Clinical and prognostic factors in children with dilated cardiomyopathy

Abstract

Dilated cardiomyopathy is characterized by impaired myocardial contractility and cardiac dilatation. Most cases in children are idiopathic despite diagnostic developments. In this study, we aimed to review children with dilated cardiomyopathy and attempt to determine the prognostic factors. Materials and methods: A total of 59 children with a diagnosis of dilated cardiomyopathy were evaluated retrospectively between January 2005 to January 2013.The age of onset of symptoms, gender, parental consanguinity, electrocardiography findings, presence of cardiomegaly in the telecardiograpy, left ventricular ejection fraction and shortening fraction on prognosis were evaluated. Results:The mean age of patients was 49.2±59.2 months and the male/female ratio was 1.03/1. Heart failure symptoms and signs were the most common cause of admission.The most common electrocardiographic finding was left ventricular hypertrophy in 32.2% and cardiomegaly was observed on telecardiography in 77.8%. The left ventricular ejection and shortening fraction of patients' were 38.4±14.5% and 18.8±8.4% at the time of the diagnosis and 51±18.6% and 26.8±11.6% at the end of the follow-up, respectively.The mean follow-up period was 27.5±27.7 months and the mortality rate was 42.4%. The main factors affecting prognosis have been found as detection of electrocardiographic changes, cardiomegaly, and lower left ventricular ejection fraction at the time of the diagnosis. Conclusion:Dilated cardiomyopathy remains a challenging disease and the prognosis is poor. In our study, the most important prognostic markers affecting survival were determined by the presence of electrocardiographic changes, cardiomegaly, and a reduced ejection fraction.