patients with and without hemidiaphragmatic paralysis.A case-control analysis was performed comparing 10 patients with documented hemidiaphragmatic paralysis against 30 patients without paralysis who were matched for diagnosis, fenestration, and age. The following early postoperative outcomes were assessed: duration of ventilator support, duration of hospital stay, incidence of ascites, prolonged effusions, and readmission.Preoperatively, there were no significant differences between the 2 groups. However, among the postoperative outcomes, the duration of hospital stay (25.4 +/- 16.6 days vs 10.8 +/- 6.3 days; P =.03), incidence of ascites (70% vs 3%; P <.001), prolonged pleural effusions (60% vs 13%; P =.007), and readmission (50% vs 7%; P =.007) were significantly greater in patients with hemidiaphragmatic paralysis than in those without hemidiaphragmatic paralysis.Hemidiaphragmatic paralysis after the modified Fontan operation is associated with an increase in early morbidity. Care should be taken to avoid injury to the phrenic nerve. Patients with prolonged effusions should be evaluated for hemidiaphragmatic paralysis.

    View details for Web of Science ID 000172244900004

    View details for PubMedID 11689788

  • Superior cavopulmonary anastomosis suppresses the activity and expression of pulmonary angiotensin-converting enzyme Malhotra, S. P., Riemer, R. K., Thelitz, S., He, Y. P., Hanley, F. L., Reddy, V. M. MOSBY-ELSEVIER. 2001: 464-469

    Abstract

    Superior cavopulmonary anastomosis is widely used for palliation of various forms of univentricular heart defects. However, clinically significant pulmonary arteriovenous malformations develop in 15% to 25% of patients after surgery.To assess altered regulation of pulmonary vascular tone caused by superior cavopulmonary anastomosis in an ovine model.Lambs, aged 35 to 45 days, underwent an end-to-end anastomosis of the superior vena cava to the right pulmonary artery. In age-matched controls, a sham operation was performed. Arteriovenous malformations were detectable by contrast echocardiography by 8 weeks after surgery. Animals (n = 24) were studied at various time points after the operations. Expression of angiotensin-converting enzyme messenger RNA, protein levels, and enzyme activity were measured in lung homogenates. Levels of angiotensin II were measured by enzyme-linked immunosorbent assay.Expression of angiotensin-converting enzyme messenger RNA and protein was significantly reduced at 1 to 5 weeks after superior cavopulmonary anastomosis. Angiotensin-converting enzyme activity in the right lung of animals subjected to superior cavopulmonary anastomosis was reduced 86% +/- 1% (standard deviation) compared with control values at 1 week (P =.003) and 77% +/- 8.5% at 2 weeks (P <.001) after surgery. This correlated with a 59% +/- 3.5% (P =.007) reduction in angiotensin II levels up to 5 weeks after cavopulmonary anastomosis. By 15 weeks after the operations, angiotensin II levels were equivalent to control levels (P =.19).Superior cavopulmonary anastomosis causes an early reversible reduction in activity and expression of angiotensin-converting enzyme, resulting in decreased circulating levels of the vasoconstrictor angiotensin II. These results suggest that the ability of the pulmonary endothelium to regulate vascular tone is inhibited after superior cavopulmonary anastomosis. Dilation of the affected vasculature induced by cavopulmonary anastomosis may contribute to the disordered vascular remodeling observed in this setting.

    View details for DOI 10.1067/mtc.2001.115698

    View details for Web of Science ID 000171027100010

    View details for PubMedID 11547295

  • Neonatal repair of truncus arteriosus: Continuing improvement in outcomes ANNALS OF THORACIC SURGERY Thompson, L. D., McElhinney, D. B., Reddy, V. M., Petrossian, E., Silverman, N. H., Hanley, F. L. 2001; 72 (2): 391-395

    Abstract

    Repair of truncus arteriosus in the neonatal and early infant periods has become standard practice at many centers. We reviewed our recent experience with repair of truncus arteriosus in neonates, with a focus on early and intermediate outcomes.From July 1992 to December 1999, 65 patients 1 month of age or less underwent primary complete repair of truncus arteriosus. Median age was 10 days, and median weight was 3.2 kg. Major associated anomalies included moderate or severe truncal valve regurgitation in 15 patients (23%), interrupted aortic arch in 8 (12%), coronary artery abnormalities in 12 (18%), and nonconfluence of the pulmonary arteries in 3 (5%). Median durations of cardiopulmonary bypass and cardioplegic arrest were 172 minutes and 90 minutes, respectively. Circulatory arrest was employed only in 7 patients undergoing concomitant repair of interrupted arch. Reconstruction of the right ventricular outflow tract was achieved with an aortic (n = 39) or pulmonary (n = 26) allograft valved conduit (median diameter, 12 mm). Replacement (n = 6) or repair (n = 5) of a regurgitant truncal valve was performed in 11 patients, and interrupted arch was repaired in 8.There were three early deaths (5%). Early reoperations included reexploration for bleeding in 3 patients, emergent replacement of a pulmonary outflow conduit that failed acutely in 1 patient, and placement of a permanent pacemaker in 1. Mechanical circulatory support was required in 1 patient. During the median follow-up of 32 months, there were two deaths. The Kaplan-Meier estimate of survival was 92% at 1 year and beyond. The only demographic, diagnostic, or operative factors significantly associated with poorer survival over time were operative weight of 2.5 kg or less (p = 0.01) and truncal valve replacement (p = 0.009). Actuarial freedom from conduit replacement among early survivors was 57% at 3 years.Repair of truncus arteriosus in the neonatal period can be performed routinely with excellent survival, even in patients with major associated abnormalities.

    View details for Web of Science ID 000170437300018

    View details for PubMedID 11515872

  • A prospective randomized study comparing volume-standardized modified and conventional ultrafiltration in pediatric cardiac surgery Thompson, L. D., McElhinney, D. B., Findlay, P., Miller-Hance, W., Chen, M. J., Minami, M., Petrossian, E., Parry, A. J., Reddy, V. M., Hanley, F. L. MOSBY-ELSEVIER. 2001: 220-228

    Abstract

    Modified ultrafiltration has been touted as superior to conventional ultrafiltration for attenuating the consequences of hemodilution after cardiac surgery with cardiopulmonary bypass in children. We conducted a prospective randomized study to test the hypothesis that modified and conventional ultrafiltration have similar clinical effects when a standardized volume of fluid is removed.From October 1998 to September 1999, 110 children weighing 15 kg or less (median weight 6.1 kg, median age 6.3 months) undergoing surgery with cardiopulmonary bypass for functionally biventricular congenital heart disease were randomized to conventional (n = 67) or arteriovenous modified ultrafiltration (n = 43) for hemoconcentration. The volume of fluid removed with both methods was standardized as a percentage of effective fluid balance (the sum of prime volume and volume added during cardiopulmonary bypass minus urine output): in patients weighing less than 10 kg, 50% of effective fluid balance was removed, whereas 60% was removed in patients weighing 10 to 15 kg. Hematocrit, hemodynamics, ventricular function, transfusion of blood products, and postoperative resource use were compared between groups.There were no significant differences between groups in age, weight, or duration of cardiopulmonary bypass. The total volume of fluid added in the prime and during bypass was greater in patients undergoing conventional ultrafiltration than in those receiving modified ultrafiltration (205 +/- 123 vs 162 +/- 74 mL/kg; P =.05), although the difference was due primarily to a greater indexed priming volume in patients having conventional ultrafiltration. There was no difference in the percentage of effective fluid balance that was removed in the 2 groups. Accordingly, the volume of ultrafiltrate was greater in patients receiving conventional than modified ultrafiltration (95 +/- 63 vs 68 +/- 28 mL/kg; P =.01). Preoperative and postoperative hematocrit levels were 35.6% +/- 6.6% and 36.3% +/- 5.6% in patients having conventional ultrafiltration and 34.4% +/- 6.7% and 38.7% +/- 7.5% in those having modified ultrafiltration. By repeated-measures analysis of variance, patients receiving modified and conventional ultrafiltration did not differ with respect to hematocrit value (P =.87), mean arterial pressure (P =.85), heart rate (P =.43), or left ventricular shortening fraction (P =.21) from baseline to the postbypass measurements. There were no differences between groups in duration of mechanical ventilation, stay in the intensive care unit, or hospitalization.When a standardized volume of fluid is removed, hematocrit, hemodynamics, ventricular function, requirement for blood products, and postoperative resource use do not differ between pediatric patients receiving conventional and modified ultrafiltration for hemoconcentration after cardiac surgery.

    View details for Web of Science ID 000170254000005

    View details for PubMedID 11479493

  • The safety and efficacy of sevoflurane anesthesia in infants and children with congenital heart disease ANESTHESIA AND ANALGESIA Russell, I. A., Hance, W. C., Gregory, G., Balea, M. C., Cassorla, L., DeSilva, A., Hickey, R. F., Reynolds, L. M., Rouine-Rapp, K., Hanley, F. L., Reddy, V. M., Cahalan, M. K. 2001; 92 (5): 1152-1158

    Abstract

    We tested the hypothesis that sevoflurane is a safer and more effective anesthetic than halothane during the induction and maintenance of anesthesia for infants and children with congenital heart disease undergoing cardiac surgery. With a background of fentanyl (5 microg/kg bolus, then 5 microg. kg(-1). h(-1)), the two inhaled anesthetics were directly compared in a randomized, double-blinded, open-label study involving 180 infants and children. Primary outcome variables included severe hypotension, bradycardia, and oxygen desaturation, defined as a 30% decrease in the resting mean arterial blood pressure or heart rate, or a 20% decrease in the resting arterial oxygen saturation, for at least 30 s. There were no differences in the incidence of these variables; however, patients receiving halothane experienced twice as many episodes of severe hypotension as those who received sevoflurane (P = 0.03). These recurrences of hypotension occurred despite an increased incidence of vasopressor use in the halothane-treated patients than in the sevoflurane-treated patients. Multivariate stepwise logistic regression demonstrated that patients less than 1 yr old were at increased risk for hypotension compared with older children (P = 0.0004), and patients with preoperative cyanosis were at increased risk for developing severe desaturation (P = 0.049). Sevoflurane may have hemodynamic advantages over halothane in infants and children with congenital heart disease.In infants and children with congenital heart disease, anesthesia with sevoflurane may result in fewer episodes of severe hypotension and less emergent drug use than anesthesia with halothane.

    View details for Web of Science ID 000168335900013

    View details for PubMedID 11323338

  • Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome Tworetzky, W., McElhinney, D. B., Reddy, V. M., Brook, M. M., Hanley, F. L., Silverman, N. H. LIPPINCOTT WILLIAMS & WILKINS. 2001: 1269-1273

    Abstract

    Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome.We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental decisions regarding care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. Of 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was terminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 patients diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patients were less likely to undergo surgery than patients diagnosed after birth (P:=0.008). Among live-born infants, there was a similar rate of nonintervention. Among patients who underwent surgery, survival was 75% (39/52). All patients who had a prenatal diagnosis and underwent surgery survived, whereas only 25 of 38 postnatally diagnosed patients survived (P:=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P:=0.02), tricuspid regurgitation (P:=0.001), and ventricular dysfunction (P:=0.004). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). Preoperative factors correlating with early mortality included postnatal diagnosis (P:=0.009), more severe acidosis (P:=0.03), need for bicarbonate or inotropes (P:=0.008 and 0.04), and ventricular dysfunction (P:=0.05).Prenatal diagnosis of HLHS was associated with improved preoperative clinical status and with improved survival after first-stage palliation in comparison with patients diagnosed after birth.

    View details for Web of Science ID 000167562200017

    View details for PubMedID 11238272

  • Late thrombosis of the native aortic root after Norwood reconstruction for hypoplastic left heart syndrome JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Brennan, T. V., Rodefeld, M. D., Tacy, T. A., Reddy, V. M., Hanley, F. L. 2001; 121 (3): 580-582

    View details for Web of Science ID 000167721100024

    View details for PubMedID 11241094

  • Perioperative administration of angiotensin converting enzyme inhibitors decreases the severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis CARDIOLOGY IN THE YOUNG Thompson, L. D., McElhinney, D. B., Culbertson, C. B., Hardy, C. E., Brook, M. M., Reddy, V. M., Hanley, F. L. 2001; 11 (2): 195-200

    Abstract

    Pleural effusions after bidirectional cavopulmonary anastomosis remain a significant cause of morbidity. Prolonged effusions in such patients have been associated with persistent elevations in plasma renin and angiotensin II.We conducted a controlled study in 36 patients (median age 8 months) undergoing bidirectional cavopulmonary anastomosis. Enalapril (5 mcg/kg) was administered intravenously within 1 hour of surgery and every 12 hours thereafter in 18 patients; when these patients were tolerating feeds, enalapril was switched to enteral captopril (3 mg/kg/day) every 8 hours. The other 18 patients did not receive perioperative angiotensin converting enzyme inhibitors. Using standardized criteria for discontinuation of chest tubes (< 2 mL/kg/day), volume and duration of pleural drainage were compared between groups. RESULTS. There were no differences between groups in demographic, diagnostic, or hemodynamic factors. There was no difference in cardiopulmonary bypass time between groups and no difference in postoperative pulmonary arterial pressures. The duration of pleural drainage was shorter (2.2+/-1.4 vs 5.9+/-1.4 days, p<0.001) and the volume less during the first 24 hours (4.7+/-1.2 vs 7.7+/-2.1 mL/kg, p<0.001) and overall (10.6+/-2.4 vs 19.6+/-4.5 mL/kg, p<0.001) in patients who received angiotensin converting enzyme inhibitors than those who did not. Readmission for persistent effusions was required in 3 patients who did not receive angiotensin converting enzyme inhibitors and none who did (p=0.11).Perioperative administration of angiotensin converting enzyme inhibitors is associated with decreased severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis.

