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Objective: The objective of this study was to assess the relationship of prenatal diagnosis of critical congenital heart disease (CHD) to preoperative and postoperative patient findings. Method: Retrospective analysis of neonates with critical CHD who underwent cardiothoracic surgery at one of four centers in North Carolina between 2008-2013. Surgical data collected by sites for submission to the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the North Carolina CHD Lifespan Database were queried. Results: There were 715 patients with STS records; 566 linked to the NC CHD database. Patients with prenatal diagnosis had a lower incidence of preoperative risk factors, including need for mechanical ventilation and presence of shock. However, prenatally diagnosed patients had worse short-term outcomes, including higher operative mortality, higher incidence of select postoperative complications, and longer LOS. There was no difference in one-year mortality. Conclusion: Our findings are consistent with current literature which suggests that prenatal diagnosis of critical CHD is associated with a more optimized preoperative clinical status. However, we found that patients with prenatal diagnoses had less favorable postoperative outcomes. This needs to be investigated further, but may be secondary to patient-specific factors, such as CHD disease severity.

Background The Long-ter M O U tcomes after the Multisystem Inflammatory S yndrome I n C hildren (MUSIC) study aims to characterize the frequency and time course of acute and long-term cardiac and non-cardiac sequelae in multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C), which are currently poorly understood. Methods This multicenter observational cohort study will enroll at least 600 patients Conclusion The MUSIC study, with the largest cohort of MIS-C patients and the longest follow-up period to date, will make an important contribution to our understanding of the acute cardiac and non-cardiac manifestations of MIS-C and the long-term effects of this public health emergency.

Patients after Fontan palliation represent a growing pediatric population requiring heart transplant (HTx) and often have lymphopenia (L) and/or hypogammaglobinemia that may be exacerbated by protein-losing enteropathy (PLE, P). The post-HTx effects of this altered immune phenotype are not well studied.In this study of the Pediatric Heart Transplant Society Registry, 106 Fontan patients who underwent HTx between 2005 and 2018 were analyzed. The impact of lymphopenia and PLE on graft survival, infection, rejection, and malignancy was analyzed at 1 and 5 years post-HTx.The following combinations of lymphopenia and PLE were noted: +L+P, n = 37; +L-P, n = 23; -L+P, n = 10; and -L-P, n = 36. Graft survival between the groups was similar within the first year after transplant (+L+P: 86%, +L-P: 86%, -L+P: 87%, -L-P: 89%, p = .9). Freedom from first infection post-HTx was greatest among -L-P patients compared to patients with either PLE, lymphopenia, or both; with a 22.1% infection incidence in the -L-P group and 41.4% in all others. These patients had a significantly lower infection rate in the first year after HTx (+L+P: 1.03, +L-P: 1, -L+P: 1.3, -L-P: 0.3 infections/year, p .001) and were similar to a non-single ventricle CHD control group (0.4 infections/year). Neither freedom from rejection nor freedom from malignancy 1 and 5 years post-HTx, differed among the groups.Fontan patients with altered immunophenotype, with lymphopenia and/or PLE, are at increased risk of infection post-HTx, although have similar early survival and freedom from rejection and malignancy. These data may encourage alternative immunosuppression strategies and enhanced monitoring for this growing subset of patients.

Background The AHA/ACC Adult Congenital Heart Disease guidelines recommend that most adults with congenital heart disease (CHD) follow-up with CHD cardiologists every 1 to 2 years because longer gaps in care are associated with adverse outcomes. This study aimed to determine the proportion of patients in North Carolina who did not have recommended follow-up and to explore predictors of loss to follow-up. Methods Patients ages ≥18 years with a healthcare encounter from 2008 to 2013 in a statewide North Carolina database with an ICD-9 code for CHD were assessed. The proportion with cardiology follow-up within 24 months following index encounter was assessed with Kaplan-Meier estimates. Cox regression was utilized to identify demographic factors associated with differences in follow-up. Results 2822 patients were identified. Median age was 35 years; 55% were female. 70% were white, 22% black, and 3% Hispanic; 36% had severe CHD. The proportion with 2-year cardiology follow-up was 61%. Those with severe CHD were more likely to have timely follow-up than those with less severe CHD (72% vs 55%, P Conclusion 39% of adults with CHD in North Carolina are not meeting AHA/ACC recommendations for follow-up. Younger and minority patients and those with non-severe CHD were particularly vulnerable to inadequate follow-up; targeted efforts to retain these patients in care may be helpful.

Objectives In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care. Data sources The curricula from the twenty 4th year pediatric cardiac critical care training programs were collated, along with the learning objectives from the Pediatric Cardiac Intensive Care Society published "Curriculum for Pediatric Cardiac Critical Care Medicine." Study selection This initiative is endorsed by the Pediatric Cardiac Intensive Care Society as a first step toward Accreditation Council for Graduate Medical Education oversight of training and American Board of Pediatrics oversight of maintenance of certification. Data extraction A taskforce was established of cardiac intensivists, including the directors of all 4th year pediatric cardiac critical care training programs. Data synthesis Using modified Delphi methodology, learning objectives, rotational requirements, and institutional requirements for providing training were developed. Conclusions In the current era of increasing specialized care in pediatric cardiac critical care, standardized training for pediatric cardiac critical care is paramount to optimizing outcomes.

