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From (1) Department of Medicine, Division of Pulmonary and Critical Care Medicine, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, dzisman@mednet.ucla.edu, rasaggar@mednet.ucla.edu, dross@mednet.ucla.edu; jbelperio@mednet.ucla.edu, jplynch@mednet.ucla.edu; (2) Department of Medicine, Division of Geriatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, akarlamangla@mednet.ucla.edu; (3) Department of Surgery, Division of Cardiothoracic Surgery, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, aardehali@mednet.ucla.edu; (4) Department of Medicine, College of Physicians and Surgeons, Joseph L. Mailman School of Public Health, Columbia University, New York, NY, sk2097@columbia.edu; (5) Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, VA, oksana.shlobin@inova.com, steven.nathan@inova.com; (6) Department of Medicine, University of Colorado Health Sciences Center, Denver, CO, marvin.schwarz@uchsc.edu
dzisman{at}mednet.ucla.edu
Abstract
Background: We have developed a method to screen for pulmonary hypertension in idiopathic pulmonary fibrosis patients, based on a formula to predict mean pulmonary artery pressure from standard lung function measurements. The objective of this study was to validate this method in a separate group of idiopathic pulmonary fibrosis patients.
Methods: Cross-sectional study of 60 idiopathic pulmonary fibrosis patients from two institutions. The accuracy of the mean pulmonary artery pressure estimation was assessed by examining the correlation between the predicted and measured mean pulmonary artery pressures and the magnitude of the estimation error. The discriminatory ability of the method for pulmonary hypertension was assessed using the area under the receiver operating characteristic curve.
Results: There was strong correlation in the expected direction between the predicted and measured mean pulmonary artery pressures (r = 0.72; p <0.0001). The estimated mean pulmonary artery pressure was within 5 mmHg of the measured mean pulmonary artery pressure 72% of the time. The area under the receiver operating characteristic curve for predicting pulmonary hypertension was 0.85, and did not differ by institution. A formula-predicted mean pulmonary artery pressure > 21 mmHg was associated with a sensitivity, specificity, positive predictive value, and negative predictive value of 95%, 58%, 51%, and 96%, respectively, for pulmonary hypertension defined as mean pulmonary artery pressure from right-heart catheterization > 25 mmHg.
Conclusions: A prediction formula for mean pulmonary artery pressure using standard lung function measurements can be used to screen for pulmonary hypertension in idiopathic pulmonary fibrosis patients.
Key Words: "idiopathic pulmonary fibrosis" "prediction" "pulmonary fibrosis" "pulmonary hypertension"
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