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The Impact of Pulmonary Hypertension on Survival in Patients With Idiopathic Pulmonary Fibrosis* FREE TO VIEW

Hassan F. Nadrous, MD; Patricia A. Pellikka, MD; Michael J. Krowka, MD, FCCP; Karen L. Swanson, DO, FCCP; Nithima Chaowalit, MD; Paul A Decker, MS; Jay H. Ryu, MD, FCCP
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*From the Divisions of Pulmonary, Critical Care and Internal Medicine (Drs. Nadrous and Ryu) and Cardiovascular Diseases (Drs. Pellikka and Chaowalit), the Department of Health Sciences Research Clinic (Mr. Decker), and the Division of Pulmonary Hypertension (Drs. Krowka and Swanson), Mayo Clinic and Foundation, 200 First Street SW, Rochester, MN.

Correspondence to: Jay H Ryu, MD, Mayo Clinic, 200 First St SW, Rochester, MN 55905; e-mail: ryu.jay@mayo.edu

Chest. 2005;128(6_suppl):616S-617S. doi:10.1378/chest.128.6_suppl.616S
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In this study, we describe survival in patients with idiopathic pulmonary fibrosis (IPF) who develop pulmonary hypertension (PH). PH may develop during the clinical course of IPF. The prevalence of PH in patients with IPF and the impact of PH on survival of these patients are not known.

We conducted a retrospective chart review of 487 patients with IPF who had been seen at the Mayo Clinic in Rochester, MN, during the period January 1, 1994, to December 31, 1996. Patients who had undergone transthoracic echocardiograms with the measurement of systolic pulmonary artery pressure (SPAP) within 3 months of the initial evaluation at the Mayo Clinic (ie, the index visit) for IPF were included. SPAP was determined using the measurement of tricuspid regurgitation velocity. Cumulative survival probabilities were estimated using the Kaplan-Meier method. The log rank test was used to compare the survival of groups of patients. A Cox proportional hazards regression model was used to assess the association of SPAP with survival. In all cases, p values of < 0.050 were considered to be statistically significant

We identified 88 patients with transthoracic echocardiographic measurement of SPAP at the time of their index visit. These patients were stratified into the following three groups based on their estimated SPAP: SPAP ≥ 0 mm Hg and ≤ 35 mm Hg, 14 patients (16%); SPAP > 35 mm Hg and ≤ 50 mm Hg, 47 patients (53%); and SPAP > 50 mm Hg, 27 patients (31%). The estimated SPAP ranged from 27.7 to 116.0 mm Hg (median, 45.4 mm Hg; mean, 48.3 mm Hg; SD, 15.5 mm Hg). For the survival analysis, time zero was the index visit. As SPAP increased, patients were more likely to die with a hazard ratio of 1.34 for each 10-mm Hg increase in SPAP (p < 0.001). When comparing survival across SPAP groups, the SPAP > 50 mm Hg group had significantly worse survival compared to the other groups (p = 0.009). The estimated 1-year and 3-year survival rates along with the median for these three groups are as follows:

PH, particularly with an SPAP of > 50 mm Hg is associated with worse survival in patients with IPF. The prevalence and prognostic significance of PH in patients with IPF as well as the impact of therapy for PH on these patients requires further exploration.

Abbreviations: IPF = idiopathic pulmonary fibrosis; PH = pulmonary hypertension; SPAP = systolic pulmonary artery pressure

Dr. Chaowalit was supported by Siriraj Hospital, Mahidol University, Bangkok, Thailand and by the Mayo Clinic.




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