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code. Patients who could be linked to a NC death certificate between 2008 and 2016 were included. Patients were divided by CHD subtypes (severe, shunt, valve, other). COD was compared between groups. Records of 35,542 patients

Background: Children with congenital heart defects (CHDs) are at risk for poor academic performance. The degree to which receipt of health care services is associated with adverse academic outcomes is not known. We examined the association between episodes of cardiac care and third-grade performance in children with CHD. Methods: We identified subjects between 1/1/2008 and 4/30/2012 among 5 centers in North Carolina. We classified children by CHD type and linked subjects to the state educational records. Any inpatient or outpatient cardiac encounter on a date of service was considered an encounter. We calculated the number of encounters by adding the number of inpatient or outpatient cardiac visits prior to the date of the end of grade (EOG) tests. We estimated the odds of failing third-grade reading or math EOG tests by episodes of care stratified at the 50thpercentile, controlling for CHD type, maternal education, sex, race/ethnicity, birth weight and gestational age. Results: A total of 184 children had third-grade EOG scores linked to health care records. The median number of episodes of care was 4 (range: 1-60). Those with visits ˃50th percentile (>4 encounters over 4.3 years) had 2.09 (95% CI: 1.04, 4.21) greater odds of failing the math EOG compared to those ≤50th percentile (1-4 encounters). The third-grade math score declined by 1.5 points (pConclusion: Children with CHD with >4 episodes of cardiac care over 4.3 years may be at risk for delays in third-grade academic performance. Strategies to minimize school absenteeism may improve academic success in this population.

The US Pediatric Heart Allocation Policy (PHAP) was revised in March 2016, with the goal of reducing waitlist mortality. We evaluated the hypothesis that these changes, which increased status exceptions, have worsened racial disparities in waitlist outcomes.Children in the Pediatric Heart Transplant Study database listed for first heart transplant from January 2012 - June 2020 were included and stratified by listing before (Era 1) or after (Era 2) the PHAP revision.A total of 4,089 children were listed during the study period. Compared with white children (n = 2648), non-white children (n = 1441) were more likely to have an underlying diagnosis of cardiomyopathy in both eras. Waitlist mortality was similar in white and non-white children in Era 1, but comparatively worse for non-white children in Era 2. In multivariable analysis controlling for diagnosis, age, and severity markers, non-white children had a significantly higher waitlist mortality only in Era 2 (Era 1: sHR 1.22 [95%CI 0.90 - 1.66] vs. Era 2: sHR 1.57 [95%CI 1.17 - 2.10]).Widening racial disparities in waitlist mortality may be an unintended consequence of the 2016 PHAP revision. Additional analyses may inform the degree to which this policy vs. unrelated changes in care differentially contribute to these disparities.

Introduction: The US Pediatric Heart Allocation Policy was recently revised with the goal of lowering waitlist mortality. Effective March 2016, these rules significantly changed the requirements for priority status. We evaluated the hypothesis that this change, which has led to an increase in status exceptions, has worsened known racial disparities in waitlist outcomes. Methods: Children in the Pediatric Heart Transplant Study database listed at a US center from January 2012 - June 2020 were included and stratified by listing before (Era 1) or after (Era 2) allocation policy change on March 22, 2016. Competing risk survival analysis was performed to evaluate endpoint of death or deterioration on the waitlist with transplant treated as a competing risk. Results: Compared with white children (n=2,648), non-white children (n=1,441) were more likely to have an underlying diagnosis of cardiomyopathy (49% vs. 39%, p Conclusions: Widening racial disparities in waitlist mortality may be an unintended consequence of the 2016 Pediatric Heart Allocation Policy revision. Additional analyses may help the transplant community understand the degree to which this policy (i.e. prioritized listing status and exceptions) vs. unrelated changes in care differentially contribute to these disparities.

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code. Patients who could be linked to a NC death certificate between 2008 and 2016 were included. Patients were divided by CHD subtypes (severe, shunt, valve, other). COD was compared between groups. Records of 35,542 patients 20 years old were evaluated. There were 15,277 infants with an annual mortality rate of 3.5 deaths per 100 live births. The most frequent COD in infants (age 1 year) were CHD (31.7%), lung disease (16.1%), and infection (11.4%). In 20,265 children (age 1 to 20 years), there was annual mortality rate of 9.7 deaths per 1000 at risk. The most frequent COD in children were CHD (34.2%), neurologic disease (10.2%), and infection (9.5%). In the severe subtype, CHD was the most common COD. In infants with shunt-type CHD disease, lung disease (19.5%) was the most common COD. The mortality rate in infants was three times higher when compared to children. CHD is the most common underlying COD, but in those with shunt-type lesions, extra-cardiac COD is more common. A multidisciplinary approach in CHD patients, where development of best practice models regarding comorbid conditions such as lung disease and neurologic disease could improve outcomes in this patient population.

Background: Home oxygen therapy is often required in children with chronic respiratory conditions. This document provides an evidence-based clinical practice guideline on the implementation, monitoring, and discontinuation of home oxygen therapy for the pediatric population. Methods: A multidisciplinary panel identified pertinent questions regarding home oxygen therapy in children, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation approach to rate the quality of evidence and strength of clinical recommendations. Results: After considering the panel’s confidence in the estimated effects, the balance of desirable (benefits) and undesirable (harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were developed for or against home oxygen therapy specific to pediatric lung and pulmonary vascular diseases. Conclusions: Although home oxygen therapy is commonly required in the care of children, there is a striking lack of empirical evidence regarding implementation, monitoring, and discontinuation of supplemental oxygen therapy. The panel formulated and provided the rationale for clinical recommendations for home oxygen therapy based on scant empirical evidence, expert opinion, and clinical experience to aid clinicians in the management of these complex pediatric patients and identified important areas for future research.

In patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life-threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.

