Modifiable Health Behaviours in Children with HCM: Lessons from the Heart Health Survey.

Obesity factors into hypertrophic cardiomyopathy (HCM)-related risk as a disease modifying environmental factor. Behaviours such as diet and sleep are seldom reported upon in children with HCM. It was our aim to report on these factors in this population.

Bioethics at the bedside: considering the adolescent voice in withdrawal of life-sustaining therapy.

Durable mechanical circulatory devices are commonly used to support children and adolescents in end-stage heart failure. However, these patients remain at high risk of acute medical complications, which may lead to significant impairment in functional capacity, altered quality of life, or death. We explore the incorporation of adolescent directives into medical decision-making in this scenario through a clinical case vignette.

Durable left ventricular assist devices in pediatrics: impact of body size on outcomes and size limitations.

Despite the range of body sizes in children, few ventricular assist devices (VAD) exist to support pediatric patients with end-stage heart failure. Large registry data identified weight < 20 kg to be associated with higher rates of VAD-related stroke, compared to > 40 kg. Moreover, patients < 1 years of age experience the highest post-implant mortality, with 1-year survival improving in an age-dependent manner.

Transcatheter Axial Pump Use in Pediatric Patients on Veno-Arterial Extracorporeal Membrane Oxygenation: An ACTION Collaborative Experience.

We report the largest pediatric multicenter experience with Impella pump use and peripheral veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support. Utilizing the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) collaborative database, we conducted a retrospective, multicenter study of all patients with cardiogenic shock requiring VA-ECMO support with subsequent Impella implant between October 2014 and December 2021. The primary outcome was defined as death while on Impella support.

Outcomes of Children With Hypoplastic Left Heart Syndrome and Heart Failure on Medical Therapy.

Background: Systemic right ventricle (RV) dysfunction is associated with lower transplant-free survival (TFS) in hypoplastic left heart syndrome (HLHS), but the likelihood of functional improvement and utility of heart failure (HF) medications is not understood.

Objectives: The authors aimed to describe TFS, HF medication use, and surgical interventions in HLHS patients with RV dysfunction with and without subsequent improvement in function.

Methods: The SickKids HF Database is a retrospective cohort that includes all pediatric HLHS patients with RV dysfunction lasting >30 days.

Nutritional status and cannula infections in pediatric patients on ventricular assist device support.

Background: Ventricular assist devices (VADs) are used to bridge pediatric patients to heart transplantation. Paracorporeal VADs require the placement of cannulas, which can create an environment for infections. We examined cannula infections in pediatric VAD patients and the role of nutritional status.

Exploring Health-Related Quality of Life in Children With Hypertrophic Cardiomyopathy and Relationship to Physical Activity.

Background: Hypertrophic cardiomyopathy is a burdensome condition that inflicts both physical and psychological impairment on those with the disease, negatively impacting health-related quality of life (HRQoL). Given the abundance of evidence suggesting a role of physical activity (PA) in modulating HRQoL in healthy populations of children, we sought to determine the relationship between HRQoL and PA in children diagnosed with hypertrophic cardiomyopathy.

Methods And Results: A multicenter prospective observational cohort study was conducted, with patients with hypertrophic cardiomyopathy aged 10 to 19 years being provided a wrist-worn activity tracker (Fitbit Charge HR) to wear for 14 days.

A prospective multicenter feasibility study of a miniaturized implantable continuous flow ventricular assist device in smaller children with heart failure.

Background: There is no FDA-approved left ventricular assist device (LVAD) for smaller children permitting routine hospital discharge. Smaller children supported with LVADs typically remain hospitalized for months awaiting heart transplant-a major burden for families and a challenge for hospitals. We describe the initial outcomes of the Jarvik 2015, a miniaturized implantable continuous flow LVAD, in the NHLBI-funded Pumps for Kids, Infants, and Neonates (PumpKIN) study, for bridge-to-heart transplant.

Unique Aspects of Hypertrophic Cardiomyopathy in Children.

Hypertrophic cardiomyopathy (HCM) is a primary heart muscle disease characterized by left ventricular hypertrophy that can be asymptomatic or with presentations that vary from left ventricular outflow tract obstruction, heart failure from diastolic dysfunction, arrhythmias, and/or sudden cardiac death. Children younger than 1 year of age tend to have worse outcomes and often have HCM secondary to inborn errors of metabolism or syndromes such as RASopathies. For children who survive or are diagnosed after 1 year of age, HCM outcomes are often favourable and similar to those seen in adults.

