Recovery From Acute Kidney Injury and CKD Following Heart Transplantation in Children, Adolescents, and Young Adults: A Retrospective Cohort Study. American journal of kidney diseases 2016; 68 (2): 212-218
Acute kidney injury (AKI) is common in children following surgery for congenital heart disease and has been associated with poor long-term kidney outcomes. Children undergoing heart transplantation may be at increased risk for the development of both AKI and chronic kidney disease (CKD). This study examines AKI rates in children, adolescents, and young adults after heart transplantation and analyzes the relationship between AKI and CKD in this population.Retrospective cohort study.88 young patients who underwent heart transplantation at Lucile Packard Children'sHospital, Stanford, CA, September 1, 2007, to November 30,2013.The primary independent variable was AKI within the first 7 postoperative days, ascertained according to the KDIGO (Kidney Disease: Improving Global Outcomes) creatinine criteria (increase in serum creatinine 1.5 times baseline within 7 days).Recovery from AKI at 3 months, ascertained as serum creatinine level< 1.5 times baseline; and development of CKD at 6 and 12 months, ascertained as estimated glomerular filtration rate< 60mL/min/1.73m(2) for more than 3 months.63 (72%) patients developed AKI; 57% had moderate (stage 2 or severe stage 3) disease. Recoveryoccurred in 39 of 63 (62%), 50% for stage 2 or 3 versus 78% for stage 1 (P=0.04). At 6 and 12 months, 3 of 82 (4%) and 4 of 76 (5%) developed CKD, respectively. At both time points, CKD was more common in those without recovery (3/22 [14%] vs 0/38 (0%); P=0.04, and 3/17 (18%) vs (0/34) 0%; P=0.03, respectively).Retrospective design, small sample size, and single-center nature of the study.AKI is common after heart transplantation in children, adolescents, and young adults. Nonrecovery from AKI is more common in patients with more severe AKI and is associated with the development of CKD during the first year.
View details for DOI 10.1053/j.ajkd.2016.01.024
View details for PubMedID 26970941
Electrocardiographic repolarization abnormalities and increased risk of life-threatening arrhythmias in children with dilated cardiomyopathy HEART RHYTHM 2016; 13 (6): 1289-1296
Life-threatening arrhythmia events (LTEs) occur in ~5% of children with dilated cardiomyopathy (DCM). While prolonged QRS duration has been shown to be associated with LTEs, electrocardiographic (ECG) repolarization findings have not been examined.We sought to determine the associations between ECG repolarization abnormalities and LTEs in children with DCM.A single-center retrospective review of children with DCM was performed. LTEs were defined as documented ventricular tachycardia or fibrillation requiring medical intervention. Three pediatric cardiologists, blinded to clinical events, evaluated ECGs obtained at the time of initial referral. Kaplan-Meier survival and Cox proportional hazards analyses were used to evaluate time to LTEs.A total of 137 patients (mean age 7.8 6.7 years; 75(55%) male patients) with DCM (mean ejection fraction 35% 16%) were included; 67 patients (49%) had a corrected JT (JTc) interval of 340 ms, 72 (53%) had a corrected QT (QTc) interval of 450 ms, and 41 (30%) had abnormal T waves. LTEs occurred in 15 patients at a median of 12 months (interquartile range 3-36 months) after the initial ECG. Patients with LTEs had a longer JTc interval (371 77 ms vs 342 41 ms; P = .02) and a longer QTc interval (488 96 ms vs 453 44 ms; P = .01). In survival analysis, a JTc interval of 390 ms (hazard ratio [HR] 4.07; 95% confidence interval [CI] 1.12-14.83; P = .03), a QTc interval of 510 ms (HR 6.95; 95% CI 1.53-31.49; P = .01), abnormal T-wave inversion (HR 11.62; 95% CI 2.75-49.00; P = .001), and ST-segment depression (HR 6.91; 95% CI 1.25-38.27; P = .03) were associated with an increased risk of LTEs, even after adjusting for QRS duration and amiodarone use.Repolarization abnormalities are common in children with DCM. Certain ECG repolarization abnormalities, such as significantly prolonged JTc and QTc intervals, may be useful in identifying patients at risk of LTEs.
View details for DOI 10.1016/j.hrthm.2016.02.014
View details for Web of Science ID 000376334800016
View details for PubMedID 26945851
Compassionate deactivation of ventricular assist devices in pediatric patients JOURNAL OF HEART AND LUNG TRANSPLANTATION 2016; 35 (5): 564-567
Despite greatly improved survival in pediatric patients with end-stage heart failure through the use of ventricular assist devices (VADs), heart failure ultimately remains a life-threatening disease with a significant symptom burden. With increased demand for donor organs, liberalizing the boundaries of case complexity, and the introduction of destination therapy in children, more children can be expected to die while on mechanical support. Despite this trend, guidelines on the ethical and pragmatic issues of compassionate deactivation of VAD support in children are strikingly absent. As VAD support for pediatric patients increases in frequency, the pediatric heart failure and palliative care communities must work toward establishing guidelines to clarify the complex issues surrounding compassionate deactivation. Patient, family and clinician attitudes must be ascertained and education regarding the psychological, legal and ethical issues should be provided. Furthermore, pediatric-specific planning documents for use before VAD implantation as well as deactivation checklists should be developed to assist with decision-making at critical points during the illness trajectory. Herein we review the relevant literature regarding compassionate deactivation with a specific focus on issues related to children.
View details for DOI 10.1016/j.healun.2016.03.020
View details for Web of Science ID 000376951900004
View details for PubMedID 27197773
Impact of ventricular assist device placement on longitudinal renal function in children with end-stage heart failure. journal of heart and lung transplantation 2016; 35 (4): 449-456
Although ventricular assist devices (VADs) restore hemodynamics in those with heart failure, reversibility of end-organ dysfunction with VAD support is not well characterized. Renal function often improves in adults after VAD placement, but this has not been comprehensively explored in children.Sixty-three children on VAD support were studied. Acute kidney injury (AKI) was defined by Kidney Disease: Improving Global Outcomes criteria. Estimated glomerular filtration rate (eGFR) was determined by the Schwartz method. Generalized linear mixed-effects models compared the pre-VAD and post-VAD eGFR for the cohort and sub-groups with and without pre-VAD renal dysfunction (pre-VAD eGFR < 90 ml/min/1.73 m(2)).The pre-VAD eGFR across the cohort was 84.0 ml/min/1.73 m(2) (interquartile range [IQR] 62.3-122.7), and 55.6% (34 of 63) had pre-VAD renal dysfunction. AKI affected 60.3% (38 of 63), with similar rates in those with and without pre-existing renal dysfunction. Within the cohort, the nadir eGFR occurred 1 day post-operatively (62.9 ml/min/1.73 m(2); IQR, 51.2-88.9 ml/min/1.73 m(2); p < 0.001). By Day 5, however, the eGFR exceeded the baseline (99.0 ml/min/1.73 m(2); IQR, 59.3-146.7 ml/min/1.73 m(2); p = 0.03) and remained significantly higher through the first post-operative week. After adjusting for age, gender, and AKI, the eGFR continued to increase throughout the entire 180-day study period ( = 0.0025; 95% confidence interval, 0.0015-0.0036; p < 0.001). Patients with pre-VAD renal dysfunction experienced the greatest improvement in the eGFR ( = 0.0051 vs = 0.0013, p < 0.001).Renal dysfunction is prevalent in children with heart failure undergoing VAD placement. Although peri-operative AKI is common, renal function improves substantially in the first post-operative week and for months thereafter. This is particularly pronounced in those with pre-VAD renal impairment, suggesting that VADs may facilitate recovery and maintenance of kidney function in children with advanced heart failure.
View details for DOI 10.1016/j.healun.2015.10.039
View details for PubMedID 26653933
Outpatient Outcomes of Pediatric Patients with Left Ventricular Assist Devices. ASAIO journal 2016; 62 (2): 163-168
Outpatient experience of children supported with continuous flow ventricular assist devices (CFVAD) is limited. We reviewed our experience with children discharged with CF-VAD support.All pediatric patients <18 years old with CF-VADs implanted at our institution were included. Discharge criteria included a stable medication regimen, completion of a VAD education program and standardized rehabilitation plan, and presence of a caregiver. Hospital re-admissions (excluding scheduled admissions) were reviewed. Adverse events were defined by INTERMACS criteria.Of 17 patients with CF-VADs, 8(47%) were discharged from the hospital (1 Heartware HVAD, 7 Heartmate II). Median age was 15.3(range 9.6-17.1) years and weight was 50.6(33.6-141) kg. Device strategies were destination therapy (n=4) and bridge to transplant (n=4). Patients spent a median 49(26-107) days hospitalized post-implant and had 2(1-5) hospital re-admissions. Total support duration was 3154 patient-days, with 2413 as outpatient. Most frequent adverse events were device malfunction and arrhythmias. There was one death due to pump thrombosis, and no bleeding or stroke events. Overall adverse event rate was 15.22 per 100-patient-months.Early experience suggests that children with CF-VADs can be safely discharged. Device malfunction and arrhythmia were the most common adverse events but were recognized quickly with structured outpatient surveillance.
View details for DOI 10.1097/MAT.0000000000000324
View details for PubMedID 26720740
A Pilot Study Assessing ECG versus ECHO Ventriculoventricular Optimization in Pediatric Resynchronization Patients. Journal of cardiovascular electrophysiology 2016; 27 (2): 210-216
Cardiac resynchronization therapy indications and management are well described in adults. Echocardiography (ECHO) has been used to optimize mechanical synchrony in these patients; however, there are issues with reproducibility and time intensity. Pediatric patients add challenges, with diverse substrates and limited capacity for cooperation. Electrocardiographic (ECG) methods to assess electrical synchrony are expeditious but have not been extensively studied in children. We sought to compare ECHO and ECG CRT optimization in children.Prospective, pediatric, single-center cross-over trial comparing ECHO and ECG optimization with CRT. Patients were assigned to undergo either ECHO or ECG optimization, followed for 6 months, and crossed-over to the other assignment for another 6 months. ECHO pulsed-wave tissue Doppler and 12-lead ECG were obtained for 5 VV delays. ECG optimization was defined as the shortest QRSD and ECHO optimization as the lowest dyssynchrony index. ECHOs/ECGs were interpreted by readers blinded to optimization technique. After each 6 month period, these data were collected: ejection fraction, velocimetry-derived cardiac index, quality of life, ECHO-derived stroke distance, M-mode dyssynchrony, study cost, and time. Outcomes for each optimization method were compared.From June 2012 to December 2013, 19 patients enrolled. Mean age was 9.1 4.3 years; 14 (74%) had structural heart disease. The mean time for optimization was shorter using ECG than ECHO (9 1 min vs. 68 13 min, P < 0.01). Mean cost for charges was $4,400 700 less for ECG. No other outcome differed between groups.ECHO optimization of synchrony was not superior to ECG optimization in this pilot study. ECG optimization required less time and cost than ECHO optimization.
View details for DOI 10.1111/jce.12863
View details for PubMedID 26515428
Obesity and Premature Loss of Mobility in Two Adolescents with Becker Muscular Dystrophy After HeartMate II Implantation. ASAIO journal 2016; 62 (1): e5-7
Weight gain is common following implantation of continuous flow ventricular assist devices (VADS). Obesity can have a significant negative impact on mobility. For adolescents with Becker Muscular Dystrophy (BMD) for whom the ability to ambulate often persists into the mid-third decade, preservation of functional ability is critical. We report two cases of Thoratec HeartMate II left ventricular assist device (LVAD) implantation in adolescents with Becker Muscular Dystrophy for whom post-operative weight gain contributed significantly to accelerated loss of ambulation and, in one case, drive line fracture in the context of repeated falls. As LVADS become an increasingly common therapy for end-stage heart failure in adolescents with BMD, care must focus not only on maintaining device functionality, but aggressive weight management and preservation of ambulation and skeletal muscle strength.
View details for DOI 10.1097/MAT.0000000000000292
View details for PubMedID 26461240
Ventricular assist devices in a contemporary pediatric cohort: Morbidity, functional recovery, and survival. journal of heart and lung transplantation 2016; 35 (1): 92-98
Limited availability of donor organs has led to the use of ventricular assist devices (VADs) to treat heart failure in pediatric patients, primarily as bridge to transplantation. How effective VAD therapy is in promoting functional recovery in children is currently not known.We report morbidity and mortality as defined by the Interagency Registry for Mechanically Assisted Circulatory Support Modified for Pediatrics (PediMACS) and the use of the Treatment Intensity Score to assess functional status for 50 VAD patients supported at a single pediatric program from 2004 to 2013.In this cohort, 30-day survival on VAD was 98%, and 180-day survival was 83%. Stroke occurred in 11 patients (22%), with 8 (16%) resulting in persistent neurologic deficit or death. The adverse event rate was 2-fold to 3-fold higher in the first 7 days of support compared with the subsequent support period. Functional status, as measured by the Treatment Intensity Score, improved with duration of support. Successful bridge to transplantation was associated with fewer adverse events during support and greater improvement in the Treatment Intensity Score during the period of support.Overall survival in this cohort is excellent. The risk of serious adverse events decreases over the first month of support. However, a clinically significant risk of morbidity and mortality persists for the duration of pediatric VAD support. Measures of functional status improve with duration of support and are associated with survival to transplantation.
View details for DOI 10.1016/j.healun.2015.06.006
View details for PubMedID 26210751
Obesity and Premature Loss of Mobility in Two Adolescents with Becker Muscular Dystrophy After HeartMate II Implantation ASAIO JOURNAL 2016; 62 (1): E5-E7
Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions. Congenital heart disease 2015; 10 (6): E278-87
Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value.We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class.We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012.A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value.Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike.
View details for DOI 10.1111/chd.12290
View details for PubMedID 26219731
Group visits in the pediatric heart transplant outpatient clinic PEDIATRIC TRANSPLANTATION 2015; 19 (7): 730-736
The "GVM" has emerged as an alternative to traditional individualized appointments in the ambulatory care setting. We hypothesized that group visits could successfully be utilized in a PHtx clinic. Seven patients, ages 1-18yr old, and their families participated in a total of 11 group visits in lieu of individualized appointments. Patients were divided into two groups based on whether they were greater or less than one yr post-transplant. Patient/provider satisfaction, medication adherence, and content retention were ascertained via questionnaires and free-response tests. Total clinic throughput time, including per-patient clinic utilization time, was compared to historical data. Six of seven patients completed the study with one dropout. Overall satisfaction ratings were 3.98 of 4 with all patients reporting that they would "strongly recommend" group visits to others. Health information retention tests demonstrated improvement between pre- and post-tests in eight of nine (89%) of the group visits. Overall clinic utilization decreased by nearly 50% while providing 70min of face-to-face time with the provider. Medication adherence neared 100% for all patients. The GVM can be successfully applied to the PHtx population with high patient and provider satisfaction, more face-to-face time, excellent content retention, and greatly improved clinic efficiency.