Introduction Renal dysfunction requires renal transplant or dialysis in up to 10% of transplant patients at 15 years. Those with chronic renal disease or single kidney are at high risk, but there has been limited study of calcineurin inhibitor (CNI)-sparing heart transplantation (HT) in pediatrics. Case Report A 12 year old female with history of Wilms tumor status post left nephrectomy developed anthracycline-induced dilated cardiomyopathy. She had decompensated heart failure bridged to transplant with milrinone. Given prior recurrent acute kidney injury (AKI) in the setting of a single kidney, she was at increased risk of receiving dialysis post-operatively, but a combined heart-kidney transplant was not felt indicated. She was induced with thymoglobulin and initially managed with concomitant mycophenolate mofetil and methylprednisolone. At approximately 3 weeks post-transplant, sirolimus was added. Within 2 week postoperatively, she developed hypertension, acute tubular necrosis, and oliguric AKI. Prior to starting Sirolimus, she also developed pleural and pericardial effusions requiring a chest tube, pericardiocentesis, and pericardial window. Despite these complications there was no evidence of transplant rejection or graft reperfusion injury on biopsy, but filling pressures were upwards of 25 mmHg bilaterally. Aggressive hypertensive management and judicious diuretic use improved her filling pressures and overall clinical status. She was discharged home 60 days after HT. At her 3 month post-op catheterization she had normal filling pressures and no signs of rejection (ISHLT grade A0, AMR grade 0) on low dose prednisone, mycophenolate, and sirolimus. Her AKI has resolved and she remains dialysis free. Summary AKI after HT is due to a constellation of renal hypoperfusion, low cardiac output, and drug-induced nephrotoxicity requiring dialysis in some patients. Prior studies showed improved renal function in those converted from CNIs to mTOR inhibitors. In our case, the morbidity associated with AKI was deemed too severe to pursue standard use of CNIs. While some pediatric patients have undergone immunosuppression with reduced CNI dosing, few have completed HT without CNI. Her case illustrates the utility of mTOR inhibitors for its nephrotoxic sparing profile while maintaining adequate immunosuppression protection and graft function.

Current knowledge of antibody-mediated rejection (AMR) after heart transplantation (HT) stems largely from adult data. Using the Pediatric Heart Transplant Study (PHTS) database, we report the incidence of AMR, describe treatment, and evaluate outcomes for treated AMR in children after HT.We queried the PHTS database for patients18 years of age undergoing primary HT between January 2010 and December 2014. An AMR episode was defined as either a biopsy consistent with pathologic AMR or a rejection event based on immunotherapy augmentation directed against antibody production. Biopsy data, treatment strategies and survival were analyzed.An episode of AMR was identified in 179 of 1,596 (11%) HT recipients and in 246 of 705 (35%) rejection episodes. AMR was diagnosed by biopsy in 182 of 246 episodes and by immunotherapy in 64 of 179 episodes. Mixed rejection was identified in 179. Freedom from AMR was 88% and 82% at 1 and 3 years, respectively. AMR therapies included intravenous immunoglobulin (IVIg) (58%), plasmapheresis (40%), rituximab (40%), bortezomib (11%) and eculizumab (0.4%). The most commonly used combination therapies included IVIg/plasmapheresis/rituximab (13%). Thirty-three patients (16%) died after developing AMR. Patient and graft survival were lower for the AMRIn his study we report the largest experience of AMR in pediatric HT recipients. AMR was common and often occurred concurrently with acute cellular rejection. There is wide variability in the treatment of AMR. Short-term patient and graft outcomes were worse for those with treated AMR.

The objectives of this review are to discuss the paradigms used to stage heart failure in children, the classification and physiologic profile of cardiomyopathies, and the acute and chronic pharmacologic management of heart failure.MEDLINE, PubMed.The etiology of chronic heart failure in pediatrics is vast. The paradigm of extrapolating adult clinical trials and technological advancements to treat heart failure in children has become a nonsustainable model. The field of pediatric heart failure continues to advance with more robust guideline-directed care and the imminent creation of a dynamic, contemporary international database. As the field involves a markedly heterogeneous patient population, it is imperative to use pediatric specific descriptors of disease impact. The fields of pediatric heart failure and critical care medicine will continue to evolve together as childhood specific registries, quality improvement guidelines, and research will lead to practice models eliciting optimal therapy for patients with heart failure in the intensive care setting.

BackgroundFontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients.Methods and resultsThis study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman’s Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6–33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (−0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (−0.32, p2versus lower-risk group=2.96 L/minute/m2, (pConclusionsHigher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.

Endomyocardial biopsy (EMB) is a common procedure used to aid in the diagnosis, prognosis and treatment of suspected pediatric cardiomyopathy. In suspected cardiomyopathy, no multicenter experience has previously reported on the safety and utility of EMBs. Retrospectively, adverse event (AE) and patient and procedural characteristics were obtained at seven institutions participating in the Congenital Cardiac Catheterization Outcomes Project for both a cardiomyopathy (n = 158) and a post-transplant surveillance (n = 2665) cohort. Descriptive information regarding biopsy indication, pathology and clinical management based on EMB findings were retrospectively obtained. High-severity AEs were more common in the cardiomyopathy cohort when compared to the post-transplant surveillance cohort. The cardiomyopathy cohort was younger, more hemodynamically vulnerable and required more cardiorespiratory support during the procedure. The eight high-severity AEs in the cardiomyopathy group included one myocardial perforation, two ECMO cannulations and three deaths following the EMB. Factors associated with high-severity AEs included performing another catheter-based intervention during the EMB and longer fluoroscopy time. Notably, an increased number of biopsy attempts did not increase the risk of an AE. Suspected myocarditis was the most common indication. Diagnostic EMB pathology and thus alteration to clinical management based on pathology occurred more frequently in patients with suspected myocarditis. In conclusion, there is an increased incidence of high-severity AEs in patients undergoing EMB for suspected cardiomyopathy. EMB may be more clinically useful in the management of suspected myocarditis. The increased risk of high-severity AEs when additional interventions are performed highlights the hemodynamic vulnerability in patients with suspected cardiomyopathy.

