Pediatric Hypertrophic Cardiomyopathy: Survival and Prognostic Indicators in the Current Era

Purpose Our objective was to provide a contemporary analysis of a pediatric hypertrophic cardiomyopathy (HCM) cohort and determine risk factors independently associated with death or heart transplantation. Methods A retrospective review of patients aged 0-18 years who were diagnosed with HCM at our tertiary-level institution between January 1, 2001 and July 1, 2017 was conducted. Survival analysis using Cox proportional hazard models was used to identify risk factors associated with a combined primary end-point of death or transplantation. Results Of 177 patients with HCM, 120 (68%) were males and 101 (57%) had genetic or familial HCM. The median age at diagnosis was 6.3 years (IQR 0.3-12.8) and at follow-up was 13.1 years (IQR 4.3-17.3). Left ventricular (LV) outflow tract obstruction was observed in 65 patients (37%), cardiac surgery was performed in 21 (12%) after diagnosis, and 20 (11%) received an implantable cardioverter defibrillator (ICD). There were 18 (10%) deaths and 5 (3%) transplants with an overall 10-year transplant-free survival of 81% (95% CI 77-85%). Independent risk factors for death or transplantation included chromosomal abnormalities, smaller baseline LV end-diastolic dimension (EDD), and presenting with Stage C or D heart failure on diagnosis (Table 1). Conclusion Pediatric HCM patients have a relatively high rate of interventions as well as death and/or transplantation. Risk factors for death or transplantation include chromosomal abnormalities, a small LV chamber size, and advanced heart failure on initial presentation.