Endomyocardiofibrosis: A Systematic Review

Endomyocardiofibrosis was described first time in Uganda as an infrequent restrictive cardiomyopathy with a poor prognosis, characterized by fibrosis of the ventricular subendocardium and severe restrictive physiology leading to difficult therapeutic management and frequently associated with hypereosinophilic syndrome. Its higher prevalence in the tropics and its relationship in some cases with hypereosinophilic endocarditis has led to the search for genetic, infectious, autoimmune and nutritional causes, but its etiology remains unclear. It is a rare cardiomyopathy, difficult to diagnose and with a nonexistent effective treatment. Imaging methods such as echocardiography and cardiac magnetic resonance are essential for the initial diagnosis, although endomyocardial biopsy establishes the definitive diagnosis. Immunosuppressive treatment is only useful in the early stages of the disease and usually ineffective if installed late when signs of heart failure are present. Surgical treatment is generally palliative.