Changes in Efficiency and Safety Culture After Integration of an I-PASS-Supported Handoff Process.
2016; 137 (2): 1-9
Isolation of the right subclavian artery in a patient with d-transposition of the great arteries.
Annals of pediatric cardiology
2015; 8 (2): 161-163
Recent publications have shown improved outcomes associated with resident-to-resident handoff processes. However, the implementation of similar handoff processes for patients moving between units and teams with expansive responsibilities presents unique challenges. We sought to determine the impact of a multidisciplinary standardized handoff process on efficiency, safety culture, and satisfaction.A prospective improvement initiative to standardize handoffs during patient transitions from the cardiovascular ICU to the acute care unit was implemented in a university-affiliated children's hospital.Time between verbal handoff and patient transfer decreased from baseline (397 ± 167 minutes) to the postintervention period (24 ± 21 minutes) (P < .01). Percentage positive scores for the handoff/transitions domain of a national culture of safety survey improved (39.8% vs 15.2% and 38.8% vs 19.6%; P = .005 and 0.03, respectively). Provider satisfaction improved related to the information conveyed (34% to 41%; P = .03), time to transfer (5% to 34%; P < .01), and overall experience (3% to 24%; P < .01). Family satisfaction improved for several questions, including: "satisfaction with the information conveyed" (42% to 70%; P = .02), "opportunities to ask questions" (46% to 74%; P < .01), and "Acute Care team's knowledgeabout my child's issues" (50% to 73%; P = .04). No differences in rates of readmission, rapid response team calls, or mortality were observed.Implementation of a multidisciplinary I-PASS-supported handoff process for patients transferring from the cardiovascular ICU to the acute care unit resulted in improved transfer efficiency, safety culture scores, and satisfaction of providers and families.
View details for DOI 10.1542/peds.2015-0166
View details for PubMedID 26743818
Implementation of Data Drive Heart Rate and Respiratory Rate parameters on a Pediatric Acute Care Unit.
Studies in health technology and informatics
2015; 216: 918-?
Isolation of the right subclavian artery (RSCA) is rare, and this finding in association with d-transposition of the great arteries (d-TGA) is extremely unusual. We present a case of an isolated RSCA in a newborn with d-TGA in whom the clinical presentation was diagnostic. We discuss the imaging modalities used to confirm the diagnosis, the embryological basis of the finding, and the surgical repair.
View details for DOI 10.4103/0974-2069.154154
View details for PubMedID 26085773
Prenatal Tricuspid Valve Size as a Predictor of Postnatal Outcome in Patients with Severe Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum
FETAL DIAGNOSIS AND THERAPY
2014; 35 (2): 101-107
The majority of hospital physiologic monitor alarms are not clinically actionable and contribute to alarm fatigue. In 2014, The Joint Commission declared alarm safety as a National Patient Safety Goal and urged prompt action by hospitals to mitigate the issue . It has been demonstrated that vital signs in hospitalized children are quite different from currently accepted reference ranges . Implementation of data-driven, age stratified vital sign parameters (Table 1) for alarms in this patient population could reduce alarm frequency.
View details for PubMedID 26262220
Course, Predictors of Diaphragm Recovery After Phrenic Nerve Injury During Pediatric Cardiac Surgery
ANNALS OF THORACIC SURGERY
2013; 96 (3): 938-942
Tricuspid valve (TV) size at birth correlates with intervention strategy in patients with severe pulmonary stenosis (SPS) or pulmonary atresia/intact ventricular septum (PA/IVS). Prenatal features that might predict postnatal TV size have not been well studied. We hypothesized that prenatal echocardiographic measurements predict the postnatal TV Z-score in fetuses with SPS and PA/IVS.We identified 16 neonates (gestational age 28 ± 4.8 weeks) with a fetal diagnosis of SPS or PA/IVS from 2001 to 2010. Measurements were performed offline. ROC (receiver operating characteristic) analysis was used to generate AUC (areas under the curve) for each of the variables.AUC was 0.94 for tricuspid to mitral valve (TV/MV) ratio, 0.88 for TV Z-score, and 0.85 for TV inflow duration. A cut-off value of >0.63 for TV/MV yielded a sensitivity of 78%, specificity of 100% for predicting postnatal TV Z-score >-3. Neonates with TV Z-score ≥-3 and all fetuses with antegrade flow across the pulmonary valve or more than moderate tricuspid regurgitation had biventricular circulation in follow-up.Fetal TV/MV >0.63 predicts favorable TV Z-score at birth in patients with SPS and PA/IVS. Antegrade pulmonary valve flow and more than moderate tricuspid regurgitation also conferred a favorable outcome.