BACKGROUND The use of ventricular assist devices (VADs) in children with heart failure may be of particular benefit to those with accompanying renal failure, as improved renal function is seen in some, but not all recipients. We hypothesized that persistent renal dysfunction at 7 days and/or 1 month after VAD implantation would predict chronic kidney disease (CKD) 1 year after heart transplantation (HT). METHODS Linkage analysis of all VAD patients enrolled in both the PEDIMACS and PHTS registries between 2012 and 2016. Persistent acute kidney injury (P-AKI), defined as a serum creatinine ≥1.5× baseline, was assessed at post-implant day 7. Estimated glomerular filtration rate (eGFR) was determined at implant, 30 days thereafter, and 12 months post-HT. Pre-implant eGFR, eGFR normalization (to ≥90 mL/min/1.73 m2 ), and P-AKI were used to predict post-HT CKD (eGFR

Heart failure is a common problem among children admitted in the intensive care unit and is associated with significant morbidity and mortality. As such, the 2014 meeting of the Pediatric Cardiac Intensive Care Society included a session on Clinical Controversies in Heart Failure and Transplantation. This review contains the summaries of the podium presentations of this session and will cover some of the challenging aspects of caring for these patients including medical and mechanical support, fluid overload states, high-risk populations including those after heart transplantation, and end-of-life considerations.

Heart failure remains a significant contributor to morbidity and mortality in pediatric patients with congenital and acquired heart diseases. However, there are several new promising therapies for acute and chronic heart failures including the development of new pediatric specific ventricular assist devices. There remains a need for collaborative research including the creation of a large-scale registry of pediatric heart failure, increased utilization of genetic testing, and an improved understanding of the optimal model of disease management that maximizes quality care.

Background— Pediatric data on the impact of pre–heart transplantation (HTx) risk factors on early post-HTx outcomes remain inconclusive. Thus, among patients with previous congenital heart disease or cardiomyopathy, disease-specific risk models for graft loss were developed with the use pre-HTx recipient and donor characteristics. Methods and Results— Patients enrolled in the Pediatric Heart Transplant Study (PHTS) from 1996 to 2006 were stratified by pre-HTx diagnosis into cardiomyopathy and congenital heart disease cohorts. Logistic regression identified independent, pre-HTx risk factors. Risk models were constructed for 1-year post-HTx graft loss. Donor factors were added for model refinement. The models were validated with the use of patients transplanted from 2007 to 2009. Risk factors for graft loss were identified in patients with cardiomyopathy (n=896) and congenital heart disease (n=965). For cardiomyopathy, independent risk factors were earlier year of transplantation, nonwhite race, female sex, diagnosis other than dilated cardiomyopathy, higher blood urea nitrogen, and panel reactive antibody >10%. The recipient characteristic risk model had good accuracy in the validation cohort, with predicted versus actual survival of 97.5% versus 95.3% (C statistic, 0.73). For patients with congenital heart disease, independent risk factors were nonwhite race, history of Fontan, ventilator dependence, higher blood urea nitrogen, panel reactive antibody >10%, and lower body surface area. The risk model was less accurate, with 86.6% predicted versus 92.4% actual survival, in the validation cohort (C statistic, 0.63). Donor characteristics did not enhance model precision. Conclusions— Risk factors for 1-year post-HTx graft loss differ on the basis of pre-HTx cardiac diagnosis. Modeling effectively stratifies the risk of graft loss in patients with cardiomyopathy and may be an adjunctive tool in allocation policies and center performance metrics.

Introduction: Although first year survival after pediatric heart transplant is improving, late-onset rejection, cardiac allograft vasculopathy (CAV), and graft failure remain significant challenges. Patients routinely undergo endomyocardial biopsy with invasive hemodynamics as the gold standard for rejection surveillance and assessment of long-term graft performance. However, it remains unclear how hemodynamic measures contribute to the monitoring of rejection, CAV, and graft dysfunction. This study aims to assess changes in hemodynamics as the graft ages in patients with or without a history of rejection or CAV. Hypothesis: We hypothesize that abnormal hemodynamics, particularly elevated filling pressures, are associated with CAV, rejection and graft aging. Methods: A total of 2,764 patients from the Pediatric Heart Transplant Study database from 1995-2014 met study criteria of survival to 1 year after transplant with available surveillance biopsy and hemodynamics. Retrospective analyses of 16,489 hemodynamic sets were performed. Results: The median age at transplant was 4.89 years [0.73, 12.95]. Average follow up time was 4.05 years [2.04, 7.17]. Fifty-six percent of patients were male, and 46% had history of congenital heart disease. When comparing hemodynamics collected at the first annual catheterization to 10 years after transplant, those who never had rejection or CAV showed statistically significant increases in right atrial pressure (RAP) (p= Conclusions: Despite freedom from CAV or rejection, RAP and PCWP increase and cardiac index decreases as the graft ages. Additionally, the diagnosis of CAV or rejection during the life of the graft leads to further derangement in graft performance, which can be detected in hemodynamics. Serial hemodynamic assessment may contribute to the ongoing monitoring of graft function in pediatric recipients.