View details for DOI 10.1111/petr.12574
View details for Web of Science ID 000362580100018
View details for PubMedID 26250489
Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions CONGENITAL HEART DISEASE 2015; 10 (6): E278-E287
Changes in Risk Profile Over Time in the Population of a Pediatric Heart Transplant Program. Annals of thoracic surgery 2015; 100 (3): 989-994
Single-center data on pediatric heart transplantation spanning long time frames is sparse. We attempted to analyze how risk profile and pediatric heart transplant survival outcomes at a large center changed over time.We divided 320 pediatric heart transplants done at Stanford University between 1974 and 2014 into three groups by era: the first 20 years (95 transplants), the subsequent 10 years (87 transplants), and the most recent 10years (138 transplants). Differences in age at transplant, indication, mechanical support, and survival were analyzed.Follow-up was 100% complete. Average age at time of transplantation was 10.4 years, 11.9 years, and 5.6 years in eras 1, 2, and 3, respectively. The percentage of infants who received transplants by era was 21%, 7%, and 18%, respectively. The indication of end-stage congenital heart disease vs cardiomyopathy was 24%, 22%, and 49%, respectively. Only 1 patient (1%) was on mechanical support at transplant in era 1 compared with 15% in era 2 and 30% in era 3. Overall survival was 72% at 5 years and 57% at 10 years. Long-term survival increased significantly with each subsequent era. Patients with cardiomyopathy generally had a survival advantage over those with congenital heart disease.The risk profile of pediatric transplant patients in our institution has increased over time. In thelast 10 years, median age has decreased and ventricular assist device support has increased dramatically. Transplantation for end-stage congenital heart disease is increasingly common. Despite this, long-term survival has significantly and consistently improved.
View details for DOI 10.1016/j.athoracsur.2015.05.111
View details for PubMedID 26228604
Changes in Risk Profile Over Time in the Population of a Pediatric Heart Transplant Program ANNALS OF THORACIC SURGERY 2015; 100 (3): 989-995
Task Force 7: Pediatric Cardiology Fellowship Training in Pulmonary Hypertension, Advanced Heart Failure, and Transplantation JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY 2015; 66 (6): 732-739
Task Force 7: Pediatric Cardiology Fellowship Training in Pulmonary Hypertension, Advanced Heart Failure, and Transplantation. SPCTPD/ACC/AAP/AHA. Circulation 2015; 132 (6): e99-e106
Quality of life in pediatric patients affected by electrophysiologic disease HEART RHYTHM 2015; 12 (5): 899-908
Treatment of electrophysiologic (EP) disease in pediatric patients has improved; however, the effects on quality of life (QOL) are unknown.The purpose of this study was to compare QOL within EP disease groups and to other congenital heart diseases, to evaluate the effects of cardiac rhythm devices on QOL, and to identify drivers of QOL in EP disease.Cross-sectional study of patient/parent proxy-reported Pediatric Cardiac Quality of Life Inventory scores (Total, Disease Impact, Psychosocial Impact) in subjects aged 8 to 18 years from 11 centers with congenital complete heart block (CCHB), ventricular tachycardia (VT), supraventricular tachycardia (SVT), and long QT syndrome (LQTS). QOL was compared between EP disease groups and congenital heart disease groups [bicuspid aortic valve (BAV), tetralogy of Fallot (TOF), and Fontan]. General linear modeling was used to perform group comparisons and to identify predictors of QOL variation.Among 288 patient-parent pairs, mean age was 12.8 3.0 years. CCHB ( = 83) showed higher patient Total QOL than other EP disease cohorts (P .02; LQTS = 73; SVT = 74). SVT ( = 75) and LQTS ( = 75) had lower patient Total scores than BAV ( = 81; P .008). Patient/parent-proxy QOL scores for all EP disease groups were not different than TOF and higher than Fontan. The presence of a cardiac rhythm device was associated with lower QOL scores in LQTS ( = 66 vs = 76; P < .01). Predictors of lower patient/parent-proxy QOL included EP disease type (P .03), increased medical care utilization (P .04), and no parental college degree (P .001).Given the significant variation in QOL in EP disease type, stratification by EP disease type and increased medical care utilization may allow for targeted interventions to improve QOL.
View details for DOI 10.1016/j.hrthm.2015.01.022
View details for Web of Science ID 000353333100011
View details for PubMedID 25602174
A novel pediatric treatment intensity score: development and feasibility in heart failure patients with ventricular assist devices JOURNAL OF HEART AND LUNG TRANSPLANTATION 2015; 34 (4): 509-515
The evolution of pharmacologic therapies and mechanical support including ventricular assist devices (VADs) has broadened the scope of care available to children with advanced heart failure. At the present time, there are only limited means of quantifying disease severity or the concomitant morbidity for this population. This study describes the development of a novel pediatric treatment intensity score (TIS), designed to quantify the burden of illness and clinical trajectory in children on VAD support.There were 5 clinical domains assessed: nutrition, respiratory support, activity level, cardiovascular medications, and care environment. A scale was developed through expert consensus. Higher scores indicate greater morbidity as reflected by intensity of medical management. To evaluate feasibility and face validity, the TIS was applied retrospectively to a subset of pediatric inpatients with VADs. The Bland-Altman method was used to assess limits of agreement.The study comprised 39 patients with 42 implantations. Bland-Altman interobserver and intraobserver comparisons showed good agreement (mean differences in scores of 0.02, limits of agreement 0.12). Trends in TIS were concordant with the overall clinical impression of improvement. Scores remained 0.6 preceding VAD implantation and peaked at 0.71 3 days after VAD implantation.We describe a pediatric VAD scoring tool, to assess global patient morbidity and clinical recovery. We demonstrate feasibility of using this TIS in a test population of inpatients on VAD support.
View details for DOI 10.1016/j.healun.2014.10.007
View details for Web of Science ID 000353251200006
Biventricular Berlin Heart EXCOR Pediatric Use Across the United States ANNALS OF THORACIC SURGERY 2015; 99 (4): 1328-1334
Biventricular assist device (BiVAD) support was a strong predictor of early mortality in the Berlin Heart EXCOR Pediatric investigational device exemption (IDE) study (Assess Safety and Probable Benefit of the EXCOR Pediatric Ventricular Assist Device [VAD]). In adults, it has been identified that 5% to 10% of the VAD population is benefited by BiVAD support over left ventricular assist device (LVAD) support. An analysis of the Berlin Heart study cohort was performed to characterize patients supported with BiVAD, examine risk factors of mortality in this group, and identify subsets of patients in whom BiVAD is associated with survival.All EXCOR Pediatric devices (Berlin Heart, Inc, The Woodlands, TX) placed in North America between May 2007 and December 2010 comprised the study cohort of 204 patients (128 [63%] LVADs and 76[37%] BiVADs). The following patient cohorts were analyzed to determine the effect of BiVAD use on survival: Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile 1 patients, patients with abnormal bilirubin levels, patients who received previous extracorporeal membrane oxygenation (ECMO), and patients with a 10-mL pump size.There were more patients with BiVADs in INTERMACS profile 1 (63% compared with 46%; p=0.018). The incidence of major bleeding, neurologic and renal dysfunction, and infection was similar between BiVAD and LVAD groups. White race, abnormal glomerular filtration rate (GFR), sites with experience of less than 5 implantations, and use of 10-mL pumps were predictors of mortality in patients who received BiVADs. BiVADs were not associated with improved survival in any patient cohort; however, they were associated with increased mortality in patients who had undergone ECMO before receiving a VAD.BiVAD support was not associated with improved survival in any identified subset of patients. Although not randomized, these results (which were corrected for multiple possible risk factors) suggest that some children supported with BiVADs might have done better with LVADs alone. Further prospective studies will be needed to identify patient cohorts that will be better served with BIVAD support.
View details for DOI 10.1016/j.athoracsur.2014.09.078
View details for Web of Science ID 000352162100042
Quality of life and metrics of achievement in long-term adult survivors of pediatric heart transplant PEDIATRIC TRANSPLANTATION 2015; 19 (1): 76-81
Many children who undergo heart transplantation will survive into adulthood. We sought to examine the QOL and capacity for achievement in long-term adult survivors of pediatric heart transplantation. Adults >18yr of age who received transplants as children (18yr old) and had survived for at least 10yr post-transplant completed two self-report questionnaires: (i) Ferrans & Powers QLI, in which life satisfaction is reported as an overall score and in four subscale domains and is then indexed from 0 (very dissatisfied) to 1 (very satisfied); and (ii) a "Metrics of Life Achievement" questionnaire regarding income, education, relationships, housing status, and access to health care. A total of 20 subjects completed the survey. The overall mean QLI score was 0.770.16. Subjects were most satisfied in the family domain (0.840.21) and least satisfied in the psychological/spiritual domain (0.70.28). Satisfaction in the domains of health/functioning and socioeconomic were intermediate at 0.78 and 0.76, respectively. Most respondents had graduated from high school, reported a median annual income >$50000/yr, and lived independently. Adult survivors of pediatric heart transplant report a good QOL and demonstrate the ability to obtain an education, work, and live independently.
View details for DOI 10.1111/petr.12384
View details for Web of Science ID 000346915200021
IVIG and graft coronary artery disease: A potentially deadly combination in pediatric heart transplant recipients PEDIATRIC TRANSPLANTATION 2015; 19 (1): 130-131
Reliability of echocardiographic measurements of left ventricular systolic function in potential pediatric heart transplant donors JOURNAL OF HEART AND LUNG TRANSPLANTATION 2015; 34 (1): 100-106
Echocardiogram reports, but not images, are usually available for the evaluation of potential donor hearts. To assess the reliability of local reports of potential pediatric heart donors, we compared echocardiographic measurements of left ventricular (LV) systolic function between local hospitals and a central echocardiography laboratory.We identified all potential donors aged <18 years managed by the California Transplant Donor Network from 2009 to 2013. Echocardiograms and reports were obtained from local hospitals. All studies were reviewed in a central laboratory by an experienced pediatric cardiologist blinded to local reports. Local and central measurements of fractional shortening (FS) were compared using the Bland-Altman method (mean difference 2 standard deviations). LV function was categorized based on FS as normal or mild, moderately, or severely depressed.There were 70 studies from 59 donors with local and central measurements of FS. The mean difference between local and central FS was 3.9 9.0. The limits of agreement ranged from -14.2 to 22. Twenty-five studies had discordant measurements of LV function, with 17 discordant by 1 category and 8 by 2 or more categories. Of 55 studies categorized as normal by local measurement, 6 were moderately to severely depressed by central review. Of 15 studies categorized as depressed by local measurement, 3 were normal by central review.Local and central measurements of LV systolic function were discordant in 36% of studies. Given such discordance, efforts to obtain and view actual echocardiographic images should be part of the standard evaluation of potential pediatric heart donors.
View details for DOI 10.1016/j.healun.2014.08.019
View details for Web of Science ID 000348273400012
Neurological Complications and Outcomes in the Berlin Heart EXCOR (R) Pediatric Investigational Device Exemption Trial JOURNAL OF THE AMERICAN HEART ASSOCIATION 2015; 4 (1)
Neurological complications and outcomes in the Berlin Heart EXCOR pediatric investigational device exemption trial. Journal of the American Heart Association 2015; 4 (1)
The Berlin Heart EXCOR() ventricular assist device has been approved for use in the United States as a bridge to heart transplantation in children. We sought to characterize neurological events in children supported with the Berlin Heart EXCOR() device.The multicenter prospective cohort consisted of all 204 children implanted with the Berlin Heart EXCOR() device at 47 centers in North America between May 2007 and December 2010. There were 73 neurological events in 59 patients, with 29% of the cohort experiencing 1 neurological event. Events included 52 strokes in 43 patients (21% of the cohort). The neurological event rate was 0.51 events per 100 patient-days. Many of the neurological events occurred early in the course of support, with 30 events recorded during the first 14 days of support. The mortality rate in participants with at least 1 neurological event was 42% (25 of 59), significantly higher than the 18% mortality rate (26 of 145) for those who did not have a neurological event (P=0.0006). Risk-factor analysis did not identify significant preimplantation predictors of neurological injury.Of children treated with the Berlin Heart EXCOR() device as a bridge to transplant, 29% experienced at least 1 neurological event. The majority of neurological events were ischemic strokes, and many of those occurred early in the course of support. Neurological injury was the leading cause of death after implantation of the Berlin Heart EXCOR() device. Risk stratification for stroke or neurological injury is not possible based on baseline preimplantation characteristics.www.clinicaltrials.gov. Unique Identifier: NCT00583661.
View details for DOI 10.1161/JAHA.114.001429
View details for PubMedID 25613996
HLA desensitization with bortezomib in a highly sensitized pediatric patient PEDIATRIC TRANSPLANTATION 2014; 18 (8): E280-E282
The International Society for Heart and Lung Transplantation Guidelines for the management of pediatric heart failure: Executive summary. [Corrected]. journal of heart and lung transplantation 2014; 33 (9): 888-909
An inpatient rehabilitation program utilizing standardized care pathways after paracorporeal ventricular assist device placement in children JOURNAL OF HEART AND LUNG TRANSPLANTATION 2014; 33 (6): 587-592
Structured rehabilitation programs in adults after ventricular assist device (VAD) placement result in improvements in physical function and exercise capacity, and have been shown to improve survival and accelerate post-transplant recovery. The objective of this study was to determine the safety and feasibility of an acute inpatient rehabilitation program for children utilizing standardized, age-appropriate, family-centered care pathways after paracorporeal VAD placement in both the ICU and acute-care inpatient settings.Between November 12, 2010 and March 15, 2013, 17 patients were referred to therapy after VAD implantation, 14 of whom were medically stable enough to participate. Beginning in the ICU, a structured physical and occupational therapy program was implemented utilizing novel age-appropriate, standardized care pathways for infants (age <1 year) and children (age 1 to 12 years). The infant and child pathways consisted of 8 and 10 goals, respectively. Retrospective review was conducted to ascertain the number of phases achieved per patient. Adverse events, defined as bleeding, physiologic instability, stroke, or device disruption during therapy, were also analyzed.The median age was 1.1 (range 0.5 to 14.4) years in the 14 patients considered medically stable enough to participate in rehabilitation. Nine of them were female. Eight patients participated in the infant standardized care pathway (SCP) and 6 participated in the child SCP. Seven patients were on biventricular support. Twelve patients were transplanted and survived. Two patients died while awaiting transplantation. There were 1,473 total days on the VAD (range 40 to 229 days). The median time to extubation was 2 days (range 1 to 8) and the median ICU stay was 6.5 days (range 3 to 152). Eleven patients achieved all goals of the SCP, including all of the patients in the child group. For the infant group, 5 patients achieved all goals of the SCP (range 5 to 8), and all but 1 patient achieved at least 7 goals of the SCP. There were no adverse events related to therapy.Standardized, family-centered inpatient rehabilitation care paths are safe for infants and children after paracorporeal device placement. Structured rehabilitation goals can be achieved by the majority of pediatric patients during VAD support. Early mobilization and inpatient rehabilitation in this cohort promotes normalization of function while awaiting cardiac transplantation.
