title: Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan.
abstract: OBJECTIVE
  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.
  
  
  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.
  
  
  RESULTS
  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/m(2), BNP <52 pg/mL, and 6-min walk distance >347 m at follow-up were predictors of good prognosis in the univariate analysis.
  
  
  CONCLUSIONS
  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.
authors: Department of Clinical Science, National Hospital Organization Okayama Medical Center, Okayama, Japan.
authors: Aiko
authors: A
authors: Ogawa
authors: Division of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan.
authors: Kentaro
authors: K
authors: Ejiri
authors: Department of Clinical Science, National Hospital Organization Okayama Medical Center, Okayama, Japan; Division of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan. Electronic address: matsubara.hiromi@gmail.com.
authors: Hiromi
authors: H
authors: Matsubara
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension / Q000235:genetics / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D005820:Genetic Testing; D006439:Hemodynamics; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016019:Survival Analysis; D015996:Survival Rate; D013997:Time Factors
keywords: Peripheral vascular disease; Pulmonary hypertension; Survival