Massive left ventricular hypertrophy in pediatric hypertrophic cardiomyopathy increases adverse event risk, yet significant regression is seen in nearly a quarter of patients.
What is the natural history and clinical trajectory of massive left ventricular hypertrophy in pediatric hypertrophic cardiomyopathy?
In pediatric HCM, massive LVH is associated with early childhood diagnosis and sarcomeric disease, carrying an increased risk of adverse events despite significant regression occurring in nearly 25% of patients.
Absolute Event Rate: 0% vs 0%
BACKGROUND: Massive left ventricular hypertrophy (LVH) is a risk factor for sudden cardiac death in children with hypertrophic cardiomyopathy (HCM), but little is understood about its natural history. METHODS: Patients with pediatric-onset HCM identified from 2 registries (SHaRe Sarcomeric Human Cardiomyopathy Registry and IPHCC International Paediatric Hypertrophic Cardiomyopathy Consortium) with or without massive LVH were compared. Massive LVH was defined as absolute maximal left ventricular wall thickness (MLVWT) ≥30 mm or MLVWT z score ≥+20 at 5 mm less than the largest recorded MLVWT in 25 patients (22%). CONCLUSIONS: In pediatric HCM, massive LVH disproportionately affects those diagnosed in early childhood with sarcomeric disease, with increased risk for adverse events. Significant MLVWT regression is seen in nearly a quarter of patients.
Recent April 17, 2026 Circulation publication; important for pediatric cardiology and adult transition; part of latest AHA journal releases.
Przybylski et al. (Fri,) reported a other. Massive left ventricular hypertrophy in pediatric hypertrophic cardiomyopathy increases adverse event risk, yet significant regression is seen in nearly a quarter of patients.