Right ventricular endomyocardial fibrosis. Diagnosis and management

The clinical course, noninvasive and invasive diagnostic findings in 4 patients (pt) with endomyocardial fibrosis are reported. All patients (16-50 years) were in functional class III-IV (NYHA). Central venous pressure with large a- and v-waves was elevated in all; liver enlargement and peripheral edema were also noticed in all pts, and ascites in 3. An apical 2/6 systolic murmur was present in 2, and a right parasternal pansystolic murmur 2-4/6 with positive Carvallo's sign in all pts. ECG was non-specific and chest x ray showed right atrial enlargement in every case. A right-sided diastolic plateau which was higher than left ventricular end-diastolic pressure was present in all pts. Echo-, angiocardiography and computed tomography of the heart revealed obliteration of the right ventricular cavity, predominantly localized at the apex. Two pts who underwent endocardial resection and tricuspid valve replacement are alive and well after 9 and 8 years, respectively. One pt died early in the postoperative period and one died waiting for surgical therapy. In conclusion, echo-, angiocardiography and computed tomographic findings are diagnostic. A satisfactory differentiation from other cardiac disorders with restrictive hemodynamics and right-sided heart failure is possible.