Endomyocardial fibrosis: a tragic conundrum or a mere negligence?

Abstract

Often termed as ‘vanishing mystery’ epidemiologically, endomyocardial fibrosis (EMF) continues to be the the most common cause of restrictive cardiomyopathy in Africa and some parts of the Indian subcontinent, where it is considered a significant public health problem. On the other hand, in developed countries, it is considered a rare phenomenon. This entity is considered incurable due to lack of targeted therapies, even after 75 years since its discovery. The authors describe here a case of an Indian male, in his early 30s who presented with complaints of dyspnoea on minimal daily activities, swelling of both legs and abdominal fullness for 3 months. The clinical features were suggestive of isolated right heart failure. He was diagnosed with EMF on echocardiography and the findings were confirmed on cardiac MRI. The patient has progressed to develop biventricular dysfunction and is being managed symptomatically with diuretics due to unavailability of targeted treatment options at present.