View details for DOI 10.1159/000357429
View details for Web of Science ID 000332498600005
View details for PubMedID 24457468
Outcomes of a modified approach to transcatheter closure of perimembranous ventricular septal defects
CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS
2013; 82 (1): 143-149
Hemidiaphragm paralysis from phrenic nerve injury is a known complication of congenital cardiac surgery. Return of diaphragm function has been reported; however, prior studies on this subject have been limited by small numbers, static assessment methods, or observation of plicated or non-plicated patients alone. To describe return of function, we reviewed fluoroscopy and ultrasonography in all diagnosed cases of diaphragmatic paralysis.Surgical cases at our institution between 1991 and 2010 were identified for patients with postoperative hemidiaphragm paralysis diagnosed by chest X-ray, ultrasound, or fluoroscopy. Follow-up ultrasound and fluoroscopic studies were reviewed for return of diaphragm function.Seventy-two cases of postoperative hemidiaphragm paralysis were identified. Forty cases were plicated prior to discharge. Plicated patients were younger at time of diagnosis (median 46 days average 3.6 months; p=0.025) and had a larger proportion of single ventricle diagnoses (48% vs 16%) compared with non-plicated patients. Twenty-six patients with paralysis were excluded in follow-up due to lack of studies documenting diaphragm function after the diagnostic study. Of the remaining 46 cases, median follow-up was 353 days (range: 6 days to 17 years). Plicated and non-plicated patients regained function at similar frequency (60% and 54.8%, respectively). Plication status, Risk Adjustment for Congenital Heart Surgery (RACHS) 1 score, age at diagnosis, and side of paralysis did not predict failure of recovery.In the current era, return of diaphragm function after phrenic nerve injury sustained during congenital cardiac surgery is a known occurrence; however, predicting recovery continues to be difficult.
View details for DOI 10.1016/j.athoracsur.2013.05.057
View details for Web of Science ID 000323940200038
View details for PubMedID 23932321
Tissue Doppler is more sensitive and reproducible than spectral pulsed-wave Doppler for fetal right ventricle myocardial performance index determination in normal and diabetic pregnancies.
Journal of the American Society of Echocardiography
2013; 26 (5): 507-514
To describe the immediate and midterm results of a modified method for transcatheter closure of perimembranous ventricular septal defects (pmVSDs).Transcatheter closure of pmVSDs has been associated with development of heart block due to impingement on the ventricular conduction system. Ventricular septal aneurysms (VSAs) are common; the VSA tissue can serve as a target for the device without necessitating direct contact with the conduction system.Between 2004 and 2011, 15 patients underwent transcatheter closure of a pmVSD utilizing a device implanted into a VSA. Catheterization reports were examined in addition to pre-closure, post-closure, and current clinical, ECG, and echocardiographic data.The median age was 20 years (4-61 years), and the most common indication for closure was increasing LV dilation. Four different Amplatzer devices were utilized. Following device implantation there was a decrease in Qp:Qs (1.7-1.1) and in RV:LV pressure ratio (0.36-0.31). There were no deaths, no device embolizations, and no new heart block or PR interval prolongation. Three patients developed a new right bundle branch block (RBBB). The median follow-up time was 1.5 years (4 months to 7.1 years). Two patients required further procedures for important residual shunting. Six continued to have a "trivial/small" residual leak, but only one had any degree (mild) of residual LV dilation. None of the complications were significantly associated with age or weight at the time of procedure, original size of the VSD, or size or type of the device used.Transcatheter closure of pmVSD with placement of the device into the VSA is safe and effective, and may result in fewer instances of atrioventricular block and valve abnormalities than have been reported with alternative methods of pmVSD device closure. Persistent VSDs and new RBBBs remain an important issue.