Administrative Databases: Friend or Foe in Paediatric Cardiomyopathy.

Background: Cardiomyopathy (CM) is a rare childhood disease associated with morbidity and mortality. Limited data exist on paediatric CM in Canada. Given the rare nature, single-centre studies are not sufficiently powered to address important questions.

Exploring the use of a digital therapeutic intervention to support the pediatric cardiac care journey: Qualitative study on clinician perspectives.

Pediatric heart disease currently effects over one million infants, children, and adolescents in the United States alone. Unlike the adult population, pediatric patients face a more uncertain path with factors relating to their growth and maturation creating levels of complexity to their care management. With mobile phones increasingly being utilized amongst adolescents, digital therapeutics tools could provide a platform to help patients and families manage their condition.

Textile-based Wearable to Monitor Heart Activity in Paediatric Population: A Pilot Study.

Background: Cardiac monitoring for children with heart disease still employs common clinical techniques that require visits to hospital either in an ambulatory or inpatient setting. Frequent cardiac monitoring, such as heart rate monitoring, can limit children's physical activity and quality of life. The main objective of this study is to evaluate the performance of a textile-based device (SKIIN) in measuring heart rate (HR) in different tasks: lying down, sitting, standing, exercising, and cooling down.

Reducing donor acceptance practice variation - Learnings from a discussion forum.

Purpose: Although waitlist mortality is unacceptably high, nearly half of donor heart offers are rejected by pediatric heart transplant centers. The Advanced Cardiac Therapy Improving Outcome Network (ACTION) and Pediatric Heart Transplant Society (PHTS) convened a multi-institutional donor decision discussion forum (DDDF) aimed at assessing donor acceptance practices and reducing practice variation.

Methods: A 1-h-long virtual DDDF for providers across North America, the United Kingdom, and Brazil was held monthly.

Symptomatic presentation influences outcomes in pediatric restrictive cardiomyopathy.

Introduction: Children with restrictive cardiomyopathy (RCM) traditionally have a poor prognosis, with most patients either dying or requiring heart transplantation within 2 years of diagnosis. The development of symptoms in RCM suggests advanced disease. However, as screening practices evolve and lead to diagnosis of early disease, identifying appropriate timing of transplant listing becomes increasingly important.

School age and adolescent heart failure following the Norwood procedure.

Background: Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described.

Methods: We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure.

Case report: A case of spinal muscular atrophy in a preterm infant: risks and benefits of treatment.

Spinal muscular atrophy (SMA) is a neuromuscular genetic disorder caused by the loss of lower motor neurons leading to progressive muscle weakness and atrophy. With the rise of novel therapies and early diagnosis on newborn screening (NBS), the natural history of SMA has been evolving. Earlier therapeutic interventions can modify disease outcomes and improve survival.

Risk of Sudden Death in Patients With RASopathy Hypertrophic Cardiomyopathy.

Background: Genetic defects in the RAS/mitogen-activated protein kinase pathway are an important cause of hypertrophic cardiomyopathy (RAS-HCM). Unlike primary HCM (P-HCM), the risk of sudden cardiac death (SCD) and long-term survival in RAS-HCM are poorly understood.

Objectives: The study's objective was to compare transplant-free survival, incidence of SCD, and implantable cardioverter-defibrillator (ICD) use between RAS-HCM and P-HCM patients.

Pitfalls and Possibilities of Ventricular Assist Device Support in Congenitally Corrected Transposition of the Great Arteries in Children.

Congenitally corrected transposition of the great arteries (ccTGAs) represents a complex form of congenital heart disease that is associated with several cardiac complications. Herein is a case series of three children with ccTGA and ventricular assist device (VAD) inserted for systemic right ventricle failure at a single institution. All patients remained hemodynamically stable postimplant and were successfully discharged from the intensive care unit to undergo postoperative rehabilitation.

Differentiation of COVID-19-Associated Multisystem Inflammatory Syndrome From Kawasaki Disease With the Use of Cardiac Biomarkers.

Background: Multisystem inflammatory syndrome in children (MIS-C) after COVID-19 shares clinical similarities to Kawasaki disease (KD). We sought to determine whether cardiac biomarker levels differentiate MIS-C from KD and their association with cardiac involvement.

Methods: Subjects included 38 MIS-C patients with confirmed prior COVID-19 and 32 prepandemic and 38 contemporaneous KD patients with no evidence of COVID-19.