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Therapy for Pulmonary Arterial Hypertension: The More, the Merrier?

Darren B. Taichman, MD, PhD
[+] Article, Author, and Disclosure Information

From University of Pennsylvania, Philadelphia, PA 19104.

Potential Financial Conflicts of Interest:Grants received: Actelion.

Requests for Single Reprints: Darren B. Taichman, MD, PhD, University of Pennsylvania Medical Center, Pulmonary, Allergy & Critical Care Division, Suite 120, PHI Building, Penn-Presbyterian Medical Center, 51 North 39th Street, Philadelphia, PA 19104; e-mail, darren.taichman@uphs.upenn.edu.

Ann Intern Med. 2008;149(8):583-585. doi:10.7326/0003-4819-149-8-200810210-00010
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Without effective therapy, patients with pulmonary arterial hypertension (PAH) have inexorably worsening dyspnea and declining exercise capacity and can die, most often of right heart failure. The disease involves dysregulated endothelial and vascular smooth-muscle cell growth, in situ thrombosis, and an imbalance of vasodilators and constrictors. The result is a noncompliant, markedly narrowed blood vessel that resists blood flow from a progressively weakening right ventricle. Less than 20 years ago, the mean survival of patients with idiopathic PAH (formerly called primary) was only 2.8 years. There was little to offer beyond supplemental oxygen, anticoagulants, and diuretics (1).

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