A validated model for sudden cardiac death risk prediction in pediatric hypertrophic cardiomyopathy
Circulation Jul 25, 2020
Miron A, Lafreniere-Roula M, Fan CPS, et al. - Considering that hypertrophic cardiomyopathy is the leading cause of sudden cardiac death (SCD) in both children and young adults, researchers conducted this international multicenter observational cohort study to establish and validate a SCD risk prediction model in pediatric hypertrophic cardiomyopathy to guide strategies for SCD prevention. Phenotype-positive patients who were diagnosed with isolated hypertrophic cardiomyopathy < 18 years of age were eligible. In total, 572 patients met eligibility requirements with 2,855 patient-years of follow-up. Data reported that the 5-year cumulative proportion of SCD events was 9.1%. Risk predictors included diagnostic age, reported nonsustained ventricular tachycardia, unexplained syncope, septal diameter z-score, left ventricular posterior wall diameter z score, left atrial diameter z score, peak left ventricular outflow tract gradient, and pathogenic variant presence. In contrast to adults, the left ventricular outflow gradient had an inverse correlation, and the family history of SCD had no connection with SCD. The research offers a validated SCD risk prediction model with > 70% prediction exactness and integrates risk factors specific to pediatric hypertrophic cardiomyopathy. An individualized risk prediction model has the ability to enhance the implementation of clinical practice guidelines and shared decision making for implantable cardioverter defibrillator insertion.
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