View details for DOI 10.1002/ccd.24774
View details for Web of Science ID 000320787100030
View details for PubMedID 23225758
Prenatal diagnosis of atrial restriction in hypoplastic left heart syndrome is associated with decreased 2-year survival
2012; 32 (5): 485-490
The aim of this study was to compare the reproducibility, agreement, and sensitivity of pulsed-wave Doppler tissue imaging (DTI) versus spectral Doppler assessment of right ventricular (RV) myocardial performance index (MPI) in midgestation fetuses in both a normal and a disease state.RV MPI was calculated using pulsed-wave DTI and spectral Doppler in normal pregnancies (n = 69) and in women with pregestational diabetes (n = 51). Intraobserver and interobserver variability and agreement were evaluated using Bland-Altman analysis. Student's t tests were used for comparisons of differences.In normal fetuses, RV MPI derived by the two methods showed no statistical difference, were interchangeable (DTI, 0.51 ± 0.10; spectral Doppler, 0.50 ± 0.12; P = .686), and were in agreement by Bland-Altman analysis. However, in fetuses of mothers with diabetes, the two methods produced different RV MPI measurements (DTI, 0.56 ± 0.10; spectral Doppler, 0.51 ± 0.12; P < .001). Intraobserver and interobserver bias was lower for DTI.The DTI method of measuring fetal RV MPI is more sensitive, has less variability and more precision, and is better able to demonstrate subtle abnormalities in cardiac function than the spectral Doppler method in diabetic versus normal pregnancies.
View details for DOI 10.1016/j.echo.2013.02.006
View details for PubMedID 23498900
Natural history of exercise function in patients with Ebstein anomaly: A serial study
AMERICAN HEART JOURNAL
2012; 163 (3): 486-491
To compare the course of hypoplastic left heart syndrome (HLHS) patients diagnosed prenatally with any degree of atrial restriction with those without evidence of atrial restriction.Retrospective, cohort.Prenatally diagnosed HLHS patients from August 1999 to January 2009 were categorized as nonrestrictive versus restrictive, defined by left atrial hypertension on pulmonary venous Doppler and/or an intact interatrial septum.Of 73 total fetal patients identified, 49 were live-born. Survival at 2 years was 29/35 [83% confidence interval (CI): 59.5%-88.9%] for the nonrestrictive group and 6/14 (43% CI: 17.7%-6.0%) for the restrictive group (p<0.0001). Of those who underwent stage 1 palliation (35 with nonrestrictive and 10 with restrictive atrial septa), both groups had a similar incidence of preoperative acidosis and need for ventilation and inotropic support. Postoperatively, there was no difference between groups in ventilator days, length of stay, or survival to discharge. There was decreased survival at 2 years in the restrictive group, 60% (CI: 26.2%-87.8%) versus 83% (CI: 66.4%-93.4%) in the nonrestrictive group. Furthermore, a disproportionate number of interstage deaths was evident in the restrictive group.Prenatal presence of any degree of atrial septal restriction in the setting of HLHS confers a significant survival disadvantage, with increases in both early and late mortality.
View details for DOI 10.1002/pd.3850
View details for Web of Science ID 000303797700015
View details for PubMedID 22511219
Muscular Infundibular Atresia Is Associated with Coronary Ostial Atresia in Pulmonary Atresia with Intact Ventricular Septum
CONGENITAL HEART DISEASE
2011; 6 (5): 444-450
The clinical manifestations of Ebstein anomaly (EA) vary greatly; criteria for surgical intervention remain undefined. Decisions regarding surgical intervention in asymptomatic/mildly symptomatic patients would be helpfully informed by a detailed, quantitative understanding of the natural history of exercise intolerance in these patients. However, past studies of exercise function in EA have been of a cross-sectional, rather than a serial, nature. We, therefore, analyzed serial cardiopulmonary exercise (CPX) tests from patients with unrepaired EA to better appreciate the natural history of their exercise function.All patients with EA who had had at least 2 CPX tests, separated by at least 6 months, between November 2002 and October 2010 were identified. Patients with prior tricuspid valve surgery were excluded from the study.Cardiopulmonary exercise data from 23 patients (64 CPX tests; 2.8 ± 1.0 tests/patient) were analyzed. The median time interval between the first and last CPX tests was 3.3 (range, 0.6-7.3) years. The percentage of predicted peak oxygen consumption declined slowly (1.87 ± 8.04 percentage points/y) during the follow-up period. The decline was more pronounced (3.04 ± 6.78 percentage points/y) in patients <18 years old. On multivariate modeling, only the change in oxygen pulse at peak exercise (a surrogate for forward stroke volume) and the change in peak heart rate over time emerged as statistically significant correlates of the change in percentage of predicted peak oxygen consumption.The exercise function of patients with EA tends to deteriorate over time. This deterioration appears to be related to a progressive decline in their ability to augment their forward stroke volume and heart rate during exercise.
