Document Type

Article

Publication Date

11-1-2013

Identifier

PMCID: PMC4111626 DOI: 10.1161/CIRCHEARTFAILURE.113.000125

Abstract

Background: Despite medical advances, children with dilated cardiomyopathy (DCM) remain at high risk of death or need for cardiac transplantation. We sought to identify predictors of disease progression in pediatric DCM.

Methods and results: The Pediatric Heart Network evaluated chronic DCM patients with prospective echocardiographic and clinical data collection during an 18-month follow-up. Inclusion criteria were age <22 years and DCM disease duration >2 months. Patients requiring intravenous inotropic/mechanical support or listed status 1A/1B for transplant were excluded. Disease progression was defined as an increase in transplant listing status, hospitalization for heart failure, intravenous inotropes, mechanical support, or death. Predictors of disease progression were identified using Cox proportional hazards modeling and classification and regression tree analysis. Of the 127 patients, 28 (22%) had disease progression during the 18-month follow-up. Multivariable analysis identified older age at diagnosis (hazard ratio=1.14 per year; P<0.001), larger left ventricular (LV) end-diastolic M-mode dimension z-score (hazard ratio=1.49; P<0.001), and lower septal peak systolic tissue Doppler velocity z-score (hazard ratio=0.81; P=0.01) as independent predictors of disease progression. Classification and regression tree analysis stratified patients at risk of disease progression with 89% sensitivity and 94% specificity based on LV end-diastolic M-mode dimension z-score ≥7.7, LV ejection fraction <39%, LV inflow propagation velocity (color M-mode) z-score <-0.28, and age at diagnosis ≥8.5 months.

Conclusions: In children with chronic stable DCM, a combination of diagnosis after late infancy and echocardiographic parameters of larger LV size and systolic and diastolic function predicted disease progression.

Journal Title

Circ Heart Fail

Volume

6

Issue

6

First Page

1214

Last Page

1222

MeSH Keywords

Adolescent; Cardiomyopathy, Dilated; Child; Child, Preschool; Disease Progression; Echocardiography, Doppler; Female; Follow-Up Studies; Heart Transplantation; Heart Ventricles; Humans; Infant; Male; Prognosis; Prospective Studies; Time Factors; Ventricular Function, Left

Keywords

cardiomyopathies; heart transplant; pediatrics

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