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The development of pulmonary arterial hypertension (PAH) can complicate many interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF). We sought to characterize the prevalence of PAH and its impact on survival in patients with advanced IPF.

Retrospective analysis of consecutive IPF patients undergoing pretransplantation right heart catheterization.

Lung transplant and IPF referral center.

PAH was defined as a mean pulmonary artery pressure (mPAP) of > 25 mm Hg. We compared demographic, spirometric, 6-min walk test (6MWT) results, and survival outcomes between those with PAH and those without PAH.

Seventy-nine patients were included in the study. PAH was present in 31.6% of patients (mean [± SD] mPAP, 29.5 ± 3.3 vs 19.1 ± 3.7 mm Hg, respectively). Those patients with PAH had a lower mean diffusing capacity of the …