Mechanical circulatory support (MCS) offers a surgical option for advanced heart failure when optimal medical therapy is inadequate. MCS therapy improves prognosis, functional status, and quality of life.1,2 The INTERMACS (Interagency Registry for Mechanically Assisted Circulatory Support) tracks patient selection and outcomes for all implanted US Food and Drug Administration–approved MCS devices. From June 2006 until December 2014, >15 000 patients received MCS, and >2000 implantations are performed annually. One-year survival with current continuous-flow devices is reported to be 80%, and 2-year survival, 70%.3 In patients awaiting heart transplantation, MCS provides a bridge to transplantation, and for others who are ineligible for heart transplantation, MCS provides permanent support or destination therapy. Indications and absolute and relative contraindications to durable MCS are listed in Table 1. View this table: Table 1. Indications and Contraindications to Durable Mechanical Support As of July 2014, 158 centers in the United States offer long-term MCS.3 Patients often live a substantial distance from the implanting center, necessitating active involvement of local first responders (emergency medical technicians, police, and fire department personnel), emergency department staff, primary care, and referring cardiologists. Because patients with MCS are becoming increasingly mobile, basic knowledge of equipment is necessary for personnel in public areas such as schools, public transportation, and airplanes/airports. Ambulatory patients with MCS can span the entire age spectrum from pediatrics to geriatrics. The aim of this document is to provide guidance for nonexperts in MCS and to facilitate the informed assessment, stabilization, and transport of the patient with MCS back to the MCS center for definitive therapy. In addition, the principles herein provide a foundation for emergency management and a framework to address the management of known MCS-associated complications and expected comorbid medical problems. Currently in the United States, the most frequently used durable devices are continuous-flow devices with …

Quality improvement science permeates the culture of health care. Infrastructure and systematic changes have been instituted with the ultimate goal of improved care delivery at reduced expenditures. Unfortunately, the scope and status of pediatric heart failure quality improvement cannot be accurately assessed as there is a paucity of publications in the literature. Nonetheless, there are models in health care that exist as is evidenced by the American College of Cardiology and the American Heart Association approved process measures for quality improvement in heart failure in the adult population. Despite these efforts, defining appropriate process measures is challenging as the ultimate goal is to positively affect the outcome. Linking process measures to outcome measures may be fraught with difficulties. However, the paradigm of process improvement has been associated with institutional adherence to guidelines and ultimately improved overall outcome. In pediatrics, our field has limited comprehensive, universal registries and trials. Similarly, heart failure in pediatrics exemplifies a heterogeneous population with many comorbid conditions. These challenges incite the need for strong advocacy for this process which will be constantly evolving. Organization and infrastructure to augment quality improvement across centers will be vital to ultimate success. Finally, via leveraging existing pediatric databases, international collaboration of committees, and the development of a comprehensive registry, guidelines for quality improvement in pediatric heart failure may be developed.

Bacterial infections represent a major cause of morbidity and mortality in heart transplant recipients. However, data describing the epidemiology and outcomes of these infections in children are limited.We analyzed the Pediatric Heart Transplant Study database of patients transplanted between 1993 and 2014 to determine the etiologies, risk factors and outcomes of children with bacterial infections post-heart transplantation.Of 4,458 primary transplants in the database, there were 4,815 infections that required hospitalization or intravenous therapy, 2,047 (42.51%) of which were bacterial. The risk of bacterial infection was highest in the first month post-transplant, and the bloodstream was the most common site (24.82%). In the early post-transplant period (30 days post-transplant), coagulase-negative staphylococci were the most common pathogens (16.97%), followed by Enterobacter sp (11.99%) and Pseudomonas sp (11.62%). In the late post-transplant period, community-acquired pathogens Streptococcus pneumoniae (6.27%) and Haemophilus influenzae (2.82%) were also commonly identified. Patients' characteristics independently associated with acquisition of bacterial infection included younger age (p0.0001) and ventilator (p0.0001) or extracorporeal membrane oxygenation (p = 0.03) use at time of transplant. Overall mortality post-bacterial infection was 33.78%, and previous cardiac surgery (p0.001) and multiple sites of infection (p = 0.004) were independent predictors of death.Bacteria were the most common causes of severe infections in pediatric heart transplant recipients and were associated with high mortality rates. The risk of acquiring a bacterial infection was highest in the first month post-transplant, and a large proportion of the infections were caused by multidrug-resistant pathogens.

Late (occurring1 year) rejection (LR) has been shown to increase mortality and morbidity after pediatric heart transplantation (HTx). The incidence of rejection has decreased in the first year after pediatric HTx in the current era. We hypothesized a similar phenomenon has occurred with LR.The Pediatric Heart Transplant Study database was used to analyze the effects of era (1993 to 1998 vs 1999 to 2007) and other factors on the prevalence of LR and its relationship to mortality, moderate-severe coronary vasculopathy (CAV) and retransplantation.Freedom from first LR (59% vs 69% 5-year post-HTx, p0.001) and recurrent LR (p0.001) was significantly lower in the current vs earlier era. LR was significantly (p0.001) associated with early rejection (ER; rejection1 year post-HTx) in both eras. Independent risk factors for LR were: earlier era (hazard ratio [HR] 1.47, 95% confidence interval [CI] 1.25 to 1.73, p0.001); non-white race (HR 1.41; 95% CI 1.19 to 1.67, p0.001); older recipient age (HR 1.05, 95% CI 1.03 to 1.06, p0.001); recipient Status 2 at transplant (HR 1.21, 95% CI 1.01 to 1.45, p = 0.037); and male donor (HR 1.17; 95% CI 1.0 to 1.37, p = 0.055). Late rejectors had a similar higher risk of mortality (odds ratio [OR] 4.20, 95% CI 3.04 to 5.81, p0.001) and incidence of moderate-severe CAV or retransplantation (OR 2.63, 95% CI 1.78 to 3.90, p0.001) in both eras.LR has decreased in the recent era in pediatric HTx recipients. Its effect on mortality and the development of CAV has not changed over time, suggesting an ongoing need for improved primary prevention strategies.

