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The ability to integrate echocardiographic for rheumatic heart disease (RHD) into RHD prevention programs is limited because of lack of financial and expert human resources in endemic areas. Task shifting to nonexperts is promising; but investigations into workforce composition and training schemes are needed. The objective of this study was to test nonexperts' ability to interpret RHD screening echocardiograms after a brief, standardized, computer-based training course. Six nonexperts completed a 3-week curriculum on image interpretation. Participant performance was tested in a school-screening environment in comparison to the reference approach (cardiologists, standard portable echocardiography machines, and 2012 World Heart Federation criteria). All participants successfully completed the curriculum, and feedback was universally positive. Screening was performed in 1,381 children (5 to 18 years, 60% female), with 397 (47 borderline RHD, 6 definite RHD, 336 normal, and 8 other) referred for handheld echo. Overall sensitivity of the simplified approach was 83% (95% CI 76% to 89%), with an overall specificity of 85% (95% CI 82% to 87%). The most common reasons for false-negative screens (n = 16) were missed mitral regurgitation (MR; 44%) and MR ≤1.5 cm (29%). The most common reasons for false-positive screens (n = 179) included identification of erroneous color jets (25%), incorrect MR measurement (24%), and appropriate application of simplified guidelines (39.4%). In conclusion, a short, independent computer-based curriculum can be successfully used to train a heterogeneous group of nonexperts to interpret RHD screening echocardiograms. This approach helps address prohibitive financial and workforce barriers to widespread RHD screening.

Objectives Handheld echocardiography (HAND) has good sensitivity and specificity for rheumatic heart disease (RHD) when performed by cardiologists. However, physician shortages in RHD-endemic areas demand less-skilled users to make RHD screening practical. We examine nurse performance and interpretation of HAND using a simplified approach for RHD screening. Methods Two nurses received training on HAND and a simplified screening approach. Consented students at two schools in Uganda were eligible for participation. A simplified approach (HAND performed and interpreted by a non-expert) was compared with the reference standard (standard portable echocardiography, performed and interpreted by experts according to the 2012 World Heart Federation guidelines). Reasons for false-positive and false-negative HAND studies were identified. Results A total of 1002 children were consented, with 956 (11.1 years, 41.8% male) having complete data for review. Diagnoses included: 913 (95.5%) children were classified normal, 32 (3.3%) borderline RHD and 11 (1.2%) definite RHD. The simplified approach had a sensitivity of 74.4% (58.8% to 86.5%) and a specificity of 78.8% (76.0% to 81.4%) for any RHD (borderline and definite). Sensitivity improved to 90.9% (58.7% to 98.5%) for definite RHD. Identification and measurement of erroneous colour jets was the most common reason for false-positive studies (n=164/194), while missed mitral regurgitation and shorter regurgitant jet lengths with HAND were the most common reasons for false-negative studies (n=10/11). Conclusions Non-expert-led HAND screening programmes offer a potential solution to financial and workforce barriers that limit widespread RHD screening. Nurses trained on HAND using a simplified approach had reasonable sensitivity and specificity for RHD screening. Information on reasons for false-negative and false-positive screening studies should be used to inform future training protocols, which could lead to improved screening performance.

Background Using 2012 World Heart Federation criteria, standard portable echocardiography (STAND) reveals a high burden of rheumatic heart disease (RHD) in resource-poor settings, but widespread screening is limited by cost and physician availability. Handheld echocardiography (HAND) may decrease costs, but World Heart Federation criteria are complicated for rapid field screening, particularly for nonphysician screeners. The aim of this study was to determine the best simplified screening strategy for RHD detection using HAND. Methods In this prospective study, STAND (GE Vivid q or i or Philips CX-50) was performed in five schools in Gulu, Uganda; a random subset plus all children with detectable mitral regurgitation or aortic insufficiency also underwent HAND (GE Vscan). Borderline or definite RHD cases were defined by 2012 World Heart Federation criteria on STAND images, by two experienced readers. HAND studies were reviewed by cardiologists blinded to STAND results. Single and combined HAND parameters were evaluated to determine the simplified screening strategy that maximized sensitivity and specificity for case detection. Results In 1,439 children (mean age, 10.8 ± 2.6 years; 47% male) with HAND and STAND studies, morphologic criteria and the presence of any mitral regurgitation by HAND had poor specificity. The presence of aortic insufficiency was specific but not sensitive. Combined criteria of mitral regurgitation jet length ≥ 1.5 cm or any aortic insufficiency best balanced sensitivity (73.3%) and specificity (82.4%), with excellent sensitivity for definite RHD (97.9%). With a prevalence of 4% and subsequent STAND screening of positive HAND studies, this would reduce STAND studies by 80% from a STAND-based screening strategy. Conclusions In resource-limited settings, HAND with simplified criteria can detect RHD with good sensitivity and specificity and decrease the need for standard echocardiography. Further study is needed to validate screening by local practitioners and long-term outcomes.

Background Considering the limited accuracy of clinical examination for early diagnosis of rheumatic heart disease ( RHD ), echocardiography has emerged as an important epidemiological tool. The ideal setting for screening is yet to be defined. We aimed to evaluate the prevalence and pattern of latent RHD in schoolchildren (aged 5–18 years) and to compare effectiveness of screening between public schools, private schools, and primary care centers in Minas Gerais, Brazil. Methods and Results The PROVAR (Rheumatic Valve Disease Screening Program) study uses nonexperts and portable and handheld devices for RHD echocardiographic screening, with remote interpretation by telemedicine, according to the 2012 World Heart Federation criteria. Compliance with study consent and prevalence were compared between different screening settings, and variables associated with RHD were analyzed. In 26 months, 12 048 students were screened in 52 public schools (n=10 901), 2 private schools (n=589), and 3 primary care centers (n=558). Median age was 12.9 years, and 55.4% were girls. Overall RHD prevalence was 4.0% borderline (n=486) and 0.5% definite (n=63), with statistically similar rates between public schools (4.6%), private schools (3.5%), and primary care centers (4.8%) ( P =0.24). The percentage of informed consents signed was higher in primary care centers (84.4%) and private schools (66.9%) compared with public schools (38.7%) ( P P P =0.02). Only age (odds ratio, 1.12; 95% confidence interval, 1.09–1.17; P RHD . Conclusions RHD screening in primary care centers seems to achieve higher coverage rates. Prevalence among schoolchildren is significantly high, with rates higher than expected in private schools of high‐income areas. These data are important for the formulation of public policies to confront RHD .

