Background: Primary pulmonary hypertension is a poorly understood disease with a difficult treatment.
Patients and method: Retrospective study of a series of 44 patients suffering from pulmonary hypertension who were studied in our center between 1992 and 2000.
Results: At diagnosis, 6 (13%) patients were classified as having NYHA functional class I, 11 (25%) had class II, 25 (57%) had class III, and 2 had class IV. Mean pulmonary artery systolic pressure by echo-doppler was 92 (range: 43-154) mmHg. Basal right catheterization showed a mean (SD) pulmonary artery pressure of 58 (18) mmHg, total basal pulmonary resistances of 1679 (1,071) din/cm2 and cardiac index of 2.2 (1) 1/minute/m2. Five patients improved with anticoagulation and calcium channel blockers therapy. Since 1998, 11 patients had been treated with continuous endovenous epoprostenol, yet only 3 (27%) had significant clinical improvement. Survival at 5 years after diagnosis was 56%. At the end of study, 7 (70%) out of 10 patients who underwent pulmonary transplantation were alive (mean: 34, range: 3-62 months).
Conclusions: Pulmonary hypertension is a disease with a poor prognosis. However, treatment with prostaglandins and pulmonary transplantation may lead to encouraging results.