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16 August 2005 | Volume 143 Issue 4 | Pages 282-292
Increased pressure in the pulmonary circulation, or pulmonary hypertension, is a common disorder that may complicate various cardiopulmonary conditions, including severe obstructive airways disease and left ventricular dysfunction. An increase in pulmonary arterial pressure that is not due to coexistent cardiopulmonary disease, known as pulmonary arterial hypertension, may occur in the absence of a demonstrable cause (idiopathic or familial); as a complication of systemic conditions, such as connective tissue disease, HIV infection, or chronic liver disease; or as a result of the use of fenfluramine anorexigens, amphetamines, or cocaine. The development of disease-specific therapies for pulmonary arterial hypertension over the past decade underscores the importance of diagnosing pulmonary hypertension early in the course of the condition and implementing a treatment strategy that is based on the condition's cause and severity. In this review, the authors present approaches to the diagnosis and management of pulmonary arterial hypertension, using a hypothetical case to highlight the key management points.
Author and Article Information
From the University of California, San Diego, Medical Center, La Jolla, California; and University of Colorado Health Sciences Center, Denver, Colorado.
Acknowledgments: The authors thank their colleagues who participated in the 3rd World Symposium on Pulmonary Artery Hypertension and who served on the ACCP Evidence-Based Guidelines panel, from whose work they have drawn in the preparation of this manuscript.
Potential Financial Conflicts of Interest: Consultancies: L.J. Rubin (Actelion, Pfizer Inc., Schering, CoTherix, United Therapeutics, Myogen), D.B. Badesch (GlaxoWellcome/GlaxoSmithKline, Actelion, Berlex, Astra Merck, AstraZeneca, Myogen, Intermune, Forest Laboratories, Encysive, Exhale Therapeutics/CoTherix, Pfizer, Scios, MondoBiotech, PR Pharmaceuticals); Honoraria: L.J. Rubin (Actelion, Myogen), D.B. Badesch (Glaxo Wellcome/GlaxoSmithKline, Actelion, Pfizer Inc., Encysive, United Therapeutics); Stock ownership or options (other than mutual funds): D.B. Badesch (Johnson & Johnson); Expert testimony: L.J. Rubin (Appetite suppressant litigation), D.B. Badesch (Appetite suppressant litigation); Grants received: L.J. Rubin (Actelion, Pfizer Inc., CoTherix, Myogen, National Institutes of Health), D.B. Badesch (Glaxo Wellcome/GlaxoSmithKline, United Therapeutics, Boehringer Ingelheim, Actelion, ICOS Corp./Texas Biotechnologies, Encysive, Pfizer Inc., Myogen, CoTherix, Lilly/ICOS, American Lung Association, American Heart Association, National Institutes of Health, Scleroderma Foundation).
Requests for Single Reprints: Lewis J. Rubin, MD, University of California, San Diego, Medical Center, 9300 Campus Point Drive, M/C 7381, La Jolla, CA 92037-7381.
Current Author Addresses: Dr. Rubin: University of California, San Diego, Medical Center, 9300 Campus Point Drive, La Jolla, CA 92037-7381.
Dr. Badesch: University of Colorado Health Sciences Center, Box C-272, 4200 East Ninth Avenue, Denver, CO 80262. REVIEW
Evaluation and Management of the Patient with Pulmonary Arterial Hypertension
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