Thrombosis has long been recognized as a potentially life-threatening complication in children with congenital heart disease (CHD), children with acquired heart disease, and in adults with CHD. High-risk groups include patients with shunt- dependent single ventricles (shunt thrombosis, 8%–12%; 4%

Previous single-center studies have shown that telemedicine improves care in newborns with suspected heart disease. The aim of this study was to test the hypothesis that telemedicine would shorten time to diagnosis, prevent unnecessary transports, reduce length of stay, and decrease exposure to invasive treatments.Nine pediatric cardiology centers entered data prospectively on patients aged6 weeks, matched by gestational age, weight, and diagnosis. Subjects born at hospitals with and without access to telemedicine constituted the study group and control groups, respectively. Data from patients with mild or no heart disease were analyzed.Data were obtained for 337 matched pairs with mild or no heart disease. Transport to a tertiary care center (4% [n = 15] vs 10% [n = 32], P = .01), mean time to diagnosis (100 vs 147 min, P.001), mean length of stay (1.0 vs 26 days, P = .005) and length of intensive care unit stay (0.96 vs 2.5 days, P = .024) were significantly less in the telemedicine group. Telemedicine patients were significantly farther from tertiary care hospitals than control subjects. The use of inotropic support and indomethacin was significantly less in the telemedicine group. By multivariate analysis, telemedicine patients were less likely to be transported (odds ratio, 0.44; 95% confidence interval, 0.23-0.83) and less likely to be placed on inotropic support (odds ratio, 0.16; 95% confidence interval, 0.10-0.28).Telemedicine shortened the time to diagnosis and significantly decreased the need for transport of infants with mild or no heart disease. The length of hospitalization and intensive care stay and use of indomethacin and inotropic support were less in telemedicine patients.

Advancements in technology and broadband have revolutionized the current practice of medicine. The field of pediatric cardiology is no exception given the need for prompt diagnosis and reliance on cardiac imaging to identify infants and children with potentially life-threatening cardiovascular disease. As the relationship between telemedicine and pediatric cardiology has advanced, it has created a need to develop a broad, comprehensive document reviewing all the various aspects of telemedicine in pediatric cardiology. For more than a decade, a significant body of literature has been published describing individual experiences and practices, yet there remains no comprehensive statement or document summarizing this rapidly advancing field. In an effort to describe the collective experience and to provide structure and guidance for pediatric cardiology practitioners and healthcare providers, we have developed a scientific statement on the use of telemedicine in pediatric cardiology. Specific areas explored in this document include both neonatal and fetal tele-echocardiography, implications for training community sonographers, pulse oximetry programs, qualitative improvement and appropriate use criteria initiatives, and remote electrophysiological monitoring. This document also includes teleconsultation and teleausculation, direct-to-consumer and home monitoring programs, and a look into the use of telemedicine and pediatric cardiology in the intensive care setting. Furthermore, a detailed review of the legislative, public policy, and legal aspects of telemedicine is provided, along with financial and reimbursement information. Several terms are used in the literature interchangeably; a brief explanation is provided to help readers of this document. The term telehealth is defined as the use of technology to bridge distances in any aspect of medicine; telemedicine is the specific application of technology to conduct clinical medicine at a distance. The term telecardiology is defined as the broad application of telemedicine in the field of cardiology specifically, and tele-echocardiography is the most common application used within this field. Echocardiography is …

In a 2012 meeting at the Centers for Disease Control and Prevention (CDC), key experts and stakeholders identified public health knowledge gaps about congenital heart defects (CHDs), namely prevalence of CHDs across the life span, long‐term outcomes of persons with CHDs, and health services

Background At our institution, the arterial switch operation for transposition of the great arteries has transitioned from the Gore-Tex patch (W.L. Gore & Associates, Flagstaff, AZ) for pulmonary artery reconstruction to redundant pantaloon pericardial patch (RPPP). The (U-shaped) coronary artery button was used for coronary reimplantation. This study investigates overall mortality and factors for neopulmonary artery, neoaortic, and coronary artery surgical reintervention. Methods We performed a retrospective chart review of all patients who underwent arterial switch between 1983 and 2007. Our surgical database, operative reports, and cardiology clinic charts were reviewed. Time to event was plotted as Kaplan-Meier curves. Predictors of time-to-event were examined using Cox proportional hazard modeling. Results A total of 258 patients underwent arterial switch during the study. Mortality declined from 15% (era I: 1983 to 1990) to 11% (era II: 1991 to 1998) to 7% (era III: 1999 to 2007). Era III had a significantly later time to death compared with era I (hazard ratio [HR] 0.62, p = 0.04). The RPPP had a lower neopulmonary artery reintervention rate compared with Gore-Tex; 9 of 225 (4%) versus 3 of 21 (14%), p = 0.008. Complex anatomy increased risk for neopulmonary reintervention (HR 3.3, p = 0.03). Surgical reintervention rate for coronary arteries was 2%. Complex coronary anatomy (HR 17.9, p = 0.01) predicted coronary reintervention. Predictors of neoaortic reintervention were prior pulmonary artery band (HR 4.3, p = 0.03), complex anatomy (HR 3.5, p = 0.01), and coronary artery anatomy (HR 3.5, p = 0.04). Conclusions Arterial switch operation mortality has decreased. Conversion to RPPP reduced neopulmonary artery reintervention. The (U-shaped) coronary artery button technique is associated with low coronary reintervention rates. Complex coronary anatomy increases coronary and aortic reintervention. Prior pulmonary artery banding and complex anatomy increase aortic reintervention.

