Abstract 9397: Predictors of Disease Progression in Pediatric Dilated Cardiomyopathy

Abstract

Objective: Identify predictors of disease progression in pediatric dilated cardiomyopathy (DCM). Methods: The Pediatric Heart Network Ventricular Volume Variability Study evaluated chronic DCM patients with serial prospective echocardiographic and clinical data collection over an 18 month follow-up. Inclusion criteria were age <22 years and DCM disease duration > 2 months with exclusion of those needing IV inotropic or mechanical support, and those listed status 1A/1B for transplant. Disease progression was defined as an increase in transplant listing status, hospitalization for heart failure, IV inotropes, mechanical support, or death during follow-up. Predictors of disease progression were identified using logistic regression and classification and regression tree (CART) analysis. Results: Of the 127 patients, 28 (22%) met criteria for disease progression during the 18 month follow-up period. Multivariable analysis (c-statistic=0.90) identified older age at diagnosis (OR=1.16 per yr, p=0.003), larger left ventricular (LV) end-diastolic m-mode dimension z-score (LVEDDz) (OR 1.77, p<0.001) and lower septal peak systolic tissue Doppler velocity z-score (OR=0.68, p=0.04) as independent predictors of disease progression. CART analysis risk-stratified patients for significant disease progression with 89% sensitivity and 94% specificity based on LVEDDz ≥7.7, LV ejection fraction <38.2%, LV inflow propagation velocity (color m-mode) z-score < -0.28, and age at diagnosis ≥ 8.5 months. (Figure 1) Conclusion: In pediatric patients with DCM, diagnosis after late infancy and echocardiographic parameters of LV size, systolic and diastolic function were independently associated with disease progression, and may be used to reliably risk stratify DCM patients.