Idiopathic endomyocardial fibrosis in a Western European: a case report

BACKGROUND: Endomyocardial fibrosis (EMF) is a rare cause of restrictive cardiomyopathy, mainly found in tropical/subtropical country. Endomyocardial fibrosis causes severe congestive symptoms and may lead to end-stage heart failure. CASE SUMMARY: A French Caucasian 44-year-old man without noticeable medical history and who had never travelled outside of France was hospitalized for a first episode of acute heart failure revealing an atypical appearance of the left ventricle. Cardiac magnetic resonance (CMR) identified EMF, but investigations did not identify any aetiology (no eosinophilia). Despite optimal management of chronic heart failure, functional class declined rapidly resulting in several hospitalizations for heart failure. The patient finally underwent an elective heart transplantation with good results at 6-month follow-up. DISCUSSION: Endomyocardial fibrosis exact physiopathology remains unclear, although association with eosinophilia has been reported. Diagnosis is challenging and is based on multi-modal imagery with a central role of CMR. There is no consensus on optimal management, medical therapy having poor outcomes and rate of peri-operative complications being high. Heart transplantation should be considered for eligible patients.