Primary pulmonary hypertension, then and now: 28 years of experience

To determine whether a better understanding of primary pulmonary hypertension has affected its clinical presentation, natural history, and prognosis, we retrospectively compared patients with primary pulmonary hypertension presenting to our institution during two different periods: 1962 to 1978 (group 1, n = 38) and 1979 to 1989 (group 2, n = 33). Demographic characteristics were similar in both groups. Dyspnea on exertion was the most frequent presenting symptom in both groups. Fatigue, cough, dizziness, right heart failure, and cyanosis were more frequent in group 1. The electrocardiographic, radiographic, and echocardiographic findings did not differ between groups. Hemodynamic measurements revealed severe pulmonary hypertension and a normal pulmonary capillary wedge pressure and cardiac index; these measurements were similar in both groups. Complications related to cardiac catheterization were more frequent in group 1 (32%) than in group 2 (3%). Causes of death were comparable in both groups, the most frequent being progressive right heart failure, sudden death, and death of unknown cause. Patients from both groups received standard treatment with digitalis, diuretics, and vasodilators; however, group 2 had a higher probability of survival than group 1. We conclude that patients with pulmonary hypertension seen in more recent years tend to present at earlier stages of disease, have fewer complications during cardiac catheterization, and probably survive longer than those seen several decades ago. A clear cause for the longer survival could not be identified, although it may be partly related to earlier presentation in the course of disease.