Background— We investigated the effect of polymorphisms in the renin-angiotensin-aldosterone system (RAAS) genes on ventricular remodeling, growth, renal function, and response to enalapril in infants with single ventricle. Methods and Results— Single ventricle infants enrolled in a randomized trial of enalapril were genotyped for polymorphisms in 5 genes: angiotensinogen, angiotensin-converting enzyme, angiotensin II type 1 receptor, aldosterone synthase, and chymase. Alleles associated with renin-angiotensin-aldosterone system upregulation were classified as risk alleles. Ventricular mass, volume, somatic growth, renal function using estimated glomerular filtration rate, and response to enalapril were compared between patients with ≥2 homozygous risk genotypes (high risk), and those with P P Conclusions— Renin-angiotensin-aldosterone system–upregulation genotypes were associated with failure of reverse remodeling after SCPC surgery, less improvement in renal function, and impaired somatic growth, the latter especially in patients receiving enalapril. Renin-angiotensin-aldosterone system genotype may identify a high-risk subgroup of single ventricle patients who fail to fully benefit from volume-unloading surgery. Follow-up is warranted to assess long-term impact. Clinical Trial Registration— http://www.clinicaltrials.gov . Unique identifier: NCT00113087.

ObjectivesThe purpose of this analysis was to assess preoperative risk factors before the first-stage Norwood procedure in infants with hypoplastic left heart syndrome and related single-ventricle lesions and to evaluate practice patterns in prenatal diagnosis, as well as the role of prenatal diagnosis in outcome.MethodsData from all live births with morphologic single right ventricle and systemic outflow obstruction screened for the Pediatric Heart Network's Single Ventricle Reconstruction Trial were used to investigate prenatal diagnosis and preoperative risk factors. Demographics, gestational age, prenatal diagnosis status, presence of major extracardiac congenital abnormalities, and preoperative mortality rates were recorded.ResultsOf 906 infants, 677 (75%) had prenatal diagnosis, 15% were preterm (

In adults without congenital heart disease, B-type natriuretic peptide (BNP) has been shown to be a very sensitive and specific marker of heart failure. The utility of BNP as a marker of clinical heart failure in children with a ventricular septal defect (VSD) has yet to be determined. A prospective, observational study evaluated BNP levels and other measures of heart failure. Eligible patients were

This review includes a brief discussion, from the perspective of the pediatric cardiologist, of the rationale for creation and maintenance of multi-institutional databases of outcomes of the treatment of patients with congenital and paediatric cardiac disease, together with a history of the evolution of such databases, and a description of the current state of the art. A number of projects designed to have broad-based impact are currently in the design phase, or have already been implemented. Not surprisingly, most of the efforts thus far have focused on catheterization procedures and interventions, although some work examining other aspects of paediatric cardiology practice is also beginning. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiology including the Central Cardiac Audit Database of the United Kingdom, national database initiatives for pediatric cardiology in Switzerland and Germany, various database initiatives under the leadership of the Working Groups of The Association for European Paediatric Cardiology, the IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry® of The American College of Cardiology Foundation® and The Society for Cardiovascular Angiography and Interventions (SCAI), the Mid-Atlantic Group of Interventional Cardiology (MAGIC) Catheterization Outcomes Project, the Congenital Cardiac Catheterization Project on Outcomes (C3PO), the Congenital Cardiovascular Interventional Study Consortium (CCISC), and the Joint Council on Congenital Heart Disease (JCCHD) National Quality Improvement Initiative. These projects, each leveraging multicentre data and collaboration, demonstrate the enormous progress that has occurred over the last several years to improve the quality and consistency of information about nonsurgical treatment for congenital cardiac disease. The paediatric cardiology field is well-poised to move quickly beyond outcome assessment and benchmarking, to collaborative quality improvement.

The purpose of this study was to compare the modified single-patch technique to the two-patch technique for infants with complete atrioventricular canal (CAVC) defects.Between January 2000 and June 2006, 55 infants underwent CAVC repair. Twenty-six patients had a modified single-patch technique; 29 patients had a two-patch technique. Trisomy 21 was present in 23 of 26 and 26 of 29 patients (p = not significant [ns]). Mean age was 4.4 +/- 1.3 months (single-patch) versus 5.5 +/- 1.9 months (two-patch, p0.02). Mean weight was 4.74 +/- 0.92 versus 5.28 +/- 1.67 kilograms (p = ns).There was one death in the modified single-patch group (postoperative day 130, liver failure) and no deaths in the two-patch group. Cross-clamp times and cardiopulmonary bypass times were shorter in the modified single-patch group (97.3 +/- 19.9 vs 123.3 +/- 28.2 minutes, p0.0003; 128 +/- 25 vs 157 +/- 37, p0.03). Rastelli classification was type A (18 vs 14), B (1 vs 0), and C (7 vs 15). Mean size of the ventricular septal defect as assessed by transesophageal echocardiogram was 9 +/- 2 mm, (single-patch) versus 10 +/- 3 mm (two-patch) (p = ns). Median postoperative length of stay did not differ (10 vs 8 days). There was no difference in the degree of postoperative left or right AV valve insufficiency as assessed by serial echocardiography. One patient (4%) required reoperation for mitral insufficiency in the modified single-patch versus three patients in the two-patch group (10%, p = ns). There were no patients with third degree atrioventricular block or that required reoperation for residual VSD in the modified single-patch group. There was one patient with third-degree AV block that required a pacemaker and one patient who had reoperation for a residual ventricular septal defect in the two-patch group (p = ns). No patient in either group required reoperation for left ventricular outflow tract obstruction.The modified single-patch technique produced results comparable with the two-patch technique in younger patients with similarly sized ventricular septal defects. Furthermore, the modified single-patch technique was performed with significantly shorter cross-clamp and cardiopulmonary bypass times.