    View details for Web of Science ID 000167755900009

    View details for PubMedID 11293738

  • Elective primary repair of acyanotic tetralogy of fallot in early infancy: Overall outcome and impact on the pulmonary valve JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Parry, A. J., McElhinney, D. B., Kung, G. C., Reddy, V. M., Brook, M. M., Hanley, F. L. 2000; 36 (7): 2279-2283

    Abstract

    We sought to determine if early primary repair of acyanotic tetralogy of Fallot (ToF) can be performed safely with low requirement for transannular patching (TAP) and thereafter allow normal right ventricular outflow tract (RVOT) growth.Early primary repair of ToF normalizes intracardiac flow patterns, which may allow subsequent normal RVOT growth. Traditionally repair is deferred until symptoms occur or children are deemed of adequate size for operative risk to be acceptable because of a perceived increased requirement for TAP in small infants.Between July 1992 and March 1999, 42 acyanotic infants aged 4 to 87 days (median 62) and weight 2.6 to 6.6 kg (median 4.55) underwent complete repair of ToF. Pulmonary annulus measured 4 to 10.5 mm (median 6.5) with "z-value" of-5.6 to +3.0 (median -1.9). RVOT reconstruction was tailored to each patient; pulmonary valvotomy was performed in 26, main pulmonary arterioplasty in 22, and infundibular patching in 2. Only 10 (24%) required TAP.Postoperative RVOT gradient was 0 to 30 mm Hg (median 10) and pRV/pLV ratio 0.3 to 0.6 (median 0.44). Pulmonary insufficiency was trivial/mild. There were no deaths. Junctional ectopic tachycardia developed in seven; only one required treatment. ICU stay was 2 to 14 days (median 4) and hospital stay 4 to 22 days (median 7). At follow-up 12 to 64 months later (median 38) there were no deaths. One child required reoperation for recurrent RVOT obstruction and two required balloon pulmonary arterioplasty. Follow-up RVOT gradient was 0 to 36 mm Hg (median 12), unchanged from early postoperative condition, and median z-value was -1.2 (-2.8 to +2.5); pulmonary insufficiency remained trivial/mild.Complete repair of acyanotic ToF can be performed in early infancy with low morbidity and mortality and low requirement for TAP. Though results are not statistically significant, early repair may allow normal RVOT growth thereafter.

    View details for Web of Science ID 000165600400039

    View details for PubMedID 11127473

  • Coronary arterial size late after the atrial inversion procedure for transposition of the great arteries: Implications for the arterial switch operation Amin, Z., McElhinney, D. B., Moore, P., Reddy, V. M., Hanley, F. L. MOSBY-ELSEVIER. 2000: 1047-1052

    Abstract

    Coronary flow reserve in the hypertrophied ventricle is reduced. One contributing factor may be the size of the proximal coronary arteries. In patients who undergo atrial inversion procedures for transposition of the great arteries, the left coronary artery supplies the pulmonary ventricle and may be smaller than the right coronary artery. We hypothesized that the dimensions of the coronary arteries may correlate with symptomatic status after atrial inversion and may be an important factor when these patients are considered for the arterial switch operation.The proximal left and right coronary arteries were measured in 9 patients with transposition and failure of the systemic right ventricle after atrial inversion, 10 asymptomatic patients after atrial inversion, and 10 patients with normal hearts. The diameters of the coronary arteries were indexed to body surface area and compared.The absolute and indexed diameters of the right coronary artery were greater in symptomatic patients than in asymptomatic patients (indexed: 3.1 +/- 0.6 vs 2.4 +/- 0.4 mm/m(2), P <.001) or control patients (2.0 +/- 0.3, P <.001), and the absolute diameter of the left coronary artery was smaller (2.9 +/- 0. 7 vs 3.6 +/- 0.5 mm, P =.003 [asymptomatic], 3.6 +/- 0.5 mm, P =.01 [control]). In symptomatic patients, the absolute and indexed diameters of the left coronary artery were smaller than those of the right (indexed: 2.1 +/- 0.6 vs 3.1 +/- 0.6 mm/m(2), P <.001). By contrast, there was no difference in asymptomatic patients (2.2 +/- 0.5 vs 2.4 +/- 0.4 mm/m(2), P =.44), and the left coronary artery was larger in normal control patients (2.2 +/- 0.4 vs 2.0 +/- 0.3 mm/m(2), P <.001).Differences in the sizes of the proximal coronary arteries may be related to symptomatic status in patients with transposition of the great arteries who have undergone an atrial inversion procedure, as well as to the efficacy of ventricular retraining. When pulmonary artery banding and subsequent arterial switch are considered for patients with a Mustard or Senning procedure and a failing systemic right ventricle, the size of the proximal coronary arteries may be an important factor and should be evaluated with preoperative imaging studies.

    View details for Web of Science ID 000165748800006

    View details for PubMedID 11088025

  • Familial absent pulmonary valve syndrome without deletions of chromosome 22q11 CARDIOLOGY IN THE YOUNG McElhinney, D. B., Hanley, F. L., Stanger, P. 2000; 10 (6): 618-620

    Abstract

    Deletions of chromosome 22q11 are common in patients with tetralogy of Fallot, and in those with absent pulmonary valve syndrome. In this report, we describe a pair of siblings with absent pulmonary valve syndrome, neither of whom had deletions of chromosome 22q11. The finding of familial absent pulmonary valve syndrome without deletion of 22q11 in our patients suggests an alternative genetic basis for this rare condition.

    View details for Web of Science ID 000165218100013

    View details for PubMedID 11117395

  • Infradiaphragmatic totally anomalous pulmonary venous return with two separate descending veins in association with right atrial isomerism ANNALS OF THORACIC SURGERY Thompson, L. D., McElhinney, D. B., Reddy, V. M., Jue, K. L., Hanley, F. L. 2000; 70 (4): 1400-1402

    Abstract

    We present the case of a 1-month-old infant with right atrial isomerism and complex functionally univentricular heart disease, in whom totally anomalous pulmonary venous return drained below the diaphragm via two separate vertical veins. One of the descending vertical veins drained the entire right lung and the left upper lobe, while the other drained the remainder of the left lung. Only one similar case has been described previously.

    View details for Web of Science ID 000089925600064

    View details for PubMedID 11081910

  • Transthoracic intracardiac catheters in pediatric patients recovering from congenital heart defect surgery: Associated complications and outcomes CRITICAL CARE MEDICINE Flori, H. R., Johnson, L. D., Hanley, F. L., Fineman, J. R. 2000; 28 (8): 2997-3001

    Abstract

    To characterize transthoracic intracardiac catheter uses and associated morbidities in pediatric patients recovering from congenital heart defect surgery and to identify potential risk factors associated with their use.Prospective data collection and review.An 18-bed pediatric intensive care unit (PICU) in a tertiary care university hospital.All pediatric patients between October 1, 1996, and September 30, 1997, who were recovering from congenital heart defect surgery and had transthoracic intracardiac catheters in place.Catheter use, associated morbidity, necessary interventions, and risk factors for complications of catheter use were identified. During this period, 523 catheters (276 right atrial, 155 left atrial, 68 common atrial, and 24 right ventricular or pulmonary artery catheters) in 351 PICU patients were studied. Mean age was 23.1+/-45.1 months (median, 4.98 months); 138 patients (39.3%) were <3 months old. The rate of occurrence of bleeding with catheter removal (mediastinal output in the hour after removal that was more than twice the previous average hourly output) was 36.7%, and bleeding occurred more frequently with left atrial catheters (47%; odds ratio, 2.0; p < .05). However, interventions after catheter removal were required for only 8.3% (42/504) of catheters removed, and hemodynamic compromise occurred with the removal of only 2.6% (13/504) of catheters. Interventions included fluid resuscitation (35 cases), pleural drainage (three cases), catheter wiring for retention (one case), chest tube suctioning (two cases), and surgical removal (one case). No associated deaths occurred. In a multivariate logistic regression analysis, age <3 months (odds ratio, 4.74), catheter location (left atrial: odds ratio, 4.97; pulmonary artery: odds ratio, 12.48), and platelet count of <50,000 (odds ratio, 8.59) were identified as risk factors associated with a need for intervention after catheter removal (p < .05). Other complications included blood cultures positive for organisms (1.5%), thrombus (0.6%), and catheter nonfunction (10.9%). Prematurity was a risk factor for thrombus and nonfunction.Use of transthoracic intracardiac catheters in pediatric patients is safe. Young infants and pediatric patients with thrombocytopenia or with catheters in the left atrial or pulmonary artery position have a greater need for interventions after catheter removal, warranting added precautions.

    View details for Web of Science ID 000088867300050

    View details for PubMedID 10966285

  • Neutrophil degranulation and complement activation during fetal cardiac bypass ANNALS OF THORACIC SURGERY Parry, A. J., Petrossian, E., McElhinney, D. B., Reddy, V. M., Hanley, F. L. 2000; 70 (2): 582-589

    Abstract

    Fetal cardiac bypass results in dysfunction of the fetoplacental unit (FPU) characterized by increased placental vascular resistance and respiratory acidosis. However the mechanisms of this dysfunction are not completely understood. To test the hypothesis that complement activation and neutrophil degranulation may contribute to the placental dysfunction associated with fetal bypass, we compared placental hemodynamics, complement activation, and neutrophil degranulation among fetuses exposed to cardiac bypass with a miniaturized bypass circuit including an in-line axial flow pump (Hemopump), fetuses undergoing bypass with a conventional roller pump circuit, and control fetuses that were similarly exposed but did not undergo bypass.Twenty-six Western Cross sheep fetuses (median 122 days gestation) were randomly assigned to undergo cardiac bypass for 30 minutes with the Hemopump circuit (n = 8), to undergo bypass for 30 minutes with the conventional (roller pump) circuit (n = 10), or to undergo identical exposure and cannulation but not bypass (n = 8, controls). Blood samples were collected to measure white cell count and differential, and C3a and lactoferrin levels prior to bypass, at the end of bypass, and 1 and 2 hours after bypass. Hemodynamics and blood gases were also monitored.There was a fall in white cell count over time that continued after bypass in all groups; neutrophils and lymphocytes were affected similarly. C3a levels rose significantly from prebypass to postbypass in the roller pump group (p<0.0001) but not in either of the other groups. Lactoferrin levels rose significantly from start of bypass in both bypass groups (Hemopump p = 0.01; roller pump p<0.0001) but not in controls. The elevation in lactoferrin level coincided with worsening placental gas exchange and deteriorating cardiac function.Complement and neutrophil activation occurred with fetal cardiac bypass but only neutrophil activation mirrored the FPU and cardiac dysfunction, suggesting that products of neutrophil activation may be important contributing factors. Improved FPU function with a bypass circuit that has less extracorporeal surface and does not require a large priming volume may be due in part to a reduction in the magnitude of this inflammatory response.

    View details for Web of Science ID 000088886300051

    View details for PubMedID 10969684

  • Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect ANNALS OF THORACIC SURGERY Amin, Z., McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L., Teitel, D. F. 2000; 70 (1): 119-123

    Abstract

    The frequency, distribution, and surgical importance of coronary artery to pulmonary artery (CAPA) collaterals have not been established. The aim of this study was to establish prevalence, anatomical pattern, and significance of CAPA in patients with pulmonary atresia and ventricular septal defect (PA/VSD).We reviewed cardiac catheterization and operative data of 87 consecutive, unselected patients who underwent one-stage complete unifocalization for PA/VSD and assessed major systemic to pulmonary collaterals from July 1992 to June 1998.CAPA collaterals were diagnosed in 9 of 87 patients (10%). The collaterals originated from the left coronary artery system in 7 patients and the right in 2. Collaterals from the left coronary system arose from the left main coronary artery in 3 patients and the circumflex in 4. All collaterals joined the central pulmonary artery, which bifurcated and supplied both lungs. One collateral from the right coronary system joined the stump of the main pulmonary artery and the other gave origin to a true left pulmonary artery, which was the sole supply to 75% of the left lung. Coronary artery enlargement was seen in 2 patients only. No patient had evidence of myocardial ischemia. Coronary collaterals comprised a dual source of pulmonary blood flow in all but 1 patient. During unifocalization, the CAPA collaterals were ligated at its origin in all cases, and the collateral from the right coronary to the left pulmonary artery was unifocalized.The prevalence of CAPA collaterals in patients with PA/VSD is approximately 10%. The diagnosis may be missed without appropriate angiograms. We recommend selective ascending aortogram or selective coronary angiogram in all patients.

    View details for Web of Science ID 000088318100029

    View details for PubMedID 10921694

  • Surgical approach to complicated cervical aortic arch: anatomic, developmental, and surgical considerations CARDIOLOGY IN THE YOUNG McElhinney, D. B., Thompson, L. D., Weinberg, P. M., Jue, K. L., Hanley, F. L. 2000; 10 (3): 212-219

    Abstract

    Abnormalities of brachiocephalic arterial branching and arch laterality are common in patients with a cervical aortic arch. In addition, structural anomalies of the arch such as obstruction, aneurysms, and tortuosity are found in a significant number of cases.Between 1990 and 1998, 6 patients underwent surgery for an obstructed right cervical arch. A significant obstruction was present at the transverse or distal arch in all patients, and was recurrent after previous repair in 2. In 1 patient, there was also a multi-lobed aneurysm of the aortic segment contiguous to the obstruction, and in 2 there was marked tortuosity of the arch. In all cases, the order of origin of the head and neck vessels was abnormal, and obstruction of 1 or more brachiocephalic vessels was found in 3. A vascular ring was present in all patients, with a right aortic arch and aberrant left subclavian artery in 4 patients and a double aortic arch with a dominant right cervical arch in 2. The descending aorta was circumflex (left-sided) in 3 patients. Three patients were repaired through a standard right posterolateral thoracotomy, and 3 through a median sternotomy. Patch augmentation aortoplasty was used in 2 patients, a tube graft from the ascending to descending aorta in 2, end to side anastomosis of the descending aorta to the proximal arch in 1, and direct anastomosis to reconstruct an atretic left-sided component of a double arch in 1.Repair was successful in all cases, with no perioperative complications. At follow-up ranging from 1 to 9 years, all patients were alive and well, with no recurrence of arch obstruction or other significant complications. Fluorescent in situ hybridization revealed microdeletion of chromosome 22q11 in 1 patient (not performed in the others).Structural anomalies of the arch are relatively common in patients with a cervical aortic arch. Such abnormalities may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the arch or its embryologic precursors. Given the highly variable anatomy of patients with a complicated cervical aortic arch, surgical considerations will vary in kind.

