Chronotropic Response to Exercise is Decreased in Patients With Congenital Heart Disease Compared to Cardiomyopathy Following Pediatric Heart Transplantation.

Background: Two common indications for pediatric heart transplantation are congenital heart disease and cardiomyopathy. Prior studies suggest differences in chronotropy on cardiopulmonary exercise testing outcomes depending on indication for heart transplantation. We aimed to determine whether the number of pretransplant sternotomies is associated with differences in heart rate response during exercise testing.

Indication for Pediatric Heart Transplant Affects Longitudinal Chronotropy on Cardiopulmonary Exercise Testing.

Studies have suggested that pediatric patients with heart transplants (HT) due to congenital heart disease (CHD) perform differently on cardiopulmonary exercise testing compared to pediatric patients with HT due to cardiomyopathy (CM). However, it is not known if this relationship changes over time. The aim of this study was to examine the differences in cardiopulmonary exercise test (CPET) parameters over time between patients with HT due to CHD versus CM.

Effect of body mass index on exercise capacity following pediatric heart transplantation.

Background: Obesity and impaired exercise tolerance following heart transplantation increase the risk of post-transplant morbidity and mortality. The aim of this study was to evaluate the effect of body mass index on markers of exercise capacity in pediatric heart transplant recipients and compare this effect with a healthy pediatric cohort.

Methods: A retrospective analysis of cardiopulmonary exercise test data between 2004 and 2022 was performed.

Rate of Change in Cardiac Magnetic Resonance Imaging Measures Is Associated With Death in Duchenne Muscular Dystrophy.

Background: Cardiovascular disease is the leading cause of death among patients with Duchenne muscular dystrophy (DMD). Identifying patients at risk of early death could allow for increased monitoring and more intensive therapy. Measures that associate with death could serve as surrogate outcomes in clinical trials.

International Pediatric Transplant Association (IPTA) position statement supporting prioritizing pediatric recipients for deceased donor organ allocation.

A position statement of the International Pediatric Transplant Association endorsing prioritizing pediatric recipients for deceased donor organ allocation, examining the key ethical arguments that serve as the foundation for that position, and making specific policy recommendations to support prioritizing pediatric recipients for deceased donor organ allocation globally.

Right Ventricular Remodeling Assessed by MRI in Duchenne Muscular Dystrophy.

Background: In Duchenne muscular dystrophy (DMD), the right ventricle (RV) tends to be relatively well preserved, but characterization remains difficult due to its complex architecture. Tissue phase mapping (TPM) is a phase contrast cine MRI technique that allows for multidirectional assessment of myocardial velocities.

Purpose: To use TPM to elucidate relationships between myocardial structure, function, and clinical variables in DMD.

A Systematic Review of Adherence to Immunosuppression among Pediatric Heart Transplant Patients.

After pediatric heart transplant, commitment to lifelong immunosuppression is crucial to maintaining graft health. However, a review of the current literature surrounding adherence to immunosuppression in pediatric heart transplant patients is lacking. This systematic review aims to summarize the current landscape of adherence to immunosuppression in pediatric heart transplant patients.

Diversity of Dystrophin Gene Mutations and Disease Progression in a Contemporary Cohort of Duchenne Muscular Dystrophy.

Abnormal dystrophin production due to mutations in the dystrophin gene causes Duchenne Muscular Dystrophy (DMD). Cases demonstrate considerable genetic and disease progression variability. It is unclear if specific gene mutations are prognostic of outcomes in this population.

Current Practices in Treating Cardiomyopathy and Heart Failure in Duchenne Muscular Dystrophy (DMD): Understanding Care Practices in Order to Optimize DMD Heart Failure Through ACTION.

Cardiac disease has emerged as a leading cause of mortality in Duchenne muscular dystrophy in the current era. This survey sought to identify the diagnostic and therapeutic approach to DMD among pediatric cardiologists in Advanced Cardiac Therapies Improving Outcomes Network. Pediatric cardiology providers within ACTION (a multi-center pediatric heart failure learning network) were surveyed regarding their approaches to cardiac care in DMD.

Implantable Cardioverter Defibrillator Use in Males with Duchenne Muscular Dystrophy and Severe Left Ventricular Dysfunction.

Duchenne muscular dystrophy (DMD) is characterized by myocardial fibrosis and left ventricular (LV) dysfunction. Implantable cardioverter defibrillator (ICD) use has not been characterized in this population but is considered for symptomatic patients with severe LV dysfunction (SLVD) receiving guideline-directed medical therapy (GDMT). We evaluated ICD utilization and efficacy in patients with DMD.

The landscape of cardiovascular care in pediatric cancer patients and survivors: a survey by the ACC Pediatric Cardio-Oncology Work Group.

Objective: To enhance the understanding of cardiovascular care delivery in childhood cancer patients and survivors.

Study Design: A 20-question survey was created by the Pediatric Cardio-oncology Work Group of the American College of Cardiology (ACC) Cardio-oncology Section to assess the care, management, and surveillance tools utilized to manage pediatric/young adult cardio-oncology patients. The survey distribution was a collaborative effort between Cardio-oncology Section and membership of the Adult Congenital and Pediatric Cardiology Section (ACPC) of the ACC.

Electrocardiograms for cardiomyopathy risk stratification in children with anthracycline exposure.

Background: Early recognition of anthracycline-induced cardiomyopathy may reduce morbidity and mortality in children, but risk stratification tools are lacking. This study evaluates whether electrocardiogram (ECG) changes precede echocardiographic abnormalities in children with anthracycline-induced cardiomyopathy.