Combined heart-lung transplantation remains as a treatment option for patients with cardiopulmonary failure. There is speculation that lung grafts protect the heart from developing graft vasculopathy after combined heart-lung transplantation. This protective mechanism is more likely, at best, a delay in the onset of coronary artery vasculopathy. We present our experiences in two cases of an aggressive form of cardiac allograft vasculopathy after combined heart-lung transplantation that resulted in the death of both patients.

No prior reports documenting the safety and diagnostic yield of cardiac catheterization and endomyocardial biopsy (EMB) in heart transplant recipients include multicenter data.Data on the safety and diagnostic yield of EMB procedures performed in heart transplant recipients were recorded in the Congenital Cardiac Catheterization Outcomes Project database at 8 pediatric centers during a 3-year period. Adverse events (AEs) were classified according to a 5-level severity scale. Generalized estimating equation models identified risk factors for high-severity AEs (HSAEs; Levels 3-5) and non-diagnostic biopsy samples.A total of 2,665 EMB cases were performed in 744 pediatric heart transplant recipients (median age, 12 years [interquartile range, 4.8, 16.7]; 54% male). AEs occurred in 88 cases (3.3%), of which 28 (1.1%) were HSAEs. AEs attributable to EMB included tricuspid valve injury, transient complete heart block, and right bundle branch block. Amongst 822 cases involving coronary angiography, 10 (1.2%) resulted in a coronary-related AE. There were no myocardial perforations or deaths. Multivariable risk factors for HSAEs included fewer prior catheterizations (p = 0.006) and longer case length (p0.001). EMB yielded sufficient tissue for diagnosis in 99% of cases. Longer time since heart transplant was the most significant predictor of a non-diagnostic biopsy sample (p0.001).In the current era, cardiac catheterizations involving EMB can be performed in pediatric heart transplant recipients with a low AE rate and high diagnostic yield. Risk of HSAEs is increased in early post-transplant biopsies and with longer case length. Longer time since heart transplant is associated with non-diagnostic EMB samples.

Renal dysfunction is a major determinant of outcome after HTx. Using a large, multi-institutional database, we sought to identify factors associated with late renal dysfunction after pediatric HTx. All patients in the PHTS database with eGFR ≥60 mL/min/1.73 m(2) at one yr post-HTx (n = 812) were analyzed by Cox regression for association with risk factors for eGFR 1 yr after HTx. Freedom from late renal dysfunction was 71% and 57% at five and 10 yr. Multivariate risk factors for late renal dysfunction were earlier era of HTx (HR 1.84; p < 0.001), black race (HR 1.42; p = 0.048), rejection with hemodynamic compromise in the first year after HTx (HR 1.74; p = 0.038), and lowest quartile eGFR at one yr post-HTx (HR 1.83; p < 0.001). Renal function at HTx was not associated with onset of late renal dysfunction. Eleven patients (1.4%) required chronic dialysis and/or renal transplant during median follow-up of 4.1 yr (1.5-12.6). Late renal dysfunction is common after pediatric HTx, with blacks at increased risk. Decreased eGFR at one yr post-HTx, but not at HTx, predicts onset of late renal dysfunction. Future research on strategies to minimize late renal dysfunction after pediatric HTx may be of greatest benefit if focused on these subgroups.

Objective. Multiple surgical approaches to the initial palliation of patients with hypoplastic left heart syndrome (HLHS) have been advocated throughout the years. We sought to examine what procedure, if any, is recommended for HLHS management in regard to physician preference, anatomical variations, and concomitant medical issues. Patients and Methods. A Web-based survey of pediatric cardiologist subscribed to PediHeart was conducted. Outcome Measures. The types of palliation recommended (Norwood palliation, Sano modification, hybrid palliation, primary cardiac transplantation, or hospice care) for patients with HLHS with anatomic or comorbid variants were queried. Counseling provided by the physicians to families was also documented as regards survival and outcomes. Results. Two hundred physicians (21% female) who averaged 12.3 years removed from training responded to the survey. US East Coast and Midwest respondents were more likely to recommend Norwood palliation (54% and 60%, respectively) and the US South and West respondents preferred Sano modification (73% and 82%, respectively). Norwood or Sano palliation was recommended over hospice care, hybrid palliation, or cardiac transplant for patients with an intact atrial septum (P < 0.05), moderate to severe tricuspid regurgitation (P < 0.05), or low birth weight defined as less than 2 kg (P < 0.05). Hospice was preferred in low-birth-weight infants over hybrid palliation or cardiac transplantation (P < 0.05). Hospice was recommended over any other palliation for premature infants (less than 30 weeks gestation), chromosomal abnormalities, or end-organ dysfunction (P < 0.05). Conclusions. This survey demonstrates that different palliative options are primarily recommended by caretakers based upon institutional location and patient characteristics. Prospective comparative trials may force a rethinking of this approach over time.

