Multi-institutional Prospective Registry in Pulmonary Hypertension associated with Respiratory Disease - Japan Respiratory PH study

281 patients with R-PH were included in this study. 43% had mild ventilatory impairment (MVI), 52% had a severe form of PH. 68% received PAH-targeted therapies. In MVI, those treated initially had better survival than patients not treated initially (3-year survival 70.6% vs. 34.2%; P=0.01), and no significant difference was obsereved in severe ventilatory impairment (49.6% vs. 32.1%; P=0.38). Responders to PAH-targeted therapy were more prevalent in the group with MVI.