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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-58408

Pepke-Zaba, J; Delcroix, M; Lang, I; Mayer, E; Jansa, P; Ambroz, D; Treacy, C; D'Arminio Monforte, A; Morsolini, M; Snijder, R; Bresser, P; Torbicki, A; Kristensen, B; Lewczuk, J; Simkova, I; Barberà, J A; de Perrot, M; Hoeper, M M; Gaine, S; Speich, R; Gomez-Sanchez, M A; Kovacs, G; Hamid, A M; Jaïs, X; Simonneau, G (2011). Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation, 124(18):1973-1981.

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Abstract

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated.
METHODS AND RESULTS: The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%- 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension-targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate.
CONCLUSIONS: Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension-targeted treatments.

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic and Policlinic for Internal Medicine
DDC:610 Medicine & health
Language:English
Date:2011
Deposited On:04 Mar 2012 10:34
Last Modified:17 Jun 2014 16:21
Publisher:American Heart Association
ISSN:0009-7322
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:10.1161/CIRCULATIONAHA.110.015008
PubMed ID:21969018
Citations:Web of Science®. Times Cited: 68
Google Scholar™
Scopus®. Citation Count: 93

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