View details for DOI 10.1016/j.ahj.2011.12.006
View details for Web of Science ID 000301597200026
View details for PubMedID 22424021
Prenatal Diagnosis of Hypoplastic Left Heart Syndrome in Current Era
AMERICAN JOURNAL OF CARDIOLOGY
2011; 108 (3): 421-427
To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum.Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome.Tertiary-care pediatric hospital.Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z-scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs.In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.
View details for DOI 10.1111/j.1747-0803.2011.00541.x
View details for Web of Science ID 000294919100006
View details for PubMedID 21718454
Longitudinal Exercise Capacity of Patients With Repaired Tetralogy of Fallot
AMERICAN JOURNAL OF CARDIOLOGY
2011; 108 (1): 99-105
We sought to evaluate the relation of a prenatal diagnosis (preDx) with morbidity and mortality during the initial hospitalization in a contemporary cohort of patients with hypoplastic left heart syndrome (HLHS). A retrospective study of patients with HLHS presenting from 1999 to 2010 was performed. Patients with genetic disorders or a gestational age <34 weeks or who had intentionally received comfort care only were excluded. Of the 81 patients meeting the study criteria, 49 had a preDx and 32 were diagnosed postnatally (postDx). Birth weight (median 3.0 vs 3.4 kg; p = 0.007) and gestational age (median 38 vs 39 weeks; p <0.001) were lower in the preDx than in the postDx patients. Preoperatively, the postDx patients were intubated more frequently (97% vs 71%, p = 0.004) and ventilated longer (median 96 vs 24 hours, p = 0.005) than the preDx patients. They also had more preoperative acidosis, multiorgan failure, tricuspid valve regurgitation, and right ventricular dysfunction. Of the 73 patients undergoing surgery, no difference in survival was seen between the preDx and postDx groups (91% vs 89%). The median duration of postoperative ventilation was 7 days and the median length of stay was 36 days for the 66 survivors, with no difference between the 2 groups. Postoperative morbidities, including chylothorax and infection, were also similar in the preDx and postDx patients. No studied preoperative factor was associated with death, duration of postoperative ventilation, or length of stay. In conclusion, our recent experience has shown that preDx of HLHS was not associated with a survival advantage, fewer postoperative complications, or shorter length of stay. Improved preoperative status was observed in the preDx patients; however, they were born earlier with a lower birthweight. What effect these factors might have on longer term morbidity remains unknown.
View details for DOI 10.1016/j.amjcard.2011.03.065
View details for Web of Science ID 000293767400015
View details for PubMedID 21624547
Blood transfusion is associated with prolonged duration of mechanical ventilation in infants undergoing reparative cardiac surgery
PEDIATRIC CRITICAL CARE MEDICINE
2011; 12 (1): 52-56
Patients with repaired tetralogy of Fallot have a reduced percentage of predicted peak oxygen consumption (VO(2)) and percentage of oxygen pulse (O(2)P%) compared to healthy controls. Because data regarding the progression of exercise intolerance in these patients is limited, we sought to analyze the serial exercise data from patients with Tetralogy of Fallot to quantify the changes in their exercise capacity over time and to identify associations with clinical and cardiac magnetic resonance imaging variables. The data from cardiopulmonary exercise tests (CPXs) from 2002 to 2010 for patients with repaired tetralogy of Fallot with ≥2 CPXs separated by ≥12 months were analyzed. Tests occurring after interventional catheterization or surgery were excluded. A total of 70 patients had 179 CPXs. They had a median age at the initial study of 23.6 years and an interval between the first and last CPX of 2.8 years. At the initial CPX, the peak VO(2) was 27.6 ± 8.8 ml/kg/min (78 ± 19% of predicted), and the peak O(2)P% was 89 ± 22% of predicted. At the most recent study, the peak VO(2) averaged 25.0 ± 7.4 ml/kg/min (73 ± 16% of predicted), and the peak O(2)P% averaged 83 ± 20% (p <0.01) for each versus the initial CPX. The decrease in the peak VO(2) was strongly associated with a decrease in O(2)P% and an increase (worsening) in the slope of the minute ventilation-versus-carbon dioxide production relation. Changes in the peak VO(2) did not correlate with concomitant changes in any other CPX variable. The rate of decrease was not related to a history of shunt palliation, age at CPX, or any other baseline clinical parameter, including cardiac magnetic resonance measurements. In conclusion, the exercise capacity of patients with repaired tetralogy of Fallot tends to decrease over time. This deterioration is variable and unpredictable and is primarily related to a decrease in the forward stroke volume at peak exercise.
