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Survival and prognostic factors in patients with primary pulmonary hypertension
1Department of Internal Medicine, College of Medicine, Pusan National University, Pusan, Korea.
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.