ENDOMYOCARDIAL FIBROSIS IN EGYPT - AN ILLUSTRATED REVIEW

The detailed features of right sided endomyocardial fibrosis are descr ibed in 15 out of 10 000 consecutive patients who all had infection wi th Schistosoma mansoni and came from rural Egypt. Laboratory investiga tions, It lead electrocardiography, chest radiography, and Doppler ech ocardiography were performed in all patients. Cardiac catheterisation and angiography were performed in eight. Endomyocardial biopsy specime ns were obtained from the right ventricles of two patients and pericar dial biopsy specimens from two. Pericardiocentesis was performed in al l patients. All patients were infected with S mansoni and had schistos omal hepatic fibrosis and ascites. Eleven had splenomegaly. All patien ts had raised cervical venous pressure with prominent Y descent and at rial fibrillation. Eosinophilia was notably absent. Echocardiography s howed apical fibrosis in the right ventricle, obliteration of the vent ricle, and moderate to massive exudative pericardial effusion in all p atients. Calcification and fibrosis extended into the right ventricula r outflow tracts in two Huge right atrial thrombi in five patients. Tr icuspid regurgitation (grades I-II) was detected in 11 patients by Dop pler ultrasonography. Haemodynamic and angiographic data confirmed the pure right sided restrictive pathophysiology. Pericardial biopsy spec imens showed perivascular inflammatory infiltrates in two patients and a schistosomal granuloma in one. Endocardial biopsy specimens showed dense fibrosis with many fibroblasts. Endomyocardial fibrosis in Egypt is unique in several aspects. It always affected only the right side of the heart. Calcification and fibrosis extended to the right ventric ular outflow tract. Pericardial inflammatory reaction was present. The relation to schistosomiasis and the link to periportal hepatic fibros is in these patients is intriguing.