Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis.

BACKGROUND

Idiopathic pulmonary fibrosis (IPF) is a disease with very high mortality.

OBJECTIVE

We sought to characterize serial changes in pulmonary artery pressures (PAP) in patients with advanced IPF who survive to transplant.

METHODS

Retrospective analysis of IPF patients comparing mean PAP at the time of initial evaluation for transplan- tation (mPAP(baseline)) with mPAP at the time of transplant (mPAP(follow-up)). The measurements were correlated with New York Heart Association (NYHA) functional class and oxygen requirements.

RESULTS

The final cohort consisted of 44 patients with serial right heart catheterization data. The mean mPAP(baseline) and mPAP(follow-up) were 22.5 and 32.7 mm Hg, respectively. 38.6% (17/44) of the patients had pulmonary hypertension (PH) at baseline. The majority of the non-PH patients developed PH during the serial time interval with a subsequent incidence of 77.8%. At the time of transplant, 86.4% of the patients had PH. There was a significant association between transplant NYHA class, severity of PH and oxygen requirements. Transplant NYHA class IV patients had a higher rate of mPAP change. The severity of PH at the time of transplant did not affect transplant outcomes.

CONCLUSION

PH is common and progressive in patients with advanced IPF who are transplant candidates. Serial change and severity of PAP elevations have a significant association with oxygen requirements and functional status, but not transplant outcomes. Whether or not progressive PH has a significant impact on outcomes without transplantation requires further study.