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Chronic thromboembolic pulmonary hypertension: an updated review

Lang, Irene Ma; Klepetko, Walterb

doi: 10.1097/HCO.0b013e328311f254
Diseases of the aorta, pulmonary and peripheral vessels: Edited by Alan C. Braverman

Purpose of review Venous thromboembolism is a spectrum of disease comprising deep vein thrombosis, thrombus in transit, acute pulmonary embolism, and chronic thromboembolic pulmonary hypertension (CTEPH) as a rare and late possible sequela.

Recent findings On the basis of a prospective long-term study, the incidence of CTEPH is estimated at 3.8% within 2 years of all patients surviving an episode of symptomatic idiopathic pulmonary embolism. Young age, a large perfusion defect, and idiopathic clinical presentation are associated with a higher probability of CTEPH. Current pathophysiological concepts suggest a misguided thrombus resolution process that is triggered by infection, inflammation, autoimmunity, and malignancy. Diagnosis and therapy of CTEPH are interdisciplinary achievements and nowadays still based on a positive lung perfusion scan and for assessment of operability on a classical pulmonary angiography. Treatment of choice is surgical pulmonary endarterectomy of the pulmonary obstructions, which leads to restoration of normal pulmonary hemodynamics at rest in nearly 80% of patients. In expert centers, surgical mortality is under 10%.

Summary CTEPH has emerged as a ‘dual’ pulmonary vascular disorder with major vessel vascular remodeling of thrombus organization, combined with a small vessel pulmonary arteriopathy that is a target for classic vasodilator treatments.

aDepartment of Internal Medicine II, Division of Cardiology, Austria

bDepartment of Surgery, Division of Cardiothoracic Surgery, Vienna General Hospital, Medical University of Vienna, Vienna, Austria

Correspondence to Irene M. Lang, MD, Professor of Vascular Biology, Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria Tel: +43 1 40 400 4614; fax: +43 1 40400 4216; e-mail:

© 2008 Lippincott Williams & Wilkins, Inc.