Despite recent advances in understanding of the pathobiology and targeted treatments of @entity120 ( @entity120 ), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of @entity120 (KORPAH) was to examine the epidemiology and prognosis of Korean @entity1 with @entity120 . KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 @entity1 were enrolled. The @entity1 ' mean age was 47.6    15.7 yr, and 503 (80.5%) were @entity1 . 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 @entity2597 ( @entity2597 ), @entity299 , and @entity814 ( @entity814 ) (49.8%, 25.4%, and 23.2%, respectively). @entity1 with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) @entity1 received a single @entity120 -specific treatment at the time of enrollment, but only 72 (18.9%) @entity1 received combination therapy. Incident cases during the registry represented 297 @entity1 ; therefore, the incidence rate of @entity120 was 1.9 @entity1 /yr/million @entity1 . The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean @entity120 @entity1 exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more @entity2597 - @entity120 in the etiology and also @entity1252 among @entity2597 - @entity120 . The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of @entity120 @entity1 .

Baseline Characteristics of the Korean Registry of XXXX .

@entity1 :: ('9606', 'Species') :: ['women', 'Patients', 'patients', 'people'] @entity2597 :: ('MESH:D003240', 'Disease') :: ['connective tissue disease', 'CTD'] @entity120 :: ('MESH:D006976', 'Disease') :: ['PAH', 'pulmonary arterial hypertension', 'Pulmonary Arterial Hypertension'] @entity1252 :: ('MESH:D008180', 'Disease') :: ['systemic lupus erythematosus'] @entity814 :: ('MESH:D010661', 'Disease') :: ['IPAH', 'idiopathic PAH'] @entity299 :: ('MESH:D006331', 'Disease') :: ['congenital heart disease']
@entity120 :: (MESH:D006976,Disease) :: ['Pulmonary Arterial Hypertension']
