The Natural History of Massive Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: A Multiregistry Analysis - PubMed
3 hours ago
- #ventricular hypertrophy
- #pediatric cardiology
- #hypertrophic cardiomyopathy
- Massive left ventricular hypertrophy (LVH) is a risk factor for sudden cardiac death in children with hypertrophic cardiomyopathy (HCM), but its natural history is poorly understood.
- A multiregistry analysis compared pediatric HCM patients with and without massive LVH (defined as MLVWT ≥30 mm or z score ≥+20 before age 18).
- Patients with massive LVH were diagnosed younger, had higher prevalence of sarcomeric genetic variants, and increased risks of HCM-related mortality, major adverse cardiac events, major ventricular arrhythmia, and heart failure.
- In serial measurements, absolute MLVWT increased significantly over time, but z scores did not differ; nearly a quarter of patients showed MLVWT regression (>5 mm decrease).
- Conclusions: Massive LVH in pediatric HCM is associated with early childhood diagnosis, sarcomeric disease, and higher adverse event risks, with significant regression observed in many cases.