View details for DOI 10.1016/j.healun.2013.12.009
View details for Web of Science ID 000336637100005
In-Hospital Arrhythmia Development and Outcomes in Pediatric Patients With Acute Myocarditis AMERICAN JOURNAL OF CARDIOLOGY 2014; 113 (3): 535-540
Cardiac arrhythmias are a complication of myocarditis. There are no large studies of in-hospital arrhythmia development and outcomes in pediatric patients with acute myocarditis. This was a retrospective 2-center review of patients 21years hospitalized with acute myocarditis from 1996 to 2012. Fulminant myocarditis was defined as the need for inotropic support within 24hours of presentation. Acute arrhythmias occurred at presentation and subacute after admission. Eighty-five patients (59% men) presented at a median age of 10years (1day to 18years). Arrhythmias occurred in 38 patients (45%): 16 acute, 12 subacute, and 9 acute and subacute (1 onset unknown). Arrhythmias were associated with low voltages on the electrocardiogram (14 of 34, 41% vs 6 of 47, 13%; odds ratio [OR] 4.78, 95% confidence interval [CI] 1.60 to 14.31) and worse outcome (mechanical support, orthotopic heart transplant, or death; OR 7.59, 95% CI 2.61 to 22.07) but were not statistically significantly associated with a fulminant course, ST changes, initial myocardial function, lactate, creatinine level, C-reactive protein and/or erythrocyte sedimentation rate, or troponin I level, after adjusting for multiple comparisons. Subacute arrhythmias were associated with preceding ST changes (10 of 15, 67% vs 15 of 59, 25%, OR 5.87, 95% CI 1.73 to 19.93). All patients surviving to discharge had arrhythmia resolution or control before discharge (10 on antiarrhythmic), with 1 exception (patient with complete heart block requiring a pacemaker). At 1-year follow-up, there were 3 recurrences of ventricular arrhythmias, but no arrhythmia-related mortality. In conclusion, arrhythmias are common in pediatric patients with myocarditis, occurring in nearly 1/2 of all hospitalized children and are associated with a worse outcome. Early identification of subacute arrhythmias using electrocardiographic changes may help management. A majority of patients do not require continued postdischarge arrhythmia treatment.
View details for DOI 10.1016/j.amjcard.2013.10.021
View details for Web of Science ID 000331161700022
Successful Bridge to Transplant with a Continuous Flow Ventricular Assist Device in a Single Ventricle Patient with an Aortopulmonary Shunt ASAIO JOURNAL 2014; 60 (1): 119-121
Ventricular assist devices are frequently used to bridge pediatric patients to cardiac transplantation; however, experience in single ventricle patients with aortopulmonary shunts remains limited. This case report addresses the challenge of balancing pulmonary and systemic circulation with a focus on the role of continuous versus pulsatile ventricular assist device support.
View details for DOI 10.1097/MAT.0000000000000007
View details for Web of Science ID 000329368600021
Hot Topics in Tetralogy of Fallot JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY 2013; 62 (23): 2155-2166
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "hot topics" to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.
View details for DOI 10.1016/j.jacc.2013.07.100
View details for Web of Science ID 000328073000002
Orthotopic heart transplantation in two infants with histiocytoid cardiomyopathy and left ventricular non-compaction PEDIATRIC TRANSPLANTATION 2013; 17 (7): E165-E167
HC is a rare cause of congestive heart failure that typically presents with malignant ventricular arrhythmias in infants, often requiring urgent intervention. Successful heart transplantation in a patient with HC has only been reported once (J Heart Lung Transplant 2004: 23: 902). The combination of HC with concurrent LVNC has only been described three times (Int J Legal Med 2009: 123: 47; Hum Pathol 2005: 36: 403; Pediatr Dev Pathol 2012: 15: 397). We report two rare cases of HC with LVNC in two infants presenting with cardiogenic shock, one requiring ECMO support who was successfully bridged to orthotopic heart transplantation with a Berlin Heart LVAD.
View details for DOI 10.1111/petr.12141
View details for Web of Science ID 000325369400004
View details for PubMedID 24099092
Long-term Cardiovascular Toxicity in Children, Adolescents, and Young Adults Who Receive Cancer Therapy: Pathophysiology, Course, Monitoring, Management, Prevention, and Research Directions A Scientific Statement From the American Heart Association CIRCULATION 2013; 128 (17): 1927-1995
QRS prolongation is strongly associated with life-threatening ventricular arrhythmias in children with dilated cardiomyopathy JOURNAL OF HEART AND LUNG TRANSPLANTATION 2013; 32 (10): 1013-1019
The incidence of sudden death in children with dilated cardiomyopathy has been estimated at < 1% annually. This number, however, may underestimate the incidence of life-threatening arrhythmias. The objective of this study was to assess the incidence of and identify risk factors for life-threatening arrhythmias in children with dilated cardiomyopathy.We conducted a retrospective record review of 183 children with dilated cardiomyopathy treated at a single center between 2000 and 2011. Life-threatening arrhythmia was defined as any ventricular arrhythmia that resulted in syncope or hypotension and required medical intervention. Risk factors for life-threatening arrhythmias were identified with univariate analyses. A prediction model was constructed with multivariate logistic regression and receiver operating characteristic curves.Nineteen patients experienced life-threatening arrhythmias, representing an annualized rate of 4.9%. Outpatient life-threatening arrhythmias occurred at a rate of 2.2% per year. Predictors of outpatient life-threatening arrhythmias were longer QRS duration (p = 0.003) and increased left ventricular posterior wall (LVPWd) thickness (p = 0.03). Only QRS duration remained significant in multivariate logistic regression (odds ratio, 1.8 for every unit increase in z-score; 95% CI, 1.01-1.9; p = 0.04). For all life-threatening arrhythmias, prolonged QRS duration was the only significant risk factor in multivariate logistic regression (odds ratio, 1.5; 95% CI, 1.1-2.2; p = 0.02).In children with dilated cardiomyopathy, as QRS duration increases, so too does the risk of life-threatening arrhythmias. Life-threatening arrhythmias occurred at an annual rate of 5%, which was much higher than the previously reported rate of sudden cardiac death in this population.
View details for DOI 10.1016/j.healun.2013.06.007
View details for Web of Science ID 000325044600010
Lower socioeconomic status is associated with worse outcomes after both listing and transplanting children with heart failure PEDIATRIC TRANSPLANTATION 2013; 17 (6): 573-581
The relationship between SES and outcomes surrounding pediatric cardiac transplantation is complex and influenced by recipient race. Broad-based studies of SES have not been performed. A retrospective review of all 5125 primary pediatric heart transplants performed in the United States between 2000 and 2011. Patients were stratified by SES based on zip code of residence and U.S. census data (low SES: 1637; mid-SES: 2253; high SES: 1235). Survival following listing and transplantation was compared across strata. Risk-adjusted long-term mortality on the waitlist was higher among low SES patients (hazard 1.32, CI 1.07-1.63). The relationship between SES and outcomes varied by race. Early risk-adjusted post-transplant outcomes were worst among high SES patients (10.8% vs. low SES: 8.9%, p<0.05). The incidence of non-compliance was higher among low SES patients (p<0.0001). Long-term risk-adjusted patient survival was poorer among low (hazard 1.41, CI 1.10-1.80) and mid-SES (1.29, 1.04-1.59) groups. Low SES is associated with worse outcomes on both the waitlist and late following transplantation. Higher SES patients had more complex transplants with higher early mortality. Further research should be directed at identifying and addressing underlying causal factors for these disparities.
View details for DOI 10.1111/petr.12117
View details for Web of Science ID 000322317700015
View details for PubMedID 23834560
Children with pulmonary arterial hypertension and prostanoid therapy: Long-term hemodynamics JOURNAL OF HEART AND LUNG TRANSPLANTATION 2013; 32 (5): 546-552
Pediatric patients with severe pulmonary arterial hypertension (PAH) are treated with intravenous epoprostenol or intravenous or subcutaneous treprostinil. Little is known about longitudinal hemodynamics and outcomes of epoprostenol, treprostinil, and transitions from epoprostenol to treprostinil.This was retrospective study of 77 pediatric patients (47 idiopathic PAH, 24 congenital heart disease-PAH) receiving epoprostenol or treprostinil from 1992 to 2010 at 2 centers. Outcomes were defined as living vs dead/transplant.Mean age at baseline was 7.7 5.2 years, with follow-up of 4.3 3.4 years. Thirty-seven patients were treated with epoprostenol, 20 with treprostinil, and 20 were transitioned from epoprostenol to treprostinil. Mean pulmonary-to-systemic vascular resistance ratio (Rp/Rs) for epoprostenol was 1.0 0.4, 0.8 0.4, 0.8 0.4, 1.0 0.4, and 1.2 0.4, respectively, at baseline, 1, 2, 3, and 4 years. For treprostinil, Rp/Rs was 0.9 0.3, 0.7 0.3, 0.5 0.2, (p < 0.01 vs baseline), and 1.1 0.2, respectively, at baseline, 1, 2, and 3 to 4 years, respectively. There were similar changes in mean pulmonary artery pressure and pulmonary vascular resistance index. The Rp/Rs 1 year after epoprostenol to treprostinil transition increased from 0.6 to 0.8 (n = 7). Changes not statistically significant unless noted. Eight patients died or received a transplant within 2 years of baseline; compared with the rest of the cohort, mean baseline Rp/Rs, right atrial pressure, and pulmonary vascular resistance index were significantly worse in this group. Thirty-nine patients remain on prostanoids, 17 are off, 16 died, and 5 received heart-lung transplant. Kaplan-Meier 5-year transplant-free survival was 70% (95% confidence interval, 56%-80%).There was improvement in Rp/Rs on both therapies at 1 to 2 years that was not sustained. The 5-year transplant-free survival was better than in similar adult studies.
View details for DOI 10.1016/j.healun.2013.01.1055
View details for Web of Science ID 000317708500012
A Novel Scoring System for Recovery after VAD Implantation in Children: Initial Feasibility ELSEVIER SCIENCE INC. 2013: S289-S289
Pediatric Outpatient VAD Experience at Stanford ELSEVIER SCIENCE INC. 2013: S288-S288
Abdominal complaints as a common first presentation of heart failure in adolescents with dilated cardiomyopathy AMERICAN JOURNAL OF EMERGENCY MEDICINE 2013; 31 (4): 684-686
We hypothesized that isolated gastrointestinal complaints (abdominal pain, nausea, anorexia, weight loss), in the absence of other symptoms, were a common mode of initial presentation in children with congestive heart failure (CHF).Ninety-eight patients younger than 18 years hospitalized with dilated cardiomyopathy at a single institution between January 1, 2000, and December 31, 2009, were included. Retrospective review of their presenting complaints was recorded and analyzed according to 3 age groups: 0 to 1 year (infants), 1 to 10 years (children), and 11 to 18 years (adolescents) of age.Respiratory symptoms were common in all age groups (range, 56%-63%). Gastrointestinal complaints were also common in all age groups (42%, 28%, and 65%, respectively) and were more frequent than respiratory complaints in adolescents. Adolescents were likely to present with abdominal pain as their only complaint (10/43, 23%). Chest pain, syncope, or cardiac arrest occurred rarely.Abdominal complaints are a common component of the presenting symptom complex of CHF in pediatric dilated cardiomyopathy in all age groups. In adolescents, abdominal complaints occur more frequently than respiratory complaints and often in the absence of any other symptoms. Unlike CHF in adults, chest pain, arrhythmia, or cardiac arrest occurs rarely at presentation in pediatric patients. Recognition of the different presenting symptoms of heart failure in children by primary providers is crucial to ensuring prompt diagnosis and timely initiation of therapy.
View details for DOI 10.1016/j.ajem.2012.12.009
View details for Web of Science ID 000316723400010
View details for PubMedID 23380118
Magnetic resonance imaging of the right ventricle in pediatric pulmonary arterial hypertension. Pulmonary circulation 2013; 3 (2): 350-355
Pulmonary arterial hypertension (PAH) causes changes in the right ventricle (RV), affecting RV size and function, ultimately leading to death. These changes have been evaluated by cardiac MRI (CMR) in adults with PAH, but not in children. Using CMR in pediatric patients with PAH, we examined how RV size and function (1) compare to normal data, (2) change over time, and (3) compare to similar studies in the adult population. Data from two institutions were retrospectively reviewed. Subjects with PAH and a CMR were included. Baseline CMR variables (right and left ventricular end-diastolic and end-systolic volumes indexed for body surface area, and calculated stroke volume and ejection fraction) were compared to normative data and follow-up CMR data. Twenty-six subjects (15 female), age 2-16 (mean 11) years, with idiopathic PAH were included. All patients were on PAH medication, and 65% on prostacyclin therapy. The baseline 6-Minute Walk Distance (6MWD; 481 137) was normal. RV volumes and ejection fraction were markedly abnormal compared to normal data (P < 0.001). Follow-up CMRs were analyzed in 15 patients. RV volumes and function and LV stroke volume showed no significant change over one year. Our pediatric patients with PAH have markedly abnormal right ventricles by CMR but have normal walk distances. The lack of change in CMR parameters over one year may represent a stable cohort and is different than similar studies in adults.
View details for DOI 10.4103/2045-8932.114763
View details for PubMedID 24015335
Low Dose Factor Eight Inhibitor Bypassing Activity (FEIBA) for Incessant Bleeding in Pediatric Patients on Mechanical Circulatory Support (MCS) ELSEVIER SCIENCE INC. 2013: S290-S290
Intermediate-term outcomes after combined heart-liver transplantation in children with a univentricular heart JOURNAL OF HEART AND LUNG TRANSPLANTATION 2013; 32 (3): 368-370
For patients with end-stage hepatic failure secondary to failing hemodynamics, combined heart-liver transplant (H-LT) remains the only option for long-term survival. We report a series of three pediatric patients who successfully underwent orthotopic H-LT for failed single-ventricle palliation. All three patients are currently living, now two, three, and five years post-transplant, and remain completely free of cardiac cellular allograft rejection despite reduced immunosuppression protocols. One patient, however, did develop acute antibody-mediated rejection in the immediate post-transplant period, suggesting that this protective effect may be less effective in attenuating humoral mechanisms of rejection.
View details for DOI 10.1016/j.healun.2012.11.023
View details for Web of Science ID 000315664600014
Cognitive outcomes in pediatric heart transplant recipients bridged to transplantation with ventricular assist devices JOURNAL OF HEART AND LUNG TRANSPLANTATION 2013; 32 (2): 212-220
Ventricular assist devices (VADs) have been associated with high rates of neurologic injury in pediatric patients during the period of support, but the delayed consequences of this type of injury have not been described in the literature.In this study we assess cognitive outcomes with indices of general intellectual functioning, including working memory, processing speed, perceptual reasoning and verbal comprehension, for pediatric heart transplant recipients who required VAD support as a bridge to transplant (n = 9). We present an aggregate of these VAD patients combined with heart transplant recipients who did not require mechanical circulatory support (n = 11), and compare the performance of all transplant patients (n = 20) to typically developing, healthy comparators (n = 12). We also present a post hoc analysis of those transplant recipients with significant medical morbidity in the first year of life, referred to as the "high-risk" transplant group (n = 5), and compare them with the "low-risk" transplant group (n = 15) and the typically developing comparators (n = 12).The mean performance of the VAD patients was in the average range for each of the examined indices of cognitive functioning. A total of 11% of the VAD patients performed in the impaired range and 78% performed in the average range, with 11% in the superior range on measures of general intellectual functioning. The typically developing participants performed significantly better than the aggregated transplant recipients on all indices except verbal comprehension. Lower cognitive performance in the combined transplant group appears to be associated with medical morbidity in the first year of life.Despite significant neurologic risk factors, this cohort of pediatric patients who were bridged to transplant with VAD demonstrated resiliency in terms of cognitive outcomes. In this heterogeneous population, it is likely that multiple factors contributed to the cognitive outcomes. As VAD use becomes more common in pediatric patients, a prospective evaluation of cognitive outcomes is warranted.
