Endomyocardial fibrosis: early and late results of surgery in 20 patients.

Twenty patients with endomyocardial fibrosis (EMF), the largest series reported to date, were operated upon between June, 1978, and June, 1980. Eleven were male and nine female, ranging in age from 6 to 23 years (mean 13.3 years). There were seven cases of right ventricular (RVEMF), six of left ventricular (LVEMF), and seven cases of bilateral EMF. All patients underwent endocardiectomy and atrioventricular valve replacement with a xenograft. Four patients had an additional valvular annuloplasty. There were four postoperative deaths (all bilateral EMF): two from low cardiac output and one each from hepatic failure and cerebral malaria. There was one late death from serum hepatitis. The other patients had a relatively difficult postoperative course, but none of the 20 patients atrioventricular block. The longest follow-up of the 15 survivors is 28 months (mean 16.7 months). All patients are symptom free. Three take digitalis and/or diuretics. Ten have been recatheterized from 6 months to 1 year after operation. Intracardiac pressures, the ventricular cineangiogram, liver, and heart size returned to normal in patients with LVEMF; in RVEMF, despite clinical improvement, most of these parameters remained abnormal. Of special interest were (1) our recognitions of an early type of LVEMF and (2) our surgical preservation of a thin juxta-annular rim of fibrosis in the right ventricle to avoid atrioventricular block. Operation is indicated in all patients with LVEMF, despite greater risk. Early intervention is advised in RVEMF to avoid irreversible liver damage and cardiac enlargement.