Abstract 13638: Risk Stratification for Sudden Death in Children With Hypertrophic Cardiomyopathy. An International Multi-Center Study

Introduction: Children with Hypertrophic Cardiomyopathy (HCM) are at high risk for sudden cardiac death (SCD). Yet, risk stratification for SCD is poorly defined in children with HCM. Methods: We collected retrospective data on HCM children at 35 centers. Inclusion criteria were age ≤20 years at diagnosis with HCM ± an ICD. Patients without ICD were included if ≥ 5 years at diagnosis with follow up ≥ 5 years. Patients with co-existent syndromes and metabolic disorders were excluded. Patients were classified as malignant HCM if they had 1) secondary prevention (SP) ICD, 2) primary prevention (PP) ICD with appropriate intervention (AI) or 3) aborted cardiac arrest without an ICD. All others were considered benign. RS was performed using 1) family history (FH) of SCD 2) unexplained syncope; 3) interventricular septal (IST) and posterior (PWT) Left ventricular (LV) wall thickness 4) non-sustained VT (NSVT) on ambulatory monitoring; 5) abnormal blood pressure response to exercise (ABPR) and 6) ECG markers of L...