A comprehensive pediatric cardio-oncology program: a single institution approach to cardiovascular care for pediatric patients with cancer and childhood cancer survivors.

Cardiovascular complications related to cancer therapies are broad and variable in onset. These complications are the leading cause of non-cancer related morbidity and mortality in childhood cancer survivors and can also impact ongoing cancer treatment. Despite this understanding, dedicated cardio-oncology programs are lacking in pediatric cardiology.

Uncertain benefit of statins in pediatric heart transplant recipients: A PHTS analysis.

Background: Cardiac allograft vasculopathy (CAV) is a leading cause of graft failure in pediatric heart transplant recipients (HTRs). Early statin use has been shown to reduce CAV incidence and all-cause mortality in adult HTRs. We sought to evaluate the contemporary prevalence and trends of statin use in pediatric HTRs and the association between statin use with CAV development and graft failure.

Treatment Strategies for Cardiomyopathy in Children: A Scientific Statement From the American Heart Association.

This scientific statement from the American Heart Association focuses on treatment strategies and modalities for cardiomyopathy (heart muscle disease) in children and serves as a companion scientific statement for the recent statement on the classification and diagnosis of cardiomyopathy in children. We propose that the foundation of treatment of pediatric cardiomyopathies is based on these principles applied as personalized therapy for children with cardiomyopathy: (1) identification of the specific cardiac pathophysiology; (2) determination of the root cause of the cardiomyopathy so that, if applicable, cause-specific treatment can occur (precision medicine); and (3) application of therapies based on the associated clinical milieu of the patient. These clinical milieus include patients at risk for developing cardiomyopathy (cardiomyopathy phenotype negative), asymptomatic patients with cardiomyopathy (phenotype positive), patients with symptomatic cardiomyopathy, and patients with end-stage cardiomyopathy.

Managing Myocardial Bridge and Right Ventricular Outflow Tract Obstruction in an Adolescent With Hypertrophic Cardiomyopathy.

We report a case of symptomatic myocardial bridge in an adolescent with dynamic right ventricular outflow tract obstruction and history of congenital pulmonary valve stenosis as well as hypertrophic cardiomyopathy. Definitive treatment was surgical infundibular myectomy and coronary unroofing, resulting in improvement in right ventricular outflow tract gradient and ischemic symptoms.

The Prevalence and Association of Exercise Test Abnormalities With Sudden Cardiac Death and Transplant-Free Survival in Childhood Hypertrophic Cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) can be associated with an abnormal exercise response. In adults with HCM, abnormal results on exercise stress testing are predictive of heart failure outcomes. Our goal was to determine whether an abnormal exercise response is associated with adverse outcomes in pediatric patients with HCM.

Rejection surveillance in pediatric heart transplant recipients: Critical reflection on the role of frequent and long-term routine surveillance endomyocardial biopsies and comprehensive review of non-invasive rejection screening tools.

Background: Despite significant medical advances in the field of pediatric heart transplantation (HT), acute rejection remains an important cause of morbidity and mortality. Endomyocardial biopsy (EMB) remains the gold-standard method for diagnosing rejection but is an invasive, expensive, and stressful process. Given the potential adverse consequences of rejection, routine post-transplant rejection surveillance protocols incorporating EMB are widely employed to detect asymptomatic rejection.

Carvedilol Does Not Improve Exercise Performance in Fontan Patients: Results of a Crossover Trial.

Increased circulating catecholamines are associated with worse exercise performance in adult heart failure patients. Patients with Fontan physiology have increased circulating catecholamines and theoretically could benefit from beta blockade. We hypothesized that carvedilol would improve exercise performance in Fontan patients.

Center Donor Refusal Rate Is Associated With Worse Outcomes After Listing in Pediatric Heart Transplantation.

Background: Pediatric donor heart acceptability differs among transplant centers. However, the impact of center donor acceptance on waitlist and posttransplant outcomes has not been investigated. The aim of our study was to investigate associations between transplant center refusal rate (RR) and outcomes after listing.

A comprehensive strategy in donor acceptance: Impact on pediatric waitlist and heart transplant outcomes.

Significant inter- and intra-center practice variability is present in pediatric donor heart acceptability. This may contribute to variation in the donor refusal rate and may impact waitlist time, morbidity, mortality, and transplant rates. In order to reduce practice variability, our center developed and implemented a comprehensive strategy regarding donor acceptance in September 2017.

Heart transplantation in an infant with Williams-Beuren syndrome and rapidly progressive ischemic cardiomyopathy.

Ischemic cardiomyopathy with resultant refractory HF may occur in patients with WBS, often as the result of coronary involvement with SVAS. The rapid development of arteriopathy at a young age raises concerns regarding transplant candidacy due to progressive stenoses at other arterial sites with potential detrimental impact on long-term heart graft function. We report a 2-month-old male infant diagnosed with mild aortic stenosis during the neonatal period, but subsequently developed rapidly progressive supravalvar and coronary artery stenoses leading to cardiogenic shock due to myocardial ischemia.

Utilization and outcomes in biventricular assist device support in pediatrics.

Objective: Patients with biventricular assist devices (BiVADs) have worse outcomes than those with left ventricular assist devices (LVADs). It is unclear whether these outcomes are due to device selection or patient factors. We used propensity score matching to reduce patient heterogeneity and compare outcomes in pediatric patients supported with BiVADs with a similar LVAD cohort.

Utilization of Neurally Adjusted Ventilatory Assist (NAVA) Mode in Infants and Children Undergoing Congenital Heart Surgery: A Retrospective Review.

We assessed the feasibility and the impact of NAVA compared to conventional modes of mechanical ventilation in ventilatory and gas exchange parameters in post-operative children with congenital heart disease. Infants and children (age < 18 years) that underwent congenital heart surgery were enrolled. Patients were ventilated with conventional synchronized intermittent mechanical ventilation (SIMV) and subsequently transitioned to NAVA during their cardiovascular intensive care unit (CVICU) stay.