Primary pulmonary hypertension in Israel - A national survey

Objectives: To characterize the incidence of patients with primary pulmonar y hypertension (PPH) in Israel and their outcomes. Methods: We have evaluated retrospectively all the patients in Israel in wh om PPH was diagnosed between the years 1988 and 1997. We looked at medical history, hemodynamic data, pulmonary function and gas exchange, and demogra phic variables. Patients were followed up for survival until November 1997. Life table analysis and Kaplan-Meier statistics were used to estimate the overall survival distribution. Regression analysis was used to examine the relations between survival and selected variables. Results: Overall, we found 44 patients with PPH. The estimated incidence of PPH in Israel is 1.4 new cases per year per million population. The mean ( +/- SD) age at diagnosis was 43 +/- 13 years. In the Jewish population, PPH was more frequent among immigrants from Europe and the United States. The mean interval from the onset of symptoms to diagnosis was 3 years (median, 2 years). The median survival time was 4 years. The 1-year, 3-year, and 5-y ear survival rates were 82%, 57%, and 43%, respectively. The major variable s influencing the survival rate were the following: interval from symptom o nset to diagnosis; and hemodynamic measurements tie, mean pulmonary artery pressure, mean right atrial pressure, and cardiac index). In comparison to rates discerned from the National Institutes of Health registry data, the s urvival rate in Israel is somewhat better and prognosis is influenced by si milar hemodynamic variables. Conclusion: PPH is a rare and fatal disease in Israel. New therapeutic moda lities such as prostacyclin therapy and Lung transplantation may improve su rvival among patients with this malignant disease.