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(Chest. 2006;130:545-552.)
© 2006 American College of Chest Physicians

Thrombotic Arteriopathy and Anticoagulation in Pulmonary Hypertension*

Sindhu R. Johnson, MD; John T. Granton, MD and Sanjay Mehta, MD, FCCP

* From the Division of Rheumatology (Dr. Johnson) and Pulmonary Hypertension Centre (Dr. Granton), University Health Network, University of Toronto, Toronto; and Centre for Critical Illness Research (Dr. Mehta), Lawson Health Research Institute, Division of Respirology, London Health Sciences Centre, London. ON, Canada.

Correspondence to: Sanjay Mehta, MD, FCCP, Southwest Ontario Pulmonary Hypertension Clinic, Division of Respirology, Department of Medicine, London Health Sciences Center, Victoria Hospital, Room E2.624, Professional Block, 800 Commissioner’s Rd East, London, ON, Canada, N6A 4G5; e-mail: sanjay.mehta{at}lhsc.on.ca

Abstract

The role of thrombotic arteriopathy in the pathophysiology of idiopathic pulmonary arterial hypertension (IPAH) and the use of anticoagulants in the treatment of IPAH are currently controversial issues. This article reviews the evidence for a role of vascular thrombosis in the pathophysiology of IPAH. There is sufficient biological rationale to support the notion that thrombotic arteriopathy is an important pathophysiologic feature of pulmonary arterial hypertension (PAH) and that its progression materially contributes to disease progression. To date, the data from observational studies suggest that anticoagulation with warfarin is an effective intervention in patients with IPAH. Its efficacy in other causes of PAH remains speculative.

Key Words: idiopathic pulmonary arterial hypertension • pulmonary hypertension • thrombosis • thrombotic arteriopathy




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