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.

Acute Kidney Injury Requiring Dialysis After Pediatric Heart Transplant.

Background: Acute kidney injury (AKI) is a common complication of pediatric heart transplant, with a subset of patients developing severe AKI requiring dialysis (AKI-D). We aimed to identify the epidemiology, risk factors, and outcomes of postoperative AKI-D in pediatric heart transplant recipients.

Methods: We retrospectively identified all pediatric first-time, single-organ heart transplants at our institution from 2014 to 2022.

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.

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.

Heart transplantation in neonatal Marfan syndrome: Saving life in a rare and fatal condition.

Background: Neonatal Marfan syndrome is a rare disease with mortality in the first year of life reported as high as 95% predominantly due to progressive heart failure from valvar regurgitation and cardiomyopathy. Multisystem involvement and uncertain prognosis have historically precluded transplant candidacy, and current management options are of limited success.

Case Report: We present a baby girl with a postnatal diagnosis of neonatal Marfan syndrome who at 1 year of age underwent mitral valve and tricuspid valve repair with postoperative profound left ventricular and moderate right ventricular dysfunction necessitating biventricular assist device (BiVAD) support and subsequent heart transplant.

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.

"Acquired" Brugada syndrome in a cardiac allograft.

Introduction: Brugada syndrome is an inherited channelopathy characterized by arrhythmia and an increased risk of sudden cardiac death (SCD). Implantation of a defibrillator for primary or secondary prevention is the only effective strategy to decrease the risk of SCD in Brugada syndrome. We present a case in which a cardiac donor had a pathogenic variant for Brugada syndrome, discovered on genetic testing after transplantation.

Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk of Death or Heart Transplantation.

Background: Left ventricular noncompaction (LVNC) is associated with genetic and phenotypic variability that influences outcomes. We aimed to identify risk factors for death or heart transplantation (HTx) in a paediatric LVNC cohort.

Methods: We reviewed patients < 18 years of age (2001-2018) with LVNC, either isolated (I-LVNC) or with dilated phenotype (D-LVNC), and at least mildly reduced left ventricular ejection fraction (EF).

When is enough, enough? Exploring ethical and team considerations in paediatric cardiac care dilemmas.

Purpose Of Review: Therapies for paediatric congenital and acquired heart disease continue to evolve and the appropriateness of pursuing life sustaining interventions at margins of standard therapy is ethically challenging.

Recent Findings: With ongoing emphasis on shared decision making, recent literature explored physician and parental perspectives on communication with families and offering interventions for complex congenital heart disease and advanced heart failure. The inclusion of parental values and views in this process is now widely accepted.

Barriers to effective, safe communication and workflow between nurses and non-consultant hospital doctors during out-of-hours.

Objective: This study aimed to evaluate the nature and type of communication and workflow arrangements between nurses and doctors out-of-hours (OOH). Effective communication and workflow arrangements between nurses and doctors are essential to minimize risk in hospital settings, particularly in the out-of-hour's period. Timely patient flow is a priority for all healthcare organizations and the quality of communication and workflow arrangements influences patient safety.