Abstract

Factors predicting life expectancy in primary pulmonary hypertension have not been well defined. Thirty four cases of primary pulmonary hypertension that had been followed up until death or for at least five years were reviewed retrospectively. Patients were divided into three groups: 18 patients who died within five years of presentation to hospital; 12 who survived more than five years; and four who improved and who lived for more than five years. The age at onset was similar in the three groups and, like symptoms and sex, did not predict life expectancy. Right heart failure during the course of the disease was associated with a poor outcome. Radiographic evidence of cardiac enlargement and evidence of right heart strain on electrocardiogram at presentation was also predictive of survival for less than five years. Pulmonary arteriolar resistance was higher and cardiac output lower in those with the shortest survival times. There was no relation between pulmonary artery pressure and length of survival. Systemic resistance varied directly with pulmonary resistance and served to maintain systemic pressure. Presentation in or after pregnancy and patency of the foramen ovale were associated with longer survival. In four patients there was evidence of regression of the disease by cardiac catheterisation and lung histology. Primary pulmonary hypertension is a heterogeneous condition in which life expectancy varies widely.