Severe left ventricular endomyocardial fibrosis presenting as biventricular failure in a young adult: A case report

Abstract

Endomyocardial Fibrosis (EMF) is a form of progressive restrictive cardiomyopathy of unclear aetiology prevalent in areas within 15° of equator including coastal areas of Kerala a few decades back. It inflicts young adults and carries a poor prognosis due to limited options for treatment. Fortunately, the incidence of cases is now declining due to improvement in health and hygiene standards. Here, we review the aetiology and pathogenesis of EMF and report a case of a young male from Mumbai (non-endemic area) presenting with progressively worsening breathlessness and signs of heart failure unresponsive to conventional medical treatment. To delineate the extent of the disease transthoracic echocardiography and cardiac Magnetic Resonance Imaging (MRI) was done which revealed infiltrative lesions in left ventricular apex with grade 2/3 mitral regurgitation. Due to progressive and severe nature of the disease the patient was managed conservatively. Through this report we would like to rekindle the interest of reader in a forgotten tropical disease which is considered rare in this geographical area but should not be missed as a cause heart failure considering its significant mortality.