View details for DOI 10.1016/j.healun.2012.11.006
View details for Web of Science ID 000314445800006
Insights into dyssynchrony in Hypoplastic Left Heart Syndrome HEART RHYTHM 2012; 9 (12): 2010-2015
Cardiac resynchronization therapy has been proposed for treatment of hypoplastic left heart syndrome (HLHS) patients with right ventricular (RV) failure. The role of dyssynchrony, however, is poorly understood in this population.The purpose of this study was to better understand the relationship between electrical and mechanical dyssynchrony in HLHS using 3-dimensional electrical mapping, tissue Doppler indices of wall motion, and vector velocity imaging.Eleven HLHS subjects with normal RV function and ten normal subjects (age 3-18 years) were studied. Electrical and mechanical activation times and dyssynchrony indices (electrical dyssynchrony index, mechanical dyssynchrony index) were calculated using 3-dimensional electrical mapping, tissue Doppler indices, and vector velocity imaging.No differences in measures of electrical dyssynchrony were seen when comparing HLHS patients and normal patients (electrical activation time 63.3 22.8 ms vs 56.2 11.2 ms, P = .38; electrical dyssynchrony index 13.7 6.3 ms vs 11.6 3.0 ms, P = .34). However, measures of mechanical dyssynchrony were markedly abnormal in HLHS patients despite normal RV function (mechanical activation time 16 11.3 ms vs 0.91.9 ms, P = .01; mechanical dyssynchrony index 7.5 5.5 vs 0.4 0.8, P<.01).Patients with HLHS and preserved RV systolic function have normal electrical activation when compared to patients with normal right and left ventricles. In contrast, these patients demonstrate mechanical dyssynchrony compared to patients with normal right and left ventricles. This finding raises important questions about the indications for cardiac resynchronization therapy in this patient population.
View details for DOI 10.1016/j.hrthm.2012.08.031
View details for Web of Science ID 000311791900029
View details for PubMedID 23085485
Prospective Trial of a Pediatric Ventricular Assist Device NEW ENGLAND JOURNAL OF MEDICINE 2012; 367 (6): 532-541
Options for mechanical circulatory support as a bridge to heart transplantation in children with severe heart failure are limited.We conducted a prospective, single-group trial of a ventricular assist device designed specifically for children as a bridge to heart transplantation. Patients 16 years of age or younger were divided into two cohorts according to body-surface area (cohort 1, <0.7 m(2); cohort 2, 0.7 to <1.5 m(2)), with 24 patients in each group. Survival in the two cohorts receiving mechanical support (with data censored at the time of transplantation or weaning from the device owing to recovery) was compared with survival in two propensity-score-matched historical control groups (one for each cohort) undergoing extracorporeal membrane oxygenation (ECMO).For participants in cohort 1, the median survival time had not been reached at 174 days, whereas in the matched ECMO group, the median survival was 13 days (P<0.001 by the log-rank test). For participants in cohort 2 and the matched ECMO group, the median survival was 144 days and 10 days, respectively (P<0.001 by the log-rank test). Serious adverse events in cohort 1 and cohort 2 included major bleeding (in 42% and 50% of patients, respectively), infection (in 63% and 50%), and stroke (in 29% and 29%).Our trial showed that survival rates were significantly higher with the ventricular assist device than with ECMO. Serious adverse events, including infection, stroke, and bleeding, occurred in a majority of study participants. (Funded by Berlin Heart and the Food and Drug Administration Office of Orphan Product Development; ClinicalTrials.gov number, NCT00583661.).
View details for DOI 10.1056/NEJMoa1014164
View details for Web of Science ID 000307310800007
View details for PubMedID 22873533
Electrical and mechanical dyssynchrony in pediatric pulmonary hypertension JOURNAL OF HEART AND LUNG TRANSPLANTATION 2012; 31 (8): 825-830
Electrical and mechanical dyssynchrony are often seen in patients with left ventricular failure. In pediatric pulmonary hypertension (PH), right ventricular failure predominates; however, the prevalence of electrical and/or mechanical dyssynchrony in these patients is unknown. We examined the prevalence of electrical and mechanical dyssynchrony in pediatric PH patients.Medical records (including, functional status, electrocardiograms and echocardiograms) of pediatric PH patients were reviewed. QRS duration z-scores were calculated to determine electrical dyssynchrony. Echo vector velocity imaging was used to calculate the mechanical dyssynchrony index (DI).Seventy-seven PH patients (idiopathic pulmonary arterial hypertension [IPAH]: n = 26; congenital heart disease: n = 41; other: n = 10) were studied. Electrical dyssynchrony was seen in 84% (p < 0.01 vs historic controls), with a mean z-score of 4.3 (95% CI 3.5 to 5.1). There was no difference between those with IPAH, z = 3.6 (95% CI 2.5 to 4.6), and those without, z = 4.7 (95% CI 3.6 to 5.8). Mechanical dyssynchrony was seen in 76% of patients (mean DI = 66 47 vs 18 8 milliseconds in historic controls, p < 0.01) in both IPAH and non-IPAH patients. Post-operative congenital heart disease patients had the largest dyssynchrony index. No correlation was found among electrical or mechanical dyssynchrony, hemodynamics or disease severity.Significant electrical and mechanical dyssynchrony is present in pediatric PH patients, regardless of etiology. The overall effect of electrical and mechanical dyssynchrony on outcomes in this patient population is still unknown. Select patients may benefit from resynchronization therapy.
View details for DOI 10.1016/j.healun.2012.04.004
View details for Web of Science ID 000306449000006
View details for PubMedID 22682994
Outcomes of Children Following a First Hospitalization for Dilated Cardiomyopathy CIRCULATION-HEART FAILURE 2012; 5 (4): 437-443
We hypothesized that children with dilated cardiomyopathy who require hospital admission are at increased risk for death or transplantation during their first hospitalization and in the first year that follows. We also assessed the value of routine data collected during that time to predict death or the need for transplantation prior to discharge and within 1 year of admission.We conducted a retrospective review of 83 pediatric patients with dilated cardiomyopathy whose initial hospitalization fell between 2004 and 2009. The mean age at hospitalization was 7 years. The majority of patients demonstrated moderate or severe left ventricular dysfunction on initial echocardiogram (80%) and/or the need for intravenous inotropes within 7 days of hospital admission (69%). Five patients (6%) died, and 15 (18%) were transplanted in the initial hospitalization. At 1 year, 11/71 (15%) had died, and 27/71 (38%) were transplanted. The overall freedom from death, transplantation, or rehospitalization at 1 year following admission was 21%. Fractional shortening, left ventricular ejection fraction, serum cholesterol, uric acid, mixed venous saturation, and atrial filling pressures were all predictive of death or transplantation during the initial hospitalization. Left ventricular ejection fraction was predictive of death or transplantation at 1 year.The first hospitalization for dilated cardiomyopathy marks a period of high risk for clinical decline, end stage heart failure, and the need for cardiac transplantation. Echocardiographic function and hemodynamic and serum measurements may aid in predicting outcomes. Despite medical management, most patients will be rehospitalized and/or require cardiac transplantation within 1 year of admission.
View details for DOI 10.1161/CIRCHEARTFAILURE.111.964510
View details for Web of Science ID 000313578100013
View details for PubMedID 22570362
Perioperative management of the morbidly obese adolescent with heart failure undergoing bariatric surgery PEDIATRIC ANESTHESIA 2012; 22 (5): 476-482
The incidence and prevalence of adolescent obesity and adolescent heart failure are increasing, and anesthesiologists increasingly will encounter patients with both conditions. A greater understanding of the physiologic challenges of adolescent heart failure as they relate to the perioperative stressors of anesthesia and bariatric surgery is necessary to successfully manage the perioperative risks faced by this growing subpopulation. Here, we present a representative case of a morbidly obese adolescent with heart failure who underwent a laparoscopic bariatric operation and review the limited available literature on perioperative management in this age group. Specifically, we review evidence and offer recommendations related to preoperative evaluation, venous thromboembolism prophylaxis, positioning, induction, airway management, monitoring, anesthetic maintenance, ventilator management, and adverse effects of the pneumoperitoneum, rhabdomyolysis, and postoperative care.
View details for DOI 10.1111/j.1460-9592.2012.03824.x
View details for Web of Science ID 000302540900011
View details for PubMedID 22385267
Is There a Rote for Cardiac Resynchronization Therapy in Pediatric Pulmonary Hypertension? ELSEVIER SCIENCE INC. 2012: S29-S29
Complement Fixation by C1q vs MFI: Detection of Clinically Relevant Antibodies ELSEVIER SCIENCE INC. 2012: S139-S140
Use of the Impella 5.0 as a bridge from ECMO to implantation of the HeartMate II left ventricular assist device in a pediatric patient PEDIATRIC TRANSPLANTATION 2012; 16 (2): 205-206
End-Stage Renal Disease and Cardiomyopathy in Children: Cardiac Effects of Renal Transplantation TRANSPLANTATION 2012; 93 (2): 182-187
The occurrence and progression of cardiomyopathy is well known in patients with end-stage renal disease (ESRD). However, the feasibility of renal transplantation in the setting of cardiac dysfunction and the effect of renal transplantation on this progression remain poorly studied in pediatric patients.A single-center, retrospective review of pediatric renal transplants between January 1, 2001, and December 31, 2010, was conducted. Six children with ESRD and severe systolic dysfunction underwent renal transplantation. Clinical data were collected and compared for the pretransplant, peritransplant, and posttransplant periods.Nutritional support, dialysis, and chronic kidney disease and heart failure therapy led to improved cardiac function before transplantation (ejection fraction 28.8%9.6% vs. 44.4%11.5%; fractional shortening 12.7%5.1% vs. 23.6%6.2%); however, normal systolic function was not achieved before transplantation in any patient. After transplantation, two patients had normalization of systolic function by hospital discharge, while the systolic function of the remaining four patients normalized during the first posttransplant year. Mean ejection fraction 1 year posttransplant was 22 units greater than before transplant. All patients experienced excellent allograft function in the peritransplant period. Mean estimated creatinine clearance 1 year posttransplant was 93.233.3 mL/min/1.73 m(2).Renal transplantation can be performed safely in children with ESRD and severe systolic dysfunction. After transplantation, systolic function continues to improve and may reach normal levels during the first posttransplant year. The presence of severe systolic dysfunction in pediatric dialysis patients should not deter referral for renal transplantation.
View details for DOI 10.1097/TP.0b013e31823be7f8
View details for Web of Science ID 000299164000013
View details for PubMedID 22146314
Characteristics and Outcome After Hospitalization for Acute Right Heart Failure in Patients With Pulmonary Arterial Hypertension CIRCULATION-HEART FAILURE 2011; 4 (6): 692-699
Although much is known about the risk factors for poor outcome in patients hospitalized with acute heart failure and left ventricular dysfunction, much less is known about the syndrome of acute heart failure primarily affecting the right ventricle (acute right heart failure).By using Stanford Hospital's pulmonary hypertension database, we identified consecutive acute right heart failure hospitalizations in patients with PAH. We used longitudinal regression analysis with the generalized estimating equations method to identify factors associated with an increased likelihood of 90-day mortality or urgent transplantation. From June 1999 to September 2009, 119 patients with PAH were hospitalized for acute right heart failure (207 episodes). Death or urgent transplantation occurred in 34 patients by 90 days of admission. Multivariable analysis identified a higher respiratory rate on admission (>20 breaths per minute; OR, 3.4; 95% CI, 1.5-7.8), renal dysfunction on admission (glomerular filtration rate <45 mL/min per 1.73 m2; OR, 2.7; 95% CI, 1.2-6.3), hyponatremia (serum sodium ?136 mEq/L; OR, 3.6; 95% CI, 1.7-7.9), and tricuspid regurgitation severity (OR, 2.5 per grade; 95% CI, 1.2-5.5) as independent factors associated with an increased likelihood of death or urgent transplantation.These results highlight the high mortality after hospitalizations for acute right heart failure in patients with PAH. Factors identifiable within hours of hospitalization may help predict the likelihood of death or the need for urgent transplantation in patients with PAH.
View details for DOI 10.1161/CIRCHEARTFAILURE.110.949933
View details for Web of Science ID 000297166100008
View details for PubMedID 21908586
Berlin Heart EXCOR Pediatric ventricular assist device Investigational Device Exemption study: Study design and rationale AMERICAN HEART JOURNAL 2011; 162 (3): 425-U41
Currently, there are no Food and Drug Administration-approved devices available that can provide long-term mechanical circulatory support to smaller children with severe heart failure as a bridge to heart transplant (HT). In recent years, the Berlin Heart EXCOR Pediatric ventricular assist device (VAD) has emerged as a potential treatment option. Systematic data on the safety and efficacy of the EXCOR are limited.The Investigational Device Exemption (IDE) clinical study is designed to evaluate the safety and probable benefit of the EXCOR to support regulatory review of the device under the Humanitarian Device Exemption regulation. The study design and rationale are reviewed in light of the well-described challenges inherent in small population studies.The Berlin Heart EXCOR IDE clinical study is a prospective, multicenter, single-arm, clinical cohort study. Children aged 0 to 16 years with severe heart failure (Interagency Registry for Mechanically Assisted Circulatory Support profile 1 or 2) due to 2-ventricle heart disease and actively listed for HT comprise the primary study cohort. The control population is a propensity-matched retrospective cohort of children supported with extracorporeal membrane oxygenation, the only bridge device available to smaller children before the EXCOR. The primary efficacy end point is survival to heart transplantation or recovery. The primary safety end point is the incidence of serious adverse events as defined by pediatric Interagency Registry for Mechanically Assisted Circulatory Support criteria. The study will enroll a total of 48 subjects in 2 cohorts based on body surface area (cohort 1 <0.7 m(2), cohort 2 0.7-1.5 m(2)) and is powered to show safety superiority to a prespecified performance goal of 0.25 serious adverse events per day of support. Children ineligible for the primary cohort will still have access to the device in a third compassionate-use cohort where adverse event data will be collected for additional safety characterization of the device.The Berlin Heart IDE clinical study will be the first bridge-to-HT VAD study designed exclusively for children. It is anticipated that the study will provide important information on the safety and efficacy of the Berlin Heart EXCOR Pediatric in children while providing valuable lessons into the design and conduct of future VAD studies in children.
View details for DOI 10.1016/j.ahj.2011.05.026
View details for Web of Science ID 000294447400003
View details for PubMedID 21884857
The impact of heart failure severity at time of listing for cardiac transplantation on survival in pediatric cardiomyopathy JOURNAL OF HEART AND LUNG TRANSPLANTATION 2011; 30 (7): 755-760
The survival benefit of heart transplantation in adult heart failure is greatest for the sickest patients and negligible for patients not requiring inotropic or mechanical support. We hypothesized a similar survival benefit of heart transplantation for childhood cardiomyopathies with heart failure.A merged data set of children registered in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study was used to assess differences in mortality before and after transplant in patients with different levels of heart failure severity. Severity was scored 2 if mechanical ventilatory or circulatory support was required, 1 if intravenous inotropes were required, or 0 if no support was required.For 332 eligible children, 12-month mortality after listing was 9% for those with a severity score of 0 (n = 105), 16% with a score of 1 (n = 118), and 26% with a score of 2 (n = 109; p = 0.002) with a 3%, 8%, and 20% mortality with severity scores at listing of 0, 1, and 2, respectively, occurring before transplant. Patients listed with a score of 0 frequently deteriorated: 50% received an allograft or died before transplant with severity scores of 1 or 2. The risk of deterioration increased with previous surgery (relative risk, 3.84; p = 0.03) in the short-term and with lower left ventricular mass z-score at time of presentation (relative risk, 1.74; p = 0.003) in the longer-term.Pediatric cardiomyopathy patients who require high levels of support receive a survival benefit from heart transplantation that is not shared by patients not requiring intravenous inotropic or mechanical support.
