Survival and Prognostic Factors in Patients with Primary Pulmonary Hypertension

OBJECTIVES: Primary pulmonary hypertension (PPH) that affects predominantly young and productive people is a progressive fatal disease of unknown cause. The objectives of this study were to characterize mortality in patients with PPH and to investigate the factors associated with their survival. METHODS: Thirteen patients with PPH were enrolled between 1988 and 1996 and followed-up through July 1999. Measurements at diagnosis included hemodynamic and pulmonary function variables in addition to information on demographic data and medical history. RESULTS: 1) The mean age of the patients with PPH enrolled into the study was 36.1 ± 9.3 years with female predominance. 2) The estimated median survival was 3.4 ± 0.6 years. 3) Decreased cardiac index was the only significant predictor of mortality (Cox proportional hazards model). CONCLUSION: Patients with PPH have a poor survival expectancy. In this limited study with a small number of patients, mortality is largely associated with decreased cardiac index.