Pre- and postoperative findings in patients with endomyocardial fibrosis.

From 1971 to 1977 endomyocardial fibrosis was diagnosed in 7 patients (6 women and 1 man) and the clinical course was followed for 36 months. Five of the 7 patients (70%) showed partial obliteration of both ventricles; in 2 patients the fibrotic lesions were confined to the left ventricle. The main haemodynamic features were restriction to ventricular filling and atrioventricular valve regurgitation. Angiocardiographically there was a small left ventricular (LV) cavity with globular configuration and a right ventricular cavity with obliteration of the corpus and small additional cavities in the apical region. LV ejection fraction ranged from 48 to 73 per cent. Endocardial thickening of the posterior wall was found in 4 of 6 echocardiographically evaluated patients, and thickening of the right ventricular anterior wall with systolic obliteration of the cavity was detected by echocardiography in 2 patients in whom angiocardiography had shown severe fibrosis of the right ventricle. The follow-up of 3 medically treated patients showed a stable course in 2 patients with biventricular fibrosis; one patient with LV endomyocardial fibrosis, however, died 6 years later in a severe low-output syndrome. The follow-up of the 4 surgically treated patients showed a good functional result 4 to 14 months after endocardial decortication and atrioventricular valve replacement. There was a significant decrease in LV end-diastolic pressure and a significant increase in cardiac output. LV end-diastolic volume increased in 3 patients as a result of the removal of the filling obstruction and the end-diastolic pressure-volume relation disclosed an increase in LV distensibility. One patient with incomplete decortication showed a slight decrease in postoperative distensibility. It is concluded that (1) endomyocardial fibrosis in Switzerland is a rare disease and is observed mainly in women; (2) the clinical picture is dominated by severe congestive heart failure whereby heart size is only moderately enlarged; (3) systolic performance is normal or only slightly depressed despite severe restriction to filling and/or atrioventricular valve regurgitation; (4) partial obliteration of the right or left ventricle may be detected by echocardiography; and (5) endocardial decortication with atrioventricular valve replacement seems to be an adequate treatment with appreciable postoperative improvement in the patient's condition.