OBJECTIVES: To characterize the incidence of patients with primary pulmonary hypertension (PPH) in Israel and their outcomes. METHODS: We have evaluated retrospectively all the patients in Israel in whom PPH was diagnosed between the years 1988 and 1997. We looked at medical history, hemodynamic data, pulmonary function and gas exchange, and demographic 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-year survival rates were 82%, 57%, and 43%, respectively. The major variables influencing the survival rate were the following: interval from symptom onset to diagnosis; and hemodynamic measurements (ie, 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 survival rate in Israel is somewhat better and prognosis is influenced by similar hemodynamic variables. CONCLUSION: PPH is a rare and fatal disease in Israel. New therapeutic modalities such as prostacyclin therapy and lung transplantation may improve survival among patients with this malignant disease.
OBJECTIVES: To characterize the incidence of patients with primary pulmonary hypertension (PPH) in Israel and their outcomes. METHODS: We have evaluated retrospectively all the patients in Israel in whom PPH was diagnosed between the years 1988 and 1997. We looked at medical history, hemodynamic data, pulmonary function and gas exchange, and demographic 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-year survival rates were 82%, 57%, and 43%, respectively. The major variables influencing the survival rate were the following: interval from symptom onset to diagnosis; and hemodynamic measurements (ie, 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 survival rate in Israel is somewhat better and prognosis is influenced by similar hemodynamic variables. CONCLUSION: PPH is a rare and fatal disease in Israel. New therapeutic modalities such as prostacyclin therapy and lung transplantation may improve survival among patients with this malignant disease.
Authors: Sophia Emmons-Bell; Catherine Johnson; Alexandra Boon-Dooley; Paul A Corris; Peter J Leary; Stuart Rich; Magdi Yacoub; Gregory A Roth Journal: Pulm Circ Date: 2022-01-18 Impact factor: 2.886
Authors: Peter Steele; Geoff Strange; John Wlodarczyk; Brad Dalton; Simon Stewart; Eli Gabbay; Anne Keogh Journal: BMC Cardiovasc Disord Date: 2010-02-22 Impact factor: 2.298
Authors: Eduardo M Moreira; Henning Gall; Maarten J G Leening; Lies Lahousse; Daan W Loth; Bouwe P Krijthe; Jessica C Kiefte-de Jong; Guy G Brusselle; Albert Hofman; Bruno H Stricker; Hossein A Ghofrani; Oscar H Franco; Janine F Felix Journal: PLoS One Date: 2015-06-23 Impact factor: 3.240
Authors: Carlos G Piscoya Roncal; Adriano A Mendes; Maria T C Muniz; Sheilla A de Oliveira; Leonidas M do Valle Neto; Nathália A de Vasconcellos Piscoya; Gustavo H B Góes; Dario C Sobral Filho; Mardi Gomberg-Maitland Journal: Int J Cardiol Heart Vasc Date: 2019-10-31