    View details for Web of Science ID 000086998800007

    View details for PubMedID 10824901

  • Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries - Experience with 85 patients Reddy, V. M., McElhinney, D. B., Amin, Z., Moore, P., Parry, A. J., Teitel, D. F., Hanley, F. L. LIPPINCOTT WILLIAMS & WILKINS. 2000: 1826-1832

    Abstract

    Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients.Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients.Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

    View details for Web of Science ID 000086812900013

    View details for PubMedID 10769284

  • Reinterventions after repair of common arterial trunk in neonates and young infants JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McElhinney, D. B., Rajasinghe, H. A., Mora, B. N., Reddy, V. M., Silverman, N. H., Hanley, F. L. 2000; 35 (5): 1317-1322

    Abstract

    To determine rates of reintervention after repair of common arterial trunk in the neonatal and early infant periods.With improving success in the early treatment of common arterial trunk, the need for reinterventional procedures in older children, adolescents and adults will become an increasingly widespread concern in the treatment of these patients.We reviewed our experience with 159 infants younger than four months of age who underwent complete primary repair of common arterial trunk at our institution from 1975 to 1998, with a focus on postoperative reinterventions.Of 128 early survivors, 40 underwent early reinterventions for persistent mediastinal bleeding or other reasons. During a median follow-up of 98 months (range, 2 to 235 months), 121 reinterventions were performed in 81 patients. Actuarial freedom from reintervention was 50% at four years, and freedom from a second reintervention was 75% at 11 years. A total of 92 conduit reinterventions were performed in 75 patients, with a single reintervention in 61 patients, 2 reinterventions in 11 patients and 3 reinterventions in 3 patients. Freedom from a first conduit reintervention was 45% at five years. The only independent variable predictive of a longer time to first conduit replacement was use of an allograft conduit at the original repair (p = 0.05), despite the significantly younger age of patients receiving an allograft conduit (p < 0.001). Reintervention on the truncal valve was performed on 22 occasions in 19 patients, including 21 valve replacements in 18 patients and repair in 1, with a freedom from truncal valve reintervention of 83% at 10 years. Surgical (n = 29) or balloon (n = 12) reintervention for pulmonary artery stenosis was performed 41 times in 32 patients. Closure of a residual ventricular septal defect was required in 13 patients, all of whom underwent closure originally with a continuous suture technique. Eight of 16 late deaths were related to reintervention.The burden of reintervention after repair of common arterial trunk in early infancy is high. Although conduit reintervention is inevitable, efforts should be made at the time of the initial repair to minimize factors leading to reintervention, including prevention of branch pulmonary artery stenosis and residual interventricular communications.

    View details for Web of Science ID 000086265900028

    View details for PubMedID 10758975

  • Management and outcomes of delayed sternal closure after cardiac surgery in neonates and infants CRITICAL CARE MEDICINE McElhinney, D. B., Reddy, V. M., Parry, A. J., Johnson, L., Fineman, J. R., Hanley, F. L. 2000; 28 (4): 1180-1184

    Abstract

    To investigate the efficacy, safety, and patterns of management of open sternotomy and delayed sternal closure in infants who were left with an open sternum after cardiac surgery and to assess these patterns for possible correlation with outcome.Retrospective chart review with statistical analysis.Pediatric cardiac surgery service at a regional referral center based in an urban university teaching hospital.All 128 patients <1 yr of age who were left with an open sternum after cardiac surgery with cardiopulmonary bypass during the 4-yr period from July, 1992 to June, 1996.Procedures for managing open sternotomy and delayed sternal closure were analyzed retrospectively. No interventions were undertaken for the study.Of the 128 patients, 14 (11%) died before sternal closure; delayed sternal closure was performed in the remaining 114. Of these 114, 13 died in the early postoperative period. During sternal closure, significant increases were noted in pulmonary arterial (from 21.1+/-7.6 mm Hg to 26.1+/-6.5 mm Hg; p = .006), left atrial (from 8.4+/-3.4 mm Hg to 11.5+/-3.7 mm Hg; p < .001), and right atrial pressures (from 7.3+/-2.5 mm Hg to 9.8+/-2.5 mm Hg; p < .001). In addition, mean airway pressure (from 7.4+/-2.0 mm Hg to 8.6+/-2.4 mm Hg; p < .001) and peak inspiratory pressure (from 29.3+/-5.4 mm Hg to 31.3+/-5.6 mm Hg; p = .004) increased. Sternal wound infection occurred in one patient.Delayed sternal closure is an effective approach to the management of neonates and infants at risk for hemodynamic, respiratory, or hemostatic instability early after cardiac surgery. Significant changes in hemodynamics and respiratory variables occur during sternal closure, often requiring adjustment of inotropic and ventilatory management. (Crit Care Med 2000; 28: 1180-1184)

    View details for Web of Science ID 000086862800044

    View details for PubMedID 10809302

  • Incidence and implications of systemic to pulmonary collaterals after bidirectional cavopulmonary anastomosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Petrossian, E., Hanley, F. L., Moore, P. 2000; 69 (4): 1222-1228

    Abstract

    Systemic to pulmonary arterial collaterals often develop after bidirectional cavopulmonary anastomosis (BCPA). It has been proposed that such collaterals may be related to perioperative outcome and duration of effusions after the modified Fontan procedure. However, the incidence and significance of collaterals after BCPA remain uncertain.To evaluate risk factors for, and significance of, such collaterals, we reviewed angiographic and clinical data for all 76 patients who underwent BCPA between January 1990 and June 1996 and had follow-up catheterization during or before 1997.The median age at BCPA was 10 months, and the median duration from BCPA to follow-up catheterization was 18 months. Arterial collaterals were detected on follow-up catheterization in 45 patients (59%). Factors associated with collateral development included a prior right-sided systemic-to-pulmonary arterial shunt, lower pre-BCPA end-diastolic ventricular pressure and pulmonary vascular resistance, and use and duration of cardiopulmonary bypass during the BCPA operation. Fourteen of the 45 patients (30%) underwent coil embolization of the collaterals. Forty-three patients have undergone extracardiac conduit Fontan, with 1 early and 1 late death. Collaterals were present in 22 of these patients, 7 of whom underwent pre-Fontan embolization. The duration from BCPA to Fontan was longer in patients with collaterals, but these patients were not more likely to have prolonged effusions than those without, and the duration of tube thoracostomy was significantly shorter in patients with collaterals. Embolization of collaterals did not affect the duration of effusions.Systemic-to-pulmonary arterial collaterals are common after BCPA. In contrast to prior reports, collaterals were not associated with a higher incidence of prolonged effusions after the Fontan procedure in our experience, and did not correlate with poor outcome.

    View details for Web of Science ID 000086808500059

    View details for PubMedID 10800823

  • Surgical intervention for complications of transcatheter dilation procedures in congenital heart disease ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Moore, P., Brook, M. M., Hanley, F. L. 2000; 69 (3): 858-864

    Abstract

    Transcatheter interventions have assumed an important role in the management of many forms of congenital heart disease. While complications of transcatheter interventions are uncommon and usually minor, significant complications requiring operation do occur on occasion. The purpose of this report is to present our experiences with seven such complications, and to review the literature on this topic.Seven patients who required operation after a transcatheter dilation procedure between 1992 and 1998 are described. Three patients required retrieval of retained foreign bodies (stents or balloons), and repair of the underlying abnormality. Two patients underwent repair of fistulas between 2 great vessels, or a great vessel and a cardiac chamber. One patient required operation for a postdilation aneurysm. One patient underwent urgent repair of severe aortic regurgitation after balloon aortic valvuloplasty.All patients survived and are doing well, with no further need for catheter or operative intervention, from 8 months to 6 years after operation. Additional reported complications requiring operation are discussed as well.Operation for complications of catheter interventions in congenital heart disease is seldom necessary. Though uncommon, a variety of such complications may occur, including vascular, valvar, intracardiac, and foreign body complications. When operation is required, results are typically very good.

    View details for Web of Science ID 000086022000044

    View details for PubMedID 10750773

  • Issues and outcomes in the management of supravalvar aortic stenosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Petrossian, E., Tworetzky, W., Silverman, N. H., Hanley, F. L. 2000; 69 (2): 562-567

    Abstract

    Supravalvar stenosis of the aorta is an uncommon congenital cardiac anomaly that involves not only the supravalvar aorta but the entire aortic root. Despite considerable attention to the importance of maintaining the integrity of the aortic root during supravalvar reconstruction, there has been little focus on the management of other components of the aortic root and left ventricular outflow tract, including the aortic valve, subvalvar region, and coronary arteries.We reviewed the records of 36 consecutive patients with supravalvar aortic stenosis who underwent repair from 1992-1998 (median age, 4 years). Discrete stenosis was present in 29 patients, whereas the remaining 7 had the diffuse form of the disease. Associated anomalies of the aortic root and adjacent structures were present in 23 patients. The median pressure gradient across the left ventricular outflow tract was 70 mm Hg. Supravalvar stenosis was relieved by extended aortoplasty with a Y-shaped patch in 18 patients, resection of the stenotic segment of ascending aorta at the sinotubular junction with end-to-end anastomosis of the ascending aorta in 7, the Ross procedure in 4, and other techniques in 7. Additional procedures included aortic valvuloplasty in 10 patients, resection of subvalvar stenosis in 11, and procedures on the coronary arteries in 2.There was 1 perioperative death, and no reoperations or other significant complications. During follow-up (median 33 months), there were no deaths and 3 reoperations for replacement of the aortic valve with a pulmonary autograft (n = 1) or mechanical prosthesis (n = 2). The median pressure gradient across the left ventricular outflow tract was 10 mm Hg.In patients with supravalvar aortic stenosis, abnormalities of the aortic valve, subaortic region, and coronary arteries are frequently present as well. Management of these issues is as critical to the long-term outcome of these patients as reconstruction of the supravalvar aorta. Aggressive valvuloplasty may help decrease the incidence of late aortic valve replacement, whereas the Ross procedure may be a preferable approach in some patients with complex outflow tract obstruction.

    View details for Web of Science ID 000085382200061

    View details for PubMedID 10735699

  • Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience Marcelletti, C. F., Hanley, F. L., Mavroudis, C., McElhinney, D. B., Abella, R. F., Marianeschi, S. M., Seddio, F., Reddy, V. M., Petrossian, E., de la Torre, T., Colagrande, L., Backer, C. L., Cipriani, A., Iorio, F. S., FONTAN, F. MOSBY-ELSEVIER. 2000: 340-344

    Abstract

    Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection.Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary.There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days.Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

    View details for Web of Science ID 000085509800031

    View details for PubMedID 10649210

  • Extracardiac conduit variation of the Fontan procedure. Advances in cardiac surgery Petrossian, E., Thompson, L. D., Hanley, F. L. 2000; 12: 175-198

    Abstract

    The ECF operation is designed to improve postoperative outcome by enhancing factors that are critical in optimal functioning of the Fontan circulation, including preservation of ventricular and pulmonary vascular function, avoidance of dysrhythmias, and prevention of stasis and flow turbulence in the Fontan circuit. Preoperative strategies include an early bidirectional Glenn procedure, and avoiding ancillary intracardiac procedures at the time of the Fontan by performing them at the time of the Glenn operation. Operative strategies include minimizing the duration of CPB by performing the conduit to pulmonary artery anastomosis off bypass, using partial instead of full CPB by cannulating the IVC alone, avoiding hypothermia, avoiding cross-clamping of the aorta, avoiding atrial incisions and suture lines, using a tubular conduit to construct the Fontan pathway, making a large conduit to pulmonary artery anastomosis, incorporating the conduit into aggressive pulmonary arterioplasties, and offsetting of the superior and inferior cavopulmonary anastomoses.

    View details for PubMedID 10949650

  • A 5-year experience with surgical repair of atrial septal defect employing limited exposure CARDIOLOGY IN THE YOUNG Khan, J. H., McElhinney, D. B., Reddy, V. M., Hanley, F. L. 1999; 9 (6): 572-576

    Abstract

    There has been a trend in recent years towards less invasive therapy for many congenital cardiac malformations. For the past 5 years, we have employed a technique of limited surgical exposure when repairing atrial defects within the oval fossa.Over the 5-year period from July 1992 to August 1997, 115 consecutive patients underwent surgical repair of an isolated atrial septal defect in the region of the oval fossa by a single surgeon. The patients had a limited midline skin incision starting at the line of the nipples and extending inferiorly across 2 to 3 intercostal spaces. A partial sternotomy was performed, sparing the manubrium. Standard instruments and cannulation techniques were used for cardiopulmonary bypass and fibrillatory arrest.There were no deaths and no major complications. The median time to extubation after leaving the operating room was 3 hours (30 minutes to 8 days). Mediastinal drains were removed the morning after surgery. The median stay in the intensive care unit was 7 hours (3 hours to 10 days), and patients were discharged from the hospital a median of 4 days postoperatively (2 to 23 days).This approach using limited exposure can be applied safely without any new instruments and without peripheral incisions or sites of vascular access, while providing a comfortable exposure for the surgeon and achieving a cosmetically superior result for the patient.

    View details for Web of Science ID 000085038300007

    View details for PubMedID 10593266

  • Is it necessary to routinely fenestrate an extracardiac fontan? JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Thompson, L. D., Petrossian, E., McElhinney, D. B., Abrikosova, N. A., Moore, P., Reddy, V. M., Hanley, F. L. 1999; 34 (2): 539-544

    Abstract

    This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan.Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined.Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients.Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy.Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.

