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(Chest. 2004;126:78S-92S.)
© 2004 American College of Chest Physicians

Prognosis of Pulmonary Arterial Hypertension*

ACCP Evidence-Based Clinical Practice Guidelines

Vallerie V. McLaughlin, MD, FCCP; Kenneth W. Presberg, MD, FCCP; Ramona L. Doyle, MD, FCCP; Steven H. Abman, MD; Douglas C. McCrory, MD, MHSc; Terry Fortin, MD and Gregory Ahearn, MD

From the University of Michigan (Dr. McLaughlin), Ann Arbor, MI; Medical College of Wisconsin (Dr. Presberg), Milwaukee, WI; Stanford University (Dr. Doyle), Stanford, CA; University of Colorado Health Sciences Center (Dr. Abman), The Children’s Hospital, Denver, CO; and Duke University Medical Center (Drs. McCrory, Fortin, and Ahearn), Durham, CA.

Correspondence to: Vallerie V. McLaughlin, MD, FCCP, University of Michigan, 1500 East Medical Center Dr, Women’s Hospital-Room L3119, Ann Arbor, MI 48109-0273; e-mail: vmclaugh{at}umich.edu

Although idiopathic pulmonary arterial hypertension is perceived as a progressive disease with a uniformly poor outcome, the natural history of disease is heterogeneous, with some patients dying within months of diagnosis and others living for decades. The course of the disease has also been altered by advances in medical therapies. The outcome of patients with other types of pulmonary arterial hypertension (PAH) has been less well characterized. Assessment of prognosis of such patients is important, as it influences both medical therapy and referral for transplantation. This chapter will provide evidence based recommendations to assess the prognosis of patients with PAH.

Key Words: cardiopulmonary exercise testing • echocardiography • epoprostenol • functional class • hemodynamics • prognosis • pulmonary hypertension • 6 min walk test • survival • vasoreactivity




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