Pulmonary function testing in patients with pulmonary arterial hypertension

Jing, Zhi-Cheng; Xu, Xi-Qi; Badesch, David B.; Jiang, Xin; Wu, Yan; Liu, Jin-Ming; Wang, Yong; Pan, Lei; Li, Hui-Ping; Pu, Jie-Lin; Zhang, Zhuo-Li; Yang, Yue-Jin

doi:10.1016/j.rmed.2009.03.009

« Previous Next »Respiratory Medicine
Volume 103, Issue 8 , Pages 1136-1142, August 2009

Pulmonary function testing in patients with pulmonary arterial hypertension

Zhi-Cheng Jing
- Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
- Fu Wai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Corresponding author: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, No. 507 Zhengmin Road, Shanghai 200433, China.
- These authors contributed equally to this article.
Xi-Qi Xu
- Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
- Aviation Industry Central Hospital, Beijing, China
- These authors contributed equally to this article.
David B. Badesch
- University of Colorado Health Sciences Center, USA
Xin Jiang
- Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
Yan Wu
- Fu Wai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Jin-Ming Liu
- Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
Yong Wang
- Beijing Shijitan Hospital, Peking University, Beijing, China
Lei Pan
- Beijing Shijitan Hospital, Peking University, Beijing, China
Hui-Ping Li
- Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
Jie-Lin Pu
- Fu Wai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Zhuo-Li Zhang
- Peking University First Hospital, Beijing, China
Yue-Jin Yang
- Fu Wai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

Received 22 November 2008; accepted 2 March 2009. published online 29 April 2009.

Summary

Background

Although previous studies have shown that peripheral airway obstruction can occur in idiopathic PAH (IPAH), pulmonary function tests have not been well-studied in patients with PAH associated with congenital heart disease (CHD-PAH) and connective tissue disease (CTD-PAH).

Methods

A multicenter prospective study was performed in PAH patients in China. Pulmonary function tests were evaluated in 190 PAH patients.

Results

Total lung capacity (TLC), residual volume (RV) and total airway resistance (Rtot) were similar in PAH patients and controls. However, measures of airflow, including vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1s (FEV₁), FEV₁/FVC and MEF₅₀ were decreased in PAH group. Single-breath diffusion capacity for carbon monoxide (DL_CO) was also decreased in PAH patients. Expiratory flow–volume curves showed reduction and a curvilinear appearance in patients with PAH. Similar changes were observed among the various subgroups of IPAH, CHD-PAH, and CTD-PAH patients. More CTD-PAH patients had abnormal DL_CO.

Conclusions

Airway obstruction is common in IPAH, CHD-PAH and CTD-PAH patients. CTD-PAH patients have lower DL_CO. Hemodynamics, serum markers and exercise capacity parameters did not correlate well with pulmonary function indices.

Keywords: Airway obstruction, Pulmonary arterial hypertension, Pulmonary function test, Pulmonary diffusion capacity