View details for DOI 10.1016/j.amjcard.2011.02.349
View details for Web of Science ID 000292785600017
View details for PubMedID 21529748
Echocardiographic Risk Stratification of Fetuses with Sacrococcygeal Teratoma and Twin-Reversed Arterial Perfusion
FETAL DIAGNOSIS AND THERAPY
2011; 30 (4): 280-288
Perioperative transfusion has adverse effects in adults undergoing cardiac surgery. We sought to investigate whether greater use of blood and blood products might be an independent predictor of prolonged postoperative recovery, indicated by duration of mechanical ventilation (DMV), after reparative infant heart surgery.Secondary analysis of prospectively collected data from two randomized trials of hematocrit strategy during cardiopulmonary bypass in infant heart surgery to explore the association of DMV with perioperative transfusion and other variables.Tertiary pediatric hospital.Two hundred seventy infants undergoing two ventricle corrective cardiac surgery without aortic arch reconstruction.In univariable analyses, longer DMV was associated with younger age and lower weight at surgery, diagnostic group, and higher intraoperative and postoperative blood product transfusion (each p < .001). In multivariable proportional hazard regression, longer total support time and greater intraoperative and early postoperative blood products per kg were the strongest predictors of longer DMV. Patients in the highest tertile of intraoperative blood products per kg had an instantaneous risk of being extubated approximately half that of patients in the lowest tertile (hazard ratio, 0.51; 95% confidence interval, 0.35, 0.73). Patients who received any blood products on postoperative day 1, compared with those who did not, had a hazard ratio for extubation of 0.65 (95% confidence interval, 0.50, 0.85).In this exploratory secondary analysis of infants undergoing two ventricular repair of congenital heart disease without aortic arch obstruction, greater intraoperative and early postoperative blood transfusion emerged as potential important risk factors for longer DMV. Future prospective clinical trials are needed to determine whether reduction in blood product administration hastens postoperative recovery after infant heart surgery.
View details for DOI 10.1097/PCC.0b013e3181e30d43
View details for Web of Science ID 000285964500019
View details for PubMedID 20453699
Exercise Function of Children with Congenital Aortic Stenosis Following Aortic Valvuloplasty during Early Infancy
CONGENITAL HEART DISEASE
2009; 4 (4): 258-264
To evaluate pre-intervention echocardiographic parameters of cardiac function in fetuses who survive without hydrops as compared to fetuses who develop hydrops or perinatal death in the setting of sacrococcygeal teratoma (SCT) and twin-reversed arterial perfusion sequence (TRAP).Clinical, echocardiographic and sonographic data of fetuses with SCT or TRAP during 1999-2009 were reviewed retrospectively. Measurements of cardiothoracic ratio (CTR), cardiac dimension Z-scores, combined ventricular output (CVO), valvular regurgitation, and cardiovascular profile scores (CVPS) were obtained.In total, 19 fetuses (11 SCT, 8 TRAP) met the inclusion criteria and 26 detailed fetal echocardiographic studies were reviewed. Outcome was poor in 7 pregnancies (group A) and good in 12 (group B). Group A had worse CVPS (8.5 vs. 10, p < 0.01) and higher CTR (0.37 vs. 0.30, p = 0.04). At least one of the following was present in each group A fetus: CTR >0.5, CVO >550 ml/min/kg, tricuspid or mitral regurgitation, or mitral valve Z-score >2. No group B fetus had any of these abnormalities. No fetus in either group had abnormal venous Doppler waveforms at presentation.Fetal echocardiography can identify abnormalities of cardiac size and systolic, but not diastolic, function in all fetuses who subsequently died or developed hydrops.