To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan.Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age19 years) or Short Form Health Survey (age ≥ 19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined.Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL (P.001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points (P = .004) and the emotional functioning score decreased by an average of 0.64 points (P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire (P.001) and Short Form Health Survey scores (P.001).Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood.ClinicalTrials.gov: NCT00132782.

Objective Calcific deposits develop in 20–40% of children with juvenile dermatomyositis (juvenile DM), contributing to disease morbidity and mortality. This study was undertaken to define the structure and composition of these deposits and to characterize their association with chronic inflammation. Methods We examined calcific deposits from 5 children with juvenile DM (2 boys and 3 girls). The crystal structure and mineral content of the deposits was analyzed by x-ray diffraction, Fourier transform infrared spectroscopy, and imaging. The protein content of the deposits, following solubilization, was assayed by Western blotting. Results All 5 children had both a young age at disease onset (mean ± SD 3.3 ± 1.9 years) and, despite therapy, persistent cutaneous inflammation (mean ± SD duration 81.3 ± 58.7 months). The bone proteins, osteopontin, osteonectin, and bone sialoprotein, were identified in the protein extracts; the only mineral detected was hydroxyapatite, but the tissue was distinct from bone, with an extremely high mineral content and an irregular distribution of mineral. Conclusion These results indicate that chronic cutaneous inflammation may contribute to the formation of hydroxyapatite-containing pathologic calcifications in children with juvenile DM.

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives. The Working Group reviewed data on the epidemiology of ACHD, long-term outcomes of complex cardiovascular malformations, issues in assessing morphology and function with current imaging techniques, surgical and catheter-based interventions, management of related conditions including pregnancy and arrhythmias, quality of life, and informatics. After research and training barriers were discussed, the Working Group recommended outreach and educational programs for adults with congenital heart disease, a network of specialized adult congenital heart disease regional centers, technology development to support advances in imaging and modeling of abnormal structure and function, and a consensus on appropriate training for physicians to provide care for adults with congenital heart disease.

Transesophageal echocardiography is more sensitive than transthoracic echocardiography (TTE) in detecting endocarditis in adults; however, pediatric data are limited. Retrospectively, 39 subjects with endocarditis were divided into adult-sized (≥60 kg) and pediatric-sized (

BACKGROUND: Rheumatic heart disease (RHD) remains a major public health concern in developing countries, and routine screening has the potential to improve outcomes. Standard portable echocardiography (STAND) is far more sensitive than auscultation for the detection of RHD but remains cost-prohibitive in resource-limited settings. Handheld echocardiography (HAND) is a lower-cost alternative. The purpose of this study was to assess the incremental value of HAND over auscultation to identify RHD. METHODS: RHD screening was completed for schoolchildren in Gulu, Uganda, by using STAND performed by experienced echocardiographers. Any child with mitral or aortic regurgitation or stenosis plus a randomly selected group of children with normal STAND findings underwent HAND and auscultation. STAND and HAND studies were interpreted by 6 experienced cardiologists using the 2012 World Heart Federation criteria. Sensitivity and specificity of HAND and auscultation for the detection of RHD and pathologic mitral or aortic regurgitation were calculated by using STAND as the gold standard. RESULTS: Of 4773 children who underwent screening with STAND, a subgroup of 1317 children underwent HAND and auscultation. Auscultation had uniformly poor sensitivity for the detection of RHD or valve disease. Sensitivity was significantly improved by using HAND compared with auscultation for the detection of definite RHD (97.8% vs 22.2%), borderline or definite RHD (78.4% vs 16.4%), and pathologic aortic insufficiency (81.8% vs 13.6%). CONCLUSIONS: Auscultation alone is a poor screening test for RHD. HAND significantly improves detection of RHD and may be a cost-effective screening strategy for RHD in resource-limited settings.

Background: Patients with significant overcirculation from large ventricular septal defects (VSDs) may have altered contractility compared to normal patients. Methods: Patients were enrolled prospectively. Patients were excluded if they were acutely ill or over age five. Left ventricular longitudinal strain and strain rate were measured in patients with VSDs referred for surgical correction and control participants with structurally normal hearts. Results: There was no difference in the average longitudinal strain or strain rate of six segments in the left ventricle. The mean for both the groups was 19.9% ( P = .95). Strain rate was 1.5 s−1 in the patients with a VSD and 1.42 s−1 in the control group ( P = .30). The left ventricular end diastolic dimension was significantly larger in the group with ventricular defects (z score 1.38 vs −0.37) than the control group ( P = .000). Conclusions: The left ventricle end-diastolic dimension in patients with ventricular defects was significantly larger, but there was no difference in longitudinal strain or strain rate.

ObjectivesThe technical performance score (TPS) has been reported in a single center study to predict the outcomes after congenital cardiac surgery. We sought to determine the association of the TPS with outcomes in patients undergoing the Norwood procedure in the Single Ventricle Reconstruction trial.MethodsWe calculated the TPS (class 1, optimal; class 2, adequate; class 3, inadequate) according to the predischarge echocardiograms analyzed in a core laboratory and unplanned reinterventions that occurred before discharge from the Norwood hospitalization. Multivariable regression examined the association of the TPS with interval to first extubation, Norwood length of stay, death or transplantation, unplanned postdischarge reinterventions, and neurodevelopment at 14 months old.ResultsOf 549 patients undergoing a Norwood procedure, 356 (65%) had an echocardiogram adequate to assess atrial septal restriction or arch obstruction or an unplanned reintervention, enabling calculation of the TPS. On multivariable regression, adjusting for preoperative variables, a better TPS was an independent predictor of a shorter interval to first extubation (P = .019), better transplant-free survival before Norwood discharge (P

To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth before stage II.From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed.Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only (n = 57). There was significant variation in feeding mode among sites (oral only 0%-81% and G-tube only 0%-56%, P.01). After adjusting for site, multivariable modeling showed G-tube feeding at discharge was associated with longer hospitalization, and N-tube feeding was associated with greater number of discharge medications (R(2) = 0.65, P.01). After adjusting for site, mean pre-stage II weight-for-age z-score was significantly higher in the oral-only group (-1.4) vs the N-tube-only (-2.2) and G-tube-only (-2.1) groups (P = .04 and .02, respectively).Feeding mode at hospital discharge after the Norwood procedure varied among sites. Prolonged hospitalization and greater number of medications at the time of discharge were associated with tube feeding. Infants exclusively fed orally had a higher weight-for-age z score pre-stage II than those fed exclusively by tube. Exploring strategies to prevent morbidities and promote oral feeding in this highest risk population is warranted.

