Long-term follow-up in primary pulmonary hypertension

Abstract

Two patients with severe primary pulmonary hypertension, who are alive 38 and 27 years after the onset of the disease, were closely followed by the authors for 24 years. No previous report of such a long course has been found. Despite pulmonary artery pressures at systemic level, effort syncope disappeared and there were long intervals without symptoms during which the 2 patients were able to take part in physical activity with minimal discomfort. Severe primary pulmonary hypertension should not always be considered a malignant disease.