The 2008 AHA/ACC Adult Congenital Heart Disease (CHD) guidelines recommended 1-2 year cardiology follow up for nearly all patients with CHD; >2 year lapse in follow up among these patients has been linked to adverse outcomes. We hypothesize that patients are not achieving this goal and demographic

Although pediatric heart failure is generally a chronic, progressive disorder, recovery of ventricular function may occur with some forms of cardiomyopathy. Guidelines for the management of chronic heart failure in adults and children have recently been published by the International Society for Heart and Lung Transplantation the American College of Cardiology, and the American Heart Association. The primary aim of heart failure therapy is to reduce symptoms, preserve long-term ventricular performance, and prolong survival primarily through antagonism of the neurohormonal compensatory mechanisms. Because some medications may be detrimental during an acute decompensation, physicians who manage these patients as inpatients must be knowledgeable about the medications and therapeutic goals of chronic heart failure treatment. Understanding the mechanisms of chronic heart failure may foster improved understanding of the treatment of decompensated heart failure.

The survival of patients with congenital heart disease (CHD) has significantly improved during the past several decades (the Figure). This accomplishment is directly attributable to the net effect of individual therapeutic successes from the innovative work of surgeons and cardiologists and the courageous devotion of patients and families. However, as the field moves forward, the need for evidence-based medicine derived from multi-institutional collaborations to address more subtle and complex questions such as functional outcome and quality of life and to provide data to guide individual practitioners has become paramount. Recognizing this, the National Heart, Lung, and Blood Institute (NHLBI) established the Pediatric Heart Network (PHN) in 2001 to provide a collaborative platform for conducting clinical studies. The need for evidence-based care and the ability of the PHN to provide this evidence were demonstrated by recent developments in the surgical management of hypoplastic left heart syndrome. Hypoplastic left heart syndrome was a uniformly fatal defect before Norwood’s innovative surgical approach in which the diminutive aorta is reconstructed using the pulmonary artery trunk and pulmonary blood flow is provided by a Blalock-Taussig shunt. 2 Recently, a previously described modification to this procedure in which pulmonary blood flow is provided by a right ventricle to pulmonary artery shunt has seen renewed interest. 3 This modification has been widely adopted but has yielded conflicting results. 4–7 In May 2005, the PHN launched the Single Ventricle Reconstruction (SVR) trial, a randomized trial comparing these 2 surgical strategies. 8 In just over 3 years, 555 infants were

Hypertrophic cardiomyopathy (HCM) is a common autosomal dominant condition with an increased risk of sudden cardiac death. Although clinical genetic testing can be used for confirmation of a clinical diagnosis as well as a predictive test, based on our clinical experience it is underutilized. Therefore, we developed and administered a questionnaire to assess potential determinants of parental interest in this testing. Of the 30 adult caregivers who participated, 80% had heard of genetic testing, whereas only 30% knew about genetic testing specifically for HCM. Once informed of the availability, 62% said they would consider testing in the future and 28% would consider it in the next year. Participants' younger age, higher education level, knowledge of carrier testing, and positive view of genetic testing were significantly associated with the participant considering HCM genetic testing for their child (por= 0.05). Based on a logistic regression model, age, education level, and knowing that HCM is an inherited disease were the best predictors of who would consider genetic testing. This study provides healthcare providers with a framework to understand caregivers' knowledge and views of genetic testing, which can be used to improve clinical care for pediatric HCM patients.

The purpose of this study was to assess the association of calcium replacement therapy with morbidity and mortality in infants after cardiac surgery involving cardiopulmonary bypass.Retrospective chart review.The cardiac intensive care unit at a tertiary care children's hospital.Infants undergoing cardiac surgery involving cardiopulmonary bypass between October 2002 and August 2004.None.Total calcium replacement (mg/kg calcium chloride given) for the first 72 postoperative hours was measured. Morbidity and mortality data were collected. The total volume of blood products given during the first 72 hrs was recorded. Infants with confirmed chromosomal deletions at the 22q11 locus were noted. Correlation and logistic regression analyses were used to generate odds ratios and 95% confidence intervals, with p.05 being significant. One hundred seventy-one infants met inclusion criteria. Age was 4 +/- 3 months and weight was 4.9 +/- 1.7 kg at surgery. Six infants had deletions of chromosome 22q11. Infants who weighed less required more calcium replacement (r = -.28, p.001). Greater calcium replacement correlated with a longer intensive care unit length of stay (r = .27, p.001) and a longer total hospital length of stay (r = .23, p = .002). Greater calcium replacement was significantly associated with morbidity (liver dysfunction [odds ratio, 3.9; confidence interval, 2.1-7.3; p.001], central nervous system complication [odds ratio, 1.8; confidence interval, 1.1-3.0; p = .02], infection [odds ratio, 1.5; confidence interval, 1.0-2.2; p.04], extracorporeal membrane oxygenation [odds ratio, 5.0; confidence interval, 2.3-10.6; p.001]) and mortality (odds ratio, 5.8; confidence interval, 5.8-5.9; p.001). Greater calcium replacement was not associated with renal insufficiency (odds ratio, 1.5; confidence interval, 0.9-2.3; p = .07). Infants with1 sd above the mean of total calcium replacement received on average fewer blood products than the total study population.Greater calcium replacement is associated with increasing morbidity and mortality. Further investigation of the etiology and therapy of hypocalcemia in this population is warranted.