To study the feasibility of transmitting full-length diagnostic-quality echocardiograms with video conferencing systems linked by ISDN.Forty-three previously video-recorded full-length echocardiograms (22 complete two-dimensional Doppler studies, 21 separate M-mode studies) were transmitted from a site 30 miles distant using desktop video conferencing systems linked with one ISDN line (128 kbps). Blinded interpretations of the original recorded and transmitted studies were made by two pediatric cardiologists (two-dimensional Doppler) and a pediatric sonographer (M-mode).Diagnoses of normal (N = 12) and abnormal (N = 10) conditions were made from the original tapes; the same interpretations were made from the transmitted studies. Twenty-one specific abnormalities in the 10 abnormal studies were seen on both original and transmitted videotapes. These abnormalities ranged from simple congenital heart defects, including ventricular septal defect, atrial septal defect, and patent ductus arteriosus, to complex ones, such as coronary artery fistula, double-outlet right ventricle, and complete atrioventricular canal. Qualitatively, there was mild degradation in gray scale, brightness, and contrast of the images. Six M-mode variables from the transmitted images had excellent concordance with the original tracings (P = 0.506 to 0.838; r = 0.86 to 0.97).Although our sample size was small, this preliminary experience indicates that video conferencing equipment utilizing ISDN technology is a reliable method for transmitting full-length diagnostic-quality echocardiographic studies from remote sites. This technology may have a significant impact on the management of pediatric patients with suspected heart disease. Further evaluation is warranted.

Background The development of coronary aneurysms as sequelae of Kawasaki disease can result in myocardial ischemia, infarction, and sudden death. Traditionally, these patients have undergone coronary angiography and nuclear stress imaging for risk stratification and follow-up. However, angiography is invasive, and both modalities expose the patient to repeated radiation, which is an important issue in children. The purpose of this study was to determine the feasibility of performing exercise stress echocardiography in children diagnosed with coronary abnormalities secondary to Kawasaki disease. Methods and Results Treadmill exercise stress echocardiographic studies were performed in 28 children ages 6 to 16 years. All had acute Kawasaki disease 1 to 10 years before study, and coronary artery abnormalities were identified during previous echocardiographic imaging. Patients were exercised using a standard Bruce protocol. Transthoracic echocardiographic images, obtained in the parasternal long, short, apical two- and four-chamber views immediately before and after exercise, were digitized for review and analysis. In baseline studies before exercise, wall motion abnormalities were identified in 2 patients; these segments became normal with exercise. Two patients developed new exercise-induced wall motion abnormalities that corresponded to angiographically defined critical stenosis of the left anterior descending coronary artery. No patients had resting or exercise-induced ECG evidence of ischemia. There were no adverse reactions, and 26 of 28 patients had normal exercise tolerance. Conclusions Among patients with coronary artery involvement resulting from Kawasaki disease, exercise stress echocardiography is a safe, noninvasive procedure and may identify children with myocardial ischemia that was not detected with ECG stress test alone.

ObjectivesWe sought to identify risk factors for mortality and morbidity during the Norwood hospitalization in newborn infants with hypoplastic left heart syndrome and other single right ventricle anomalies enrolled in the Single Ventricle Reconstruction trial.MethodsPotential predictors for outcome included patient- and procedure-related variables and center volume and surgeon volume. Outcome variables occurring during the Norwood procedure and before hospital discharge or stage II procedure included mortality, end-organ complications, length of ventilation, and hospital length of stay. Univariate and multivariable Cox regression analyses were performed with bootstrapping to estimate reliability for mortality.ResultsAnalysis included 549 subjects prospectively enrolled from 15 centers; 30-day and hospital mortality were 11.5% (63/549) and 16.0% (88/549), respectively. Independent risk factors for both 30-day and hospital mortality included lower birth weight, genetic abnormality, extracorporeal membrane oxygenation (ECMO) and open sternum on the day of the Norwood procedure. In addition, longer duration of deep hypothermic circulatory arrest was a risk factor for 30-day mortality. Shunt type at the end of the Norwood procedure was not a significant risk factor for 30-day or hospital mortality. Independent risk factors for postoperative renal failure (n=46), sepsis (n=93), increased length of ventilation, and hospital length of stay among survivors included genetic abnormality, lower center/surgeon volume, open sternum, and post-Norwood operations.ConclusionsInnate patient factors, ECMO, open sternum, and lower center/surgeon volume are important risk factors for postoperative mortality and/or morbidity during the Norwood hospitalization.

Objectives In the Single Ventricle Reconstruction trial, infants undergoing the Norwood procedure were randomly allocated to undergo a right ventricle-to-pulmonary artery shunt or a modified Blalock-Taussig shunt. Apart from shunt type, subjects received the local standard of care. We evaluated variation in perioperative care during the Norwood hospitalization across 14 trial sites. Methods Data on preoperative, operative, and postoperative variables for 546 enrolled subjects who underwent the Norwood procedure were collected prospectively on standardized case report forms, and variation across the centers was described. Results Gestational age, birth weight, and proportion with hypoplastic left heart syndrome were similar across sites. In contrast, all recorded variables related to preoperative care varied across centers, including fetal diagnosis (range, 55%-85%), preoperative intubation (range, 29%-91%), and enteral feeding. Perioperative and operative factors were also variable across sites, including median total support time (range, 74-189 minutes) and other perfusion variables, arch reconstruction technique, intraoperative medication use, and use of modified ultrafiltration (range, 48%-100%). Additional variation across centers was seen in variables related to postoperative care, including proportion with an open sternum (range, 35%-100%), median intensive care unit stay (range, 9-44 days), type of feeding at discharge, and enrollment in a home monitoring program (range, 1%-100%; 5 sites did not have a program). Overall, in-hospital death or transplant occurred in 18% (range across sites, 7%-39%). Conclusions Perioperative care during the Norwood hospitalization varies across centers. Further analysis evaluating the underlying causes and relationship of this variation to outcome is needed to inform future studies and quality improvement efforts.

