Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator: An international multicenter study

S. Balaji
M. P. DiLorenzo
F. A. Fish
S. P. Etheridge
P. F. Aziz
M. W. Russell
S. Tisma
A. Pflaumer
N. Sreeram
P. Kubus
I. H. Law
M. J. Kantoch
N. J. Kertesz
M. Strieper
C. C. Erickson
J. P. Moore
S. J. Nakano
H. R. Singh
A. D. Blaufox, Zucker School of Medicine at Hofstra/Northwell
M. Shah


© 2019 Heart Rhythm Society Background: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). Objective: The purpose of this study was to determine predictors of LAE in children with HCM. Methods: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. Results: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P =.0025), LV posterior wall thickness z score (HR 1.02; P <.005), and LV outflow gradient < 30 mm Hg (HR 2.0; P <.006). LV posterior wall thickness z score ≥ 5 was associated with LAE. Conclusion: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.