Clinical trials are abundant in adult cardiovascular medicine; however, they are rare in pediatric cardiology. Pediatric cardiac trial design may be impacted by the heterogeneous nature of the underlying cardiac defects, as well as by a strong emotional response from parents whose child will undergo a surgical intervention. The purpose of this study was to assess factors that may have an impact on parents considering enrollment of their child in a clinical trial at the time of surgical intervention. A voluntary, self-administered questionnaire (14 questions) was provided to parents of children 16 years of age or younger during the preadmission testing period. Demographic and procedure-related variables were collected for each patient. A total of 119 surveys were analyzed over a 1.5-year period. Only 8% of the parents had their child participate in a clinical trial in the past. Fifty-six percent of the parents preferred that their child's cardiologist or surgeon explain clinical trial details, with 23% preferring the principal investigator and 3% preferring the research coordinator. Fifty percent of the parents were favorably disposed to participate in a clinical trial if the drug or device was currently used by their child's doctor, and 19% were encouraged to participate if the drug or device was approved for use in adults. The majority of parents (64%) preferred to be asked about participating in a trial within 1 month prior to the planned procedure, and 40% preferred to discuss trial details at a remote time in an outpatient location. Sixty-three percent of parents believed that most of the medications currently used in children were already approved by the Food and Drug Administration. Most parents (91%) believed that clinical trials conducted in children will help improve pediatric health care; 74% believed that their child may receive potential benefit from enrolling in a trial. Finally, 43% believed that funding for trials should come from government and health care agencies, as opposed to pharmaceutical companies (24%). This survey reveals the importance of the attending physician and timing in educating parents regarding a cardiac critical care clinical trial. These data may impact the design and successful conduct of future trials.

OBJECTIVE Hyperglycemia in critical care populations has been shown to be a risk factor for increased morbidity and mortality. Minimal data exist in postoperative pediatric cardiac patients. The goal of this study was to determine whether hyperglycemia in the postoperative period was associated with increased morbidity or mortality. DESIGN Retrospective chart review. SETTING Tertiary care, free-standing pediatric medical center with a dedicated cardiac intensive care unit. PATIENTS We included 184 patients

Background Modification of the Norwood procedure has been reported to improve immediate postoperative mortality compared with the classic Norwood. Interstage mortality has not been shown to be improved with the modified Norwood probably because of the small number of patients from each institution. The goal of this study was to determine if meta-analysis would provide sufficient data to prove statistical difference in interstage mortality for the modified Norwood procedure. Methods PubMed was searched using six different terms individually for articles from January 2003 to October 2004. Manuscripts that compared the classic to modified Norwood were reviewed. Mantel-Haenszel analysis was used to evaluate the relationship between treatment method and mortality stratified across hospitals. The Breslow-Day procedure tested homogeneity of odds ratio across hospitals. Separate analyses were performed for inpatient and interstage periods. Results A total of 4,545 citations was screened. Five manuscripts met the criteria. Seventy-two patients undergoing classic Norwood and 84 patients undergoing modified Norwood survived to initial hospital discharge. The Breslow-Day statistic supported homogeneity of odds ratios for survival across hospitals (χ 2 = 2.09, df=4, p = 0.72). Odds of interstage death was 11.6 times greater (2.2 to 62.1, 95% CI) for the classic Norwood compared with the modified Norwood procedure. This difference was statistically significant by the Mantel-Haenszel χ 2 (11.0, p = 0.001). The Breslow-Day statistic supported homogeneity of the odds ratios across hospitals (χ 2 = 3.1, df=4, p = 0.53). Conclusions The modified Norwood procedure has a significantly lower interstage mortality compared with the classic Norwood procedure. A large randomized study is needed to determine whether these results remain consistent.

Cerebral saturation (SCO2) monitors are noninvasive tools that continuously measure saturations in the cerebral cortex, a predominately venous bed. The purpose of this study was to see if a trend existed between measurements of SCO2 and mixed venous saturation values (SVO2) for patients on extracorporeal life support (ECLS). Six patients required ECLS for cardiac failure after congenital cardiac surgery, and one patient required ECLS for pulmonary failure. Patients were divided into two groups, those without systemic/pulmonary venous mixing (n=3, Group I) and those with mixing due to an intraatrial shunt or left ventricular vent (n=4, Group II). The age of patients was 0.49-0.5 years (mean9-SD), weight was 5.29-2.3 kg, and time on ECLS was 8.39-4.8 days. No significant abnormalities were seen on head imaging. A total of 786 paired data points were collected. Mean values were different; however, there was a significant trend between SCO2 and SVO2 for the entire sample (R2=0.66, p B-0.001). Cerebral saturation trends follow mixed venous trends and, therefore, may be helpful in combination with other physical and laboratory findings in the care of the critically ill child.

To identify factors associated with mortality in children with heart disease managed with extracorporeal membrane oxygenation (ECMO).Retrospective chart review.Tertiary care university-affiliated children's hospital.All pediatric cardiac intensive care unit patients managed with ECMO between January 1, 1995, and June 30, 2001.None.During the study period, 137 patients were managed with ECMO in the pediatric cardiac intensive care unit. Of the 137 patients, 80 (58%) survivedor =24 hrs after decannulation, and 53 (39%) survived to hospital discharge. Patients managed with ECMO following cardiac surgery were analyzed separately from patients not in the postoperative period. Factors associated with an increased probability of mortality in the postoperative patients were age1 month, male gender, longer duration of mechanical ventilation before ECMO, and development of renal or hepatic dysfunction while on ECMO. Single ventricle physiology and failure to separate from cardiopulmonary bypass were not associated with an increased risk of mortality. Cardiac physiology and indication for ECMO were not associated with mortality rate. Although longer duration of ECMO was not associated with increased mortality risk, patients with longer duration of ECMO were less likely to survive without heart transplantation.In a series of 137 patients managed with ECMO in a pediatric cardiac intensive care unit, survival to hospital discharge was 39%. In postoperative patients only, mortality risk was increased in males, patients1 month old, patients with a longer duration of mechanical ventilation before initiation of ECMO, and patients who developed renal or hepatic failure while on ECMO.