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Background: Risk assessment in heart transplantation is critical for candidate selection, but current models inadequately assess individual risk of postoperative mortality. We sought to identify risk factors and develop a scoring system to predict mortality after heart transplantation in children., Methods: The records of patients undergoing heart transplantation at our institution from 2010 through 2016 were reviewed. Clinical characteristics were recorded and compared between survivors and nonsurvivors. We used Cox proportional hazard modeling of factors associated with postoperative mortality to develop a risk factor score., Results: There were 74 patients who underwent heart transplantation at a mean age of 8.8 ± 6.6 years. Congenital heart disease was the most common indication, comprising 48.6% of the cohort. Overall mortality was 18.9%, with 10 of 14 dying within 30 days of the operation or during the initial postoperative admission (early mortality). Preoperative factors associated with overall mortality were single-ventricle congenital heart disease (hazard ratio [HR], 3.2; p = 0.042), biventricular assist device (HR, 4.8; p = 0.043), history of four or more sternotomies (HR, 3.9; p = 0.023), panel reactive antibody exceeding 10% (HR, 4.4; p = 0.013), any previous operation at another institution (HR, 3.2; p = 0.038), and pulmonary vein disease (HR, 4.7; p = 0.045). Each risk factor was assigned a point value, based on similar magnitude of the HRs. A score of 4 or higher predicted mortality with 57% sensitivity and 90% specificity., Conclusions: In this single-center pediatric cohort, postheart transplantation mortality could be predicted using patient-specific risk factors. The cumulative effect of these risk factors predicted mortality with high specificity., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Hypertrophic cardiomyopathy has a range of clinical severity in children. Treatment options are limited, mainly on account of small patient size. Disopyramide is a sodium channel blocker with negative inotropic properties that effectively reduces left ventricular outflow tract gradients in adults with hypertrophic cardiomyopathy, but its efficacy in children is uncertain. A retrospective chart review of patients ⩽21 years of age with hypertrophic cardiomyopathy at our institution and treated with disopyramide was performed. Left ventricular outflow tract Doppler gradients before and after disopyramide initiation were compared as the primary outcome measure. Nine patients received disopyramide, with a median age of 5.6 years (range 6 days-12.9 years). The median left ventricular outflow tract Doppler gradient before initiation of disopyramide was 81 mmHg (range 30-132 mmHg); eight patients had post-initiation echocardiograms, in which the median lowest recorded Doppler gradient was 43 mmHg (range 15-100 mmHg), for a median % reduction of 58.2% (p=0.002). With median follow-up of 2.5 years, eight of nine patients were still alive, although disopyramide had been discontinued in six of the nine patients. Reasons for discontinuation included septal myomectomy (four patients), heart transplantation (one patient), and side effects (one patient). Disopyramide was effective for the relief of left ventricular outflow tract obstruction in children with hypertrophic cardiomyopathy, although longer-term data suggest that its efficacy is not sustained. In general, it was well tolerated. Further study in larger patient populations is warranted.

While the epidemiology of adult heart failure has been extensively researched, this systematic review addresses the less well characterized incidence and prevalence of pediatric HF. The search strategy used Cochrane methodology and identified 83 unique studies for inclusion. Studies were categorized according to whether the HF diagnosis was reported as primary (n = 10); associated with other cardiovascular diseases (CVDs) (n = 49); or associated with non-CVDs (n = 24). A narrative synthesis of the evidence is presented. For primary HF, the incidence ranged from 0.87/100,000 (UK and Ireland) to 7.4/100,000 (Taiwan). A prevalence of 83.3/100,000 was reported in one large population-based study from Spain. HF etiology varied across regions with lower respiratory tract infections and severe anemia predominating in lower income countries, and cardiomyopathies and congenital heart disease major causes in higher income countries. Key findings for the other categories included a prevalence of HF associated with cardiomyopathies ranging from 36.1% (Japan) to 79% (US); associated with congenital heart disease from 8% (Norway) to 82.2% (Nigeria); associated with rheumatic heart diseases from 1.5% (Turkey) to 74% (Zimbabwe); associated with renal disorders from 3.8% (India) to 24.1% (Nigeria); and associated with HIV from 1% (US) to 29.3% (Brazil). To our knowledge, this is the first systematic review of the topic and strengthens current knowledge of pediatric HF epidemiology. Although a large body of research was identified, heterogeneity in study design and diagnostic criteria limited the ability to compare regional data. Standardized definitions of pediatric HF are required to facilitate cross-regional comparisons of epidemiological data.

