cad a22 4500 316949302 20181103042250.0 cr unu---uuuuu 140620s2011 xx s 000 0 eng 10.5167/uzh-58408 doi 10.1161/CIRCULATIONAHA.110.015008 doi (ZORA)oai:www.zora.uzh.ch:58408 610 ddc 21969018 pmid Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry [Elektronische Daten] [J Pepke-Zaba, M Delcroix, I Lang, E Mayer, P Jansa, D Ambroz, C Treacy, A D'Arminio Monforte, M Morsolini, R Snijder, P Bresser, A Torbicki, B Kristensen, J Lewczuk, I Simkova, J A Barberà, M de Perrot, M M Hoeper, S Gaine, R Speich, M A Gomez-Sanchez, G Kovacs, A M Hamid, X Jaïs, G Simonneau] openAccess eu-repo 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. Clinic and Policlinic for Internal Medicine zora Pepke-Zaba J. joint author Delcroix M. joint author Lang I. joint author Mayer E. joint author Jansa P. joint author Ambroz D. joint author Treacy C. joint author D'Arminio Monforte A. joint author Morsolini M. joint author Snijder R. joint author Bresser P. joint author Torbicki A. joint author Kristensen B. joint author Lewczuk J. joint author Simkova I. joint author Barberà J A. joint author de Perrot M. joint author Hoeper M M. joint author Gaine S. joint author Speich R. joint author Gomez-Sanchez M A. joint author Kovacs G. joint author Hamid A M. joint author Jaïs X. joint author Simonneau G. joint author Circulation 124(18):1973-1981 https://www.zora.uzh.ch/id/eprint/58408/1/Circulation-2011-Pepke-Zaba-1973-81.pdf text/html WWW-Backlink auf das Repository 1 Journal Article PeerReviewed zora ZORA 856 40 https://www.zora.uzh.ch/id/eprint/58408/1/Circulation-2011-Pepke-Zaba-1973-81.pdf text/html WWW-Backlink auf das Repository ZORA 700 1- Pepke-Zaba J. joint author ZORA 700 1- Delcroix M. joint author ZORA 700 1- Lang I. joint author ZORA 700 1- Mayer E. joint author ZORA 700 1- Jansa P. joint author ZORA 700 1- Ambroz D. joint author ZORA 700 1- Treacy C. joint author ZORA 700 1- D'Arminio Monforte A. joint author ZORA 700 1- Morsolini M. joint author ZORA 700 1- Snijder R. joint author ZORA 700 1- Bresser P. joint author ZORA 700 1- Torbicki A. joint author ZORA 700 1- Kristensen B. joint author ZORA 700 1- Lewczuk J. joint author ZORA 700 1- Simkova I. joint author ZORA 700 1- Barberà J A. joint author ZORA 700 1- de Perrot M. joint author ZORA 700 1- Hoeper M M. joint author ZORA 700 1- Gaine S. joint author ZORA 700 1- Speich R. joint author ZORA 700 1- Gomez-Sanchez M A. joint author ZORA 700 1- Kovacs G. joint author ZORA 700 1- Hamid A M. joint author ZORA 700 1- Jaïs X. joint author ZORA 700 1- Simonneau G. joint author ZORA 773 0- Circulation 124(18):1973-1981 BK010053 XK010053 XK010000 ZORA ZORA ZORA Journal Article openAccess