Methods: We performed a retrospective analysis of 589 pediatric cancer patients who received anthracyclines at a tertiary referral center.

Risk Factors for Cardiac and Non-cardiac Causes of Death in Males with Duchenne Muscular Dystrophy.

As survival and neuromuscular function in Duchenne muscular dystrophy (DMD) have improved with glucocorticoid (GC) therapy and ventilatory support, cardiac deaths are increasing. Little is known about risk factors for cardiac and non-cardiac causes of death in DMD. A multi-center retrospective cohort study of 408 males with DMD, followed from January 1, 2005 to December 31, 2015, was conducted to identify risk factors for death.

Use of the terminal complement inhibitor eculizumab in paediatric heart transplant recipients.

Antibody-mediated rejection is a major clinical challenge that limits graft survival. Various modalities of treatment have been reported in small studies in paediatric heart recipients. A novel approach is to use complement-inhibiting agents, such as eculizumab, which inhibits cleavage of C5 to C5a thereby limiting the formation of membrane attack complex and terminal complement-mediated injury of tissue-bound antibodies.

Use of advanced heart failure therapies in Duchenne muscular dystrophy.

Background: As survival and neuromuscular function in Duchenne Muscular Dystrophy (DMD) improve with glucocorticoid therapy and respiratory advances, the proportion of cardiac deaths is increasing. Little is known about the use and outcomes of advanced heart failure (HF) therapies in this population.

Methods: A retrospective cohort study of 436 males with DMD was performed, from January 1, 2005-January 1, 2018, with the primary outcome being use of advanced HF therapies including: implantable cardioverter defibrillator (ICD), left ventricular assist device (LVAD), and heart transplantation (HTX).

Characteristics of Clinically Diagnosed Pediatric Myocarditis in a Contemporary Multi-Center Cohort.

The objective of this study was to describe a contemporary cohort of pediatric patients hospitalized for clinically suspected myocarditis. A retrospective chart review was performed at seven tertiary pediatric hospitals. Electronic medical records were searched between 2008 and 2012 for patients ≤18 years admitted with an ICD-9 code consistent with myocarditis.

Risk Factors for Cardiac Arrest or Mechanical Circulatory Support in Children with Fulminant Myocarditis.

In children with fulminant myocarditis (FM), we sought to describe presenting characteristics and clinical outcomes, and identify risk factors for cardiac arrest and mechanical circulatory support (MCS). A retrospective review of patients with FM admitted at our institution between January 1, 2004, and June 31, 2015, was performed. We compared characteristics and outcomes of FM patients who received cardiopulmonary resuscitation (CPR) and/or were placed on MCS (CPR/MCS group) to those who did not develop these outcomes (Control group).

Heart transplantation for the failing Fontan.

Background: Patients with failing Fontan circulation are at high risk for complications after heart transplantation (HTx) because of multiple prior operations, elevated panel reactive antibody, hepatic dysfunction, coagulopathy, protein-losing enteropathy (PLE), and poor nutrition. The purpose of this review was to evaluate the outcome of HTx for these patients, including those who are status post-Fontan conversion.

Methods: Of 206 heart transplants at Ann & Robert H.

Bilateral coronary artery fistulas and left ventricle noncompaction in a neonate: diagnosis and management.

This report describes a neonate with heart failure resulting from bilateral coronary artery fistulas and left ventricle noncompaction. The patient underwent successful surgical ligation of the fistulas.

Real-time three-dimensional echocardiographic assessment of left ventricular systolic dyssynchrony in healthy children.

Background: The use of resynchronization therapy for the treatment of left ventricular (LV) systolic dysfunction in children has been expanding. Because QRS duration is not a reliable indicator of the presence or severity of dyssynchrony in every case, additional methods of quantitation of dyssynchrony are needed. The purpose of this study was threefold: (1) to define normal values for LV real-time quantitative three-dimensional echocardiographic (3DE) dyssynchrony indices (DIs), (2) to analyze the feasibility and observer variability of 3DE DIs in a wide range of children, and (3) to determine the effects of age, heart rate, body surface area, and LV end-diastolic volume on these parameters.

Late sinus and atrial tachycardia after pediatric heart transplantation might predict poor outcome.

Our objective was to examine clinical/electrocardiogram (ECG) predictors and outcomes of arrhythmias beyond 1 year after pediatric heart transplantation (HTx). We performed a retrospective chart review of 94 1-year HTx survivors, 1988-2006. Clinical records identified patients with arrhythmias occurring >1 year after HTx requiring pharmacotherapy, excluding acute rejection.

A comparison of Tei index versus systolic to diastolic ratio to detect left ventricular dysfunction in pediatric patients.

Background: The aim of this study was to determine the accuracy, sensitivity, specificity, predictive values, and likelihood ratios of the left ventricular (LV) Tei index (TX) and the ratio of systolic duration to diastolic duration (S/D) to detect ventricular dysfunction.

Methods: LV systolic and diastolic function were studied in a cohort of 68 children, 25 normal and 43 abnormal. Systolic dysfunction was defined as the presence of all of 3 criteria: ejection fraction < 50%, fractional shortening < 27%, and tissue Doppler systolic S wave < 1.

Transcription repression and blocks in cell cycle progression in hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is characterized by abnormally developed atrial septum and a severe underdevelopment of the left side of the heart. Despite significant advances in its surgical management, little is known about the molecular abnormalities in this syndrome. To gain molecular insights into HLHS, expression profiling by gene-chip microarray (Affymetrix U133 2.