Pediatric Hypertrophic Cardiomyopathy: The High-Risk Profile of Massive Left Ventricular Hypertrophy

Background

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy, affecting 1 in 500 individuals globally. In children, massive left ventricular hypertrophy (LVH), defined as a maximal left ventricular wall thickness (MLVWT) ≥30 mm or a z score ≥+20, is a recognized risk factor for sudden cardiac death (SCD). However, the natural history and long-term outcomes of massive LVH in pediatric HCM remain poorly characterized, creating gaps in risk stratification and management strategies.

Study Design