Background: Patient safety reporting systems (PSRSs) may not detect teamwork or coordination process errors that affect all dimensions of quality defined by the Institute of Medicine. This study aimed to develop and observe the performance of a novel tool, the Coordination Process Error Reporting Tool (CPERT), as a prospective clinical surveillance mechanism for teamwork errors in the pediatric cardiac ICU., Methods: Providers and parents used the qualitative nominal group technique to identify coordination process error examples. Using categories developed from these discussions, the CPERT was designed and observed to assess agreement among providers and with the PSRS. For each patient at the end of each observed shift, the nurse, frontline clinician, and attending physician were invited to complete the CPERT online. Responses among providers were compared to assess interobserver agreement. Patients with errors identified by the CPERT were matched 1:1 with patients without CPERT errors within the same shift. The PSRS and medical record were reviewed to judge whether a coordination process error occurred and whether patients with CPERT errors differed from controls., Results: Eight categories of errors were identified and incorporated into the CPERT. During 10 shifts (218 patients), the CPERT completion rate was 74%. Fifty-one patient shifts had errors identified by the CPERT (23%); these patients did not differ significantly from those without CPERT- reported errors. Only 5 CPERT-reported errors (10%) were identified by two or more providers. Of the 51 CPERT- reported errors, 43 (84%) were not documented in the PSRS., Conclusion: The CPERT detects coordination process errors not identified through PSRS, making it or similar tools potentially useful for improvement efforts., (Copyright 2016 The Joint Commission.)

Hyponatremia is a common finding in adults hospitalized with heart failure (HF) and is associated with longer hospital stays and increased mortality. The significance of hyponatremia in children with HF is not known. We sought to determine the incidence of hyponatremia and association with clinical outcome in children hospitalized with HF. Admission and inpatient serum sodium concentrations were analyzed in 141 consecutive children hospitalized with acute decompensated HF. Inclusion criteria include patients (age, birth to 21 years) with biventricular hearts who were hospitalized for HF from January 2007 to December 2012. The primary composite end point was death, cardiac transplantation, or the use of mechanical circulatory support (MCS) during hospitalization. Data for 141 patients were included in the analysis. The cohort included 48 patients (34%) with preexisting HF. Mean serum sodium at admission was 136 ± 4 mmol/L (range 124 to 150 mmol/L). Hyponatremia (serum sodium <135 mmol/L) was present in 45 patients (32%) at admission. Seventy-one patients (75%) with normal serum sodium concentrations at admission subsequently developed acquired hyponatremia during their hospitalization. Hyponatremia persisted at discharge in 17 of 66 patients (26%). Fifty-eight patients (41%) reached the composite end point during hospitalization (death, n = 15; cardiac transplantation, n = 27; MCS, n = 46). Hyponatremia at admission was independently associated with death, cardiac transplantation, or the use of MCS during hospitalization (odds ratio 3.1, p = 0.02). In conclusion, hyponatremia occurs commonly in children hospitalized with acute decompensated HF and is associated with increased risk of in-hospital mortality, cardiac transplantation, and need for MCS., (Copyright © 2016. Published by Elsevier Inc.)

Background: Value-based health care is a proposed driver for reimbursement under the Affordable Care Act, with value broadly defined as outcomes divided by cost. Data on value-based health care in pediatric heart failure are scarce., Methods and Results: A retrospective analysis of the Healthcare Cost and Utilization Project Kids' Inpatient Database and Nationwide Inpatient Sample was performed for pediatric and adult cardiomyopathy and heart failure-related hospitalizations. The study included 5,689 pediatric and 473,416 adult hospitalizations. Pediatric cardiomyopathy and heart failure hospitalizations were significantly longer than adult hospitalizations (mean ± SE 16.2 ± 0.7 days vs 6.8 ± 0.1 days; P < .001). Overall mortality was greater for pediatric hospitalizations (7.7% vs 5.6%; P < .001), although it decreased over time for both pediatric and adult hospitalizations. Charges were greater for pediatric hospitalizations, both overall ($116,483 ± $5,735 vs $40,662 ± $1,419; P < .001) and for all years evaluated., Conclusions: In a value-based model, pediatric cardiomyopathy and heart failure-related hospitalizations are associated with worse outcomes and greater charges than adult hospitalizations. More research is needed to understand the cost effectiveness of pediatric heart failure treatment and to reduce the burden on the health care system., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Information exchanged during handoffs contributes importantly to a team's shared mental model. There is no established instrument to measure shared clinical understanding as a marker of handoff quality., Objective: To study the reliability, validity, and feasibility of the pediatric cardiology Patient Knowledge Assessment Tool (PKAT), a novel instrument designed to measure shared clinical understanding for pediatric cardiac intensive care unit patients., Design: To estimate reliability, 10 providers watched 9 videotaped simulated handoffs and then completed a PKAT for each scenario. To estimate construct validity, we studied 90 handoffs in situ by having 4 providers caring for an individual patient each complete a PKAT following handoff. Construct validity was assessed by testing the effects of provider preparation and patient complexity on agreement levels., Setting: A 24-bed pediatric cardiac intensive care unit in a freestanding children's hospital., Results: Video simulation results demonstrated score reliability. Average inter-rater agreement by item ranged from 0.71 to 1.00. During in situ testing, agreement by item ranged from 0.41 to 0.87 (median 0.77). Construct validity for some items was supported by lower agreement rates for patients with increased length of stay and increased complexity., Discussion: Results suggest that the PKAT has high inter-rater reliability and can detect differences in understanding between handoff senders and receivers for routine and complex patients. Additionally, the PKAT is feasible for use in a real-time clinical environment. The PKAT or similar instruments could be used to study effects of handoff improvement efforts in inpatient settings., (© 2014 Society of Hospital Medicine.)