View details for DOI 10.1016/j.healun.2011.01.718
View details for Web of Science ID 000291898800003
View details for PubMedID 21419658
Implementation of a Comprehensive Interdisciplinary Care Coordination of Infants and Young Children on Berlin Heart Ventricular Assist Devices JOURNAL OF CARDIOVASCULAR NURSING 2011; 26 (3): 231-238
Young children supported on a ventricular assist device (VAD) can have prolonged hospitalizations awaiting heart transplantation. The adult VAD literature demonstrates that comprehensive programs optimize transplantation outcomes. The goal of this intervention was to create an interdisciplinary program to optimize care coordination and delivery in young children requiring a VAD.This study was a case review.We have supported 8 infants and young children with Berlin Heart VAD. These children's hospitalizations have been more complex than those of our older VAD patients, and they have required intensive care for prolonged periods. An interdisciplinary group evaluated our practices and identified areas for potential improvement. The focus group from our intensive care unit introduced multiple interventions to optimize interdisciplinary care coordination and consistency of practice. These included (1) interdisciplinary care guidelines for chronically, critically ill patients; (2) institution of a primary cardiovascular intensive care unit (CVICU) physician and nurse practitioner system; (3) introduction of a psychological intervention for families to minimize the impact of their extended CVICU stay; (4) implementation of early jejunal feeds; (5) a focused developmental and rehabilitation protocol, 6) implementation of a structured approach to minimizing blood transfusions; (7) increased structure for our interdisciplinary rounds to optimize team communication; (8) comprehensive nursing education including wound care, anticoagulation management, and laboratory draws; and (9) a protocol for off-unit rehabilitative/developmental interventions. These changes in both practice and approach for young VAD patients have also been of benefit to other chronically, critically ill children in the CVICU, as well as to our CVICU team.Comprehensive interdisciplinary care coordination and standardized practice can be achieved in the critical care setting for complex pediatric heart failure patients who require long-term VAD support.
View details for DOI 10.1097/JCN.0b013e3181f29a2e
View details for Web of Science ID 000289404100010
View details for PubMedID 21099697
Cardiomyopathy and heart failure in children: anesthetic implications PEDIATRIC ANESTHESIA 2011; 21 (5): 577-584
The purpose of this article is to provide a brief but systematic overview of heart failure and cardiomyopathy in children and the anesthetic management of these patients. We will begin with disease definitions and descriptions of the disorders. Our review will include the epidemiology and etiology of the more prevalent underlying causes of heart failure, the principal pathophysiology of the specific cardiomyopathies, as well as the common therapies in use today in both inpatient and outpatient settings. Important implications for anesthetic management will be highlighted.
View details for DOI 10.1111/j.1460-9592.2011.03561.x
View details for Web of Science ID 000289469500013
View details for PubMedID 21481080
GENETIC DETERMINANTS OF DRAMATIC IMPROVEMENT IN LEFT VENTRICULAR FUNCTION IN PATIENTS WITH HEART FAILURE ELSEVIER SCIENCE INC. 2011: E2041-E2041
Longitudinal Hemodynamic Changes in Pediatric Patients with Severe Pulmonary Arterial Hypertension (PAH) on Prostanoid Therapy ELSEVIER SCIENCE INC. 2011: S72-S72
MRI Evaluation of the Right Ventricle in Pediatric Pulmonary Arterial Hypertension ELSEVIER SCIENCE INC. 2011: S81-S81
Electrical Dyssynchrony in Pediatric Pulmonary Arterial Hypertension ELSEVIER SCIENCE INC. 2011: S224-S225
Clinical usefulness of a novel C1q assay to detect immunoglobulin G antibodies capable of fixing complement in sensitized pediatric heart transplant patients JOURNAL OF HEART AND LUNG TRANSPLANTATION 2011; 30 (2): 158-163
Donor-specific antibodies (DSA) against human leukocyte antigens complicate transplantation with the potential for acute antibody-mediated rejection (AMR). Complement-fixing antibodies are required to initiate the complement cascade. Not all DSAs, however, can fix complement.A novel C1q assay was developed to detect the sub-set of immunoglobulin G (IgG) antibodies capable of fixing complement. Sera from 18 pediatric heart transplant patients were analyzed for DSAs using a Luminex platform (Luminex Inc, Austin, TX) and commercially available single-antigen bead assay kits. Biopsy specimens were assessed for AMR using histopathologic criteria and immunohistochemical staining.During the study period, 5 patients had AMR; of these, 2 were C1q virtual crossmatch positive (VXM+) and had persistent C1q DSAs after transplant, and 3 were C1q VXM- but antibody developed immediately after transplant. A positive C1q assay in the immediate post-transplant period had a positive predictive value (PPV) of 100% and a negative predictive value (NPV) of 100%, with 100% sensitivity and 100% specificity (Fisher exact p = 0.001). Of 11 patients who were IgG VXM+, 5 had AMR; the IgG VXM had a PPV of 45% and NPV of 100%, with 100% sensitivity and 54% specificity (Fisher exact p = 0.101).The C1q assay can detect a sub-set of antibodies capable of fixing complement and predicts AMR early after transplant. Avoiding only the donor antigens that would be recognized by the C1q assay may accelerate time to transplant by expansion of the donor pool and potentially allows transplantation of previously "incompatible" organs.
View details for DOI 10.1016/j.healun.2010.08.020
View details for Web of Science ID 000286545200008
View details for PubMedID 20951058
Bridging children of all sizes to cardiac transplantation: The initial multicenter North American experience with the Berlin Heart EXCOR ventricular assist device JOURNAL OF HEART AND LUNG TRANSPLANTATION 2011; 30 (1): 1-8
Beginning in 2000 and accelerating in 2004, the Berlin Heart EXCOR (Berlin Heart Inc Woodlands, TX) became the first pediatric-specific ventricular assist device (VAD) applied throughout North America for children of all sizes. This retrospective study analyzed the initial Berlin Heart EXCOR pediatric experience as a bridge to transplantation.Between June 2000 and May 2007, 97 EXCOR VADs were implanted in North America at 29 different institutions. The analysis is limited to 73 patients (75%) from 17 institutions, for which retrospective data were available.Median age and weight at VAD implant were 2.1 years (range, 12 days-17.8 years) and 11 kg (range, 3-87.6 kg), respectively. The primary diagnoses were dilated cardiomyopathy in 42 (58%), congenital heart disease in 19 (26%), myocarditis in 7 (10%), and other cardiomyopathies in 5 (7%). Pre-implant clinical condition was critical cardiogenic shock in 38 (52%), progressive decline in 33 (45%), or other in 2 (3%). Extracorporeal membrane oxygenation was used as a bridge to EXCOR in 22 patients (30%). Device selection was left VAD (LVAD) in 42 (57%) and biventricular assist devices (BiVAD) in 31 (43%). The EXCOR bridged 51 patients (70%) to transplant and 5 (7%) to recovery. Mortality on the EXCOR was 23% (n = 17) overall, including 35% (11 of 31) in BiVAD vs 14% (6 of 42) in LVAD patients (p = 0.003). Multivariate analysis showed younger age and BiVAD support were significant risk factors for death while on the EXCOR.This limited but large preliminary North American experience with the Berlin Heart EXCOR VAD as a bridge to cardiac transplantation for children of all ages and sizes points to the feasibility of this approach. The prospective investigational device evaluation trial presently underway will further characterize the safety and efficacy of the EXCOR as a bridge to pediatric cardiac transplantation.
View details for DOI 10.1016/j.healun.2010.08.033
View details for Web of Science ID 000286287000001
View details for PubMedID 21145473
Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS)-Defined Morbidity and Mortality Associated With Pediatric Ventricular Assist Device Support at a Single US Center The Stanford Experience CIRCULATION-HEART FAILURE 2010; 3 (6): 682-688
The use of ventricular assist devices (VADs) to bridge pediatric patients to heart transplantation has increased dramatically over the last 15 years. In this report, we present the largest US single-center report of pediatric VAD use to date. We present detailed descriptions of morbidity and mortality associated with VAD support, using standard Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) criteria for pediatrics to facilitate the comparison of these results to other studies.We retrospectively identified 25 patients younger than 18 years with 27 episodes of mechanical circulatory support using VADs as bridge to heart transplantation from January 1998 to December 2007. Survival to transplant for the entire cohort was 74%. The most common major morbidities, as defined by INTERMACS criteria for a pediatric population, were respiratory failure, major localized infections, major bleeding events, hepatic dysfunction, and right heart failure. Major neurological events occurred in 48% of the study population. The median time to the first occurrence of an adverse event was less than 14 days for respiratory failure, right heart failure, major localized infection, and major bleeding. Patients who died before transplantation had significantly more adverse events per day of support than did those who were successfully transplanted. Episodes of major bleeding, tamponade, acute renal failure, respiratory failure, and right heart failure were all associated with increased risk of mortality.INTERMACS criteria can be successfully used to analyze pediatric VAD outcomes. These data serve as a baseline for future studies of VAD support in children and indicate good survival rates but considerable morbidity.
View details for DOI 10.1161/CIRCHEARTFAILURE.109.918672
View details for Web of Science ID 000284261600011
View details for PubMedID 20807863
Outpatient management of pediatric heart failure. Heart failure clinics 2010; 6 (4): 515-?
The management of heart failure in children is becoming a specialized discipline within pediatric cardiology. Unlike the treatment of heart failure in adults, for which an extensive body of literature supports current treatment regimens, management of heart failure in children is largely guided by extrapolation from adult studies and expert opinion. This review focuses on the current state-of-the-art with respect to the outpatient management of heart failure in children.
View details for DOI 10.1016/j.hfc.2010.05.007
View details for PubMedID 20869651
Outcomes of Children With Restrictive Cardiomyopathy Listed for Heart Transplant: A Multi-institutional Study JOURNAL OF HEART AND LUNG TRANSPLANTATION 2009; 28 (12): 1335-1340
Restrictive cardiomyopathy (RCM) in children often has a progressive nature, with a high risk of clinical deterioration and death. Heart transplantation (HTx) is a widely accepted therapy that offers long-term survival, but criteria for and outcomes after listing have not been well defined.A multi-institutional, prospective, event-driven data registry of 3,147 patients aged < 18 years listed for HTx from January 1993 to December 2006 was used to assess risk factors and survival of 145 listed RCM patients.Mean age at listing was 8.1 years, with 44% listed as United Network of Organ Sharing status 1, 33% on inotropic support, 10% on a ventilator, and 5% on mechanical support. At 1 year, 82% of these patients survived to HTx, whereas 9% died waiting. Univariate risk factors for death while waiting included younger age (p < 0.001), ventilator dependence (p < 0.001), status 1 (p < 0.001), and inotrope usage (p < 0.001). Use of multiple support devices at listing (ventilator, extracorporeal membrane oxygenation, ventricular assist device, intraaortic balloon pump) was also an important risk factor for early phase death while waiting (relative risk; 9.01, p < 0.0001). Survival after listing was 63% at 10 years and compared favorably with survival for non-cardiomyopathy patients (p = 0.01).Children with RCM awaiting HTx have a generally low waitlist mortality and reasonable overall survival. Children requiring mechanical support and infants had a significantly higher risk of death while waiting. Further study is warranted to identify factors important in determining the optimal timing of listing in children with RCM before the need for inotropic or mechanical support.
View details for DOI 10.1016/j.healun.2009.06.028
View details for Web of Science ID 000272943500017
View details for PubMedID 19783176
Acute Effects of Single-site Pacing from the Left and Right Ventricle on Ventricular Function and Ventricular-Ventricular Interactions in Children with Normal Hearts CONGENITAL HEART DISEASE 2009; 4 (5): 356-361
We studied, as a physiological benchmark, acute effects of right ventricular (RV) apical, RV outflow, and left ventricular (LV) pacing in children with normal cardiac function on LV and RV function and ventricular-ventricular interactions.The design of the study was a prospective, acute intervention.The study was conducted in a tertiary care electrophysiology laboratory. Population and Methods. Seven children (mean +/- SD, 12 +/- 4 years) were paced after accessory pathway ablation, at baseline (AOO), and with atrioventricular pacing (DOO) from the RV apex, RV outflow, and left ventricle.Right ventricular dP/dT(max) and RV dP/dT(neg) (high-fidelity transducer-tipped catheters, Millar Instruments, Houston, TX, USA), cardiac index (Fick), blood pressure, and QRS duration were measured at each pacing condition. Intra- and interventricular mechanical dyssynchrony, systolic- and diastolic peak tissue velocities, and isovolumic acceleration were recorded by tissue Doppler imaging at the lateral mitral, septal, and tricuspid annuli at each condition. Results at each pacing condition were compared by repeated-measures analysis of variance. Results. Pacing prolonged QRS duration, causing electrical dyssynchrony (86 +/- 19 ms [baseline], 141 +/- 44 ms [RV apex], 121 +/- 18 ms [RV outflow], and 136 +/- 34 ms [LV], P < .01). Right ventricular outflow pacing caused LV intraventricular delay (63 +/- 52 vs. 12 +/- 7 ms, P < .05). Right ventricular apical pacing caused interventricular delay (61 +/- 29 vs. 25 +/- 18 ms, P < .05). There were no significant changes in blood pressure, cardiac index, RV dp/dT(max), RV dP/dT(neg), regional tissue velocities, or isovolumic acceleration during any of the pacing conditions, indicating preserved ventricular function and hemodynamics. No important ventricular-ventricular interactions were seen.In children with normal cardiac anatomy and function, single-site RV apical, RV outflow, and LV pacing induce electromechanical dyssynchrony without significantly changing ventricular function or hemodynamics, or adversely affecting ventricular-ventricular interactions.
View details for DOI 10.1111/j.1747-0803.2009.00327.x
View details for Web of Science ID 000207893900007
View details for PubMedID 19740190
Right ventricular resynchronization: Moving beyond proof of concept HEART RHYTHM 2009; 6 (6): 857-859
A Novel Non-Invasive Method of Estimating Pulmonary Vascular Resistance in Patients With Pulmonary Arterial Hypertension JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2009; 22 (5): 523-529
The assessment of pulmonary vascular resistance (PVR) plays an important role in the diagnosis and management of pulmonary arterial hypertension (PAH). The main objective of this study was to determine whether the noninvasive index of systolic pulmonary arterial pressure (SPAP) to heart rate (HR) times the right ventricular outflow tract time-velocity integral (TVI(RVOT)) (SPAP/[HR x TVI(RVOT)]) provides clinically useful estimations of PVR in PAH.Doppler echocardiography and right-heart catheterization were performed in 51 consecutive patients with established PAH. The ratio of SPAP/(HR x TVI(RVOT)) was then correlated with invasive indexed PVR (PVRI) using regression and Bland-Altman analysis. Using receiver operating characteristic curve analysis, a cutoff value for the Doppler equation was generated to identify patients with PVRI > or = 15 Wood units (WU)/m2.The mean pulmonary arterial pressure was 52 +/- 15 mm Hg, the mean cardiac index was 2.2 +/- 0.6 L/min/m2, and the mean PVRI was 20.5 +/- 9.6 WU/m2. The ratio of SPAP/(HR x TVI(RVOT)) correlated very well with invasive PVRI measurements (r = 0.860; 95% confidence interval, 0.759-0.920). A cutoff value of 0.076 provided well-balanced sensitivity (86%) and specificity (82%) to determine PVRI > 15 WU/m2. A cutoff value of 0.057 increased sensitivity to 97% and decreased specificity to 65%.The novel index of SPAP/(HR x TVI(RVOT)) provides useful estimations of PVRI in patients with PAH.