    View details for Web of Science ID 000083039200033

    View details for PubMedID 10440170

  • Resection of subaortic stenosis; can a more aggressive approach be justified? EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY Parry, A. J., Kovalchin, J. P., Suda, K., McElhinney, D. B., Wudel, J., Silverman, N. H., Reddy, V. M., Hanley, F. L. 1999; 15 (5): 631-638

    Abstract

    Discrete subaortic stenosis causes left ventricular outflow tract (LVOT) obstruction and often produces aortic regurgitation (AR) which alone may precipitate surgical intervention. Conventional resection relieves the obstruction, but the recurrence rate is high, and the AR is little changed as the thick fibrous membrane which extends onto the valve leaflets remains. We studied whether an aggressive surgical approach could reduce both the severity of AR and rate of recurrence of obstruction associated with discrete subaortic stenosis, and whether this aggressive approach could be justified.Between June 1992 and April 1996, 37 patients aged 0.5-35 years (median 7.5) underwent resection of a discrete subaortic membrane. Ten underwent re-operation for recurrent obstruction and eight followed previous ventricular septal defect closure. LVOT gradient was measured using the modified Bernoulli equation and AR was graded on a scale of 0-4 (0 = none, 4 = severe). Postoperative assessment was performed early (<7 days) and at mid-term (27.0 months; range 2-59 months).There was significant improvement in AR from mild/moderate to none/trivial (P = 0.019) immediately postoperatively and LVOT gradient from 66.9+/-30.4 to 15.1+/-12.2 mmHg (P < 0.0001). By stepwise logistic regression preoperative gradient correlated significantly with postoperative mild/moderate AR (P = 0.015) and LVOT gradient (P = 0.0036). Preoperative mild/moderate AR also correlated with postoperative mild/moderate AR (P = 0.034). Five patients developed complete heart block, four undergoing reoperation for recurrent obstruction, and one preoperatively had right bundle branch block from previous ventricular septal defect repair. At mid-term follow-up there was no increase in AR or LVOT gradient (14.8+/-12.8 mmHg). Early post-operative AR was the strongest predictor of late mild/moderate AR (P = 0.02). Early post-operative gradient was a weaker predictor (P = 0.04). Pre-operative and early post-operative gradient were significant predictors of late gradient (P = 0.0038; <0.0001, respectively). No patient required reoperation for recurrent obstruction; one underwent late aortic valve replacement for severe AR.An aggressive surgical approach to discrete subaortic stenosis produces excellent relief of obstruction and frees the valve leaflets, significantly reducing associated AR at early and mid-term follow-up with low morbidity for primary operation. Long-term follow-up is required to confirm whether this early benefit is maintained.

    View details for Web of Science ID 000080692000021

    View details for PubMedID 10386409

  • Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography CARDIOLOGY IN THE YOUNG McElhinney, D. B., Silverman, N. H., Brook, M. M., Hanley, F. L., Stanger, P. 1999; 9 (3): 300-304

    Abstract

    Tricuspid regurgitation as a manifestation of an isolated congenital anomaly of the tricuspid valve is rare. Cross-sectional and color Doppler echocardiography allow improved evaluation of tricuspid valvar function. As a result, the heterogeneous category of congenital tricuspid valvar dysplasia may be better understood from a functional point of view. We are reporting a distinct entity in which tricuspid valvar regurgitation results from failure of coaptation due to short tendinous cords tethering the septal leaflet.Three children with significant primary tricuspid regurgitation were evaluated, treated, and followed. On echocardiographic evaluation, a central regurgitant jet of moderate or severe degree was directed toward the atrial septum through poorly coapting tricuspid valvar leaflets, which did not approximate due to tethering of the septal leaflet by abnormally short cords. In one patient, the tricuspid valve was otherwise normal; in the other two the leaflets and cords were also thickened. Two patients underwent surgery at 9 and 11 years of age. The cords tethering the septal leaflet were augmented by interposing appropriate lengths of expanded polytetrafluoroethylene suture and performing commissural annuloplasty. Both patients are asymptomatic 33 and 42 months postoperatively, with mild residual tricuspid regurgitation that has not changed since surgery. The other patient, an 8 month-old infant, has not yet undergone surgery.Asymmetric tendinous cords of the tricuspid valve causing tethering of the septal leaflet is a distinct cause of tricuspid regurgitation that can be recognized with echocardiography. Although rare, the importance of recognizing this lesion lies in its being readily amenable to surgical repair.

    View details for Web of Science ID 000083408600010

    View details for PubMedID 10386700

  • Compression of the central airways by a dilated aorta in infants and children with congenital heart disease ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Pian, M. S., Moore, P., Hanley, F. L. 1999; 67 (4): 1130-1136

    Abstract

    Children with congenital heart disease often experience respiratory symptoms in the preoperative and perioperative periods, which can complicate their management. An uncommon but important cause of respiratory insufficiency in such children is external airway compression.We operated on 5 patients (median age, 6 months) with significant respiratory distress attributable to compression of the central airways by a dilated ascending aorta before or after repair of concomitant cardiovascular defects. Four of these patients had right aortic arch and 3 had pulmonary atresia with a ventricular septal defect and major aortopulmonary collaterals. In all patients, aortopexy was performed at the time of operation for the cardiovascular defects (n = 3) or after symptoms developed in the postoperative period (n = 2). The 3 patients in whom airway compression produced symptoms preoperatively also underwent reduction ascending aortoplasty.Symptoms resolved immediately after operation in 3 patients, whereas symptoms persisted in the other 2 patients and tracheostomy was required. At follow-up of 20 months to 5 years, all patients are alive and well, with mild or moderate respiratory symptoms in the 2 patients who required tracheostomy, both of whom were decannulated within 13 months.External airway compression can cause significant morbidity in patients with congenital heart defects other than vascular rings. In patients with respiratory symptoms in the context of a lesion that involves increased aortic outflow during intrauterine life and consequently, an enlarged ascending aorta, such as tetralogy of Fallot with pulmonary atresia, airway compression should be considered as a cause, especially if a right aortic arch is present or the patient also has pulmonary atresia with a ventricular septal defect and collaterals. Attempts to address this problem surgically may provide substantial relief, but increasing duration of airway compression is likely to lead to tracheal or bronchial malacia and persistent symptoms even after the compression is relieved.

    View details for Web of Science ID 000080115300050

    View details for PubMedID 10320262

  • Early results of the extracardiac conduit Fontan operation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Petrossian, E., Reddy, V. M., McElhinney, D. B., Akkersdijk, G. P., Moore, P., Parry, A. J., Thompson, L. D., Hanley, F. L. 1999; 117 (4): 688-695

    Abstract

    Among the modifications of the Fontan operation, the extracardiac approach may offer the greatest potential for optimizing early postoperative ventricular and pulmonary vascular function, insofar as it can be performed with short periods of normothermic partial cardiopulmonary bypass and without cardioplegic arrest in most cases. In this study, we reviewed our experience with the extracardiac conduit Fontan operation, with a focus on early postoperative outcomes.Between July 1992 and April 1997, 51 patients (median age 4.9 years) underwent an extracardiac conduit Fontan operation. Median cardiopulmonary bypass time was 92 minutes and has decreased significantly over the course of our experience. Intracardiac procedures were performed in only 5 patients (10%), and the aorta was crossclamped in only 11 (22%). Intraoperative fenestration was performed in 24 patients (47%). There were no early deaths. Fontan failure occurred in 1 patient who was a poor candidate for the Fontan procedure. Transient supraventricular tachyarrhythmias occurred in 5 patients (10%). Median duration of chest tube drainage was 8 days. Factors significantly associated with prolonged resource use (mechanical ventilation, inotropic support, intensive care unit stay, and hospital stay) included longer bypass time and higher Fontan pressure. At a median follow-up of 1.9 years, there was 1 death from bleeding at reoperation.The extracardiac conduit Fontan procedure can be performed with minimal mortality and morbidity. Improved results may be related to advantages of the extracardiac approach and improved preservation of ventricular and pulmonary vascular function.

    View details for Web of Science ID 000079520000008

    View details for PubMedID 10096963

  • Congenital obstructive lesions of the right aortic arch ANNALS OF THORACIC SURGERY McElhinney, D. B., Tworetzky, W., Hanley, F. L., Rudolph, A. M. 1999; 67 (4): 1194-1202

    Abstract

    In the setting of normal cardiac situs, a right-sided aortic arch is uncommon. When a right arch does occur, it is typically in conjunction with other congenital cardiovascular anomalies, especially defects with abnormal right ventricular outflow. Congenital obstruction of a right arch, caused by coarctation, interruption, or cervical arch, is extremely rare.We reviewed our experience and all reported cases of right aortic arch with coarctation of the aorta, interrupted arch, or obstruction of a cervical arch in the setting of normal cardiac situs and topology.Since 1992, 4 such patients have undergone repair at our institution, including 1 with interrupted arch, 1 with coarctation of a mirror image arch, and 2 with obstruction of a cervical arch. In addition to these 4 patients, 38 others have been described in the published reports: 15 with interrupted arch, 19 with coarctation, and 4 with obstruction of a cervical arch. Associated cardiac defects were uncommon, except for ventricular septal defect in patients with interrupted arch, but abnormalities of the brachiocephalic vessels were frequent. Except for most of the patients with interrupted right arch, the majority of patients described have undergone successful surgical repair.Although obstructive arch lesions are often grouped together, the etiologies of coarctation of the aorta, interrupted arch, and cervical arch with obstruction almost certainly differ. The rarity of such lesions among patients with right aortic arch may be explained in part by the fact that the fetal hemodynamic conditions associated with persistence of a right arch do not facilitate flow-related arch obstruction. In this review, we discuss these issues in detail, along with specific surgical considerations in the management of obstruction lesions of the right aortic arch.

    View details for Web of Science ID 000080115300078

    View details for PubMedID 10320289

  • The use of 'pericardial hoods' for maintaining exact coronary artery geometry in the arterial switch operation with complex coronary anatomy EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY Parry, A. J., Thurm, M., Hanley, F. L. 1999; 15 (2): 159-164

    Abstract

    Complex coronary artery anatomy is the major risk factor for the arterial switch operation. Of the many approaches described the 'trap door' technique for coronary reimplantation is most flexible and allows safer transfer in complex arterial configurations. However, we have occasionally been concerned regarding torsion of the vessels with this approach. We therefore explored the role of trap-door augmentation with pericardial hoods to maintain exact coronary geometry during coronary transfer.Between February 1992 and December 1997, 80 patients underwent an arterial switch procedure at our institution. Sixty-seven patients underwent direct coronary reimplantation. In ten, coronary/great vessel anatomy was considered unfavourable and the trap-door approach was adopted primarily. In two an augmented trap-door was performed as the primary procedure and in the last patient Aubert's approach was used. In five patients during rewarming, ischaemic changes were noted on the electrocardiogram and/or regional wall motion abnormalities on transoesophageal echocardiography. This prompted revision of the appropriate coronary anastomosis. In three it was considered the anastomosis was kinked due to angulation of the button; in two the coronary was overstretched. In four, revision of the anastomosis was by pericardial hood augmentation.In all patients there was normalization of the electrocardiogram and immediate improvement in cardiac function documented by transoesophageal echocardiography. No early or late death occurred in the pericardial hood group nor were there any readmissions for any reason.Pericardial augmentation of trap-door aortic anastomoses allows for the maintenance of exact coronary artery geometry during the arterial switch procedure and minimizes the risk of myocardial ischaemia. We believe it broadens the application of the arterial switch procedure to even the most complex coronary anatomy and is a useful adjunct to the other techniques of coronary transfer.

    View details for Web of Science ID 000080762600009

    View details for PubMedID 10219548

  • Results of 102 cases of complete repair of congenital heart defects in patients weighing 700 to 2500 grams Reddy, V. M., McElhinney, D. B., Sagrado, T., Parry, A. J., Teitel, D. F., Hanley, F. L. MOSBY-ELSEVIER. 1999: 324-330

    Abstract

    Published data suggest that low birth weight is a risk factor for poor outcome in corrective surgery for many cardiac defects. Congenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative operations, with definitive repair delayed. The morbidity associated with such approaches is high.Since 1990 complete repair of congenital heart defects (other than patent ductus arteriosus) has been performed in 102 infants no larger than 2500 g (median 2100 g, range 700-2500 g), including 16 no larger than 1500 g. Defects included ventricular septal defect (n = 22), tetralogy of Fallot complexes (n = 20), transposition complexes (n = 13), aortic coarctation (n = 12), interrupted arch (n = 10), truncus arteriosus (n = 8), atrioventricular septal defect (n = 6), total anomalous pulmonary venous return (n = 5), and other (n = 6).Preoperative morbidity was more common among patients referred late for surgical correction. There were 10 early deaths (10%) attributable to cardiac failure (n = 4), arrhythmia (n = 1), multiorgan failure (n = 1), sepsis (n = 1), idiopathic coronary artery intimal necrosis (n = 1), foot gangrene (n = 1), and pulmonary hemorrhage (n = 1). No patient had postbypass intracerebral hemorrhage. At follow-up (median 36 months) there were 8 late deaths, and 8 patients underwent 10 reinterventions. There was no evidence of neurologic sequelae attributable to the operation.In general, delaying repair of congenital heart defects in low birth weight infants does not confer a benefit and is associated with higher preoperative morbidity. Complete repair of both simple and complex lesions can be achieved in such cases with good results. Growth after repair approximates the normal curve for low birth weight infants without heart disease. It is recommended that such infants, especially when they have symptoms, undergo early surgical repair rather than prolonged medical management or other forms of palliation.

    View details for Web of Science ID 000078353700026

    View details for PubMedID 10049033

  • Endothelin receptor blockade prevents the rise in pulmonary vascular resistance after cardiopulmonary bypass in lambs with increased pulmonary blood flow JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Petrossian, E., Parry, A. J., Reddy, V. M., Akkersdijk, G. P., McMullan, D. M., Thompson, L., Hendricks-Munoz, K. D., Hallak, H., Hanley, F. L., Fineman, J. R. 1999; 117 (2): 314-322

    Abstract

    Children with increased pulmonary blood flow may experience morbidity as the result of increased pulmonary vascular resistance after operations in which cardiopulmonary bypass is used. Plasma levels of endothelin-1, a potent vasoactive substance implicated in pulmonary hypertension, are increased after cardiopulmonary bypass.In a lamb model of increased pulmonary blood flow after in utero placement of an aortopulmonary shunt, we characterized the changes in pulmonary vascular resistance induced by hypothermic cardiopulmonary bypass and investigated the role of endothelin-1 and endothelin-A receptor activation in postbypass pulmonary hypertension.In eleven 1-month-old lambs, the shunt was closed, and vascular pressures and blood flows were monitored. An infusion of a selective endothelin-A receptor blocker (PD 156707; 1.0 mg/kg/h) or drug vehicle (saline solution) was then begun 30 minutes before cardiopulmonary bypass and continued for 4 hours after bypass. The hemodynamic variables were monitored, and plasma endothelin-1 concentrations were determined before, during, and for 6 hours after cardiopulmonary bypass.After 90 minutes of hypothermic cardiopulmonary bypass, both pulmonary arterial pressure and pulmonary vascular resistance increased significantly in saline-treated lambs during the 6-hour study period (P <.05). In lambs pretreated with PD 156707, pulmonary arterial pressure and pulmonary vascular resistance decreased (P <. 05). After bypass, plasma endothelin-1 concentrations increased in all lambs; there was a positive correlation between postbypass pulmonary vascular resistance and plasma endothelin-1 concentrations (P <.05).This study suggests that endothelin-A receptor-induced pulmonary vasoconstriction mediates, in part, the rise in pulmonary vascular resistance after cardiopulmonary bypass. Endothelin-A receptor antagonists may decrease morbidity in children at risk for postbypass pulmonary hypertension. This potential therapy warrants further investigation.