Heart failure is a common late complication in adults with congenital heart defects, both repaired and unrepaired. The onset of clinical heart failure is associated with increased morbidity and mortality. Some patients with congenital heart disease may benefit from medications shown to improve survival in the population with acquired heart failure, but these same therapies may be of no benefit to other patients. Further studies are needed to better guide the choice of medical therapies., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Allografts are used for vascular reconstruction in many forms of congenital heart disease. Although allografts induce anti-human leukocyte antibody (HLA) formation, much about this response is unknown., Methods: Three groups of patients aged 8 to 18 years old underwent analysis for class I and II anti-HLA antibodies using Luminex. Groups were defined by timing of allograft exposure and diagnosis at Norwood for hypoplastic left heart syndrome (neonatal group), at Glenn for single-ventricle lesions not requiring arch reconstruction (infant group), and cardiac defects repaired during infancy without allografts (controls). Patients had significant anti-HLA (sensitization) if mean fluorescence intensity was ≥ 1500., Results: The study enrolled 29 patients (median age, 10.1 years). Significant class I anti-HLA antibodies were seen in 44% (8 of 18) of the neonatal group, 25% (1 of 4) of the infant group, and 14% (1 of 7) of controls; class II anti-HLA antibodies were seen in 44% (8 of 18) of the neonatal group, 25% (1 of 4) of the infant group, and 29% (2 of 7) of controls. All patients received fresh whole blood, but the neonatal group had greater exposure (p = 0.001). There was less sensitization with increasing time from last receipt of allograft(s) or blood transfusion (p = 0.05)., Conclusions: Exposure to allograft at the Norwood procedure is associated with long-term sensitization to anti-HLA antibodies in 56% of patients. Sensitization also occurs in those without prior exposure to allografts, may decrease over time, and appears related to whole blood. These findings have implications for those in whom heart transplant is considered late in the clinical course., (Copyright © 2013 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Purpose of Review: Hypertrophic cardiomyopathy (HCM) is the second most common childhood heart muscle disease and is the most common cause of unexpected sudden cardiac death in young people. The purpose of this review is to describe the risk of sudden death, to assess which of the criteria conventionally used as indication for a primary prevention defibrillator placement in adults are applicable in children with HCM, and to review differences in management between Europe and North America., Recent Findings: There is no uniform 'annual mortality rate' in childhood, and the risk of sudden death is at its highest level between 8 and 16 years. There are no significant differences between sudden death fatality rates in childhood HCM in Europe, North America and Australia when patients presenting with sudden death as the first sign of disease are excluded. The criteria for primary prevention defibrillator placement in adults with HCM are, with the exception of previous cardiac arrest and ventricular tachycardia on Holter monitoring, not predictive of risk in childhood HCM, and further research is required to establish better criteria for primary prevention in children., Summary: Most children diagnosed with HCM have a favorable prognosis with life-style modification with additional medical therapy, surgical therapy, and/or defibrillator placement in selected patients. The challenges inherent in identification of the patients most likely to benefit from invasive therapies and avoidance of these therapies in those least likely to benefit (and most likely to experience harm) exist on both sides of the Atlantic.

While the Fontan operation has facilitated the survival of a generation of children born with congenital heart disease resulting in a functional single ventricle, it does not recreate a normal circulation. Over time, survivors of the Fontan operation are at risk for ventricular dysfunction, plastic bronchitis, protein-losing enteropathy and chronic Fontan failure. New techniques and therapies are emerging to address the long-term risks associated with Fontan physiology, but as the number of survivors continues to grow, the recognition of the limitations of this circulation is increasing. Novel investigations of possible mechanical devices designed to function as a subpulmonary ventricle are underway, but are still many years away from clinical use. In the meantime, continued development of medical therapeutics targeted at the specific problems of the Fontan circulation will be beneficial and might reduce the need for cardiac transplantation.