Background— Angiotensin-converting enzyme inhibitor therapy improves clinical outcome and ventricular function in adults with heart failure. Infants with single-ventricle physiology have poor growth and are at risk for abnormalities in ventricular systolic and diastolic function. The ability of angiotensin-converting enzyme inhibitor therapy to preserve ventricular function and improve somatic growth and outcomes in these infants is unknown. Methods and Results— The Pediatric Heart Network conducted a double-blind trial involving 230 infants with single-ventricle physiology randomized to receive enalapril (target dose 0.4 mg · kg −1 · d −1 ) or placebo who were followed up until 14 months of age. The primary end point was weight-for-age z score at 14 months. The primary analysis was intention to treat. A total of 185 infants completed the study. There were 24 and 21 withdrawals or deaths in the enalapril and placebo groups, respectively ( P =0.74). Weight-for-age z score was not different between the enalapril and placebo groups (mean±SE −0.62±0.13 versus −0.42±0.13, P =0.28). There were no significant group differences in height-for-age z score, Ross heart failure class, brain natriuretic peptide concentration, Bayley scores of infant development, or ventricular ejection fraction. The incidence of death or transplantation was 13% and did not differ between groups. Serious adverse events occurred in 88 patients in the enalapril group and 87 in the placebo group. Conclusions— Administration of enalapril to infants with single-ventricle physiology in the first year of life did not improve somatic growth, ventricular function, or heart failure severity. The results of this randomized trial do not support the routine use of enalapril in this population. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00113087.

Background Angiotensin converting enzyme (ACE) inhibitors are known to improve clinical outcome and ventricular function in adults with heart failure. Infants with single-ventricle physiology show abnormalities in ventricular function as well as poor growth. The ability of an ACE inhibitor to preserve ventricular function and improve growth in these infants is unknown. Methods The Pediatric Heart Network designed a randomized, double-blind trial to compare outcomes in infants with single-ventricle physiology receiving enalapril or placebo. Neonates ≤45 days old were eligible. The primary outcome is weight-for-age Z -score at 14 months of age. Secondary outcomes include other measures of somatic growth, laboratory and functional measures of heart failure, developmental indices, measures of ventricular size and function, and the relationship of the renin-angiotensin-aldosterone system genotype to the response to enalapril. The incidence and spectrum of adverse events will also be compared between treatment groups. Results A total of 1,245 neonates were screened and 533 (43%) were eligible. The consent rate was 43%; 230 subjects were enrolled. Parental reluctance to participate was the primary reason for non-consent in 79% of the eligible nonconsenting patients. Randomized patients were older, more likely to be male, and more likely to have hypoplastic left heart syndrome than the eligible patients who did not enroll. Conclusions The results of this randomized trial will make an important contribution to the management of infants with single-ventricle physiology by determining whether initiation of ACE inhibition therapy in the neonatal period improves growth, clinical outcome, and ventricular function.

Over the past decade, concerns have been raised regarding the safety of a variety of psychotropic medications in children and adolescents, the appropriate selection of patients for therapy, and the indications for cardiovascular monitoring. In 1999, concerns over potential cardiovascular effects of psychotropic drugs, especially tricyclic antidepressants1,2 but including stimulants, prompted the American Heart Association (AHA) scientific statement “Cardiovascular Monitoring of Children and Adolescents Receiving Psychotropic Drugs.”3 At that time, no specific cardiovascular monitoring was recommended for the use of stimulant medications. Since that time, a constellation of circumstances have come together, necessitating a second look at this complicated issue. These circumstances include an increased awareness of the presence of attention deficit/hyperactivity disorder (ADHD) in the general population and in children with preexisting cardiac conditions; public concerns about the side effects and toxicities of medications, especially psychotropic medications in children; and regulatory factors and warnings issued by the US Food and Drug Administration (FDA) and by the pharmaceutical industry in response to the FDA. This writing group was convened in response to FDA concerns with regard to the safety of the ADHD drugs and with regard to the identification of children with underlying cardiovascular abnormalities. At a time when there is much discussion of the side effects of drugs and of the use of psychotropic drugs in children in the media and lay literature, it is particularly important for the medical profession to play a significant role in critically evaluating the use of stimulant medication in children, including those who may have undiagnosed heart disease and those who are known to have heart disease. The writing group for “Cardiovascular Monitoring of Children and Adolescents With Heart Disease Receiving Medications for Attention Deficit/Hyperactivity Disorder” reviewed the literature relevant to this topic since the last publication of the AHA scientific …

The intent of this review is to provide the clinician with a summary of what is currently known about the contribution of genetics to the origin of congenital heart disease. Techniques are discussed to evaluate children with heart disease for genetic alterations. Many of these techniques are now available on a clinical basis. Information on the genetic and clinical evaluation of children with cardiac disease is presented, and several tables have been constructed to aid the clinician in the assessment of children with different types of heart disease. Genetic algorithms for cardiac defects have been constructed and are available in an appendix. It is anticipated that this summary will update a wide range of medical personnel, including pediatric cardiologists and pediatricians, adult cardiologists, internists, obstetricians, nurses, and thoracic surgeons, about the genetic aspects of congenital heart disease and will encourage an interdisciplinary approach to the child and adult with congenital heart disease.

citation patterns in hypertrophic cardiomyopathy. Am Heart J 1981; 101:233-4. 7. Bharati S, Strassbert B, Bilitch M, Salibi H, Mandel W, Rosen MK, Lev M. Anatomic substrate for preexcitation in idiopathic myocardial hypertrophy with fibroelastosis of the left ventricle. Am J Cardiol 1981;48:47-58. 8. MacRae C, Ghaisas N, McGarry K, McKenna W, Seidman JG, Seidman CE. Familial hypertrophic cardiomyopathy with Wolff-ParkinsonWhite syndrome maps to a locus on chromosome 7q3. Circulation 1994; 90:I-25.

