Circulation. 2006;113:2011-2020
doi: 10.1161/CIRCULATIONAHA.105.602565
doi: 10.1161/CIRCULATIONAHA.105.602565
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(Circulation. 2006;113:2011-2020.)
© 2006 American Heart Association, Inc.
Contemporary Reviews in Cardiovascular Medicine |
Chronic Thromboembolic Pulmonary Hypertension
From the Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany (M.M.H.); Department of Cardiothoracic and Vascular Surgery, Johannes Gutenberg University, Mainz, Germany (E.M.); Centre of Maladies Vasculaires Pulmonaires, Hôpital Antoine Béclère, Clamart, France (G.S.); and Pulmonary Vascular Program, University of California, San Diego (L.J.R.).
Correspondence to Marius M. Hoeper, MD, Department of Respiratory Medicine, Hannover Medical School, 30623 Hannover, Germany. E-mail hoeper.marius@mh-hannover.de
Key Words: embolism • pulmonary hypertension
An extract of the first 250 words of the full text is provided, because this article has no abstract. |
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Introduction |
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Chronic thromboembolic pulmonary hypertension (CTEPH) has emerged as one of the leading causes of severe pulmonary hypertension. The disease is notoriously underdiagnosed, and the true prevalence is still unclear. CTEPH is characterized by intraluminal thrombus organization and fibrous stenosis or complete obliteration of pulmonary arteries.1 The consequence is an increased pulmonary vascular resistance resulting in pulmonary hypertension and progressive right heart failure. Vascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for patients with CTEPH,2 but not all patients are eligible for surgery. Recent research has provided evidence suggesting that the mechanistic view of CTEPH as a disease caused solely by obliteration of central pulmonary arteries due to organized thrombi may have been too simplistic. Pulmonary embolism, either as a single episode or as recurrent episodes, is thought to be the initiating event followed by progressive pulmonary vascular remodeling. This concept explains the clinical observation that CTEPH patients may have severe pulmonary hypertension out of proportion to the pulmonary vascular obliteration seen on a pulmonary angiogram. Thus, treatment of CTEPH often requires a multidisciplinary approach and may involve surgery, medical treatment, or both. However, many aspects of the pathogenesis of CTEPH are poorly understood, the diagnostic approach to these patients has not been standardized, and neither randomized controlled trials nor guidelines have been published in this field.
In July 2005, a group of international experts met in Zurich, Switzerland, for a comprehensive discussion of CTEPH. The members of this group are listed in the Appendix (in the online-only Data
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