Children and adults with congenital heart disease (CHD) can require interventions that result in immunologic alterations that are different than those seen in patients with cardiomyopathies. Patients with CHD can be exposed to heart surgeries, blood products, valved and non-valved allograft tissue, and mechanical circulatory support, all of which can alter the immunologic status of these patients. This change in immunologic status is most commonly manifested as the development of anti-human leukocyte antigen (HLA) antibodies. This review will delineate a) the causes of anti-HLA anti-body production (often referred to as allosensitization); b) preventive strategies for anti-HLA antibody production before transplantation; c) treatment strategies for those patients who develop anti-HLA antibodies before transplantation; d) consequences of HLA allosensitization after transplantation; and e) treatment of HLA allosensitization and antibody-mediated rejection after transplantation.

Background: Although decellularized cryopreserved valved allografts (DCAs) have reduced immunogenicity, proof of clinical superiority over standard cryopreserved allografts (SCAs) is lacking. To assess functional results and durability, we studied a group of patients with DCAs implanted between 2000 and 2005 and compared them with a similar group with SCAs., Methods: From July 2000 until January 2005, 47 patients underwent insertion of a DCA between the right ventricle and pulmonary arteries. The DCA patients were compared with 47 age-matched and diagnosis-matched controls receiving SCAs. All patients received pulmonary allografts and were matched for valve position (orthotopic versus heterotopic). We analyzed each group for survival, reoperation, reintervention (surgical or catheter-based), stenosis, and regurgitation., Results: There were no differences between groups with respect to weight, age, valve size, or survival. Actuarial freedom from reintervention at 8 years was 79% for DCAs as compared with 63% for SCAs (p = 0.31, log-rank). Echocardiogram in the DCA group (median 66 months) showed a slightly lower median peak gradient of 16 mm Hg (range, 0 to 82 mm Hg) as compared with 22 mm Hg (range, 0 to 63) in the SCA group (median 61 months, p = 0.051, Wilcoxon). However, when conduits 18 mm or less in diameter were compared, DCA patients had a median peak gradient of 10 mm Hg (range, 0 to 43) compared with 25 mm Hg in SCAs (range, 0 to 55 mm Hg, p = 0.03). There were no differences in the degree of allograft insufficiency in either group., Conclusions: Decellularized cryopreserved valved allografts have a nonsignificant trend toward lower peak valve gradient and reintervention in comparison with SCAs. Small valve sizes (18 mm or less) show a slight but significant improvement in peak gradient, but no advantage in valve insufficiency. These findings and a significantly higher cost (>$3,000) make further direct comparisons necessary before widespread use of DCAs can be justified., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2010 Elsevier Inc. All rights reserved.)

The proper ethical conduct of studies to evaluate drugs in children is of paramount importance to all those involved in these types of studies. This report is an updated revision to the previously published guidelines from the American Academy of Pediatrics in 1995. Since the previous publication, there have been great strides made in the science and ethics of studying drugs in children. There have also been numerous legislative and regulatory advancements that have promoted the study of drugs in children while simultaneously allowing for the protection of this particularly vulnerable group. This report summarizes these changes and advances and provides a framework from which to guide and monitor the ethical conduct of studies to evaluate drugs in children.

There is a growing population of adult patients with congenital heart defects in the developed world. Most have been repaired, but few have been cured. Many have myocardial dysfunction. Most have exercise intolerance. Some have heart failure. As a group, they show neurohormonal activation similar to that seen in an adult heart failure population with acquired heart disease. Currently, the patients at greatest risk of heart failure are those without a systemic left ventricle, such as Mustard and Senning repairs of transposition of the great arteries (TGA), congenitally corrected TGA, and patients who have had a Fontan procedure. Exercise intolerance may predict hospitalization and death in such patients. For those patients with systemic left ventricles, it would seem reasonable to use the heart failure guidelines developed for patients with acquired heart disease. For those patients without a systemic left ventricle (e.g., a systemic right ventricle or single ventricle), there is currently no foundation for evidence-based therapy.

Cryopreserved valved allografts are frequently used in the repair of congenital heart defects in children. Although the longevity of these grafts is generally good in most patients, there continue to be ongoing problems with allograft dysfunction and subsequent failure, particularly in infants and young children. The aim of this review is to discuss the immunogenicity of cryopreserved allograft tissue and measures that may minimize the deleterious effect of the immune system on allograft function and durability.

