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Validation of a Method To Screen for Pulmonary Hypertension in Advanced Idiopathic Pulmonary Fibrosis^*

^* From the Division of Pulmonary and Critical Care Medicine, (Drs. Zisman, Saggar, Ross, Belperio, and Lynch) and Division of Geriatrics (Dr. Karlamangla), Department of Medicine, and Division of Cardiothoracic Surgery (Dr. Ardehali), Department of Surgery, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA; Department of Medicine (Dr. Kawut), College of Physicians and Surgeons, Joseph L. Mailman School of Public Health, Columbia University, New York, NY; Advanced Lung Disease and Transplant Program (Drs. Shlobin and Nathan), Inova Fairfax Hospital, Falls Church, VA; and Department of Medicine (Dr. Schwarz), University of Colorado Health Sciences Center, Denver, CO.

Correspondence to: David A. Zisman, MD, MS, FCCP, Director, Interstitial Lung Disease Center, David Geffen School of Medicine at UCLA, 37–131 Center for Health Sciences, Los Angeles, CA 90095; e-mail: dzisman{at}mednet.ucla.edu

Abstract

Background: We have developed a method to screen for pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) patients, based on a formula to predict mean pulmonary artery pressure (MPAP) from standard lung function measurements. The objective of this study was to validate this method in a separate group of IPF patients.

Methods: Cross-sectional study of 60 IPF patients from two institutions. The accuracy of the MPAP estimation was assessed by examining the correlation between the predicted and measured MPAPs and the magnitude of the estimation error. The discriminatory ability of the method for PH was assessed using the area under the receiver operating characteristic curve (AUC).

Results: There was strong correlation in the expected direction between the predicted and measured MPAPs (r = 0.72; p < 0.0001). The estimated MPAP was within 5 mm Hg of the measured MPAP 72% of the time. The AUC for predicting PH was 0.85, and did not differ by institution. A formula-predicted MPAP > 21 mm Hg was associated with a sensitivity, specificity, positive predictive value, and negative predictive value of 95%, 58%, 51%, and 96%, respectively, for PH defined as MPAP from right-heart catheterization > 25 mm Hg.

Conclusions: A prediction formula for MPAP using standard lung function measurements can be used to screen for PH in IPF patients.

Key Words: idiopathic pulmonary fibrosis • prediction • pulmonary fibrosis • pulmonary hypertension

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