Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan

Aims Idiopathic/heritable pulmonary arterial hypertension (I/HPAH) carries a poor prognosis despite the therapeutic options available. Patient survival from Western countries has been reported, but data from Asia are scarce. Main methods We retrospectively reviewed 56 patients with I/HPAH treated at a single referral center in Japan. Survival analyses were conducted using the Kaplan–Meier method with the log-rank test. Variables associated with survival were determined using a Cox proportional hazard model. Key findings There were 41 women (73%) and the mean age at the diagnosis was 32 ± 17 years. Mean survival time from the diagnosis was 14.9 ± 0.8 years (95% CI, 13.4–16.4 years), with 1-, 2-, 3-, 5- and 10-year survival rates of 98, 96, 96, 96 and 78%, respectively. In patients who underwent follow-up right-heart catheterization > 3 months after initial catheterization, mean pulmonary arterial pressure (mPAP) was decreased significantly from 63 ± 15 to 35 ± 10 mm Hg with an improved cardiac index. Patients with high levels of brain natriuretic peptide (BNP) or low oxygen saturation at baseline showed worse survival. At follow-up, 98% of patients were on PAH-targeted drugs. WHO functional classes I and II, mPAP < 42.5 mm Hg, cardiac index > 2.5 L/min/m², BNP < 52 pg/mL, and 6-min walk distance > 347 m at follow-up were predictors of good prognosis in the univariate analysis. Significance The study revealed a long-term survival of Japanese patients with I/HPAH. Hemodynamic parameters improved significantly after treatment, which might be related to high prescription rates of PAH-targeted drugs. Multicenter studies are needed to reveal the prognostic factors for I/HPAH.