Sensitization to HLA antigens and the subsequent development of HLA antibodies in children under consideration for heart transplantation is a significant impediment to survival after listing. This is related both to the frequent need for prospective donor-specific crossmatching (thus limiting donor availability and increasing pretransplant morbidity and mortality), and to the increased risk of adverse outcomes after transplantation. This article will review the scope of this problem in children under consideration for heart transplantation, the different methods available for diagnosing HLA sensitization, the known causes of HLA sensitization, the consequences of these preformed antibodies on outcomes before and after heart transplantation, and the different methods of preventing and treating this sensitization that are currently available. Improved methods of diagnosing, preventing, and treating this problem can only lead to better outcomes for children who require heart transplantation.

We report the first successful transcatheter closure of a large traumatic ventricular septal defect in a 16-year-old adolescent. The risks of standard open heart repair using cardiopulmonary bypass were felt to be prohibitive due to coexistent injuries. This case demonstrates the feasibility of transcatheter therapy for traumatic ventricular septal defects., (Copyright 2004 Wiley-Liss, Inc.)

Background: Recognition of the immunogenicity of standard cryopreserved allografts has led to the development of new decellularized allografts (CryoValve SG; CryoLife, Inc, Kennesaw, Ga). This preliminary study examined the HLA antibody response to these decellularized allografts and compared it with the response to standard allograft material., Methods: We prospectively measured the frequency of panel-reactive HLA class I (HLA-A, HLA-B, and HLA-C) and class II (HLA-DR/DQ) alloantibodies in 14 children (age 8.5 +/- 7.9 years) receiving decellularized, cryopreserved allografts, including 6 undergoing allograft patch insertion and 8 with a valved pulmonary allograft. We compared them with 20 historical control subjects (age 1.7 +/- 2.4 years) undergoing implantation of standard cryopreserved allografts, 8 with valves and 12 with allograft patch. All patients had panel-reactive antibody levels measured before and at 1, 3, and 12 months after the operation. HLA class I and class II panel-reactive antibody levels were determined with a sensitive flow cytometry technique., Results: We found panel-reactive antibody levels in decellularized allografts to be elevated slightly from preoperative levels for both class I and class II antibodies at 1, 3, and 12 months (P >.05). The panel-reactive antibody level for both class I and class II antibodies were significantly lower for decellularized allografts as compared to standard allografts. Functionally, the allografts were similar with decellularized valved grafts showing a peak echo-determined systolic gradient of 13 +/- 15 mm Hg at 8 +/- 2.6 months postoperatively as compared to a gradient of 24 +/- 18 mm Hg measured 12 +/- 6 months postoperatively in standard allografts (P =.11)., Conclusions: Decellularized grafts elicited significantly lower levels of class I and class II HLA antibody formation at 1, 3, and 12 months after implantation than did standard cryopreserved allografts. Early hemodynamic function of decellularized grafts was similar to that of standard cryopreserved allograft valves. Further experience is necessary to determine whether the reduced immunogenicity of decellularized allografts will truly allow tissue ingrowth and improved long-term durability in patients.

Background: Carvedilol is a medication with both beta-receptor and alpha-receptor blocking properties that has been approved for the treatment of heart failure in adults. Little is known about its safety, efficacy, pharmacokinetics, and dosing profile in children., Methods: The primary objective of this study is to evaluate the efficacy of carvedilol administered twice daily for 8 months in terms of its effect compared with placebo on a composite measure of clinical outcomes in children with symptomatic systemic ventricular systolic dysfunction and heart failure. The secondary objectives are to determine the effect of carvedilol on individual components of a composite of clinical outcomes (hospitalizations for worsening heart failure, all-cause mortality and cardiovascular hospitalizations, all cause mortality, heart failure symptoms, and patient and physician global assessment); determine the effect of carvedilol on echocardiographic indices of ventricular function and remodeling; characterize the pharmacokinetics of carvedilol in pediatric patients with heart failure; characterize the effects carvedilol on neurohormonal systems; and provide data for the selection of an optimal titration schedule and daily dose of carvedilol in children with heart failure. This study will enroll 150 children between birth and 17 years of age with chronic symptomatic heart failure caused by systemic ventricular systolic dysfunction., Conclusion: This study will determine whether carvedilol improves symptoms in children with heart failure as a result of systemic ventricular systolic dysfunction. The study also will provide information on echocardiographic changes of ventricular performance and neurohormonal levels in children with heart failure before and after treatment with carvedilol, in addition to pharmacokinetics of carvedilol in children.