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Published online before print July 11, 2007
Eur Respir J 2007, doi:10.1183/09031936.00107206
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ORIGINAL ARTICLE

Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant

A.F. Shorr 1*, J.L. Wainright 2, C.S. Cors 2, C.J. Lettieri 3, S.D. Nathan 4

1 Pulmonary and Critical Care Medicine Section, Washington Hospital Center, Washington, DC
2 United Network for Organ Sharing, Richmond, VA
3 Pulmonary and Critical Care Medicine Service, Walter Reed Army Medical Center, Washington, DC
4 INOVA Transplant Center, Fairfax Inova Hospital, Falls Church, VA

* To whom correspondence should be addressed. E-mail: afshorr{at}dnamail.com.


   Abstract

Pulmonary hypertension (PH) may complicate idiopathic pulmonary fibrosis (IPF) but the prevalence of PH in IPF remains undefined. We sought to describe the prevalence of PH in IPF.

We analyzed the lung transplant (LT) registry for the US (January 1995 to June 2004) and identified IPF patients who had undergone right heart catheterization (RHC). We defined PH as a mPAP of ≥25 mm Hg and severe PH as an mPAP of >40 mm Hg. Independent factors associated with PH were determined.

Of 3, 457 persons listed, 2, 525 (73.0%) had undergone RHC. PH affected 46.1% of subjects. Approximately 9% had severe PH. Variables independently associated with mild to moderate PH were: need for oxygen, PCWP, and FEV1. Independent factors related to severe PH included: pCO2, age, FEV1, PCWP, need for oxygen, and race. A sensitivity analysis in subjects with PCWPs of <15 mm Hg did not appreciably alter our findings.

PH is common in IPF awaiting LT, but the elevations in mPAP are moderate. Lung volumes alone do not explain the PH. Given the prevalence of PH and its relationship with surrogate markers for quality of life (e.g. ADLs), future trials of therapies for this may be warranted.

Keywords:  Epidemiology, hemodynamics, idiopathic pulmonary fibrosis, pulmonary hypertension




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