Chest
Original ResearchDiffuse Lung DiseaseSildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction
Section snippets
Setting and Participants
The complete STEP-IPF protocol has been previously published.6 See e-Appendix 2 for a full description of the study methods. This was a double-blind, placebo-controlled trial of sildenafil in patients with advanced IPF and was conducted at 14 IPFnet centers (e-Appendix 1). All subjects provided written informed consent. The study was approved by institutional review boards at participating institutions. Eligibility criteria included consensus criteria-defined IPF and carbon monoxide diffusing
Results
Of 180 subjects enrolled into STEP-IPF, echocardiograms from 119 were available for independent review (sildenafil, n = 56; placebo, n = 63) (Fig 1). The remaining 61 echocardiograms could not be transferred to the echocardiogram core for review due to inability of clinical centers to obtain institutional review board permission for data transfer. No significant difference was detected in the high-resolution CT image scoring or pathology scoring for patients treated with sildenafil vs placebo
Discussion
We have demonstrated that sildenafil treatment results in significantly better exercise capacity and QOL in IPF for subjects with RVSD present on echocardiogram. It has previously been demonstrated that PH results in exercise limitation in patients with IPF.14 Prior work suggests that sildenafil leads to preferential pulmonary artery vasodilation in well-ventilated lung tissue and may improve ventilation-perfusion matching and gas exchange in IPF.4 Hence, STEP-IPF hypothesized that sildenafil
Acknowledgments
Author contributions: Drs Han and Anstrom had full access to all of the data and take responsibility for the integrity and accuracy of the data analysis.
Dr Han: contributed to study conception and design; data collection, analysis, and interpretation; writing of the manuscript; and served as principal author.
Dr Bach: contributed to data analysis and interpretation, and writing of the manuscript.
Dr Hagan: contributed to data analysis and interpretation, and writing of the manuscript.
Mr Yow:
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Cited by (0)
Funding/Support: This work is supported by funding from National Institutes of Health/National Heart, Lung and Blood Institute [Grants K23 HL093351 and U10HL080509 (data coordinating center), U10HL80413, U10HL80274, U10HL80370, U10HL80371, U10HL80383, U10HL80411, U10HL80509, U10HL80510, U10HL80513, U10HL80543, U10HL80571, and U10HL80685 (clinical centers)]; by the Cowlin Fund at the Chicago Community Trust; by Pfizer Inc, which donated sildenafil and matching placebo; and by Masimo Corp, which donated pulse oximeters.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.
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A complete list of study participants is located in e-Appendix 1.