    View details for Web of Science ID 000078353700024

    View details for PubMedID 9918974

  • Role of the endothelium in placental dysfunction after fetal cardiac bypass JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Rajasinghe, H. A., Liddicoat, J. R., Hendricks-Munoz, K., Fineman, J. R., Hanley, F. L. 1999; 117 (2): 343-351

    Abstract

    Fetal cardiac bypass causes placental dysfunction, characterized by increased placental vascular resistance, decreased placental blood flow, hypoxia, and acidosis. Vasoactive factors produced by the vascular endothelium, such as nitric oxide and endothelin 1, are important regulators of placental vascular tone and may contribute to this placental dysfunction.To investigate the role of the vascular endothelium in placental dysfunction related to fetal cardiac bypass, we studied 3 groups of fetal sheep. In the first group (n = 7) we determined placental hemodynamic responses before and after bypass to an endothelium-dependent vasodilator (acetylcholine), an endothelium-independent vasodilator (nitroprusside), and endothelin 1. In the second group (n = 8) a nonspecific endothelin receptor blocker (PD 145065) was administered and placental hemodynamic values were measured before and after bypass. In the third group (n = 5) endothelin 1 levels were measured before and after bypass.Before fetal cardiac bypass exogenous endothelin 1 decreased placental blood flow by 9% and increased placental resistance by 9%. After bypass endothelin 1 decreased placental flow by 47% and increased resistance by 106%. There was also a significant attenuation of the placental vascular relaxation response to acetylcholine after bypass, whereas the response to nitroprusside was not significantly altered. In fetuses that received the PD 145065, placental vascular resistance increased significantly less than in control fetuses (28% versus 62%). Similarly, placental blood flow decreased significantly more (from 6. 3 +/- 3.1 to 28.3 +/- 10.4 pg/mL; P =.01) in control fetuses than in fetuses receiving PD 145065 (33% versus 20%). Umbilical venous endothelin 1 levels increased significantly in fetuses exposed to fetal bypass but did not change in control fetuses.The basal endothelial regulatory mechanisms of placental vascular tone were deranged after fetal cardiac bypass. Endothelin receptor blockade, which substantially reduced postbypass placental dysfunction, and other interventions aimed at preserving endothelial function may be effective means of optimizing fetal outcome after cardiac bypass.

    View details for Web of Science ID 000078353700030

    View details for PubMedID 9918976

  • Echocardiographic diagnosis alone for the complete repair of major congenital heart defects JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Tworetzky, W., McElhinney, D. B., Brook, M. M., Reddy, V. M., Hanley, F. L., Silverman, N. H. 1999; 33 (1): 228-233

    Abstract

    The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair.Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects.To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery.Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality.This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.

    View details for Web of Science ID 000078088300036

    View details for PubMedID 9935035

  • Multiple ventricular septal defects: How and when should they be repaired? JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Seddio, F., Reddy, V. M., McElhinney, D. B., Tworetzky, W., Silverman, N. H., Hanley, F. L. 1999; 117 (1): 134-139

    Abstract

    Congenital heart lesions with multiple ventricular septal defects remain a surgical challenge. Traditional approaches often rely on either ventriculotomy for exposure or palliation with pulmonary artery banding. However, indications for repair versus palliation and for various approaches to surgical exposure are not clearly defined.From July 1992 to January 1998, 45 patients with multiple (>/=2) ventricular septal defects (37 with associated lesions) underwent surgery. Median age was 86 days; all but 4 patients were infants. The mean number of defects was 3.7, and almost half of the patients had more than 3 defects. Apical muscular defects were present in 62% of patients. Thirty-one patients underwent primary complete repair through a right atriotomy or trans-semilunar valve approach (group 1), 8 had palliation (group 2), and 6 underwent complete repair after prior palliation elsewhere (group 3). No patient had a ventriculotomy.One early death occurred in a group 1 patient. Four patients who had had palliation (50%) underwent early reoperation for pulmonary artery band revision because of failure to thrive or band removal after spontaneous closure of the defects. At follow-up (median 22 months), there was 1 death in a group 2 patient (palliation) and 1 other group 2 patient required cardiac transplantation. The only late reoperation was for removal of the pulmonary artery band and closure of multiple apical defects in a group 2 (palliation) patient. No patients who underwent repair have hemodynamically significant residual defects.In our experience, palliation of multiple ventricular septal defects is associated with greater morbidity than primary repair. Multiple defects can almost always be repaired adequately in early infancy without ventriculotomy, although "Swiss-cheese" septum may be an indication for palliation.

    View details for Web of Science ID 000077898200027

    View details for PubMedID 9869767

  • Accessory and anomalous atrioventricular valvar tissue causing outflow tract obstruction - Surgical implications of a heterogeneous and complex problem JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. 1998; 32 (6): 1741-1748

    Abstract

    The purpose of this study was to determine the effect of accessory or anomalous atrioventricular valvar apparatus on relief of outflow tract obstruction.Outflow tract obstruction due to accessory tissue or anomalous attachments of the atrioventricular valvar apparatus is an unusual but well-recognized problem. In addition to obstruction, anomalous attachments of the atrioventricular valvar apparatus may interfere with procedures to relieve outflow tract obstruction or perform outflow tract reconstruction.Since 1992, we have operated on 21 patients (median age 4 years) with systemic (n=13), pulmonary (n=5) or bilateral (n=3) outflow tract obstruction due to accessory atrioventricular valvar tissue and/or anomalous attachments of the subvalvar apparatus. Primary diagnoses were isolated obstruction of the systemic outflow tract or aortic arch (n=7), transposition complexes (n=6), previously repaired atrioventricular septal defect (n=3), functionally single ventricle (n=3) and ventricular septal defect with pulmonary outflow obstruction (n=2). Outflow tract gradients ranged from 20-110 mm Hg (median 58 mm Hg).Complete relief of obstruction due to atrioventricular valvar anomalies was possible in 14 patients. In six patients, the planned procedure either had to be modified or only partial relief of the obstruction was achieved. In the remaining patient, who had borderline functionally single ventricle heart disease (unbalanced atrioventricular septal defect) and systemic outflow obstruction due to accessory and functional valvar apparatus, support was withdrawn because the parents refused univentricular palliation and the valvar anomalies precluded a Ross-Konno procedure. There were two early deaths. At follow-up ranging from 1 to 66 months (median 27 months), there was one death, and there has been no recurrence of outflow tract obstruction or residual atrioventricular valvar tissue.Outflow tract obstruction caused by accessory or anomalous atrioventricular valvar structures is an uncommon and heterogeneous group of conditions that can have significant surgical implications. In the majority of cases, tailoring of surgical techniques will permit complete relief of obstruction. However, such anomalies may limit standard surgical options and necessitate an innovative approach in some patients.

    View details for Web of Science ID 000076981500022

    View details for PubMedID 9822104

  • Alternative approach to the repair of Ebstein's malformation: Intracardiac repair with ventricular unloading ANNALS OF THORACIC SURGERY Marianeschi, S. M., McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. 1998; 66 (5): 1546-1550

    Abstract

    Moderate to severe Ebstein's malformation remains a surgical challenge. Although the various approaches that have been used are appropriate and successful in many patients, there are many for which these approaches are suboptimal. To improve the prognosis for patients across the full spectrum of Ebstein's malformation, alternative surgical approaches are necessary.From December 1995 to October 1997, 10 patients (median age, 9 years) with moderate or severe Ebstein's malformation and mild to severe tricuspid regurgitation had partial biventricular repair with reduction of right ventricular volume load. All patients were symptomatic in New York Heart Association functional class II (n = 9) or III (n = 1). In addition to bidirectional cavopulmonary anastomosis and closure of intracardiac defects in all patients, 6 underwent tricuspid valve repair using a variety of procedures, most often simple horizontal annuloplasty.There were no deaths. Early reoperation was required in 1 patient (atrial septostomy on the day after operation for right ventricular failure) and another required revision of the tricuspid valve repair 10 months postoperatively for recurrent regurgitation. At follow-up ranging from 2 to 24 months, all patients are in New York Heart Association class I and have trivial tricuspid regurgitation, including the 4 who had no tricuspid valvuloplasty performed.We have presented an alternative approach to the management of severe Ebstein's malformation that focuses on both the tricuspid valve and the right ventricle. Just as tricuspid valve repair and reduction of regurgitation will likely improve right ventricular performance, reducing the volume load on the ventricle may improve both ventricular (right and left) and tricuspid valve function. All patients have demonstrated improved exercise tolerance and right heart function at follow-up ranging to 24 months. Additional experience will be necessary to evaluate this strategy more completely.

    View details for Web of Science ID 000077703300014

    View details for PubMedID 9875749

  • Intramyocardial hematoma causing cardiac tamponade after repair of Ebstein malformation: Erroneous echocardiographic diagnosis as intracavitary thrombus JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Momenah, T. S., McElhinney, D. B., Brook, M. M., Teitel, D. F., Hanley, F. L., Silverman, N. H. 1998; 11 (11): 1087-1089

    Abstract

    Intramyocardial hematoma is an uncommon lesion, most often occurring after chest trauma, aortic valve disease, acute myocardial infarction, or coronary artery bypass surgery. We describe a 17-year-old patient who experienced cardiac tamponade after repair of Ebstein malformation. The malformation was caused by an enlarging intramyocardial hematoma that extended from the right atrium to the atrialized right ventricle. The hematoma was incorrectly diagnosed by echocardiography as an intracavitary thrombus, and the correct diagnosis was recognized only at the time of surgical intervention.

    View details for Web of Science ID 000077038900014

    View details for PubMedID 9812104

  • Extracardiac conduit Fontan procedure without cardiopulmonary bypass ANNALS OF THORACIC SURGERY McElhinney, D. B., Petrossian, E., Reddy, V. M., Hanley, F. L. 1998; 66 (5): 1826-1828

    Abstract

    There are a number of potential advantages of extracardiac conduit cavopulmonary anastomosis for palliation of functional single ventricle heart disease, including the ability to perform the operation with no aortic cross-clamping and with minimal duration of extracorporeal circulation. In many patients, it may be possible to perform the procedure without cardiopulmonary bypass altogether. In this report, we present our technique for performing the extracardiac conduit Fontan operation without cardiopulmonary bypass.

    View details for Web of Science ID 000077703300095

    View details for PubMedID 9875809

  • Repair of secundum atrial septal defect: Limiting the incision without sacrificing exposure ANNALS OF THORACIC SURGERY Khan, J. H., McElhinney, D. B., Reddy, V. M., Hanley, F. L. 1998; 66 (4): 1433-1435

    Abstract

    A simple and effective technique for repair of secundum atrial septal defect is described. The heart is exposed through a limited midline skin incision and partial sternotomy, and the atrial septal defect is closed through a right atriotomy with ascending aortic and dual venous cannulation. This approach achieves a cosmetically superior result with standard instrumentation and cardiopulmonary bypass techniques, without compromising exposure or using peripheral incisions.

    View details for Web of Science ID 000076692900087

    View details for PubMedID 9800859

  • Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot revisited: making a case for primary repair in infancy CARDIOLOGY IN THE YOUNG McElhinney, D. B., Reddy, V. M., Silverman, N. H., Brook, M. M., Hanley, F. L. 1998; 8 (4): 455-461

    Abstract

    Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot is a rare combination of congenital cardiac anomalies. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Between July 1992 and August 1997, nine patients underwent repair of complete atrioventricular septal defect with tetralogy of Fallot. One patient, aged 9.6 years at the time of repair, had previously undergone construction of a modified Blalock-Taussig shunt. Primary complete repair was performed in the other 8 patients at ages ranging from 2.5 to 16 months (median 4.6 months), and all but one were infants. All patients had a Rastelli type C defect, a single ventricular septal defect with inlet and outlet components, and malalignment of the muscular outlet septum with subpulmonary stenosis. A single patch technique, with closure of the zone of apposition ('cleft') in the left atrioventricular valve, was used in all eight patients undergoing primary repair, while a double patch was employed in the previously palliated older patient. In all cases of repair using a single patch, the anterosuperior bridging leaflet was divided obliquely to the right, following the malaligned outlet septum, in order to avoid subaortic obstruction. Repair of the right ventricular outflow tract included infundibular myectomy in eight, pulmonary valvotomy in four, infundibular or transannular patching in three and one, respectively, and reconstruction with a valved allograft conduit in two patients. There was no early mortality or significant morbidity. At a median follow-up of 45 months, there had been one death related to non-cardiac causes and no reinterventions. Left atrioventricular valvar regurgitation was moderate or mild in two patients, and right atrioventricular valvar regurgitation was mild in one patient. No patient had more than mild pulmonary regurgitation or a gradient across the right ventricular outflow tract in excess of 18 mm Hg. Our results demonstrate that primary repair of atrioventricular septal defect with tetralogy of Fallot can be performed with excellent early and mid-term results in young infants. Although it has been suggested that a technique utilizing oblique division of the anterosuperior bridging leaflet may lead to high rates of atrioventricular valvar regurgitation, medium-term atrioventricular valvar function in the present cohort of patients has been excellent.

    View details for Web of Science ID 000077254300010

    View details for PubMedID 9855099

  • Tetralogy of Fallot with major aortopulmonary collaterals: Early total repair PEDIATRIC CARDIOLOGY McElhinney, D. B., Reddy, V. M., Hanley, F. L. 1998; 19 (4): 289-296

    Abstract

    Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals is a complex lesion distinguished by marked heterogeneity of pulmonary blood supply. Over the past two decades, investigators have developed various approaches to the management of this anomaly generally based on the concept of staged unifocalization of pulmonary blood supply. Although such approaches may represent an improvement on the natural history of this lesion, they remain inadequate for a substantial portion of patients born with tetralogy of Fallot and major aortopulmonary collaterals. Since 1992, our approach has been to perform one-stage complete unifocalization through a midline approach in all but a few extremely complicated patients. We aim to repair these patients early in infancy, with an emphasis on native tissue-tissue reconstruction, in order to optimize prospects for survival with a good functional outcome in as many patients as possible. In this review, we present our philosophy and our experience with unifocalization and repair in 72 patients.