View details for DOI 10.1159/000330762
View details for Web of Science ID 000298661500005
View details for PubMedID 22086180
The Longitudinal Course of Cardiomyopathy in Friedreich's Ataxia During Childhood
2009; 30 (3): 306-310
The objectives of this study were to characterize the exercise function of patients treated with balloon aortic valvuloplasty at 6 years old with a history of neonatal AS to undergo exercise testing.We enrolled 30 patients (median age 13.1 years) who underwent balloon aortic valvuloplasty at a median age of 12 days. At time of exercise testing, the median maximum Doppler AS gradient was 34 mm Hg (0-70 mm Hg); 11 patients had moderate or severe aortic regurgitation. All patients were asymptomatic. Overall, peak oxygen consumption (VO(2)) was below normal (87 +/- 18% predicted; P < .001), and was severely depressed (
View details for DOI 10.1111/j.1747-0803.2009.00304.x
View details for Web of Science ID 000207893700007
View details for PubMedID 19664028
Children with cardiomyopathy: complications after noncardiac procedures with general anesthesia
2007; 17 (8): 775-781
Clinical heart disease was recognized in the first descriptions of Friedreich's ataxia (FA). Cardiac manifestations reported for this progressive neurologic disease include hypertrophic cardiomyopathy, dilated cardiomyopathy, and electrophysiologic disturbances. Longitudinal data for childhood cases are limited. This study aimed to define the longitudinal course of the cardiac abnormalities with FA diagnosed during childhood and to correlate the presence of cardiomyopathy with clinical and genetic factors.A retrospective chart review was conducted, with prospective, blinded interpretation of echocardiograms and electrocardiograms. All the patients with a diagnosis of FA referred to the cardiology department of a single institution from 1974 to 2004 were included in the study.This study investigated a total of 113 echocardiograms for 28 patients. Overall, the group had left ventricular hypertrophy and normal systolic function, with a median mass z-score of 2.48 (range, -3.8 to 35.6) and a median ejection fraction (EF) of 61% (range, 23-81%). Of the 28 patients, 23 (82%) had two or more echocardiograms. The median follow-up time to the most recent echocardiogram was 5.1 years (range, 0.4-16.5 years). Many in this longitudinal follow-up cohort (57%) showed hypertrophic cardiomyopathy on at least one echocardiogram, with the last follow-up assessment showing systolic dysfunction for 38% of these patients. There was a slow nonlinear decline in systolic function over time, with the mean EF decreasing more rapidly as age increased (p = 0.02) and maintenance of EF in the normal range until the age of 22 years. Of the 12 patients with systolic dysfunction and follow-up echocardiograms, 10 showed improvement to the normal EF range on at least one echocardiogram, and 5 remained normal through the last study. None of the trends in cardiac function and morphology correlated with frataxin GAA repeat length (the primary genetic defect in FA) or ambulatory status. One patient required an implantable defibrillator. There were no deaths or heart transplantations.Overall, patients with FA have preserved cardiac function with increased mass throughout childhood. Because many patients who experience depressed systolic function show improvement in subsequent studies, evaluation for potentially reversible causes of heart failure should be conducted. Relative clinical stability during childhood and maintenance of normal systolic function into the second decade may be helpful for parent and patient education.
View details for DOI 10.1007/s00246-008-9305-1
View details for Web of Science ID 000264884500015
View details for PubMedID 18716706
Children with cardiomyopathy (CM) often undergo procedures that require general anesthesia (GA) but little is known about anesthesia-related adverse events or postprocedural outcomes.After approval, all children with CM who underwent nonopen heart surgical procedures and/or diagnostic imaging under GA at a tertiary children's hospital during January 2002 to May 2005 were identified from a clinical database. Based on their preprocedure fractional shortening (FS) on echocardiogram, systemic ventricular dysfunction was categorized as mild (FS 23-28%), moderate (FS 16-22%), or severe (FS < 16%) and those with normal (FS > 28%) were excluded from review.Twenty-six patients underwent 34 procedures under GA, of whom 13 (38%) had mild or moderate ventricular dysfunction and 21 (62%) had severe dysfunction. Common procedures included pacer/defibrillator placement (43%) and imaging studies (18%). Eighteen complications were noted in 12 patients. Fifteen (83%) complications occurred in patients with severe ventricular dysfunction. One patient with severe ventricular dysfunction died (3% mortality). Hypotension requiring inotropic support was the most frequent complication (61%). Children with severe ventricular dysfunction often required hospital support pre- and postprocedure with 67% requiring intensive care. Hospital stay was longer for patients with severe ventricular dysfunction compared with children with mild or moderate ventricular dysfunction (P = 0.006).The 30-day mortality rate was low but complications were common, especially in patients with severe ventricular dysfunction. For these patients, we recommend early consideration of perioperative intensive care support to optimize cardiovascular therapy and monitoring.
View details for DOI 10.1111/j.1460-9592.2007.02245.x
View details for Web of Science ID 000247582600007
View details for PubMedID 17596222