title: Survival of Japanese Patients With Idiopathic/Heritable Pulmonary Arterial Hypertension.
abstract: Idiopathic/heritable pulmonary arterial hypertension has a poor prognosis despite the available therapeutic options. Survival of Japanese patients with this disease entity has not been reported in the multicenter setting. A retrospective study of 141 patients with idiopathic/heritable pulmonary arterial hypertension treated at 3 pulmonary hypertension centers in Japan from 1992 to 2012 investigated survival and determinants of survival. Mean survival time from treatment initiation was 14.7 ± 0.8 years (95% confidence interval, 13.1 to 16.3 years) and the 1-, 3-, 5-, and 10-year survival rates were 97.9%, 92.1%, 85.8%, and 69.5%, respectively. Patients showed significant improvement in exercise capacity and hemodynamics after treatment. Patients with 6-minute walk distance >372 m, mean pulmonary arterial pressure ≤46 mm Hg, and cardiac index >2.5 L/min/m2at follow-up had a significantly better prognosis. Most patients (99.2%) were receiving pulmonary hypertension-targeted drugs at follow-up. Use of endothelin receptor antagonists and intravenous epoprostenol were related to survival in the univariate analysis. Among the patients who were on intravenous epoprostenol therapy, those with endothelin receptor antagonists had a significantly better prognosis, whereas patients on warfarin had a significantly worse prognosis. In conclusion, survival of Japanese patients with idiopathic/heritable pulmonary arterial hypertension in this study was good, showing improvement in hemodynamic parameters supported by pulmonary hypertension-targeted drugs.
authors.affiliation: Department of Clinical Science, National Hospital Organization Okayama Medical Center, Okayama, Japan. Electronic address: aiko-oky@umin.ac.jp.
authors.forename: Aiko
authors.initials: A
authors.lastname: Ogawa
authors.affiliation: Division of Cardiology, Department of Medicine, Kyorin University Hospital, Tokyo, Japan.
authors.forename: Toru
authors.initials: T
authors.lastname: Satoh
authors.affiliation: Department of Cardiology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Tamura
authors.affiliation: Department of Cardiology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Fukuda
authors.affiliation: Department of Clinical Science, National Hospital Organization Okayama Medical Center, Okayama, Japan.
authors.forename: Hiromi
authors.initials: H
authors.lastname: Matsubara
mesh_terms: D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D011464:Epoprostenol / Q000627:therapeutic use; D017079:Exercise Tolerance / Q000502:physiology; D065627:Familial Primary Pulmonary Hypertension / Q000097:blood / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D014655:Vascular Resistance; D000070857:Walk Test; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D020097:Natriuretic Peptide, Brain; D011464:Epoprostenol