Endomyocardial fibrosis related sudden cardiac death; two autopsied case-reports from Egypt

Abstract

Endomyocardial fibrosis (EMF) is an idiopathic tropical disorder that is characterized by the development of restrictive cardiomyopathy. Neglected EMF can cause sudden cardiac death (SCD) in adults. Conclusive diagnosis of EMF depends on autopsy after death. In an effort to attract the interest of the community for this rare disease, we report two cases of SCD that were diagnosed as EMF during autopsy in Egypt. Both cases were thoroughly investigated with emphasis on death circumstances and post-mortem anatomical and histopathological findings. The two cases were for adult males presented with SCD following a quarrel with a negative medical history and family history regarding cardiac diseases. No trauma or drug abuse. The autopsy revealed hypertrophied hearts, thick fibrosed endocardium, patchy myocardial fibrosis, and filling of the apex by fibrosis and calcifications. In one of them, there was a huge mural thrombus reaching the level of the mitral valve that totally occluded the cavity of the left ventricle. Histopathologically, fibrosis was confirmed, and no eosinophils were detected. In contrast to previously reported cases in Egypt, the left ventricle was solely affected. Despite the rarity of the disease outside the tropics, the frequency of EMF cases is more likely to be more than the number of reported cases. EMF should be considered as possible cause of SCD during autopsy. Further studies are needed to clarify the etiology and epidemiology of EMF.

Keywords: Endomyocardial fibrosis; Restrictive cardiomyopathy; Sudden cardiac death.