    View details for Web of Science ID 000074204200004

    View details for PubMedID 9636252

  • The effects of inhaled nitric oxide on postoperative pulmonary hypertension in infants and children undergoing surgical repair of congenital heart disease ANESTHESIA AND ANALGESIA Russell, I. A., Zwass, M. S., Fineman, J. R., Balea, M., Rouine-Rapp, K., Brook, M., Hanley, F. L., Silverman, N. H., Cahalan, M. K. 1998; 87 (1): 46-51

    Abstract

    The role of inhaled nitric oxide in the immediate post-bypass period after surgical repair of congenital heart disease is uncertain. In a controlled, randomized, double-blind study, we tested the hypothesis that inhaled nitric oxide (NO) would reduce pulmonary hypertension immediately after surgical repair of congenital heart disease in 40 patients with preoperative evidence of pulmonary hypertension (mean pulmonary arterial pressure [MPAP] exceeding 50% of mean systemic arterial pressure [MSAP]). Patients were then followed in the intensive care unit (ICU) to document the incidence of severe pulmonary hypertension. Of the patients, 36% (n = 13) emerged from bypass with MPAP > 50% MSAP. In these patients, inhaled NO reduced MPAP by 19% (P = 0.008) versus an increase of 9% in the placebo group. No effect on MPAP was observed in patients emerging from bypass without pulmonary hypertension (n = 23). Inhaled NO was required five times in the ICU, always in the patients who had emerged from cardiopulmonary bypass with pulmonary hypertension (5 of 13 [38%] versus 0 of 23). We conclude that, in infants and children undergoing congenital heart surgery, inhaled NO selectively reduces MPAP in patients who emerge from cardiopulmonary bypass with pulmonary hypertension and has no effect on those who emerge without it.In a randomized double-blind study, inhaled nitric oxide selectively reduced pulmonary artery pressures in pediatric patients who developed pulmonary hypertension (high blood pressure in the lungs) immediately after cardiopulmonary bypass and surgical repair.

    View details for Web of Science ID 000074557500011

    View details for PubMedID 9661544

  • Partial biventricular repair for complex congenital heart defects: An intermediate option for complicated anatomy or functionally borderline right complex heart JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Silverman, N. H., Marianeschi, S. M., Hanley, F. L. 1998; 116 (1): 21-27

    Abstract

    Partial biventricular repair consists of bidirectional cavopulmonary anastomosis in the presence of separated systemic and pulmonary circulations, with antegrade flow of inferior caval return through an intact or reconstructed pulmonary outflow tract. This versatile procedure may be used as a definitive repair for patients with a functional right heart complex incapable of supporting an entire cardiac output or in patients with complicated anatomy.From July 1992 to April 1997, 23 patients (median age 5.2 years) underwent partial biventricular repair. In 15 of these cases the entire repair, including bidirectional cavopulmonary anastomosis, intracardiac repair, and right ventricular outflow reconstruction, was performed as a planned procedure at our institution. The other eight patients had previously been placed on a Fontan track and had undergone bidirectional cavopulmonary anastomosis; their circulations were converted to a partial biventricular circulation.There were no early deaths. Complete atrioventricular block developed in two patients with straddling tricuspid valve. At a median follow-up of 17 months, there were no late deaths and three patients had undergone reintervention. Partitioning of the pulmonary arteries to create a classic Glenn anastomosis with antegrade flow to the left lung was performed in one case; another patient underwent an atrial septectomy, and the third patient required revision of tricuspid valve repair. All patients are in New York Heart Association functional class I.Partial biventricular repair is a versatile strategy that can be used to manage a variety of forms of complex congenital heart disease. Cases for which the repair is useful include those in which complete biventricular repair is unlikely to be achieved because of limited size or function of the right side of the heart and those in which a patient with a ventricle capable of supporting inferior vena caval return was previously placed on a Fontan track.

    View details for Web of Science ID 000074833500002

    View details for PubMedID 9671893

  • Rare forms of isolation of the subclavian artery: echocardiographic diagnosis and surgical considerations CARDIOLOGY IN THE YOUNG McElhinney, D. B., Silverman, N. H., Brook, M. M., Reddy, V. M., Hanley, F. L. 1998; 8 (3): 344-351

    Abstract

    Isolation of the subclavian artery is an unusual anomaly in which the subclavian artery arises not from the aortic arch but from a pulmonary artery via an arterial duct. Such isolation most often occurs with a right aortic arch, and in lesions frequently associated with a right arch, such as tetralogy of Fallot. Since 1994, we have undertaken surgery in four young infants with isolated subclavian arteries and unusual associated anomalies, including one with atrioventricular septal defect and common valvar orifice, two with interruption of a left aortic arch and one with interruption of a right aortic arch. In both patients with interrupted left arch, the isolated subclavian artery was diagnosed preoperatively by echocardiography. We emphasize the significant surgical issues.

    View details for Web of Science ID 000075607500012

    View details for PubMedID 9731649

  • Repair of congenital tricuspid valve abnormalities with artificial chordae tendineae ANNALS OF THORACIC SURGERY Reddy, V. M., McElhinney, D. B., Brook, M. M., Silverman, N. H., Stanger, P., Hanley, F. L. 1998; 66 (1): 172-176

    Abstract

    Congenital abnormalities of the tricuspid valve, including Ebstein's malformation, dysplasia, straddling, and those found in pulmonary atresia with intact septum and congenitally corrected transposition, are an uncommon cause of tricuspid regurgitation. Congenital tricuspid valve anomalies are found as a spectrum of disease in which both the leaflets and the subvalvar apparatus are often involved. Tricuspid valve repair is complicated in such patients because the chordae tendineae are often abnormally short and thick. Replacement or augmentation of chordae tendineae has proved to be a useful component of mitral valve repair. In the present report, we describe the techniques and results of chordal augmentation in the repair of congenital tricuspid valve abnormalities.Since July 1992, tricuspid valve repair has been performed in 5 children with severe tricuspid regurgitation secondary to congenital abnormalities of the tricuspid valve with significant chordal pathology. As a component of the repair, chordal replacement or augmentation was performed using expanded polytetrafluoroethylene suture.Intraoperative and postoperative echocardiographic assessment showed good mobility of the tricuspid valve leaflets and trivial to mild tricuspid regurgitation. There were no complications and no early or late mortality. At follow-up of 34 to 60 months (median, 49 months), tricuspid valve function has remained excellent in 4 of the 5 patients. In the remaining patient, progressive regurgitation of the right ventricle to pulmonary artery allograft conduit has led to right ventricular dilatation, with a secondary increase in tricuspid regurgitation from trivial to moderate.Chordal replacement or augmentation with expanded polytetrafluoroethylene suture is a useful technique in the repair of congenitally dysplastic tricuspid valves with abnormal chordal structures.

    View details for Web of Science ID 000074992000038

    View details for PubMedID 9692459

  • Congenital cardiac anomalies with vein of Galen malformations in infants ARCHIVES OF DISEASE IN CHILDHOOD McElhinney, D. B., Halbach, V. V., Silverman, N. H., Dowd, C. F., Hanley, F. L. 1998; 78 (6): 548-551

    Abstract

    Published reports and personal experience are reviewed relating to patients under 1 year of age diagnosed with a vein of Galen malformation and congenital heart disease. Including five patients from this institution, a total of 23 patients (12 neonates) with congenital heart disease and a vein of Galen malformation have been reported. Six of these had sinus venosus atrial septal defect and nine had aortic coarctation.

    View details for Web of Science ID 000074060300011

    View details for PubMedID 9713012

  • Geometric mismatch of pulmonary and aortic anuli in children undergoing the Ross procedure: Implications for surgical management and autograft valve function JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Phoon, C. K., Brook, M. M., Hanley, F. L. 1998; 115 (6): 1255-1262

    Abstract

    There is often substantial mismatch between the diameters of the pulmonary and aortic anuli in young patients with systemic outflow tract disease. To implant the autologous pulmonary valve in the aortic position under such circumstances, it is necessary to adapt the geometry of the systemic outflow tract. The effects of such adaptations on autograft function in children are not well known.To determine factors predictive of autograft regurgitation, we analyzed 41 cases of children who have undergone the Ross procedure. The diameter of the pulmonary valve was greater (by at least 3 mm) than that of the aortic valve in 20 cases, equal (within 2 mm) in 12 cases, and less (by at least 3 mm) in nine cases, with differences ranging from +10 to -12 mm. In 12 patients with a larger pulmonary anulus, aortoventriculoplasty was used to correct the mismatch. In patients with a larger aortic anulus, the mismatch was corrected by gradual adjustment along the circumference of the autograft, rather than by tailoring of the native aortic anulus.At follow-up (median 31 months), two patients had undergone reoperation on the neoaortic valve for moderate regurgitation. In the remaining 38 cases, autograft regurgitation was as follows: none or trivial in 30, mild in seven, and moderate in one. There was no correlation between regurgitation and age, geometric mismatch, or previous or concurrent procedures.Subtle technical factors that may result in distortion of the valve complex are probably more important determinants of autograft regurgitation than are indication for repair, geometric mismatch, or previous or concomitant outflow tract procedures. Significant mismatch of the semilunar anuli is not a contraindication to the Ross procedure in children.

    View details for Web of Science ID 000074093600004

    View details for PubMedID 9628666

  • Atrioventricular valve function after single patch repair of complete atrioventricular septal defect in infancy: How early should repair be attempted? JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Brook, M. M., Parry, A. J., Hanley, F. L. 1998; 115 (5): 1032-1038

    Abstract

    Though repair of complete atrioventricular septal defect in infancy has become routine at most centers, it is not unusual for very young infants to be managed medically because of concerns about the fragility of the atrioventricular valve tissue.Since July 1992, seventy-two infants have undergone primary repair of complete atrioventricular septal defects at a median age of 3.9 months (40% < 3 months). A single-patch technique was used in all patients. The cleft was closed completely in 61 patients and partially (n = 10) or not at all (n = 1) in select patients at risk for valve stenosis. Left atrioventricular valve annuloplasty was performed in 18 patients. On the basis of transesophageal echocardiographic findings, 10 patients were returned to bypass for revision of the valve repair.There was one early death in a patient with single left papillary muscle, no early reoperations, and no new permanent arrhythmias. Only three patients had moderate left atrioventricular valve regurgitation at discharge. During a median follow-up of 24 months, there was one late death and five reoperations for left atrioventricular valve regurgitation (n = 2) and/or systemic outflow obstruction (n = 4). Follow-up left atrioventricular valve regurgitation was moderate in three patients, mild in 14, and none/trace in 54. Age had no relation to postoperative atrioventricular valve regurgitation, death, or reoperation.Despite concerns about fragility of valve tissue in very young patients, excellent results can be achieved with meticulous techniques. From neonates to older infants, age at repair does not influence outcome or valve function.

    View details for Web of Science ID 000073609200011

    View details for PubMedID 9605072

  • Left pulmonary artery kinking caused by outflow tract dilatation after transannular patch repair of tetralogy of fallot ANNALS OF THORACIC SURGERY McElhinney, D. B., Parry, A. J., Reddy, V. M., Hanley, F. L., Stanger, P. 1998; 65 (4): 1120-1126

    Abstract

    Stenosis of the branch pulmonary arteries after tetralogy of Fallot repair can result from several mechanisms. In patients with free pulmonary regurgitation and right ventricular dilatation after transannular patch repair, we have observed that the pulmonary outflow tract can dilate and elongate craniad and rotate to the left, resulting in kinking and obstruction of the previously normal left pulmonary artery.Ten patients referred for reoperation after tetralogy repair with severe pulmonary regurgitation and right ventricular outflow tract dilatation were found to have left pulmonary artery kinking. In 8 of these patients kinking was the sole or partial cause of left pulmonary artery obstruction, whereas there was no documented obstruction in the other 2. All patients underwent right ventricular outflow tract reconstruction and left pulmonary artery repair with removal of the redundancy at the kink point. Patching alone is not an effective method of repair in this condition, because the mechanism of obstruction is not corrected and the patch material can become redundant and lead to recurrent obstruction.All patients underwent successful pulmonary outflow tract reconstruction. Left pulmonary artery kinking was corrected in all patients, and relief of obstruction was attained in the 8 patients with stenosis. At follow-up ranging from 9 to 58 months, no patient has evidence of recurrent left pulmonary artery stenosis.Left pulmonary artery kinking should be suspected at long-term follow-up after tetralogy repair in patients with significant pulmonary regurgitation and right-sided dilatation, even if previous evaluations showed no evidence of left pulmonary arterial abnormality. Because unilateral obstruction caused by kinking may lead to asymmetric pulmonary flow, it can exacerbate pulmonary regurgitation and right ventricular dilatation, in effect accelerating the processes that led to kinking in the first place. Kinking can be relieved successfully with the techniques described in this report.

    View details for Web of Science ID 000073160100049

    View details for PubMedID 9564939

  • Trends in the management of truncal valve insufficiency ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Rajasinghe, H. A., Mora, B. N., Silverman, N. H., Hanley, F. L. 1998; 65 (2): 517-524

    Abstract

    The single semilunar valve in patients with truncus arteriosus frequently is dysplastic and dysfunctional. Truncal valve insufficiency has been associated with poor outcome. Although the management of truncal valve insufficiency has evolved over the years, approaches to this problem vary considerably and remain a serious dilemma in many cases.We reviewed the records of 89 patients with unrepaired truncus arteriosus and mild (n = 37), moderate (n = 33), or severe (n = 19) truncal valve insufficiency who were admitted to our institution between 1975 and 1995. Eight patients (7 neonates) with moderate or severe insufficiency died before surgical intervention, and 4 patients underwent palliative pulmonary artery banding. The remaining 77 patients underwent repair. The median age at repair was 3.2 months (range, 2 days to 15 years; 83% infants), and it decreased from 4 months between 1975 and 1985 to 1 month between 1986 and 1995. Truncal valve replacement (mechanical = 6, allograft = 4) was performed in 10 patients, and 5 patients underwent valve repair.All 4 patients who underwent pulmonary artery banding died either early or late. The hospital (or 30-day) mortality rate after repair was 34% (26/77). At a median follow-up of 10 years, 11 hospital survivors had died, with overall 1- and 10-year actuarial survival rates of 56% and 48%, respectively, and poorer survival among patients with severe truncal valve insufficiency (p = 0.02). Late truncal valve replacement (n = 24) had been performed in 21 patients. Freedom from truncal valve replacement was better in patients with mild truncal insufficiency than in those with moderate or severe preoperative insufficiency (p < 0.001). Four late deaths were related directly to reoperation for truncal valve replacement or to prosthetic valve dysfunction. Three of the 4 neonates who received allograft root replacements died within 7 months of repair, and severe allograft valve insufficiency requiring replacement 1 year after operation developed in the fourth.The prospects for patients with truncal valve insufficiency have been improving over time. Nevertheless, the results in patients with severe insufficiency continue to be poor.

