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Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction.

Publication ,  Journal Article
Han, MK; Bach, DS; Hagan, PG; Yow, E; Flaherty, KR; Toews, GB; Anstrom, KJ; Martinez, FJ; IPFnet Investigators,
Published in: Chest
June 2013

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with pulmonary vasculopathy. OBJECTIVE: The purpose of this study was to determine whether sildenafil improves 6-min walk distance (6MWD) in subjects with IPF and right ventricular dysfunction. METHODS: The IPFnet, a network of IPF research centers in the United States, conducted a randomized trial examining the effect of sildenafil on 6MWD in patients with advanced IPF, defined by carbon monoxide diffusing capacity < 35% predicted. A substudy examined 119 of 180 randomized subjects where echocardiograms were available for independent review by two cardiologists. Right ventricular (RV) hypertrophy (RVH), right ventricular systolic dysfunction (RVSD), and right ventricular systolic pressure (RVSP) were assessed. Multivariable linear regression models estimated the relationship between RV abnormality, sildenafil treatment, and changes in 6MWD, St. George's Respiratory Questionnaire (SGRQ), the EuroQol instrument, and SF-36 Health Survey (SF-36) from enrollment to 12 weeks. RESULTS: The prevalence of RVH and RVSD were 12.8% and 18.6%, respectively. RVSP was measurable in 71 of 119 (60%) subjects; mean RVSP was 42.5 mm Hg. In the subgroup of subjects with RVSD, subjects treated with sildenafil experienced less decrement in 6MWD (99.3 m; P = .01) and greater improvement in SGRQ (13.4 points; P = .005) and EuroQol visual analog scores (17.9 points; P = .04) than subjects receiving placebo. In the subgroup with RVH, sildenafil was not associated with change in 6MWD (P = .13), but was associated with greater relative improvement in SGRQ (14.8 points; P = .02) vs subjects receiving placebo. Sildenafil treatment in those with RVSD and RVH was not associated with change in SF-36. CONCLUSIONS: Sildenafil treatment in IPF with RVSD results in better preservation of exercise capacity as compared with placebo. Sildenafil also improves quality of life in subjects with RVH and RVSD.

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Published In

Chest

DOI

EISSN

1931-3543

Publication Date

June 2013

Volume

143

Issue

6

Start / End Page

1699 / 1708

Location

United States

Related Subject Headings

  • Walking
  • Ventricular Dysfunction, Right
  • Vasodilator Agents
  • Surveys and Questionnaires
  • Sulfones
  • Statistics, Nonparametric
  • Sildenafil Citrate
  • Respiratory System
  • Respiratory Function Tests
  • Regression Analysis
 

Citation

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Han, M. K., Bach, D. S., Hagan, P. G., Yow, E., Flaherty, K. R., Toews, G. B., … IPFnet Investigators, . (2013). Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest, 143(6), 1699–1708. https://doi.org/10.1378/chest.12-1594
Han, MeiLan K., David S. Bach, Peter G. Hagan, Eric Yow, Kevin R. Flaherty, Galen B. Toews, Kevin J. Anstrom, Fernando J. Martinez, and Fernando J. IPFnet Investigators. “Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction.Chest 143, no. 6 (June 2013): 1699–1708. https://doi.org/10.1378/chest.12-1594.
Han MK, Bach DS, Hagan PG, Yow E, Flaherty KR, Toews GB, et al. Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest. 2013 Jun;143(6):1699–708.
Han, MeiLan K., et al. “Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction.Chest, vol. 143, no. 6, June 2013, pp. 1699–708. Pubmed, doi:10.1378/chest.12-1594.
Han MK, Bach DS, Hagan PG, Yow E, Flaherty KR, Toews GB, Anstrom KJ, Martinez FJ, IPFnet Investigators. Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest. 2013 Jun;143(6):1699–1708.

Published In

Chest

DOI

EISSN

1931-3543

Publication Date

June 2013

Volume

143

Issue

6

Start / End Page

1699 / 1708

Location

United States

Related Subject Headings

  • Walking
  • Ventricular Dysfunction, Right
  • Vasodilator Agents
  • Surveys and Questionnaires
  • Sulfones
  • Statistics, Nonparametric
  • Sildenafil Citrate
  • Respiratory System
  • Respiratory Function Tests
  • Regression Analysis