View details for DOI 10.1016/j.echo.2009.01.021
View details for Web of Science ID 000266091400017
View details for PubMedID 19307098
Pacing-Induced Electromechanical Ventricular Dyssynchrony Does Not Acutely Influence Right Ventricular Function and Global Hemodynamics in Children with Normal Hearts JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY 2009; 20 (5): 539-544
Right ventricular (RV) pacing may be detrimental to ventricular function. However, the acute effects of electromechanical dyssynchrony on RV function are not well characterized in children. We studied acute effects of electromechanical dyssynchrony, induced by RV apical and RV outflow pacing, in children with normal hearts, evaluating electromechanical synchrony, hemodynamic response, and RV function.Seventeen children (mean +/- SD, 12 +/- 4 years) with normal cardiac structure/function were paced after accessory pathway ablation, at baseline (AOO), and with AV pacing (DOO) from the RV apex and RV outflow. QRS duration was determined from surface ECG. Intra- and interventricular mechanical dyssynchrony and regional ventricular function were determined using tissue Doppler imaging. Global RV systolic and diastolic functions were assessed by RV dP/dT(max) and RV dP/dT(neg) using pressure-tipped transducers. Regional RV function was assessed by tissue Doppler imaging. Cardiac index (CI) and blood pressures were measured.RV apical and outflow pacing induced significant electromechanical dyssynchrony manifested by lengthening of the QRS duration, increased LV intraventricular delay (49 +/- 34 ms, 53 +/- 43 ms, respectively, P < 0.001), and increased interventricular delay (60 +/- 29 ms, 55 +/- 37 ms, P < 0.0001) versus AOO pacing. However, there was no change in blood pressure, CI, RV dp/dT(max), RV dP/dT(neg), or regional tissue Doppler velocities, indicating preserved hemodynamics and preserved global and regional RV systolic and diastolic function.In children with normal cardiac function and structure, pacing-induced electromechanical dyssynchrony did not acutely affect RV systolic and diastolic function and did not acutely alter global hemodynamics. Therefore, electromechanical dyssynchrony may only be an important therapeutic target in the setting of decreased RV function.
View details for DOI 10.1111/j.1540-8167.2008.01354.x
View details for Web of Science ID 000265409900012
View details for PubMedID 19017336
Exercise Capacity Is Decreased in Children With Chronic Right Ventricular Pacing and Congenital Complete Heart Block ELSEVIER SCIENCE INC. 2009: A359-A359
Evaluation of Polymorphisms in Candidate Genes in the Dramatic Response to Pharmacologic Therapy of Heart Failure LIPPINCOTT WILLIAMS & WILKINS. 2008: E64-E65
Overrepresentation of neuronal development pathways in heart failure patients who dramatically responded to pharmaceutical therapy CHURCHILL LIVINGSTONE INC MEDICAL PUBLISHERS. 2008: S41-S41
Does size matter? Clinical applications of scaling cardiac size and function for body size CIRCULATION 2008; 117 (17): 2279-2287
Extensive evidence is available that cardiovascular structure and function, along with other biological properties that span the range of organism size and speciation, scale with body size. Although appreciation of such factors is commonplace in pediatrics, cardiovascular measurements in the adult population, with similarly wide variation in body size, are rarely corrected for body size. In this review, we describe the critical role of body size measurements in cardiovascular medicine. Using examples, we illustrate the confounding effects of body size. Current cardiovascular scaling practices are reviewed, as are limitations and alternative relationships between body and cardiovascular dimensions. The experimental evidence, theoretical basis, and clinical application of scaling of various functional parameters are presented. Appropriately scaled parameters aid diagnostic and therapeutic decision making in specific disease states such as hypertrophic cardiomyopathy and congestive heart failure. Large-scale studies in clinical populations are needed to define normative relationships for this purpose. Lack of appropriate consideration of body size in the evaluation of cardiovascular structure and function may adversely affect recognition and treatment of cardiovascular disease states in the adult patient.
View details for DOI 10.1161/CIRCULATIONAHA.107.736785
View details for Web of Science ID 000255394300014
View details for PubMedID 18443249
Subclinical late cardiac toxicity in childhood cancer survivors - Impact on self-reported health CANCER 2008; 112 (8): 1835-1844
The authors analyzed how self-reported health and self-reported modified New York Heart Association (NYHA) cardiac function scores were related to cardiac systolic function, cardiac risk factors, and cancer treatment history in childhood cancer survivors who reported no symptoms of cardiac disease.Long-term survivors of pediatric cancer who were treated between 1971 and 1995 (current ages, 16-39.7 years) underwent noninvasive clinical and laboratory cardiac risk evaluation and responded to selected subscales of the Medical Outcomes Study 36-item Short Form Health Survey. Results were compared with survivor history of anthracycline therapy alone or with radiotherapy (n=127 patients; mean, 10 years after diagnosis) versus no anthracycline therapy (n=32 patients; mean, 11 years after diagnosis).Sex, current age, highest school grade completed, race, age at diagnosis, diagnostic group, years off therapy, fractional shortening (FS), heart rate, and smoking status were found to be independently predictive of self-reported health. Interaction between female sex and higher low-density lipoprotein values and between diagnosis and abnormal FS variably predicted low reported vitality and low reported modified New York Heart Association (NYHA) scores. Echocardiographic findings, cardiac risk factors, and treatment history explained 13% to 28% of the variance in perceived health and self-reported modified NYHA scores.Systolic function and cardiac risk factors were linked to lower self-reported health and NYHA scores even in the absence of clinically evident cardiotoxicity.
View details for DOI 10.1002/cncr.23378
View details for Web of Science ID 000255018900024
View details for PubMedID 18311782
Right ventricular function in cardiovascular disease, part I - Anatomy, physiology, aging, and functional assessment of the right ventricle CIRCULATION 2008; 117 (11): 1436-1448
Pneumatic paracorporeal ventricular assist device in infants and children: Initial Stanford experience JOURNAL OF HEART AND LUNG TRANSPLANTATION 2008; 27 (2): 173-177
Mechanical circulatory support with the Berlin Heart EXCOR pediatric ventricular assist device (VAD) has been used successfully in Europe for children with cardiac failure. Eighty-seven devices have been placed in North America through February 2007. We describe our single-center experience in 8 children.Eight children (ages 4 to 55 months), with median weight of 9.6 kg and body surface area of 0.48 m(2), received the Berlin Heart VAD as a bridge to transplantation. All patients were in cardiogenic shock requiring multiple inotropes. Primary diagnoses were idiopathic dilated cardiomyopathy (n = 4), congenital heart disease (n = 3) and restrictive cardiomyopathy (n = 1). After device insertion, all patients were treated with an anti-coagulant (heparin or coumadin) and one or more platelet inhibitors (aspirin with clopidogrel or dipyridamole).Five patients received support with a left ventricular assist device (LVAD) and 3 with a biventricular device (BiVAD). Duration of support ranged from 2 to 234 days (median 57 days). Five patients (63%) were successfully bridged to transplantation; of these, 4 were discharged home and 1 died from early graft failure. Five patients developed post-operative neurologic events. Of these 5 events, 4 could be explained by embolism or hemorrhage. Device exchange was performed in 4 patients in the intensive care unit.In selected children, the Berlin Heart VAD can be used as a bridge to transplantation. In contrast to the published European experience, neurologic events occur frequently. Anti-coagulation and platelet inhibition strategies continue to evolve. Device exchange is technically feasible at the bedside and should be considered at the earliest visualization of thrombus formation.
View details for DOI 10.1016/j.healun.2007.11.567
View details for Web of Science ID 000253258800005
View details for PubMedID 18267223
Monitoring for cardiovascular disease in survivors of childhood cancer: Report from the cardiovascular disease task force of the children's oncology group PEDIATRICS 2008; 121 (2): E387-E396
Curative therapy for childhood cancer has improved significantly in the last 2 decades such that, at present, approximately 80% of all children with cancer are likely to survive > or = 5 years after diagnosis. Prevention, early diagnosis, and treatment of long-term sequelae of therapy have become increasingly more significant as survival rates continue to improve. Cardiovascular disease is a well-recognized cause of increased late morbidity and mortality among survivors of childhood cancer. The Children's Oncology Group Late Effects Committee and Nursing Discipline and Patient Advocacy Committee have recently developed guidelines for follow-up of long-term survivors of pediatric cancer. A multidisciplinary task force critically reviewed the existing literature to evaluate the evidence for the cardiovascular screening recommended by the Children's Oncology Group guidelines. In this review we outline the clinical manifestations of late cardiovascular toxicities, suggest modalities and frequency of monitoring, and address some of the controversial and unresolved issues regarding cardiovascular disease in childhood cancer survivors.
View details for DOI 10.1542/peds.2007-0575
View details for Web of Science ID 000252877600058
View details for PubMedID 18187811
Outcomes of children with restrictive cardiornyopathy listed for heart transplant: A multi-institutional study ELSEVIER SCIENCE INC. 2008: S188-S189
Carvedilol for children and adolescents with heart failure - A Randomized controlled trial JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION 2007; 298 (10): 1171-1179
Although beta-blockers improve symptoms and survival in adults with heart failure, little is known about these medications in children and adolescents.To prospectively evaluate the effects of carvedilol in children and adolescents with symptomatic systemic ventricular systolic dysfunction.A multicenter, randomized, double-blind, placebo-controlled study of 161 children and adolescents with symptomatic systolic heart failure from 26 US centers. In addition to treatment with conventional heart failure medications, patients were assigned to receive placebo or carvedilol. Enrollment began in June 2000 and the last dose was given in May 2005 (each patient received medication for 8 months).Patients were randomized in a 1:1:1 ratio to twice-daily dosing with placebo, low-dose carvedilol (0.2 mg/kg per dose if weight <62.5 kg or 12.5 mg per dose if weight > or =62.5 kg), or high-dose carvedilol (0.4 mg/kg per dose if weight <62.5 kg or 25 mg per dose if weight > or =62.5 kg) and were stratified according to whether each patient's systemic ventricle was a left ventricle or not.The primary outcome was a composite measure of heart failure outcomes in patients receiving carvedilol (low- and high-dose combined) vs placebo. Secondary efficacy variables included individual components of this composite, echocardiographic measures, and plasma b-type natriuretic peptide levels.There was no statistically significant difference between groups for the composite end point based on the percentage of patients who improved, worsened, or were unchanged. Among 54 patients assigned to placebo, 30 improved (56%), 16 worsened (30%), and 8 were unchanged (15%); among 103 patients assigned to carvedilol, 58 improved (56%), 25 worsened (24%), and 20 were unchanged (19%). The rates of worsening were lower than expected. The odds ratio for worsened outcome for patients in the combined carvedilol group vs the placebo group was 0.79 (95% CI, 0.36-1.59; P = .47). A prespecified subgroup analysis noted significant interaction between treatment and ventricular morphology (P = .02), indicating a possible differential effect of treatment between patients with a systemic left ventricle (beneficial trend) and those whose systemic ventricle was not a left ventricle (nonbeneficial trend).These preliminary results suggest that carvedilol does not significantly improve clinical heart failure outcomes in children and adolescents with symptomatic systolic heart failure. However, given the lower than expected event rates, the trial may have been underpowered. There may be a differential effect of carvedilol in children and adolescents based on ventricular morphology.clinicaltrials.gov Identifier: NCT00052026.
View details for Web of Science ID 000249374200022
View details for PubMedID 17848651
Right ventricular mechanical dyssynchrony in children with hypoplastic left heart syndrome JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2007; 20 (9): 1073-1079
Mechanical dyssynchrony predicts response to cardiac resynchronization therapy in adults with heart failure. Children with hypoplastic left heart syndrome (HLHS) are susceptible to right ventricular (RV) failure; however, mechanical dyssynchrony has not been studied in this population with newly available methodologies. We investigated RV mechanical dyssynchrony in children with HLHS using vector velocity imaging.We used vector velocity imaging to quantify the SD of time to peak velocity, strain, and strain rate among 6 RV segments to define intraventricular RV synchrony in 16 children with HLHS and RV and left ventricular (LV) synchrony in 16 healthy age-matched control subjects. We further investigated relations between QRS duration and mechanical dyssynchrony and between mechanical dyssynchrony and systolic function.Children with HLHS had significant RV mechanical dyssynchrony versus LV and RV control subjects (strain 37 +/- 35 vs 8 +/- 8 milliseconds, P = .003 [LV], 9 +/- 11 milliseconds, P = .005 [RV]; strain rate 31 +/- 37 vs 10 +/- 13 milliseconds, P = .04 [LV], 14 +/- 15 milliseconds, P = .09 [RV]). There was no significant relationship between QRS duration and mechanical dyssynchrony and no obvious relation between the degree of mechanical dyssynchrony and the RV fractional area of change.Children with HLHS have RV mechanical dyssynchrony unrelated to surface electrocardiographic QRS duration. This may contribute to RV dysfunction and may indicate the usefulness of cardiac resynchronization therapy in this population.
View details for DOI 10.1016/j.echo.2007.02.015
View details for Web of Science ID 000249454900007
View details for PubMedID 17566698
Noninvasive evaluation of late anthracycline cardiac toxicity in childhood cancer survivors JOURNAL OF CLINICAL ONCOLOGY 2007; 25 (24): 3635-3643
PURPOSEl: Childhood cancer survivors treated with anthracyclines and cardiac radiation are at risk for late-onset cardiotoxicity. The purpose of this study was to delineate the relationship between clinical factors and abnormalities of noninvasive cardiac testing (NICT).Participants were recruited from a long-term follow-up clinic. Study measures comprised physical examination, laboratory evaluation, echocardiogram, and ECG. Mean fractional shortening (FS) and afterload were compared for survivors who did (at risk [AR]) and did not (no risk [NR]) receive potentially cardiotoxic modalities, and with values expected for comparable age- and sex-matched controls.The 278 study participants (mean age, 18.1 years; median age, 16.8 years; range, 7.5 to 39.7 years) included 223 survivors AR for cardiotoxicity after treatment with anthracyclines (median dose +/- standard deviation [SD], 202 +/- 109 mg/m(2)) and/or cardiac radiation. Mean FS (+/- SD) was lower for AR (0.33 +/- 0.06) compared with NR survivors (0.36 +/- 0.05; P = .004) and normative controls (0.36 +/- 0.04; P < .001). Mean afterload (+/- SD) was higher for AR (58 +/- 21 g/cm(2)) compared with NR survivors (46 +/- 15 g/cm(2); P < .001) and normative controls (48 +/- 13 g/cm(2); P < .001). The distribution of FS and afterload among NR survivors did not differ from that of controls. After adjustment for age group at diagnosis and time since completion of therapy, anthracycline dose predicted decline in distribution of FS (P < .001) and increase in distribution of afterload (P < .001). Treatment with anthracycline doses >or= 100 mg/m(2) increased the risk of abnormal NICT; survivors who received >or= 270 mg/m(2) had a 4.5-fold excess risk of abnormal NICT (95% CI, 2.1 to 9.6) compared with controls.Childhood cancer survivors treated with anthracycline doses >or= 270 mg/m(2) are at greatest risk for abnormalities of FS and afterload.
