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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front. Med.    2016, Vol. 10 Issue (3) : 336-344     DOI: 10.1007/s11684-016-0461-z
RESEARCH ARTICLE |
Clinical characteristics of pulmonary hypertension in bronchiectasis
Lan Wang1,Sen Jiang2,Jingyun Shi2,Sugang Gong1,Qinhua Zhao1,Rong Jiang1,Ping Yuan1,Bigyan Pudasaini1,Jing He1,Zhicheng Jing1,3,Jinming Liu1,*()
1. Department of Cardio-Pulmonary Circulation
2. Department of Radiology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China
3. 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
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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±93.3 m. The mean pulmonary arterial pressure (PAP) was 41.5±11.7 mmHg, cardiac output was 5.2±1.4 L/min, and pulmonary vascular resistance was 561.5±281.5 dyn·s·cm5. 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.

Keywords bronchiectasis      hemodynamics      pulmonary hypertension     
Corresponding Authors: Jinming Liu   
Just Accepted Date: 13 July 2016   Online First Date: 10 August 2016    Issue Date: 30 August 2016
URL:  
http://academic.hep.com.cn/fmd/EN/10.1007/s11684-016-0461-z     OR     http://academic.hep.com.cn/fmd/EN/Y2016/V10/I3/336
Fig.1  Correlations between systolic PAP estimated during echocardiography and (A) meanPAP measured during RHC or (B) by systolic PAP. (C) Accuracy of estimated systolic PAP compared with systolic PAP measured by RHC in relation to PAP. ……: -10 mmHg and+10 mmHg.
Characteristics With PH(n = 36) Without PH(n = 35) P value
Age (year) 51.5±12.4 62.2±13.5 <0.001
Female (number [%]) 22 (61.1) 19 (54.3) 0.634
Body mass index (kg/m2) 19.8±4.0 20.7±4.0 0.345
Dyspnea (number [%]) 36 (100.0) 31 (88.6) 0.038
Hemoptysis (number [%]) 16 (44.4) 18 (51.4) 0.559
History of right heart failure or lower limb edema (number [%]) 26 (72.2) 10 (28.6) <0.001
Syncope at exercise (number [%]) 0 (0) 1 (2.8) 0.310
Finger clubbing (number [%]) 18 (50.0) 3 (8.6) <0.001
FEV1/FVC (%) 56.0±15.6 59.8±15.1 0.350
FEV1 (% pred., median [IQR]) 27.5 (20.9–30.7) 36.7 (26.4–58.8) 0.009
TLC (% pred.) 112.3±18.9 110.0±27.7 0.740
DLco (% pred.) 39.4±18.7 69.2±34.5 0.011
PaCO2 (mmHg, median [IQR]) 51.7 (46.5–58.7) 41.5 (38.4–43.9) <0.001
PaO2 (mmHg) 57.8±14.0 78.6±17.6 <0.001
RA dimension (mm) 46.1±11.2 33.5±4.8 <0.001
RV dimension (mm) 43.2±9.2 29.7±4.6 <0.001
LVEF (%) 69.2±10.6 68.8±7.0 0.873
TRV (m/s) 4.0±0.6 2.5±0.3 <0.001
sPAP (mmHg) 70.6±21.4 31.3±6.2 <0.001
TAPSE (mm) 17.3±3.5 20.5±3.1 <0.001
RAP (mmHg) 7.9±3.9 6.0±1.5 0.015
Image type (number [%]) <0.001
Cystic bronchiectasis 15 (41.7) 9 (25.7)
Cylindrical bronchiectasis 3 (8.3) 16 (45.7)
Mixed bronchiectasis 18 (50.0) 10 (28.6)
Extent of bronchiectasis (median [IQR]) 17.0 (16.0–17.8) 15.0 (11.0–17.0) 0.001
Six-minute walk distance (m) 300.8±93.3 401.2±89.7 0.001
NT-proBNP (pg/ml, median [IQR]) 864.0 (517.0–1737.0) 96.0 (43.0-228.0) 0.005
Tab.1  Characteristics of bilateral bronchiectasis patients with and without PH
Characteristic No. of patients Value
PVR (dyn·s·cm-5) 33 561.5±281.5
PAWP (mmHg) 33 8.7±3.5
sPAP (mmHg) 36 68.5±17.1
dPAP (mmHg) 36 23.3±8.8
mPAP (mmHg) 36 41.5±11.7
RAP (mmHg) 36 5.0±4.4
CO (L/min) 36 5.2±1.4
CI (L/(min•m2)) 36 3.5±0.8
SvO2 (%) 33 59.1±9.7
MAP (mmHg) 36 90.8±19.8
Tab.2  Hemodynamic data at the time of diagnosis of pulmonary hypertension
Fig.2  Correlation between mean pulmonary arterial pressure (PAP) and arterial oxygen saturation (SaO2). Regression lines are indicated.
Fig.3  Outcome of patients who received pulmonary arterial hypertension (PAH) therapy. NYHA: New York Heart Association.
Variables No. of patients Pretreatment Post-treatment P
LVEF (%) 24 70.6±15.1 69.3±11.1 0.692
TRV (m/s) 24 4.2±0.8 3.6±0.5 0.012
sPAP (mmHg) 24 82.4±27.0 65.5±20.9 0.025
TAPSE (mm) 24 15.7±2.2 18.3±4.5 0.063
RAP (mmHg) 24 8.9±3.7 8.5±2.8 0.773
NT-proBNP (pg/ml, median [IQR]) 15 748.5 (351.8–1658.5) 504.0 (161.5–3016.5) 0.925
PaO2 (mmHg) 18 58.8±14.3 52.4±11.3 0.394
PaCO2 (mmHg) 18 52.5±8.2 51.5±9.5 0.175
SaO2 (%) 18 85.8±8.3 82.4±9.0 0.769
DA-aO2 (mmHg) 18 50.3±37.5 51.8±25.7 0.117
Tab.3  Clinical variables before and after treatment with PAH-specific therapy
Fig.4  Overall survival in patients with bronchiectasis and pulmonary hypertension.
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