Abstract

Endocardial fibroelastosis is a cardiomyopathy rarely seen in the present age. Here, we report a case of 14-months-old baby who presented with failure to thrive, sweating while feeding, a dilated left ventricle, and left ventricular dysfunction. Chest x-ray revealed cardiomegaly with pulmonary venous hypertension. Electrocardiogram showed left ventricular hypertrophy with strain pattern. Echocardiogram revealed dilated left ventricle with severe systolic dysfunction which was labelled as endocardial fibroelastosis. As there were no associated lesions, it was labelled as primary endocardial fibroelastosis (pEFE). The baby was managed conservatively. Here, we would like to highlight the fact that primary endocardial fibroelastosis (pEFE) can masquerade as idiopathic dilated cardiomyopathy. Progressive ventricular dilation and ventricular dysfunction portends poor prognosis, though few of them may be managed with conservative treatment.

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