View details for DOI 10.1200/JCO.2006.09.7451
View details for Web of Science ID 000249415900015
View details for PubMedID 17704413
Children with cardiomyopathy: complications after noncardiac procedures with general anesthesia PEDIATRIC ANESTHESIA 2007; 17 (8): 775-781
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
Mechanical dyssynchrony in children with systolic dysfunction secondary to cardiomyopathy: A Doppler tissue and vector velocity imaging study JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2007; 20 (6): 756-763
Mechanical dyssynchrony is common in adults with heart failure and its presence predicts response to cardiac resynchronization therapy. However, mechanical dyssynchrony and its quantification by echocardiography have not been extensively studied in children with cardiomyopathy. We investigated mechanical dyssynchrony in children with systolic dysfunction secondary to cardiomyopathy using Doppler tissue imaging (DTI) and vector velocity imaging (VVI).We used DTI and VVI to quantify mechanical dyssynchrony in 22 children with systolic dysfunction secondary to cardiomyopathy and in 25 healthy control subjects. We analyzed DTI results corrected for cardiac dimensions and evaluated correlation between electrical and mechanical dyssynchrony and between mechanical dyssynchrony and systolic function.DTI and VVI revealed significant mechanical dyssynchrony among children with cardiomyopathy. Intraventricular and interventricular delays as defined by DTI, and the SD of time to peak velocity, strain, and strain rate as defined by VVI were 2 to 3 times higher in patients with cardiomyopathy as compared with control subjects. There was no significant relationship between electrical and mechanical dyssynchrony.Children with systolic dysfunction secondary to cardiomyopathy have mechanical dyssynchrony, unrelated to electrical dyssynchrony, which can be measured by recent echocardiographic techniques including DTI and VVI. Children with cardiomyopathy and mechanical dyssynchrony are potential candidates for cardiac resynchronization therapy.
View details for DOI 10.1016/j.echo.2006.11.007
View details for Web of Science ID 000247090900012
View details for PubMedID 17543748
Indications for heart transplantation in pediatric heart disease - A scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young; the Councils on Clinical Cardiology, Cardiovascular Nursing, and Cardiovascular Surgery and Anesthesia; and the Quality of Care and Outcomes Research Interdisciplinary Working Group CIRCULATION 2007; 115 (5): 658-676
Since the initial utilization of heart transplantation as therapy for end-stage pediatric heart disease, improvements have occurred in outcomes with heart transplantation and surgical therapies for congenital heart disease along with the application of medical therapies to pediatric heart failure that have improved outcomes in adults. These events justify a reevaluation of the indications for heart transplantation in congenital heart disease and other causes of pediatric heart failure.A working group was commissioned to review accumulated experience with pediatric heart transplantation and its use in patients with unrepaired and/or previously repaired or palliated congenital heart disease (children and adults), in patients with pediatric cardiomyopathies, and in pediatric patients with prior heart transplantation. Evidence-based guidelines for the indications for heart transplantation or retransplantation for these conditions were developed.This evaluation has led to the development and refinement of indications for heart transplantation for patients with congenital heart disease and pediatric cardiomyopathies in addition to indications for pediatric heart retransplantation.
View details for DOI 10.1161/CIRCULATIONAHA.106.180449
View details for Web of Science ID 000244000800018
View details for PubMedID 17261651
Noninvasive assessment of pulmonary arterial capacitance by echocardiography JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2007; 20 (2): 186-190
Pulmonary arterial capacitance (PAC) has been associated with right ventricular (RV) workload and mortality in pulmonary hypertension, but is not routinely evaluated in children. We investigated whether PAC can be estimated noninvasively by echocardiography.We retrospectively determined PAC in 31 children with pulmonary hypertension, using echocardiography, and compared the results with those obtained at cardiac catheterization.PAC derived from echocardiography was similar to that derived from catheterization (mean +/- SD 1.16 +/- 1.0 vs 1.10 +/- 0.95 mL(3) x mm Hg(-1), P = not significant) and the two correlated well (r = 0.74, P < .0001). There was a highly significant polynomial relationship between PAC and RV anterior wall thickness indexed to body surface area (R(2) = 0.54, P < .0001), but not between pulmonary vascular resistance and RV wall thickness. Pulmonary vascular resistance and PAC did not correlate.Echocardiography can reliably estimate PAC, which strongly correlates with RV hypertrophy, a surrogate for RV work.
View details for DOI 10.1016/j.echo.2006.08.009
View details for Web of Science ID 000244162000013
View details for PubMedID 17275705
Percutaneous translurninal coronary angioplasty in a two-month old with coronary stenosis presenting as congenital cardiomyopathy: Acute results and intermediate follow-up CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS 2006; 68 (4): 632-636
Congenital or nonsurgically acquired obstructive coronary artery disease in infancy is exceedingly rare, and previous reports of catheter-based therapies in very young patients have been solely in patients having undergone arterial switch or other cardiovascular surgical procedures. We describe a previously unreported cause for ventricular dysfunction and heart failure in an infant, isolated congenital circumflex coronary artery stenosis, and successful treatment with percutaneous transluminal coronary angioplasty. Technical considerations, immediate results, and 3-year follow-up are provided.
View details for DOI 10.1002/ccd.20750
View details for Web of Science ID 000241048000023
View details for PubMedID 16969876
Unifocalization of major aortopulmonary collaterals in single-ventricle patients ANNALS OF THORACIC SURGERY 2006; 82 (3): 934-939
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
A novel echocardiographic Doppler method for estimation of pulmonary arterial pressures JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2006; 19 (5): 559-562
Current noninvasive methods for estimating diastolic and mean pulmonary artery pressures (PAp) in children are cumbersome and have limited accuracy. We hypothesized that systolic PAp correlates with diastolic and mean PAp, and that this correlation can be used to estimate diastolic and mean PAp from Doppler flow data.We recorded PAp in 112 patients 30 years or younger catheterized for heart failure, heart transplant, pulmonary hypertension, or congenital heart disease. We derived the relationship of systolic PAp to diastolic and mean PAp. We then applied these relations to systolic PAp measured by tricuspid regurgitation (TR) Doppler flow in a subset of 17 patients with pulmonary hypertension to predict mean and diastolic PAp, and correlated the results.An excellent linear relation was found between systolic PAp and both the diastolic and mean PAp measured at cardiac catheterization (r = 0.95, P < .0001; r = 0.98, P < .0001, respectively). The calculated diastolic PAp calculated from TR Doppler correlated well with invasive data (31 +/- 13 vs 30 +/- 11 mm Hg, respectively, not significant; r = 0.85, P < .0001) and surpassed existing methods that are based on pulmonary regurgitation for predicting diastolic PAp. Similarly, mean PAp calculated from TR Doppler flow correlated well with invasive data (r = 0.86, P < .0001).A strong linear relationship between systolic and diastolic PAp allows for easy and accurate noninvasive estimation of diastolic and mean PAp from TR Doppler flow.
View details for DOI 10.1016/j.echo.2005.12.020
View details for Web of Science ID 000237488600014
View details for PubMedID 16644441
Heterotopic heart transplant combined with postoperative sildenafil use for the treatment of restrictive cardiomyopathy ANNALS OF THORACIC SURGERY 2006; 81 (4): 1505-1507
We report successful management of a 22-month-old child with restrictive cardiomyopathy and severe pulmonary hypertension using the heterotopic heart transplant technique. Additional lessons learned from postoperative management, including the novel use of Sildenafil (Viagra, Pfizer, NY) for controlling pulmonary arterial pressure are described.
View details for DOI 10.1016/j.athoracsur.2005.02.069
View details for Web of Science ID 000236239200062
View details for PubMedID 16564308
A novel echo-doppler method to predict diastolic and mean pulmonary artery pressures: Derivation and validation by catheterization ELSEVIER SCIENCE INC. 2006: 249A-249A
Anthracyclines cause endothelial injury in pediatric cancer patients: A pilot study JOURNAL OF CLINICAL ONCOLOGY 2006; 24 (6): 925-928
The vascular endothelium plays a central role in the regulation of arterial vasomotor tone, releasing nitric oxide for vasodilation. Endothelial-dependent vasodilation can be assessed in vivo, using high resolution ultrasound to measure changes in diameter of the brachial artery. Animal studies have demonstrated that anthracyclines can damage the endothelium and impair the vasodilatory response of arteries; however, there are no comparable data in humans. This is a pilot study assessing endothelial toxicity from anthracyclines in pediatric cancer patients.Fourteen control patients and 14 cancer patients (4 to 21 years) were studied. Cancer patients had completed chemotherapy containing no less than 300 mg/m2 of anthracyclines 2 to 60 months before study. Brachial artery diameters were measured at rest and 1 minute after blood pressure cuff occlusion. Brachial artery reactivity (BAR) was calculated as percent change between baseline and after cuff deflation measurements. Results were compared using unpaired, two-tailed t-test.Baseline characteristics, including age, percentage of females, blood pressure, and resting vessel diameters were similar between the two groups. BAR in the controls averaged 6.7% with a standard deviation (SD) of 3.3%, while BAR in patients receiving anthracyclines averaged 3.8% with an SD of 3.4%, demonstrating a significant decrease (P < .05) in vasomotor reactivity in the treated group.These results suggest that anthracyclines cause impaired endothelial function, an important and newly recognized toxicity. Since endothelial dysfunction is an early event in atherogenesis, there may be important clinical implications from these findings. Further study is required to confirm these preliminary results in a larger cohort.
View details for DOI 10.1200/JCO.2005.03.5956
View details for Web of Science ID 000235469700017
View details for PubMedID 16484703
Real-time color-flow CMR in adults with congenital heart disease JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE 2006; 8 (6): 809-815
CMR is valuable in the evaluation of congenital heart disease (CHD). Traditional flow imaging sequences involve cardiac and respiratory gating, increasing scan time and susceptibility to arrhythmias. We studied a real-time color-flow CMR system for the detection of flow abnormalities in 13 adults with CHD. All 16 congenital flow abnormalities previously detected by echocardiography were visualized using color-flow CMR, including atrial septal defects (n = 4), ventricular septal defects (n = 9), aortic coarctation (n = 1), Blalock-Taussig shunt (n = 1) and Fontan shunt (n = 1). Real-time color-flow CMR can identify intra- and extra-cardiac flow abnormalities in adults with congenital heart disease.
View details for DOI 10.1080/10976640600777728
View details for Web of Science ID 000241485600007
View details for PubMedID 17060103
New developments in echocardiographic methods to assess right ventricular function in congenital heart disease CURRENT OPINION IN CARDIOLOGY 2005; 20 (2): 84-88
Evaluation of right ventricular (RV) function in patients with congenital heart disease (CHD) is an essential component of clinical management. The complex geometry of the RV makes this a challenging task and necessitates the use of alternative methods from those used to assess left ventricular (LV) function. Recent developments in echocardiographic techniques have enhanced our ability to accurately assess RV function. We focus this review on literature published since September 2003.In this review we survey recent literature pertaining to advances in echocardiographic techniques used in assessing RV function. These new methods use two-dimensional echocardiography, Doppler echocardiography, tissue Doppler imaging (TDI), and strain rate imaging. Doppler techniques offer unique advantages for RV function in that they are independent of geometry and relatively independent of loading conditions.Echocardiography can assess RV function in a reliable fashion in children and adults with congenital heart disease. Appropriate use of echocardiography can minimize the utilization of more invasive imaging modalities such as magnetic resonance imaging and can guide clinical decisions regarding the necessity of obtaining such imaging.
View details for Web of Science ID 000228074200004
View details for PubMedID 15711192
Congenital junctional ectopic tachycardia and congenital complete atrioventricular block: A shared etiology? HEART RHYTHM 2005; 2 (3): 313-315
International society for heart and lung transplantation: Practice guidelines for management of heart failure in children JOURNAL OF HEART AND LUNG TRANSPLANTATION 2004; 23 (12): 1313-1333
Identifying cardiac transplant rejection in children: Diagnostic utility of echocardiography, right heart catheterization and endomyocardial biopsy data JOURNAL OF HEART AND LUNG TRANSPLANTATION 2004; 23 (3): 323-329
There has been a continued search for alternative diagnostic techniques that do not necessitate endomyocardial biopsy for diagnosing rejection in cardiac transplant recipients. The purpose of this study is to evaluate the role of echocardiography and hemodynamic catheterization data compared with endomyocardial biopsy results, in rejection surveillance for the pediatric heart transplant recipient.A prospective, blinded evaluation was performed utilizing echocardiographic and standard right heart catheterization parameters to predict acute rejection episodes.Forty-nine patients underwent 281 biopsies. Two groups were defined: those with Grade <2 rejection and those with grade > or =2 rejection. None of the echocardiographic variables showed significant differences between the study groups and all group data were within normal limits. Mixed venous saturation, mean right atrial pressure, right ventricular end-diastolic pressure and mean pulmonary artery pressure were found to be statistically significant between groups. Receiver-operator characteristic (ROC) curves were constructed to determine the extent to which the various parameters were clinically useful. The ROC found little clinical usefulness for all variables, including those found to be statistically significant.Differences in both echocardiographic and hemodynamic data were not clinically significant between the 2 groups of patients. Although many of the catheterization-derived parameters were statistically significant, they did not permit effective discrimination between groups. This is the only clinically relevant application of such data and may explain the conflicting previous reports. It is only through analyses such as ROC that the clinical application (or lack thereof) can be appreciated in this population.
View details for DOI 10.1016/S1053-2498(03)00209-2
View details for Web of Science ID 000220155700009
View details for PubMedID 15019642
A visual approach for the accurate determination of echocardiographic left ventricular ejection fraction by medical students JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2003; 16 (8): 824-831
Previously published reports show that there is significant intraobserver, interobserver, and interinstitutional variability in the determination of left ventricular (LV) ejection fraction (EF) by echocardiography. With the increased deployment of echocardiography (eg, handheld devices), there exists a need for developing a simple, intuitive approach for evaluating LVEF that allows a wider range of physicians to accurately and rapidly determine LVEF.We sought to create a system for assessing LVEF that relies on recognition and matching of patterns, rather than on mathematic calculations and geometric assumptions.A library of videoclips of cardiac function was compiled from 54 patients who spanned the spectrum of LVEF. LVEFs were calculated for these patients using standard echocardiographic methods, with further validation of a subsample using cardiac magnetic resonance imaging measurement of LVEF. The library of images was used to create a software tool for assessing LVEF on the basis of a "template-matching" approach. The software tool was then tested on medical students (N=13) to determine whether it enabled relatively untrained individuals to make accurate LVEF estimates.Using a template-matching approach for interpretation of echocardiograms, medical students were able to accurately estimate LVEF after only a limited introduction to echocardiography. Their LVEF estimates showed good correlation and agreement with gold standard (r = 0.88, standard square of the estimate = 6.0, limits of agreement = +12.0%, -15.6%).A new visual approach for assessing cardiac function using template matching can accurately estimate LVEF. With minimal training, medical students can make LVEF estimates that correlate well with gold standard. The application of this new approach includes allowing for the interpretation of LVEF from echocardiograms to be performed by a broader spectrum of physicians.
