Abstract 4956: A Risk Stratification Analysis of Predictors of Death or Transplant in Children with Hypertrophic Cardiomyopathy

Abstract

We previously reported initial results from a univariate analysis of baseline predictors of death or heart transplant in children with hypertrophic cardiomyopathy (HCM). We present here the final multivariate analysis resulting in risk stratification rules for 6 HCM etiologic subgroups in children. The Pediatric Cardiomyopathy Registry collected longitudinal data on 882 children with HCM from 1990 –2003. The primary outcome was the earlier of death or heart transplantation. Cox regression was used to identify the 3 most important risk factors at time of diagnosis for each HCM subgroup; all echo measures were expressed as z-scores. Multivariate analysis revealed that children with idiopathic HCM diagnosed by 1 year were at higher risk of poor outcome if they had CHF, low BSA, or elevated LV mass, while risk factors for children diagnosed after 1 year were female gender, depressed fractional shortening, and elevated wall thickness. Risk factors for children with malformation syndrome were Black race, CHF and increased wall thickness; Black race, younger age, and elevated LV mass for children with inborn errors of metabolism; younger age, CHF, and increased wall thickness for children with mixed restrictive HCM; and decreased weight, family history of heart disease, and increased wall thickness for children with mixed dilated HCM. Children with multiple risk factors present at diagnosis had a significantly worse prognosis compared to those with 0 or 1 risk factors. (Figure ) Based on etiologic subtype and number of risk factors present at diagnosis, clinicians can reliably estimate the risk of death of transplant in children with HCM.