title: Predictors of Long-Term Outcome in Children with Hypertrophic Cardiomyopathy.
abstract: To date limited data are available to predict the progression to end-stage heart failure (HF) with subsequent death (non-SCD), need for heart transplantation, or sudden cardiac death (SCD) in children with hypertrophic cardiomyopathy (HCM). We aimed to determine predictors of long-term outcome in children with HCM. A total of 112 children (median 14.1, IQR 7.8-16.6 years) were followed up for the median of 6.5 years for the development of morbidity and mortality, including arrhythmic and HF-related secondary end points. HF end point included HF-related death or heart transplant, and arrhythmic end point included resuscitated cardiac arrest, appropriate ICD discharge, or SCD. Overall, 23 (21 %) patients reached the pre-defined composite primary end point. At 10-year follow-up, the event-free survival rate was 76 %. Thirteen patients (12 %) reached the secondary arrhythmic end point, and 10 patients (9 %) reached the secondary HF end point. In multivariate model, prior cardiac arrest (r = 0.658), QTc dispersion (r = 0.262), and NSVT (r = 0.217) were independent predictors of the arrhythmic secondary end point, while HF (r = 0.440), LV posterior wall thickness (r = 0.258), LA size (r = 0.389), and decreased early transmitral flow velocity (r = 0.202) were all independent predictors of the secondary HF end point. There are differences in the risk factors for SCD and for HF-related death in childhood HCM. Only prior cardiac arrest, QTc dispersion, and NSVT predicted arrhythmic outcome in patients aged <18 years. LA size, LV posterior wall thickness, and decreased early transmitral flow velocity were strong independent predictors of HF-related events.
authors.affiliation: Department of Pediatric Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland. l.ziolkowska@czd.pl.
authors.forename: Lidia
authors.initials: L
authors.lastname: Ziółkowska
authors.affiliation: Department of Pediatric Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Turska-Kmieć
authors.affiliation: Department of Coronary Artery Disease and Structural Heart Disease, Institute of Cardiology, 04-628, Warsaw, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Petryka
authors.affiliation: Department of Pediatric Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland.
authors.forename: Wanda
authors.initials: W
authors.lastname: Kawalec
mesh_terms: D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications*; D002648:Child; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000209:etiology; D017147:Defibrillators, Implantable; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000401:mortality*; D016027:Heart Transplantation / Q000706:statistics & numerical data*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D018570:Risk Assessment; D012307:Risk Factors
keywords: Cardioverter–defibrillator; Children; Heart failure; Heart transplant; Hypertrophic cardiomyopathy; Sudden cardiac death