title: Clinical characteristics and survival of Korean idiopathic pulmonary arterial hypertension patients based on vasoreactivity.
abstract: We aimed to identify a vasoreactive subset of patients with idiopathic pulmonary arterial hypertension (IPAH) in Korea and to show their clinical characteristics and prognosis. Data on patients who were diagnosed with IPAH at Asan Medical Center between January 1994 and March 2013 were retrospectively collected. Acute vasodilator testing was performed with inhaled nitric oxide during diagnostic right heart catheterization. A positive acute response was defined as a reduction in mean pulmonary arterial pressure (PAP) ≥10 mmHg to an absolute level of mean PAP <40 mmHg without a decrease in cardiac output. Among a total of 60 IPAH patients included for analysis, 9 (15%) showed a positive acute response to acute vasodilator testing. Acute responders showed significantly lower peak velocity of a tricuspid regurgitation jet on echocardiography (4.1±0.3 m/s vs. 4.6±0.6 m/s; P=0.01) and significantly lower mean PAP hemodynamically (47±10 mmHg vs. 63±17 mmHg; P=0.003) than non-responders at baseline. The survival rate of acute responders was 88% at 1, 3, 5, and 10 yr, respectively, which was significantly higher than that of non-responders (85%, 71%, 55%, and 40%, respectively; P=0.029). In conclusion, Korean IPAH patients with vasoreactivity showed better baseline hemodynamic features and survival than those without vasoreactivity.
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Jee Hwan
authors.identifier: 0000-0001-6653-998X
authors.initials: JH
authors.lastname: Ahn
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Byung Ju
authors.identifier: 0000-0002-1396-7398
authors.initials: BJ
authors.lastname: Kang
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Sun In
authors.identifier: 0000-0003-2575-2084
authors.initials: SI
authors.lastname: Hong
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Jung Su
authors.identifier: 0000-0001-5644-0150
authors.initials: JS
authors.lastname: Lee
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. ; Center for Pulmonary Hypertension and Venous Thromboembolism, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Jae Seung
authors.identifier: 0000-0003-4130-1486
authors.initials: JS
authors.lastname: Lee
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Yeon-Mok
authors.identifier: 0000-0003-0116-4683
authors.initials: YM
authors.lastname: Oh
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. ; Center for Pulmonary Hypertension and Venous Thromboembolism, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Sang-Do
authors.identifier: 0000-0001-8189-4509
authors.initials: SD
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D015203:Reproducibility of Results; D056910:Republic of Korea / Q000453:epidemiology; D012307:Risk Factors; D012680:Sensitivity and Specificity; D015996:Survival Rate; D014655:Vascular Resistance / Q000187:drug effects*; D014665:Vasodilator Agents; D055815:Young Adult
chemical_list: D014665:Vasodilator Agents
keywords: Calcium Channel Blockers; Hypertension, Pulmonary; Survival Rate; Vasodilator Agents