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Original Research: Diffuse Lung Disease |

Echocardiographic and Hemodynamic Predictors of Mortality in Idiopathic Pulmonary FibrosisMortality in Idiopathic Pulmonary Fibrosis

Belinda N. Rivera-Lebron, MD; Paul R. Forfia, MD, FCCP; Maryl Kreider, MD; James C. Lee, MD; John H. Holmes, PhD; Steven M. Kawut, MD, FCCP
Author and Funding Information

From the Department of Medicine (Drs Rivera-Lebron, Forfia, Kreider, Lee, and Kawut), Penn Cardiovascular Institute (Drs Forfia and Kawut), and Center for Clinical Epidemiology and Biostatistics (Drs Holmes and Kawut), Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

Correspondence to: Steven M. Kawut, MD, FCCP, Pulmonary, Allergy and Critical Care Division, Perelman School of Medicine, University of Pennsylvania, 711 Blockley Hall, 423 Guardian Dr, Philadelphia, PA 19104; e-mail: kawut@upenn.edu


Drs Holmes and Kawut contributed equally to this work.

Some of the results of this study were presented in abstract form at the American Thoracic Society International Conference, May 18-23, 2012, San Francisco, CA; in the form of an oral presentation at the International Society for Heart and Lung Transplantation, April 18-21, 2012, Prague, Czech Republic; and have been published in abstract form (Rivera-Lebron BN, Forfia P, Kawut SM, Holmes JH. Tricuspid annular displacement (TAPSE) assessment of right ventricular dysfunction in idiopathic pulmonary fibrosis [abstract]. Am J Respir Crit Care Med. 2012;185(suppl):A1908).

Funding/Support: This study was supported by National Institutes of Health [Grants HL007586 and HL007891 to Dr Rivera-Lebron and Grant K24 HL103844 to Dr Kawut].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(2):564-570. doi:10.1378/chest.12-2298
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Background:  Idiopathic pulmonary fibrosis (IPF) can lead to the development of pulmonary hypertension, which is associated with an increased risk of death. In pulmonary arterial hypertension, survival is directly related to the capacity of the right ventricle to adapt to elevated pulmonary vascular load. The relative importance of right ventricular function in IPF is not well understood. Our objective was to evaluate right ventricular echocardiographic and hemodynamic predictors of mortality in a cohort of patients with IPF referred for lung transplant evaluation.

Methods:  We performed a retrospective cohort study of 135 patients who met 2011 American Thoracic Society/European Respiratory Society criteria for IPF and who were evaluated for lung transplantation at the Hospital of the University of Pennsylvania.

Results:  Right ventricle:left ventricle diameter ratio (hazard ratio [HR], 4.5; 95% CI, 1.7-11.9), moderate to severe right atrial and right ventricular dilation (HR, 2.9; 95% CI, 1.4-5.9; and HR, 2.7; 95% CI, 1.4-5.4, respectively) and right ventricular dysfunction (HR, 5.5; 95% CI, 2.6-11.5) were associated with an increased risk of death. Higher pulmonary vascular resistance was also associated with increased mortality (HR per 1 Wood unit, 1.3; 95% CI, 1.1-1.5). These risk factors were independent of age, sex, race, height, weight, FVC, and lung transplantation status. Other hemodynamic indices, such as mean pulmonary artery pressure and cardiac index, were not associated with outcome.

Conclusions:  Right-sided heart size and right ventricular dysfunction measured by echocardiography and higher pulmonary vascular resistance by invasive hemodynamic assessment predict mortality in patients with IPF evaluated for lung transplantation.

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