Plasma cell granulomas are lesions of uncertain histogenesis arising in a variety of locations, most commonly the lung. Treatment for these lesions is complete surgical excision if possible. Unresectable pulmonary lesions respond to oral corticosteroids and radiation therapy. We report the long-term outcome of two unusual pediatric cases of cardiac plasma cell granulomas originating within the right ventricle and posterior aspect of the left ventricle. The limited literature reports advocate surgical resection for this entity, with no discussion of alternative treatment strategies for unresectable lesions. We prospectively evaluated the response to postoperative oral steroid therapy, as complete surgical excision was not possible in either case. Sequential echocardiography demonstrated additional significant reduction in the size of the masses and the patients remain asymptomatic at 9 and 5.5 years follow-up, without evidence of obstruction or recurrence. Oral corticosteroids should be considered as a treatment option for any unresectable plasma cell granuloma.

The number of adults who have survived with congenital heart disease is increasing rapidly. Although prevalence studies have not been performed, estimates of expected numbers of people reaching adulthood with congenital heart disease (CHD) can now be made because birth prevalence has been studied and mortality rates have begun to stabilize. Birth prevalence for all forms of congenital heart disease detected in the first year of life is estimated at 8.1 per thousand live births on the basis of data from the Centers for Disease Control1; estimates for disease of sufficient severity to result in a catheterization, surgery, or death in the first year of life are 2.3 per thousand, using population-based data derived from the Pediatric Cardiac Care Consortium.2 In 1990, the National Center for Health Statistics reported the number of US live births to be 4 158 212.3 Age-specific mortality data were obtained from the complete life table for the US population4 and from population-based death rates during hospital admission using data from 6 states in 1992. Estimates of numbers of individuals reaching adulthood born in 1990 with congenital heart disease of sufficient severity to be diagnosed or treated during the first year of life are shown in the Figure. Disregarding all patients born before 1990 and those not diagnosed in the first year and assuming stable mortality in early adulthood, by 2020, nearly 760 000 individuals will have CHD, with 200 000 in the more severe subgroup. Number of children born after 1990 with congenital heart disease estimated to reach adulthood. □ indicates diagnosed in the first year of life; ⋄, undergoing catheterization or surgery in the first year of life. This striking increase in the estimated number of patients with congenital heart disease who will require competent care within the …

To describe neurodevelopmental outcomes in infants with single ventricle (SV) physiology and determine factors associated with worse outcomes.Neurodevelopmental outcomes for infants with SV enrolled in a multicenter drug trial were assessed at 14 months of age using the Bayley Scales of Infant Development-II. Multivariable regression analysis was used to identify factors associated with worse outcomes.Neurodevelopmental testing was performed at 14 ± 1 months in 170/185 subjects in the trial. Hypoplastic left heart syndrome was present in 59% and 75% had undergone the Norwood operation. Mean Psychomotor Developmental Index (PDI) and mental developmental index (MDI) were 80 ± 18 and 96 ± 14, respectively, (normal 100 ± 15, P.001 for each). Group-based trajectory analysis provided a 2-group model ("high" and "low") for height z-score trajectory and brain type natriuretic peptide (BNP) trajectory. The predicted PDI scores were 15 points higher in the "high" height z-score trajectory compared with the "low" cluster (P.001). A higher number of serious adverse events during the trial was associated with lower PDI scores (P = .02). The predicted MDI scores were 13-17 points lower in "low height trajectory-high BNP trajectory" group compared with the other 3 groups (P.001). MDI scores were also lower in subjects who required extracorporeal membrane oxygenation during the neonatal hospitalization (P = .01) or supplemental oxygen at discharge (P = .01).Neurodevelopmental outcome at 14 months of age is impaired in infants with SV physiology. Low height trajectory and high BNP trajectory were associated with worse neurodevelopmental outcomes. Efforts to improve nutritional status alone may not improve neurodevelopmental outcomes.

ObjectiveThe study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that might favor the modified Blalock–Taussig shunt or a right ventricle-to-pulmonary artery shunt.MethodsWe used competing risks methodology to analyze death without transplantation, cardiac transplantation, and survival without transplantation. Parametric time-to-event modeling and bootstrapping were used to identify independent predictors.ResultsData from 549 subjects (follow-up, 2.7 ± 0.9 years) were analyzed. Mortality risk was characterized by early and constant phases; transplant was characterized by only a constant phase. Early phase factors associated with death included lower socioeconomic status (P = .01), obstructed pulmonary venous return (P

To describe growth patterns in infants with single ventricle physiology and determine factors influencing growth.Data from 230 subjects enrolled in the Pediatric Heart Network Infant Single Ventricle Enalapril Trial were used to assess factors influencing change in weight-for-age z-score (z) from study enrollment (0.7 ± 0.4 months) to pre-superior cavopulmonary connection (SCPC; 5.1 ± 1.8 months, period 1) and pre-SCPC to final study visit (14.1 ± 0.9 months, period 2). Predictor variables included patient characteristics, feeding regimen, clinical center, and medical factors during neonatal (period 1) and SCPC hospitalizations (period 2). Univariate regression analysis was performed, followed by backward stepwise regression and bootstrapping reliability to inform a final multivariable model.Weights were available for 197 of 230 subjects for period 1 and 173 of 197 subjects for period 2. For period 1, greater gestational age, younger age at study enrollment, tube feeding at neonatal hospitalization discharge, and clinical center were associated with a greater negative z (poorer growth) in multivariable modeling (adjusted R(2) = 0.39, P.001). For period 2, younger age at SCPC and greater daily caloric intake were associated with greater positive z (better growth; R(2) = 0.10, P = .002).Aggressive nutritional support and earlier SCPC are modifiable factors associated with a favorable change in weight-for-age z-score.