    View details for Web of Science ID 000072128100046

    View details for PubMedID 9485257

  • Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants < 6 months of age AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Silverman, N. H., Hanley, F. L. 1998; 81 (2): 195-201

    Abstract

    The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.

    View details for Web of Science ID 000071617500015

    View details for PubMedID 9591904

  • Cytokine response to fetal cardiac bypass JOURNAL OF MATERNAL-FETAL INVESTIGATION Reddy, V. M., McElhinney, D. B., Rajasinghe, H. A., Rodriguez, J. L., Hanley, F. L. 1998; 8 (1): 46-49
  • An institutional experience with the bidirectional cavopulmonary shunt: do we know enough about it? CARDIOLOGY IN THE YOUNG Reddy, V. M., McElhinney, D. B., Moore, P., Bristow, J., Haas, G. S., Hanley, F. L. 1997; 7 (3): 284-293
  • Measurement of collateral blood flow in a porcine model of aortic coarctation by velocity-encoded cine MRI JOURNAL OF MAGNETIC RESONANCE IMAGING Chernoff, D. M., Derugin, N., Rajasinghe, H. A., Hanley, F. L., Higgins, C. B., Gooding, C. A. 1997; 7 (3): 557-563

    Abstract

    The purpose of this study was to investigate the time course of development of collateral blood flow in an animal model of aortic coarctation. A juxtaductal aortic stenosis (model coarctation) was surgically created in five juvenile pigs. MRI was performed preoperatively, 1 to 2 days postoperatively, and 2 to 10 weeks postoperatively. Aortic blood flow was measured by velocity-encoded cine MR (VENC-MR). The percent change in aortic blood flow (delta BF) from proximal to distal descending thoracic aorta was calculated, and a multiple-comparison paired t test used to assess changes in delta BF over time. Invasive flow measurements were obtained in one animal before sacrifice using an ultrasonic probe. delta BF preoperatively was -2 +/- 8% (mean +/- SE). delta BF increased to 32 +/- 7% (mean +/- SE, P = .022) 2 days postoperatively and 55 +/- 19% (P = .032) 2 to 8 weeks postoperatively. Invasive measurements were in qualitative agreement with the VENC-MR data. VENC-MR is an accurate noninvasive method of measuring collateral blood flow in aortic coarctation. Recruitment and development of collateral flow pathways occur rapidly in an animal model.

    View details for Web of Science ID A1997XA92300017

    View details for PubMedID 9170042

  • Outcomes after bidirectional cavopulmonary shunt in infants less than 6 months old Reddy, V. M., McElhinney, D. B., Moore, P., Haas, G. S., Hanley, F. L. ELSEVIER SCIENCE INC. 1997: 1365-1370

    Abstract

    We sought to assess the results after bidirectional cavopulmonary shunt (BCPS) in infants < 6 months old and to identify risk factors for poor outcome.Although BCPS is a well established procedure for the palliation of patients with a single-ventricle heart, there have been very few reports of outcomes after BCPS in young infants.Since 1990, 42 infants between 0.8 and 6.0 months of age (mean [+/-SD] 3.7 +/- 1.4) have undergone BCPS for primary (n = 16) or secondary (n = 26) palliation of tricuspid atresia (n = 13), hypoplastic left heart syndrome (n = 10) or other forms of functional single-ventricle heart (n = 19). Accessory pulmonary blood flow was included in 18 patients. Preoperative and perioperative data were gathered on retrospective review of patient records, and follow-up was conducted by means of direct physician contact or record review.The overall hospital mortality rate, including that associated with reoperations, was 4.8% (2 of 42 patients). Seven patients (17%) required reoperation related to the BCPS or pulmonary blood flow in the early postoperative period: Procedures included take-down of the BCPS in four patients, with one early death, and procedures to decrease pulmonary blood flow in three patients. Age < 1 month correlated significantly with early death and with early failure of the BCPS (death or take-down). Follow-up of the 37 patients discharged with intact BCPS was obtained at a mean +/-SD of 14.3 +/- 11.3 months postoperatively, during which time three patients died (at 6.5 +/- 2.5 months). The 2-year actuarial survival rate for patients undergoing BCPS at < 6 months of age was 86%. Overall freedom from death or take-down (including early and late events) was significantly lower in patients < 2 months old than in those > 2 months old. Four patients have undergone successful Fontan completion (18.3 +/- 2.9 months postoperatively), and one patient whose BCPS was taken down subsequently underwent successful restoration of a BCPS.Outcomes after BCPS in young infants are comparable to those in older infants and children. However, our current preference is to defer this procedure until after 2 months of age.

    View details for Web of Science ID A1997WW57700033

    View details for PubMedID 9137237

  • Long-term follow-up of truncus arteriosus repaired in infancy: A twenty-year experience Rajasinghe, H. A., McElhinney, D. B., Reddy, V. M., Mora, B. N., Hanley, F. L. MOSBY-YEAR BOOK INC. 1997: 869-878

    Abstract

    There have been few reports of long-term follow-up after truncus arteriosus repair in infancy.A retrospective review was performed to assess long-term outcomes among 165 patients who survived the initial hospital stay after complete repair of truncus arteriosus since 1975. The median age at truncus repair over this 20-year experience was 3.5 months (range 2 days to 36 years), and 81% of patients were less than 1 year of age. Previous pulmonary artery banding had been performed in 15 patients, and two patients had undergone prior repair of interrupted aortic arch. Significant procedures performed along with truncus repair included truncal valve replacement (n = 10) or repair (n = 5) and repair of interrupted aortic arch (n = 4).Patients were followed up for up to 20.4 years (median 10.5 years). Twenty-five patients were lost at cross-sectional follow-up, with a total of 67 patient-years of follow-up available on these patients. There have been 23 late deaths, eight of which occurred within 6 months of repair and 13 of which occurred within 1 year. Ten of the late deaths were related to reoperations. Actuarial survival among all hospital survivors was 90% at 5 years, 85% at 10 years, and 83% at 15 years and was essentially identical for infants alone. A significant independent risk factor for poorer long-term survival was truncus with moderate to severe truncal valve insufficiency before repair. During the follow-up period, 107 patients underwent 133 conduit reoperations. Median time to conduit reoperation was 5.5 years, and the only factor significantly associated with shorter time to conduit replacement was smaller conduit size at initial repair. In addition, 26 patients underwent 30 truncal valve replacements. Six patients required truncal valve replacement before any conduit-related reintervention, with two associated deaths. Actuarial freedom from truncal valve replacement among patients with no prerepair truncal valve insufficiency was 95% at 10 years. Actuarial freedom from truncal valve replacement was significantly lower among patients with truncal insufficiency before initial repair (63% at 10 years). At follow-up, all patients except three were in New York Heart Association functional class I.Ten- to 20-year survival and functional status are excellent among infants undergoing complete repair of truncus arteriosus. Conduit replacement or revision is almost inevitably necessary in this group of patients.

    View details for Web of Science ID A1997WZ62100008

    View details for PubMedID 9159620

  • Revision of previous fontan connections to extracardiac or intraatrial conduit cavopulmonary anastomosis McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L. ELSEVIER SCIENCE INC. 1996: 1276-1282

    Abstract

    In patients who have received an atriopulmonary Fontan connection, complications such as right pulmonary vein obstruction, atrial arrhythmias, and thromboembolism are often secondary to right atrial enlargement. When such complications develop despite good ventricular function, there are few management options available. Extracardiac or intraatrial conduit cavopulmonary anastomosis, which improves central systemic venous flow patterns, avoids atrial distention, and does not involve the extensive atrial suturing required by other forms of cavopulmonary anastomosis, may provide relief for this group of patients.Between October 1992 and October 1995, 7 patients presented 8 to 20 years after atriopulmonary connection with severe right atrial dilatation (7), Fontan pathway obstruction (4), progressive congestive heart failure (4), atrial tachydysrhythmias (3), right atrial thrombus (1), obstruction of right pulmonary veins by an enlarged right atrium (1), and subaortic stenosis (1). After evaluation of the options, they underwent revision of the atriopulmonary connection to extracardiac (5) or intraatrial (2) conduit cavopulmonary anastomosis.One patient with severe cachexia, in whom transplantation was contraindicated for social reasons, died in the early postoperative period of massive effusions. Two patients eventually required permanent pacing for atrial dysrhythmias (1) or complete heart block secondary to subaortic fibromuscular resection (1), and 2 demonstrated marked improvement in unstable preoperative rhythm disturbances. At a median follow-up of 17 months, 4 of the 6 survivors were functioning at higher New York Heart Association levels than preoperatively, and 1 had recently undergone heart transplantation.In properly selected patients with atrial complications, revision of a prior Fontan connection to extracardiac or intraatrial conduit cavopulmonary anastomosis appears to be a viable option.

    View details for Web of Science ID A1996VQ16700007

    View details for PubMedID 8893557

  • Homografts in congenital heart disease: Current applications and future directions McElhinney, D. B., Reddy, V. M., Hanley, F. L. ISRAEL JOURNAL MED SCIENCES. 1996: 880-885

    Abstract

    The first clinical use of homograft tissue in cardiovascular surgery was in 1948, when Gross used cadaveric arterial grafts to construct systemic to pulmonary artery shunts in patients with tetralogy of Fallot, and to repair coarctation of the aorta. Eighteen years later, a valved homograft was used for the first time in the treatment of congenital heart disease for reconstruction of the right ventricular outflow tract in a child with pulmonary atresia. Since these pioneering advances, valve and vascular homografts have become central to the management of congenital anomalies of the heart and great vessels. The primary use for homografts in congenital heart surgery today is establishment of a valved connection between the right ventricle and pulmonary arteries in children with tetralogy of Fallot with pulmonary atresia or other complicating factors, truncus arteriosus, transposition complexes, and double-outlet right ventricle, and in patients undergoing the Ross procedure. Homograft reconstruction of the left ventricular outflow tract has also been performed for many years in children with aortic insufficiency or recurrent aortic stenosis, but early homograft degeneration in young children has been a significant problem. Many surgeons are turning away from homografts in the systemic circulation in favor of the pulmonary autograft. Homograft is also widely used as a vascular patch material. In the present report, we discuss the various uses of homografts in congenital heart surgery, the benefits and drawbacks of homografts in young patients, and some of the future possibilities for homograft development and application in patients with congenital heart disease.

    View details for Web of Science ID A1996VU63200023

    View details for PubMedID 8950257

  • The Ross procedure in children Reddy, V. M., McElhinney, D. B., Hanley, F. L. ISRAEL JOURNAL MED SCIENCES. 1996: 888-891

    Abstract

    Although it has been almost 30 years since Ross described his technique for aortic valve replacement using the pulmonary autograft, only recently has there been widespread interest in the use of the Ross procedure for aortic valve replacement in children with congenital heart disease. Since July 1992 we have performed the Ross procedure in 46 patients, 40 of whom were < or = 18 years of age at the time of surgery. In 10 children the procedure was combined with Konno ventriculoplasty, and in 3 others a Ross-Konno procedure was used for biventricular repair of borderline hypoplastic left heart syndrome. The only early deaths were in two of the patients with borderline hypoplastic left heart syndrome. There has been no late mortality, and two patients have required reoperation: one for recurrent distal aortic arch obstruction, and one for moderate-severe autograft insufficiency. Freedom from reoperation at 30 months is 89%. Aside from one patient with moderate autograft insufficiency and eight with mild regurgitation, all patients have trace or no aortic insufficiency at follow-up ranging from 1 to 38 months (median 22 months). The Ross procedure is an important advance in the treatment of aortic valve disease in pediatric patients. It is the only available replacement for the aortic valve that has growth potential, and there is ample evidence that the pulmonary autograft holds up well in the systemic circulation.

    View details for Web of Science ID A1996VU63200025

    View details for PubMedID 8950259

  • Morphologic determinants favoring surgical aortic valvuloplasty versus pulmonary autograft aortic valve replacement in children VANSON, J. A., Reddy, V. M., Black, M. D., Rajasinghe, H., Haas, G. S., Hanley, F. L. MOSBY-YEAR BOOK INC. 1996: 1149-1156

    Abstract

    The pulmonary autograft is being used with increasing frequency to replace the diseased aortic valve in the pediatric population. Attempted surgical aortic valvuloplasty with an unacceptable result and return to cardiopulmonary bypass for aortic valve replacement with a pulmonary autograft results in prolonged bypass time and increased potential for morbidity. Therefore, the ability to predict an unsuccessful outcome for valvuloplasty would be of significant clinical benefit. This issue is addressed in the present study. Methods: Twenty-two patients (median age 5.7 years, range 3 weeks to 14 years) with bicuspid (n = 11), tricuspid (n = 9), or quadricuspid (n = 2) aortic valves underwent valvuloplasty for aortic stenosis (n = 9), aortic regurgitation (n = 7), or a combination (n = 6). Previous related procedures included balloon aortic valvuloplasty (n = 3) and open surgical valvotomy (n = 1). Median pressure gradient across the aortic valve was 80 mm Hg. Surgical valvuloplasty techniques included thinning of leaflets (n = 18), commissurotomy (n = 15), suspension of reconstructed leaflet to the aortic wall (n = 10), closure of leaflet fenestration (n = 5), shortening of free edge of prolapsed cusp (n = 4), repair of torn leaflets (n = 3), and augmentation of scarred leaflets with autologous pericardium (n = 3). Concomitant subvalvular and supravalvular stenosis were repaired in nine and four patients, respectively. In five patients, during the same hospital stay, a failed valvuloplasty was converted into a valve replacement with a pulmonary autograft because of residual or resultant stenosis (n = 3) or regurgitation (n = 2). Results: No early or late deaths occurred. At a median follow-up of 16.3 months the median pressure gradient across the aortic valve in the 15 patients with preoperative stenosis or combined stenosis and regurgitation was 16 mm Hg (p < 0.01 versus preoperative gradient). Of the 22 patients, the aortic valve functioned normally (defined as < or = mild stenosis or regurgitation, or both) in 14 patients (including five patients with valve replacement); four patients had stenosis (gradients 40, 45, 60, and 60 mm Hg), two patients had regurgitation, and two patients had combined stenosis (gradients 40 and 50 mm Hg) and regurgitation. Three of the patients with recurrent stenosis underwent secondary surgical valvuloplasty without improvement. Outcome after valvuloplasty was examined according to valve structure: six of nine tricuspid valves functioned normally, whereas only three of 13 nontricuspid valves functioned normally (P = 0.07). Patients with a nontricuspid aortic valve and regurgitation had a high probability of requiring immediate valve replacement (P = 0.009). The actuarial freedom from significant native valve stenosis or regurgitation at 24 months was 82% for tricuspid valves and 36% for nontricuspid valves (P = 0.007). Conclusions: (1) Surgical aortic valvuloplasty should be the preferred approach when the aortic valve is tricuspid. (2) In contrast, aortic valve replacement with a pulmonary autograft should be the preferred strategy in the presence of a nontricuspid aortic valve (especially when the aortic valve is regurgitant) and after failed surgical valvuloplasty.

