PMCID: PMC4111626 DOI: 10.1161/CIRCHEARTFAILURE.113.000125
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 age2 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
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.
CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00123071.
Circ Heart Fail
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
cardiomyopathies; heart transplant; pediatrics
Molina, Kimberly M.; Shrader, Peter; Colan, Steven D.; Mital, Seema; Margossian, Renee; Sleeper, Lynn A.; Shirali, Girish S.; Barker, Piers; Canter, Charles E.; Altmann, Karen; Radojewski, Elizabeth; Tierney, Elif Seda Selamet; Rychik, Jack; Tani, Lloyd Y.; and Pediatric Heart Network Investigators, "Predictors of disease progression in pediatric dilated cardiomyopathy." (2013). Manuscripts, Articles, Book Chapters and Other Papers. 908.