title: Baseline Characteristics of the Korean Registry of Pulmonary Arterial Hypertension.
abstract: Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 ± 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.
authors.affiliation: Department of Cardiovascular Medicine, Gachon Cardiovascular Research Institute, Gachon University Gil Medical Center, Incheon, Korea.
authors.forename: Wook-Jin
authors.initials: WJ
authors.lastname: Chung
authors.affiliation: Division of Pulmonology, Department of Internal Medicine, Hallym University Gangdong Sacred Heart Hospital, Seoul, Korea.
authors.forename: Yong Bum
authors.initials: YB
authors.lastname: Park
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Hospital, Bucheon, Korea.
authors.forename: Chan Hong
authors.initials: CH
authors.lastname: Jeon
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Yonsei University Severance Hospital, Seoul, Korea.
authors.forename: Jo Won
authors.initials: JW
authors.lastname: Jung
authors.affiliation: Department of Preventive Medicine, Gachon University School of Medicine, Incheon, Korea.
authors.forename: Kwang-Phil
authors.initials: KP
authors.lastname: Ko
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Korea University Ansan Hospital, Ansan, Korea.
authors.forename: Sung Jae
authors.initials: SJ
authors.lastname: Choi
authors.affiliation: Division of Cardiology, Department of Internal Medicine, Soonchunhyang University Hospital, Bucheon, Korea.
authors.forename: Hye Sun
authors.initials: HS
authors.lastname: Seo
authors.affiliation: Division of Pulmonology, Department of Internal Medicine, University of Ulsan, Asan Medical Center, Seoul, Korea.
authors.forename: Jae Seung
authors.initials: JS
authors.lastname: Lee
authors.affiliation: Division of Cardiology, Department of Internal Medicine, Seoul St. Mary's Hospital, The Catholic University of Korea, Seoul, Korea.
authors.forename: Hae Ok
authors.initials: HO
authors.lastname: Jung
mesh_terms: D000328:Adult; D000368:Aged; D003240:Connective Tissue Diseases / Q000150:complications; D003625:Data Collection; D016208:Databases, Factual; D065627:Familial Primary Pulmonary Hypertension / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications; D006801:Humans; D020407:Internet; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000503:physiopathology*; D012042:Registries; D056910:Republic of Korea / Q000453:epidemiology; D015996:Survival Rate; D055815:Young Adult
keywords: Hypertension, Pulmonary; Korean; Prognosis; Pulmonary Arterial Hypertension