Key result
Heart transplantation restores functional capacity in advanced endomyocardial fibrosis with severe systolic dysfunction.
Why the study?
Endomyocardial fibrosis is a rare cause of restrictive cardiomyopathy mainly found in tropical regions, with unclear physiopathology and challenging diagnosis in non-endemic areas.
Case Report (n=1)
No
Answers are grounded in this paper and cite their sources.
Offers a potential treatment avenue for idiopathic endomyocardial fibrosis in non-endemic regions; extends limited evidence but remains hypothesis-generating.
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.
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Théry et al. (2020) conducted a case report in Idiopathic endomyocardial fibrosis (n=1). Heart transplantation was evaluated. Orthotopic heart transplantation was successfully performed in a 44-year-old Caucasian man with idiopathic endomyocardial fibrosis and severe systolic dysfunction, resulting in functional class I status at 6-month follow-up.
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