[Case of primary pulmonary hypertension supported with non-invasive positive pressure ventilation].

We report a case of primary pulmonary hypertension supported with non-invasive positive pressure ventilation (NPPV). A 20-year-old woman was diagnosed as primary pulmonary hypertension at the age of 15 and we prepared for registration of cadaver lung transplantation at the age of 20. She suffered from hemoptysis and was transferred to our ICU. We performed NPPV with continuous positive airway pressure of 4-6 cmH2O. Her systolic pulmonary artery pressure estimated with echocardiography was reduced, lung congestion on chest X ray was improved, and arterial oxygenation was improved. Considering that her condition would deteriorate only with medical therapy, we planned living donor lung transplantation. However, she died from massive hemoptysis before lung transplantation.