Objectives . The aim of this study was to retrospectively evaluate the sensitivity of noninvasive surveillance (physical examination, echocardiography) of rejection in accurately predicting histologically documented rejection episodes. Additionally, the usefulness of routine scheduled biopsy and its safety in pediatric patients was explored. Background . Endomyocardial biopsy has been utilized as the standard for rejection surveillance after heart transplantation in adults, but its role in documenting clinically suspected rejection and in routine surveillance of pediatric patients has not been agreed upon. Methods . Heart transplantation was performed in 14 neonates and 21 children. The immunosuppressive regimen consisted of cyclosporine, azathioprine and prednisone. All patients underwent routine noninvasive rejection surveillance that included clinical examination and echocardiography. In the neonates, biopsy was performed quarterly beginning 6 months after transplantation, after cessation of prednisone therapy. In the children, biopsy was performed 15 times in the 1st year. A minimum of five biopsy samples were interpreted using the Working Formulation for Heart Transplant Rejection. Results . In the neonates, 37 biopsies were performed. Evidence of rejection was present in only three biopsy samples obtained during eight episodes (38%) of clinically suspected rejection. In 29 biopsies performed when rejection was not clinically suspected, each biopsy was free of cellular infiltrate. In the children, 291 biopsies were performed. Evidence of rejection was present in only seven biopsies (41%) from 17 episodes of clinically suspected rejection. Cellular rejection was discovered during routine rejection surveillance biopsies in asymptomatic patients in 23 (8.4%) of 274 biopsies. Conclusions . In neonates with clinically suspected rejection, endomyocardial biopsy identified which patients did not require rejection therapy. Endomyocardial biopsy surveillance did not detect any unsuspected cases of rejection. In children, noninvasive rejection surveillance was less reliable even in asymptomatic patients, suggesting that periodic endomyocardial biopsy should be utilized.

Quinidine syncope and factors associated with it are well known among adult patients treated for cardiac arrhythmias. To define factors that may influence the occurrence of syncope in children taking quinidine, the clinical, anatomic, electrocardiographic, roentgeno-graphic and pharmacologic data were compared in six patients with syncope (Group A) and 22 patients without syncope (Group B). There was a significant (chi-square = 10.2, p = 0.001) relation between heart disease and quinidine syncope: all six Group A (syncopal) patients had heart disease whereas 15 of the 22 Group B (non-syncopal) patients had no structural heart disease. In contrast, no significant difference was noted between Group A and Group B patients in mean age (11.4 versus 11.4 years), mean quinidine serum concentration (2.9 versus 2.3 μ/ml), mean corrected QT interval before quinidine (0.43 versus 0.40 second) or mean corrected QT interval during quinidine therapy (0.46 versus 0.46 second) or between those taking digitalis and those not. Two of the six Group A (syncopal) patients died during therapy, one 6 days after initiating therapy and one suddenly at home 6 months after beginning quinidine. Another two of the six Group A patients exhibited hypokalemia (both 2.9 mEq/liter) at the time of syncope, 2 weeks and 6 months, respectively, after initiation of quinidine therapy; both survived. Syncope occurred within 8 days of initiation of quinidine therapy in three of the six patients. Sustained ventricular tachycardia was observed during quinidine associated arrhythmia in three of six patients with syncope; nonsustained ventricular tachycardia or complex ventricular ectopic activity while on this therapy was observed before syncope in the other three patients in Group A. This experience indicates that quinidine-related syncope in children is more likely to occur in patients with structural heart disease, may be associated with hypokalemia, is a result of ventricular tachycardia and may be fatal. In the presence of heart disease, in-hospital observation during the first 14 days and maintenance of normokalemia is a prerequisite for quinidine therapy in children.

Congenital heart defects are the leading cause of infant mortality due to birth defects. In countries with low infant mortality rates, almost half of infant deaths are attributable to congenital heart defects directly or indirectly.1,2 Early studies in developed western countries have reported overall congenital heart defect prevalence estimates of 4 to 10 per 1000 live births3–5 and 2.6 to 4.4 per 1000 births for major defects,6–8 defined as defects requiring catheter or surgical intervention during the first year of life. The prevalence of congenital heart defects among fetuses is estimated to be even higher, at 14.6 per 1000 fetuses.9 Because complex congenital heart defects are common among fetuses10 and may result in spontaneous abortion or stillbirth, the total prevalence of congenital heart defects among stillbirths and live births may be higher than that reported among live births alone. The prevalence of congenital heart defects may also be underestimated because minor defects may be asymptomatic and undetected among neonates. More comprehensive surveillance among fetuses and neonates would improve prevalence estimates for congenital heart defects and help assess prevention efforts. With the wider use of prenatal and postnatal sonography to diagnose congenital heart defects, observed prevalence estimates have risen. Although sonographic investigations to detect congenital malformations are routinely performed between 13 and 22 weeks’ gestation in most European counties,11–13 routine sonography may still miss some defects, especially outflow tract defects and small septal defects.14 Specialized fetal or neonatal echocardiography is usually performed only when there is a risk of a congenital heart defect or it is suspected. Thus, the total prevalence of congenital heart defects in a general population is unclear regardless of medical indication. In most parts of China, sonographic screening for malformations was seldom used during routine prenatal health care until the 1990s. Thus, early congenital heart defect prevalence estimates of 2.7 to 6.6 per 1000 live births in China based on postnatal case ascertainment alone might be lower than those reported in some developed countries.15,16 Recently, hospital-based studies of prenatal congenital heart defect detection have been conducted in China,17–19 and 1 large population-based study, which performed echocardiography on fetuses and neonates suspected of having congenital heart defects, reported a prevalence rate of 8.2 per 1000 births.20 Given the recent increased use of fetal sonography in China, a surveillance program was initiated in 4 areas surrounding Shanghai to investigate the prevalence of congenital heart defects among all fetuses and neonates in a Chinese general population regardless of medical indication.