    View details for Web of Science ID A1996UQ38300007

    View details for PubMedID 8642815

  • Extending the limits of the Ross procedure Reddy, V. M., Rajasinghe, H. A., McElhinney, D. B., VANSON, J. A., Black, M. D., Silverman, N. H., Hanley, F. L. ELSEVIER SCIENCE INC. 1995: S600-S603

    Abstract

    The potential for growth and the proven long-term durability of the native pulmonary valve make it ideal for replacement of the diseased aortic valve, especially in growing children. The use of the autologous pulmonary valve can be further extended to patients with complex left ventricular outflow tract obstruction and to neonates and infants.Between June 1993 and May 1995, 35 patients underwent the Ross procedure at our center. Of these, 15 (43%) had complex left ventricular outflow tract obstruction and 7 (20%) were infants, including 3 neonates. The autologous pulmonary valve was implanted as a root replacement with coronary reimplantation in all patients. Additional left ventricular outflow tract procedures performed were ventricular myectomy in 7 patients and a Konno type aortoventriculoplasty in 11 patients.There was one early death in a patient with borderline hypoplastic left heart syndrome. At a median follow-up of 9 months (range, 0.2 to 22 months) there were no late deaths or reinterventions. The autologous pulmonary valve function was excellent, with 1 (2.8%) patient having moderate insufficiency.Autologous pulmonary valve is an excellent option for aortic valve replacement in all age groups. Its use can be readily extended to neonates, infants, and patients with complex left ventricular obstruction requiring additional left ventricular outflow tract procedures.

    View details for Web of Science ID A1995TW27300030

    View details for PubMedID 8604945

  • Routine primary repair of tetralogy of Fallot in neonates and infants less than three months of age Reddy, V. M., Liddicoat, J. R., McElhinney, D. B., Brook, M. M., Stanger, P., Hanley, F. L. ELSEVIER SCIENCE INC. 1995: S592-S596

    Abstract

    Although primary repair of tetralogy of Fallot is increasingly undertaken in infancy, complete repair is generally performed in only selected symptomatic neonates.From July 1992 through March 1995, 30 consecutive neonates and young infants with tetralogy of Fallot underwent routine primary repair. Group I (n = 10) consisted of patients with tetralogy of Fallot and pulmonary atresia (n = 5) or severe pulmonary stenosis (n = 5) who were duct dependent and were repaired in the neonatal period. Group II (n = 11) consisted of patients who were asymptomatic with arterial oxygen saturation between 75% and 90% (adequate pulmonary blood flow). Group III (n = 9) consisted of patients with "pink" tetralogy of Fallot (arterial oxygen saturation > 90%). Patients in groups II and III were electively scheduled for repair at about 2 months of age.The postrepair peak systolic right ventricular-to-peak systolic left ventricular pressure ratio did not correlate (p = 0.96) with the branch pulmonary artery size. One patient died 2 months after operation, despite good hemodynamics, of uncontrollable diffuse subcutaneous edema due to familial distichiasis lymphedema syndrome. There were no late deaths. At a median follow-up of 19 months, 1 patient underwent balloon dilation of branch pulmonary arteries. Follow-up echocardiography revealed a 30 to 60 mm Hg right ventricle-to-pulmonary artery gradient in 3 patients.Excellent early and midterm results can be accomplished with routine primary repair of tetralogy of Fallot in early infancy regardless of age, symptomatic status, coronary anatomy, and the size of branch pulmonary arteries as long as they arborize normally.

    View details for Web of Science ID A1995TW27300028

    View details for PubMedID 8604943

  • Performance of right ventricle to pulmonary artery conduits after repair of truncus arteriosus: a comparison of Dacron-housed porcine valves and cryopreserved allografts. Seminars in thoracic and cardiovascular surgery Reddy, V. M., Rajasinghe, H. A., McElhinney, D. B., Hanley, F. L. 1995; 7 (3): 133-138

    Abstract

    Repair of complex congenital heart defects often requires the use of extracardiac conduits. These repairs can be technically accomplished with excellent early results. However, the long-term performance of various conduits is less than optimal. In this report, we examined the long-term outcome of xenograft valved Dacron conduits and cryopreserved allograft valved conduits used for reconstruction of the right ventricular outflow tract in young patients with truncus arteriosus. A retrospective review was performed on 222 patients who underwent primary surgical repair of truncus arteriosus between January 1975 and December 1994 using a xenograft valved synthetic conduit (group I; n = 175) or with a cryopreserved valved allograft conduit (group II; n = 47). Median age at repair was 121 days in group I and 70 days in group II; median weight of patients in both groups was the same (4.2 kg). Conduit-related early deaths occurred in 5.7% (10/175) of patients in group I and none in group II. In a cohort of age-matched patients, actuarial freedom from conduit-related reintervention at 5 years follow-up was significantly better (P = .037) for the cryopreserved valved allograft conduits when compared with xenograft valved synthetic conduits. However, this difference was not apparent by 7 years. Multivariate analysis showed conduit size (P = .0005) as a significant predictor of early conduit-related reintervention. This study shows that mid-term performance of cryopreserved allografts (which have the advantage of technical ease of insertion of availability of small sizes for use in neonates), is better than the xenograft Dacron conduits.(ABSTRACT TRUNCATED AT 250 WORDS)

    View details for PubMedID 7548319

  • MIDLINE ONE-STAGE COMPLETE UNIFOCALIZATION AND REPAIR OF PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT AND MAJOR AORTOPULMONARY COLLATERALS Reddy, V. M., Liddicoat, J. R., Hanley, F. L. MOSBY-YEAR BOOK INC. 1995: 832-845

    Abstract

    Traditionally patients with pulmonary atresia, ventricular septal defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. We have taken a different approach to this lesion. Between August 1992 and March 1994, ten patients aged 1.43 months to 37.34 years (median 2.08 years) at the severe end of the morphologic spectrum of this lesion underwent a one-stage complete unifocalization and repair from a midline sternotomy approach. The median Nakata index of true pulmonary arteries was 50.0 (range 0 to 103.13) and they provided vascular supply to up to nine lung segments (median 5 segments). The number of collaterals per patient ranged from two to five with a median of four. The collaterals provided vascular supply to a median of 15 lung segments per patient (range 11 to 20). Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. In only one patient (37.34 years old) was it necessary to use a non-native conduit for peripheral pulmonary artery reconstruction. The ventricular septal defect was left open in one patient (5 years old) because of diffuse distal hypoplasia and stenosis of the pulmonary arteries and the collaterals. The postrepair peak systolic right ventricular/left ventricular pressure ratio ranged from 0.31 to 0.58 (median 0.47). There were no early deaths. Complications were bleeding necessitating reexploration in one patient, phrenic nerve palsy in three patients, and severe bronchospasm in three patients. Follow-up (median 8 months, range 2 to 19 months) was complete in all patients. One patient was reoperated on for pseudoaneurysm of the central homograft conduit and then again for stenosis of the left lower lobe collateral. After this last operation at 13 months after the initial repair she died of a preventable cardiac arrest caused by pneumothorax. The patient with open ventricular septal defect underwent balloon dilation of the unifocalized pulmonary arteries, with a current pulmonary/systemic flow ratio of 1.4 to 1.8:1, and is awaiting ventricular septal defect closure. One other patient underwent balloon dilation of the reconstructed right pulmonary artery, with a good result. All survivors (9/10) are clinically doing well. This approach establishes normal cardiovascular physiology early in life, eliminates the need for multiple systemic-pulmonary artery shunts and use of prosthetic material, and minimizes the number of operations required.(ABSTRACT TRUNCATED AT 400 WORDS)

    View details for Web of Science ID A1995QX36800002

    View details for PubMedID 7739241

  • TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE IN NEWBORNS WITH VISCERAL HETEROTAXY ANNALS OF THORACIC SURGERY Heinemann, M. K., Hanley, F. L., VANPRAAGH, S., Fenton, K. N., Jonas, R. A., Mayer, J. E., Castaneda, A. R. 1994; 57 (1): 88-91

    Abstract

    Children with visceral heterotaxy often present with total anomalous pulmonary venous drainage (TAPVD) associated with univentricular congenital heart disease. We reviewed our experience with the primary surgical management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitted to our institution and underwent primary palliation. Twenty-one of them had TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were seen with obstruction of the anomalous connection and underwent emergency operation. In 7 patients, repair of TAPVD was combined with a systemic-pulmonary artery shunt because of additional obstruction of the pulmonary blood supply, with two deaths. One patient had primary shunting and then repair of TAPVD. Four patients underwent repair of TAPVD alone. Two of them then showed signs of insufficient pulmonary blood flow, received a shunt in a second procedure, and subsequently died. Early mortality in the group with obstructed TAPVD was thus 4 (33%) of 12 patients. Statistical analysis of all 38 patients (univariate analysis, chi 2 testing) showed that neither the presence of TAPVD (p = 0.7) nor TAPVD repair alone (p = 0.8) or with shunting (p = 0.8) was a definite risk factor for early death. The performance of a shunt during the first operation, however, was associated with lower early mortality (p = 0.03). Total anomalous pulmonary venous drainage is a common finding in newborns with visceral heterotaxy. Its presence and its subsequent early repair (requiring cardiopulmonary bypass) do not increase the mortality risk. The need of a concomitant shunt in obstructed TAPVD can initially be underestimated.

    View details for Web of Science ID A1994MR31800016

    View details for PubMedID 8279925

  • REPAIR OF TRUNCUS ARTERIOSUS IN THE NEONATE JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Hanley, F. L., Heinemann, M. K., Jonas, R. A., Mayer, J. E., Cook, N. R., Wessel, D. L., Castaneda, A. R. 1993; 105 (6): 1047-1056

    Abstract

    From September 1986 through December 1991, 63 patients with truncus arteriosus underwent surgical repair. The management approach evolved over the period of the study from elective primary repair at 3 months of age to elective primary repair in the early neonatal period. Thirty variables were examined as potential risk factors for the outcome events of death, reoperation, and presence of pulmonary vascular morbidity. According to both univariate and multivariate techniques, severe truncal valve regurgitation, interrupted aortic arch, coronary artery anomalies, and age at repair greater than 100 days were important risk factors for perioperative death. In the 33 patients without these risk factors, early survival was 100%. In the 30 patients with one or more of these risk factors, survival was 63%. Pulmonary hypertensive episodes were fewer, and duration of ventilator dependence and pulmonary artery pressure were significantly less in patients undergoing the operation before 30 days of age. Seven patients required reoperation for right ventricular outflow tract obstruction at a mean follow-up time of 23.4 months, with no deaths. In conclusion, interrupted aortic arch, severe truncal valve insufficiency, coronary anomalies, and repair later than 100 days of age were risk factors for death after repair of truncus arteriosus. In the absence of these associations, truncus arteriosus can be repaired with excellent surgical outcome in the neonatal and early infancy period. Repair in the early neonatal period reduces the prevalence of postoperative pulmonary vascular morbidity.

    View details for Web of Science ID A1993LG08700011

    View details for PubMedID 8501932

  • FATE OF SMALL HOMOGRAFT CONDUITS AFTER EARLY REPAIR OF TRUNCUS ARTERIOSUS ANNALS OF THORACIC SURGERY Heinemann, M. K., Hanley, F. L., Fenton, K. N., Jonas, R. A., Mayer, J. E., Castaneda, A. R. 1993; 55 (6): 1409-1412

    Abstract

    Neonatal repair of truncus arteriosus is being performed in a number of centers, often with the use of small homograft conduits. The fate of the homograft and the risk of replacement were the subjects of this study. Between January 1987 and October 1991, 43 infants aged less than 3 months (range, 3 to 90 days) underwent primary repair of truncus arteriosus including implantation of a valved homograft conduit (diameter, 7 to 12 mm). Twenty-nine had follow-up of more than 6 months (range, 6 to 65 months; mean, 21.9 months). After a mean period of 31 months (range, 8 to 65 months), 7 patients showed obstruction with right ventricular pressures at least 75% systemic and underwent either a conduit change (n = 5) or a patch augmentation (n = 2). Mean cardiopulmonary bypass time at reoperation was 99 minutes; mortality was zero. Five other children are known to have a right ventricular pressure of 50% to 60% systemic, 2 having undergone balloon dilation. Statistical comparison of the patients with conduit reoperation or high right ventricular pressure (n = 12) with the rest of the population (n = 17) revealed an elevated pulmonary artery to right ventricular pullback gradient on postoperative day 1 after the repair (7.7 versus 1.3 mm Hg; p = 0.001) and choice of an aortic over a pulmonary homograft (100% versus 64.7%; p = 0.065) as significant risk factors. Age and weight at repair, postoperative pulmonary artery pressure, length of follow-up, and size of the homograft showed no significant differences between the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

    View details for Web of Science ID A1993LH44100011

    View details for PubMedID 8512389

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