Objectives

Dilated cardiomyopathy(DCM) is the most important cause of heart transplantation in the paediatric population. We aim to report our single institution outcomes of dilated cardiomyopathy in children, and explore variables affecting survival and the need for heart transplantation.

Methods

We performed a retrospective review of 56 children diagnosed with DCM from September, 2007 to September, 2015 in a tertiary centre of pediatric cardiology center. Demographic data, clinical presentation, etiology, treatment and outcome were assessed.

Methods

We performed a retrospective review of 56 children diagnosed with DCM from September, 2007 to September, 2015 in a tertiary centre of pediatric cardiology center. Demographic data, clinical presentation, etiology, treatment and outcome were assessed.

Results

During 8 years, we identified 56 patient; 51,7 % were boys. The age range of the patients was from 1 months to 17 years. The mean Fractional Shortening was 18%, the mean left ventricular end-systolic dimension (LVESD) z-score was +7,7. The majority of children (66%) had idiopathic disease. The most common known cause was myocarditis (21%). %14 of patients with familial DCM. Overall, 20 patients (33%) died and 9 patients (16%) were transplanted. One patient died after one year from transplantation. Within 1-year of diagnosis,13 patients died, 6 underwent transplantation and 8 patients were need mechanical circulatory support. The 1- and 5-year rates of death or transplantation were 34% and 44%, respectively. Estimation of freedom from transplantation by cause revealed that patients with idiopathic or familial DCM had the worst outcomes with 52 % freedom from transplantation at 5 years after diagnosis. Patients with myocarditis had a 5-year rate of freedom from death or transplantation of 66%.

Independent risk factors at DCM diagnosis time for subsequent death or transplantation were older age, the presence of congestive heart failure, lower left ventricular fractional shortening Z score, and etiology of DCM (P<0.01 for all).