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.
Highlights
Hypertrophic cardiomyopathy (HCM) is a relatively common genetic cardiac disease, and it accounts for 42 % of childhood cardiomyopathy [14]
Limited data are available to predict which children will progress to end-stage heart failure (HF) with subsequent death or need for heart transplantation (HT) or who will die from sudden cardiac death (SCD) [4, 37]
The aim of this study was to determine predictors of long-term outcome in children with hypertrophic cardiomyopathy (HCM) including known adult risk factors for SCD and previously proposed pediatric risk factors for death
Summary
Hypertrophic cardiomyopathy (HCM) is a relatively common genetic cardiac disease, and it accounts for 42 % of childhood cardiomyopathy [14]. HCM is the most common cause of sudden cardiac death (SCD) in the young (including competitive athletes) [9, 18]. The incidence of SCD in children with HCM ranges from 1 [3, 27] to 6 % [8, 32, 33]. Specific risk factors for SCD in adults with HCM have been proposed [6, 9, 11, 17, 38], while a few series have been focused on children [5, 15, 20, 24, 31, 33, 34, 37]
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