Chest
Volume 140, Issue 2, August 2011, Pages 301-309
Journal home page for Chest

Original Research
Pulmonary Vascular Disease
Survival of Chinese Patients With Pulmonary Arterial Hypertension in the Modern Treatment Era

https://doi.org/10.1378/chest.10-2327Get rights and content

Background

In a previous study of Chinese patients with idiopathic pulmonary arterial hypertension (IPAH) in the nontargeted therapy era (defined as the time before 2006 when new pulmonary arterial hypertension-specific drugs were not available in China), we reported 1- and 3-year survival estimates of only 68% and 39%, respectively. However, it is not yet known whether the survival of patients with pulmonary arterial hypertension is improved in the modern treatment era (defined in China as after 2006).

Methods

A retrospective cohort study was undertaken in 276 consecutive patients with newly diagnosed incident IPAH and connective tissue disease-related pulmonary arterial hypertension (CTDPAH) who were referred between 2007 and 2009. Baseline characteristics and survival rates in the two groups were compared.

Results

The 1- and 3-year survival estimates were 92.1% and 75.1%, respectively, in patients with IPAH, and 85.4% and 53.6%, respectively, in patients with CTDPAH. Patients with CTDPAH had a significantly lower mean pulmonary artery pressure, more pericardial effusion, and more severe impairment of the diffusion capacity of the lung for carbon monoxide than patients with IPAH. A diagnosis of CTDPAH, World Health Organization functional class III or IV, single-breath diffusion capacity of the lung for carbon monoxide < 80% predicted, and the presence of pericardial effusion were independent predictors of mortality. The 1- and 3-year survival rates of male patients were 93.5% and 77.5%, respectively, in those with IPAH, and 71.1% and 47.4%, respectively, in those with CTDPAH.

Conclusions

The survival rates of patients with pulmonary arterial hypertension have improved in China in the modern treatment era, despite the high costs of treatment and financial constraints. However, the survival rates of patients with CTDPAH are inferior to those of patients with IPAH. Our study also indicates poorer survival rates in male patients with CTDPAH.

Section snippets

Study Patients

We performed a retrospective cohort study of consecutive patients who received a diagnosis of IPAH or CTDPAH between 2007 and 2009 at five national referral pulmonary vascular centers in China, located in the northern, northeastern, central, southern, and eastern regions, according to a standardized diagnostic and treatment approach for PAH. Details of all newly diagnosed incident cases were recorded in a database at the time of diagnosis before any targeted treatments for PAH were started. The

Demographic Characteristics

A total of 276 patients were treated, including 173 patients who received a diagnosis of IPAH and 103 patients who received a diagnosis of CTDPAH. In patients with CTDPAH, systemic lupus erythematosus, scleroderma, Sjögren syndrome, mixed connective tissue disease, and arthritis accounted for 38%, 22%, 11%, 10%, and 4% of the population, respectively. The baseline demographic and clinical characteristics of the two etiologic groups are shown in Table 1. Most patients in both groups were women.

Discussion

The present study is one of the largest cohort studies from China concerning the clinical characteristics and survival of patients with PAH in the modern treatment era. Several clinical trials in Chinese patients with PAH have demonstrated the benefit of newer targeted therapies.16, 17 Therefore, it is important to examine the outcome of targeted treatment with these agents for various PAH subtypes in China.

In an earlier study, Schachna et al18 identified increasing age as a risk factor for PAH

Acknowledgments

Author contributions: Dr Zhang: contributed to recruiting the patients, drafting the submitted article, interpreting the data, performing the statistical analysis of the data, and approving the final manuscript.

Dr Dai: contributed to recruiting the patients, drafting the submitted article, interpreting the data, performing the statistical analysis of the data, and approving the final manuscript.

Dr Xie: contributed to enrolling the subjects, data acquisition, and approving the final manuscript.

References (28)

  • LJ Rubin

    Primary pulmonary hypertension

    N Engl J Med

    (1997)
  • HW Farber et al.

    Pulmonary arterial hypertension

    N Engl J Med

    (2004)
  • VV McLaughlin et al.

    Survival in primary pulmonary hypertension: the impact of epoprostenol therapy

    Circulation

    (2002)
  • S Provencher et al.

    Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension

    Eur Heart J

    (2006)
  • Cited by (0)

    Drs Zhang and Dai contributed equally to this article.

    Funding: This study was sponsored by the Shanghai Pujiang Program [Grant 08PJ1408600] and the Shanghai Science and Technology Division of Non-governmental International Cooperation Projects [Grant 08410701600].

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).

    View full text