Endomyocardial fibrosis as a cause of Budd–Chiari syndrome and fatal pulmonary embolism

Abstract 

A 24-year-old-man had right-sided heart failure of 3 months' duration. A Doppler echocardiogram revealed atrium and right ventricular enlargement, obliteration of the right ventricular apex, and a mass with an echolucent center measuring 20×21 mm in the right ventricular outlet. He died of pulmonary embolism. At autopsy, a huge organized thrombus obliterating the right ventricular apex passing through the tricuspid valve to the right atrium and then extending to the inferior vena cava up to the suprahepatic veins was seen. Histologically, an intense fibrotic thickening of the endomyocardium extending into the myocardium was observed. Cardiac thrombosis associated with endomyocardial fibrosis should be added to the list of causes of Budd–Chiari syndrome.

Keywords: Endomyocardial fibrosis, Budd–Chiari syndrome, Pulmonary embolism, Heart failure, Cardiac thrombosis, Inferior vena cava