Long-term survival outcome for pre-capillary pulmonary hypertension at a Japanese single center

In recent years, several treatment options for patients with pre-capillary pulmonary hypertension (PH) have improved the short-term prognosis. However, the long-term survival for pre-capillary PH has not been well investigated. This study sought to investigate the long-term survival for pre-capillary PH in Kurume University Hospital. A total of 144 patients with pre-capillary PH (110 women, mean age 55.1 ± 17.9 years) were enrolled. The maximal duration of followup was 15 years with a mean followup of 5.77 years. The 15 year survival was 59.1% for pre-capillary PH, 68.5% for pulmonary arterial hypertension (PAH), and 44.3% for chronic thromboembolic PH. The 5 year survival was 50.9% for PH due to lung disease (PH-LD), indicating the worst in the pre-capillary PH subgroups. The survival for portopulmonary hypertension was the lowest among PAH groups, and PAH associated with connective tissue disease and congenital heart disease decreased 10 years after diagnosis. A 6 min walk distance and elevated brain natriuretic peptide were significantly associated with survival outcome in pre-capillary PH patients and diastolic pulmonary arterial pressure was related to survival for PH-LD. The survivals were different among pre-capillary PH groups in our hospital. Above all, the long-term survival was better than in previous reports.