View details for DOI 10.1067/S0894-7317(03)00400-0
View details for Web of Science ID 000184604400007
View details for PubMedID 12878991
Electrical resynchronization - A novel therapy for the failing right ventricle CIRCULATION 2003; 107 (18): 2287-2289
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
Validation of the Doppler PR interval in the fetus JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2002; 15 (9): 1029-1030
Carbohydrate-deficient glycoprotein syndrome-associated pericardial effusion treated with corticosteroids and salicylic acid PEDIATRIC CARDIOLOGY 2002; 23 (4): 469-471
We describe an infant with a persistent pericardial effusion who was diagnosed with carbohydrate-deficient glycoprotein syndrome (CDGS)-Ia. She was born with mild dysmorphic features and common cardiac abnormalities. However, she re-presented at 2.5 months of age with a pericardial effusion. We decided to embark on a therapeutic trial of corticosteroids and salicylic acid therapy in an attempt to avoid pericardectomy. After 3 weeks of medical treatment the effusion resolved. This experience allows us to propose that medical management with corticosteroids and salicylic acid can be considered as an alternative to surgical therapy for CDGS-I patients with persistent pericardial effusions.
View details for DOI 10.1007/s00246-002-1497-1
View details for Web of Science ID 000176665700018
View details for PubMedID 12170369
Comparison of contrast echocardiography versus cardiac catheterization for detection of pulmonary arteriovenous malformations AMERICAN JOURNAL OF CARDIOLOGY 2002; 89 (3): 281-285
Because clinical diagnosis of pulmonary arteriovenous malformations (AVMs) is difficult and cardiac catheterization invasive, contrast echo has been used to aid in the diagnosis. Correlation between contrast echo and catheterization data in these patients remains poorly defined. We examined the ability to detect pulmonary AVMs by contrast echocardiography, pulmonary angiography, and pulmonary vein saturations in patients who have undergone cavopulmonary (Glenn) shunts. Pulmonary vein saturations were obtained from catheterization reports. Blinded observers reviewed angiographic and contrast echo data retrospectively in 27 patients who had undergone a Glenn shunt and in 19 controls with biventricular hearts. Contrast echo was positive in 68 of 99 lungs (69%). Angiography showed AVMs in 65 of 98 lungs (66%). Pulmonary vein desaturation was found in 13 of 45 lungs sampled (29%). Only 10 of 38 lungs with positive contrast echo had pulmonary vein desaturation, but only 1 patient had pulmonary vein desaturation without positive contrast echo. The degree of desaturation did not correlate with severity of contrast echo return; 6 of 11 patients with 3+ contrast echo studies had normal pulmonary vein saturations. In a control group of patients with biventricular hearts, only 1 of 19 (5.3%) contrast echos was weakly positive. Contrast echo correlates poorly with angiography and pulmonary vein saturation for evaluation of AVMs. Contrast echo is extremely sensitive and often positive despite normal pulmonary vein saturation. Assessment of pulmonary vein desaturation in 100% oxygen may improve its sensitivity and correlation with contrast echo. Additional studies are needed to follow patients with mildly positive contrast echo studies to enable determination of their clinical relevance.
View details for Web of Science ID 000173624800006
View details for PubMedID 11809429
Survey of current practices in use of amiodarone and implantable cardioverter defibrillators in pediatric patients with end-stage heart failure AMERICAN JOURNAL OF CARDIOLOGY 2001; 88 (7): 809-?
Lipoprotein abnormalities are highly prevalent in pediatric heart transplant recipients. Pediatric transplantation 2000; 4 (3): 193-199
The role of hyperlipidemia in graft coronary artery disease (GCAD) is controversial although hyper-triglyceridemia is an independent risk factor. Recent studies show that 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) inhibitors decrease the incidence of GCAD in adults. The incidence of GCAD in pediatric patients is lower than in adults; it is not clear whether age-related differences in lipid metabolism account for some of this protection. This study was performed to: characterize the lipoprotein profile in children after heart transplantation; demonstrate that total cholesterol (TC) is a poor marker for underlying lipoprotein abnormalities; and to compare lipid abnormalities in patients who had been converted from cyclosporin A (CsA) to tacrolimus. Seventy-one determinations of fasting lipoprotein profiles were performed in a cohort of 28 children. Each child had at least two determinations on separate occasions. TC, low-density lipoprotein (LDL), and serum triglyceride (TG) levels were categorized as abnormal if greater than the 75th percentile for age and gender. A high-density lipoprotein (HDL) level less than the 25th percentile was considered abnormal. Immunosuppression included CsA or tacrolimus, azathioprine, and prednisone. We found that 90% of the patients studied had abnormalities of either TG or HDL. In contrast, LDL tended to be normal when adjusted for age and gender. TC was a poor indicator of any underlying abnormality in TG, LDL, or HDL. In patients converted to tacrolimus, no significant differences were found in the levels of TG, LDL or HDL compared with each patient's respective values while receiving CsA. Hence, lipoprotein abnormalities among pediatric heart transplant recipients are highly prevalent. TC is a poor screening tool in the evaluation for lipid abnormalities. Lipoprotein profiles remain statistically unchanged after conversion from CsA to tacrolimus.
View details for PubMedID 10933319
Outcome while awaiting heart transplantation in children: A comparison of congenital heart disease and cardiomyopathy JOURNAL OF HEART AND LUNG TRANSPLANTATION 2000; 19 (8): 751-755
Outcomes for children who undergo heart transplantation differ for children with congenital heart disease as compared to those with structurally normal hearts. Similar data have not been reported for these groups of patients for the morbidity and mortality associated with waiting for a donor. We report these data.A retrospective review was performed for all pediatric patients who were listed for heart transplantation at Stanford from 1977 to 1996, comparing mortality and major morbidity for patients with congenital heart disease and those with cardiomyopathy and structurally normal hearts.There were 96 patients who met study criteria, of whom 67 were successfully transplanted. The median waiting time was 23 days. Survival at 30 days was 93% and at 90 days was 81%, with no difference between groups. Major complications were identified in 38% of patients with structurally normal hearts, vs 9% of patients with congenital heart disease (p < 0.001).Overall mortality is similar for patients with congenital heart disease and those with structurally normal hearts while listed for heart transplantation, but patients with congenital heart disease have fewer episodes of major morbidity during this time.
View details for Web of Science ID 000089041400005
View details for PubMedID 10967268
Safety and utility of the routine surveillance biopsy in pediatric patients 2 years after heart transplantation JOURNAL OF PEDIATRICS 2000; 136 (2): 238-242
The standard for diagnosing allograft rejection after heart transplantation is the endomyocardial biopsy, but the value of routine surveillance biopsies after 2 years after transplant is controversial. The objective of this study was to determine the necessity and safety of surveillance biopsies and to correlate rejection with signs and symptoms beyond the second post-transplant anniversary in pediatric patients.We reviewed the results of 899 biopsies and coincident clinical histories in 56 pediatric patients, comprising 314 patient-years of follow-up. Patients were classified as having symptoms or not based on a blinded review of their clinical status and echocardiograms. Biopsies were classified as negative or positive with established criteria.After biopsies performed less than 2 years after transplant or as a follow-up for a positive biopsy were excluded, 481 biopsies were available for analysis, of which 20 (4%) were positive. Positive biopsies were found in 15 (3%) of 456 biopsies in patients without symptoms compared with 5 (20%) of 25 biopsies in patients with symptoms. Patients with symptoms were 6 times more likely to have a positive biopsy compared with patients without symptoms. Of the positive rejection episodes, 75% occurred in patients without symptoms.Although rejection is uncommon in pediatric patients greater than 2 years after transplant, episodes of treatable allograft rejection can occur in the absence of clinical signs and symptoms. This study emphasizes the safety of and the need to continue to perform routine surveillance biopsies in patients without symptoms, even after the second post-transplant year.
View details for Web of Science ID 000085289400023
View details for PubMedID 10657832
Brachial artery reactivity: A modified technique with applicability to children JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 1999; 12 (10): 850-852
Brachial artery reactivity noninvasively tests endothelial function. Cuff pressures of 180 to 300 mm Hg have been used. These pressures are ill advised in neonatal and premature infants. We therefore evaluated the use of substantially lower occlusive pressures. Fifteen adult subjects were enrolled. One arm was occluded to 200 mm Hg, and the other arm was occluded to 40 mm Hg above the resting systolic pressure. The occlusion time was 4.5 minutes. The brachial artery was measured at baseline and 1 minute after cuff deflation. Low pressure occlusions averaged 155.7 +/- 7.8 mm Hg versus 200 mm Hg for high pressure occlusions (P <.0001). One of 15 patients with low pressure occlusions reported discomfort versus 7 of 15 with high pressure occlusions (P <.05). Brachial artery reactivity increased by 8.0% +/- 2.5% with low pressure occlusions versus 7.5% +/- 2.7% with high pressure occlusions (P = not significant). Brachial artery reactivity can be assessed at lower occlusive pressures. This may extend the applicability of the technique to children and neonates.
View details for Web of Science ID 000083255200011
View details for PubMedID 10511654
QT dispersion predicts ventricular arrhythmia in pediatric cardiomyopathy patients referred for heart transplantation JOURNAL OF HEART AND LUNG TRANSPLANTATION 1999; 18 (8): 781-785
QT dispersion has been used in stratifying risk for sudden death in adults with dilated cardiomyopathy, but its role in the pediatric population has not been delineated.We reviewed electrocardiograms in pediatric patients with dilated cardiomyopathy referred for heart transplantation, to evaluate the role of QT dispersion in predicting malignant arrhythmias in these patients. Three groups were defined: Group I (n = 13) had dilated cardiomyopathy and malignant ventricular arrhythmias, Group II (n = 13) had dilated cardiomyopathy with no ventricular arrhythmias and Group III (n = 30) consisted of normals. QT dispersion was defined as the duration of the shortest QT subtracted from that of the longest. In addition, the standard deviation of the QT intervals was calculated for each ECG, using 12 leads.QT dispersion was significantly prolonged in Group I (97 +/- 33 msec) compared to Group II (74 +/- 19 msec) and Group III (42 +/- 17 msec). QT standard deviation was also prolonged in Group I (30 +/- 11 msec) vs Group II (22 +/- 5 msec) and Group III (13 +/- 4 msec). Using a threshold value of 90 msec for QT dispersion or 25 msec for QT standard deviation, a sensitivity of 78% and a specificity of 70% was obtained for identifying patients who would subsequently develop ventricular arrhythmias.In pediatric heart transplant candidates with dilated cardiomyopathy, QT dispersion and QT standard deviation identify patients at higher risk for the development of malignant ventricular arrhythmia. This simple test can be helpful in the evaluation and management of these patients awaiting transplantation.
View details for Web of Science ID 000082347500007
View details for PubMedID 10512525
Gender and age do not alter actuarial incidence of cardiac dysfunction following anthracycline chemotherapy of childhood malignancy NATURE PUBLISHING GROUP. 1999: 30A-30A
A new therapeutic approach to the fetus with congenital complete heart block: Preemptive, targeted therapy with dexamethasone OBSTETRICS AND GYNECOLOGY 1998; 92 (4): 689-691
Therapy of established congenital complete heart block in the fetus has resulted in improved survival but persistence of heart block. This exposes the infant to the morbidity of heart block, including the risk of sudden death and pacemaker implantation.A 35-year-old gravida 2, para 1, with Sjogren syndrome and a previous pregnancy complicated by congenital complete heart block presented during her second pregnancy. Intensive fetal monitoring with echocardiography was employed. Early evidence of myocardial dysfunction and dysrhythmia was found, dexamethasone therapy was initiated, and the dysfunction and dysrhythmia resolved. The pregnancy went to term without further complication.This represents a new and successful strategy to identify very early signs of myocardial disease in a fetus at high risk of congenital complete heart block, enabling targeted, preemptive therapy.
View details for Web of Science ID 000076159900022
View details for PubMedID 9764666
Arrhythmias and thromboembolic complications after the extracardiac Fontan operation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 1998; 115 (3): 499-505
Late morbidity and mortality after the Fontan operation are largely due to atrial arrhythmias, ventricular failure, and thrombus formation. The extracardiac Fontan procedure avoids extensive atrial manipulation and suture lines, theoretically minimizing the impetus for these events. We examined our experience with the extracardiac Fontan operation with particular attention to thromboembolism and arrhythmias.We retrospectively reviewed the medical and surgical records of all 16 patients who underwent an extracardiac Fontan operation between July 1993 and May 1996. Fifteen patients (94%) were in sinus rhythm before the operation. In the immediate postoperative period, seven (44%) had arrhythmias consisting of accelerated junctional rhythm and ectopic atrial rhythm. No associated hemodynamic compromise and no early deaths occurred. Patients were followed up for 3 to 34 months after the Fontan operation. Arrhythmias were detected in eight patients (50%) on surface electrocardiograms, and seven (44%) showed evidence of sinus node dysfunction on 24-hour Holter monitor studies. Thrombi were found in three patients (19%). All patients were asymptomatic, with no evidence of conduit obstruction by echocardiogram.The incidence of hemodynamically significant tachyarrhythmias appears to be reduced after the extracardiac Fontan operation. A significant percentage of patients have evidence of sinus node dysfunction, suggesting the presence of other surgical or nonsurgical factors responsible for this finding. Our incidence of thrombotic events is similar to previous reports with other Fontan modifications. It appears to be a reasonable option to maintain these patients on anticoagulation indefinitely.
View details for Web of Science ID 000072718800002
View details for PubMedID 9535435
THROMBOSIS OF THE PULMONARY-ARTERY STUMP AFTER DISTAL LIGATION JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 1995; 110 (5): 1563-1565
Thromboembolic complications after Fontan operations. Circulation 1995; 92 (9): II287-93
Despite the increasing recognition of thromboembolic complications of the Fontan procedure, data characterizing such events are limited. The total cavopulmonary connection is believed to be less prone to this complication than other modifications of Fontan operations. We examined our experience with thromboembolism after Fontan operations to better characterize these events and their relation to the type of Fontan operation performed.We retrospectively identified 70 patients who underwent a Fontan operation between January 1978 and March 1994. Patients were divided into three groups: (1) total cavopulmonary connection, (2) atriopulmonary connection, and (3) conduit interposition. Fourteen patients (20%) developed a thromboembolic complication during a mean (+/- SD) follow-up of 5.2 +/- 4.7 years. The rate of thrombosis was similar in each group. The time from Fontan operation to thrombosis averaged 6.1 +/- 5.0 years. The overall rate of thromboembolic events was 3.9 per 100 patient-years. Twelve of the 14 thrombi were located within the venous circulation, 1 was in the left ventricle, and the location of 1 was undetermined. Six of the patients (43%) were asymptomatic, 3 (21%) presented with cerebrovascular events, and 5 (36%) presented with other symptoms. Thromboembolic events occurred from the perioperative period to 15 years after surgery.Thromboembolic complications occur frequently after the Fontan operation and its modifications and are a cause of significant morbidity. The time of presentation varies greatly. The rate of thrombosis appears to be similar in all modifications of the Fontan operation.
View details for PubMedID 7586425
THROMBOEMBOLIC COMPLICATIONS AFTER FONTAN OPERATIONS CIRCULATION 1995; 92 (9): 287-293
MYOCARDIAL-INFARCTION IN THE PERINATAL-PERIOD SECONDARY TO MATERNAL COCAINE ABUSE - A CASE-REPORT AND LITERATURE-REVIEW ARCHIVES OF PEDIATRICS & ADOLESCENT MEDICINE 1994; 148 (10): 1092-1096
GENETIC-ASPECTS OF HEART-DISEASE IN THE NEWBORN SEMINARS IN PERINATOLOGY 1993; 17 (2): 61-75