title: Clinical characteristics of pulmonary hypertension in bronchiectasis.
abstract: Pulmonary hypertension (PH), as a complication of bronchiectasis, is associated with increased mortality. However, hemodynamic characteristics and the efficacy of pulmonary arterial hypertension (PAH) therapies in patients with bronchiectasis and PH remain unknown. Patients with bilateral bronchiectasis and concurrent PH were included in the study. Patient characteristics at baseline and during follow-up, as well as survival, were analyzed. This observational study was conducted in 36 patients with a mean age of 51.5 years (range, 17‒74 years). The 6 min walking distance was 300.8 ± 3.3 m. The mean pulmonary arterial pressure (PAP) was 41.5 ± 1.7 mmHg, cardiac output was 5.2 ± 1.4 L/min, and pulmonary vascular resistance was 561.5 ± 281.5 dyn∙s∙cm(-5). The mean PAP was > 35 mmHg in 75% of the cases. Mean PAP was inversely correlated with arterial oxygen saturation values (r =-0.45, P = 0.02). In 24 patients who received oral PAH therapy, systolic PAP was reduced from 82.4 ± 27.0 mmHg to 65.5 ± 20.9 mmHg (P = 0.025) on echocardiography after a median of 6 months of follow-up. The overall probability of survival was 97.1% at 1 year, 83.4% at 3 years, and 64.5% at 5 years. Given the results, we conclude that PH with severe hemodynamic impairment can occur in patients with bilateral bronchiectasis, and PAH therapy might improve hemodynamics in such patients. Prospective clinical trials focusing on this patient population are warranted.
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Lan
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Radiology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Sen
authors.initials: S
authors.lastname: Jiang
authors.affiliation: Department of Radiology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Jingyun
authors.initials: J
authors.lastname: Shi
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Sugang
authors.initials: S
authors.lastname: Gong
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Qinhua
authors.initials: Q
authors.lastname: Zhao
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Rong
authors.initials: R
authors.lastname: Jiang
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Ping
authors.initials: P
authors.lastname: Yuan
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Bigyan
authors.initials: B
authors.lastname: Pudasaini
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.
authors.forename: Jing
authors.initials: J
authors.lastname: He
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China.|Thrombosis and Vascular Medicine Center, State Key Laboratory of Cardiovascular Disease, Fu Wai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, 100037, China.
authors.forename: Zhicheng
authors.initials: Z
authors.lastname: Jing
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, 200433, China. jinmingliu2013@126.com.
authors.forename: Jinming
authors.initials: J
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001987:Bronchiectasis / Q000150:complications*; D002681:China; D004452:Echocardiography; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000401:mortality / Q000503:physiopathology*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult
keywords: bronchiectasis; hemodynamics; pulmonary hypertension