04 February 2019 by Gul Ongen

The Europe, Middle East and Africa (EMEA) chronic thromboembolic pulmonary hypertension (CTEPH) registry: study design and baseline demographics

The EMEA CTEPH registry is an ongoing, international, prospective, Phase IV observational study investigating the epidemiology, diagnosis, and treatment of CTEPH patients in EMEA countries.

Key inclusion criteria were ≥18 years, and CTEPH diagnosed by RHC. Patients will be monitored for 36 months post-enrollment. Primary assessments are change from initial to final visit in routine clinical parameters (i.e. 6MWD, WHO functional class, and hemodynamics). Secondary assessments include patient demographics, CTEPH subtype, diagnostic and treatment patterns, PEA eligibility and outcomes, and long-term outcomes (clinical parameters, mortality, complications, hospitalizations, healthcare provider visits).

Recruitment closed with 231 patients enrolled. Data presented are from the July 2018 data cut (n=205). The table shows patient baseline characteristics. Most patients were prevalent (173 [84%]; diagnosed >6 months prior to initial visit and received PH-targeted therapy); 32 patients (16%) were incident (diagnosed <6 months prior to initial visit and received no PH-targeted therapy). The commonest symptoms were shortness of breath (94%), fatigue (43%), edema (36%), and chest discomfort (30%). The commonest techniques used at diagnosis were echocardiography (85%), ECG (72%), computer tomography angiography (67%), and RHC (66%). At initial visit, 42% of patients underwent RHC, and 94 patients (46%) were considered operable. Of the 64 patients (31%) who underwent PEA before initial visit, 50% experienced recurrent/residual PH, and 50% were not yet assessed. For the 109 inoperable patients, the reasons were non-patient related (23%), comorbidities (42%), and distal disease (35%). At initial visit, 34% of patients were not receiving PH-targeted therapy. In those 136 patients receiving therapy, the commonest were PDE5i (49%), riociguat (40%), and ERA (21%); 71% were receiving monotherapy and 25% were receiving combination therapy.

This registry is the first of its kind in EMEA countries and is expected to provide insight into diagnosis and treatment of CTEPH in these regions.


Key Contributors

Gül Öngen,1 Bahri Akdeniz,2 Düzenli Mehmet Akif,3 Alexander Chernyavsky,4 Georges Dabar,5 Majdy Idrees,6 Elena Khludeeva,7 Hakan Kültürsay,8 Vera Lukianchikova,9 Tamila Martynyuk,10 Nesrin Moğulkoç,8 Murat A. Mukarov,11 Bülent Mutlu,12 Gülfer Okumuş,13 Anuar Omarov,14 Zeynep Pinar Önen,15 Hussam Sakkijha,16 Nadezhda Shostak,17 Maria Simakova,18 Talant Sooronbaev,19 Lale Tokgözoğlu,20 Tatyana Tomskaya,21 Hüseyin Yıldırım,22 Dmitry Zateyschchikov,23 Klaus Hechenbichler,24 Stefanie Nörtemann,24 Nagihan Turgut,25 Kai Vogtländer,25 Abdullah Aldalaan26 1Istanbul University, Cerrahpasa Faculty of Medicine, Istanbul, Turkey; 2Eylul University Faculty of Medicine, Izmir, Turkey; 3Necmettin Erbakan University, Meram Faculty of Medicine, Konya, Turkey; 4Meshalkin Novosibirsk Research Institute of Circulation Pathology Academician, Novosibirsk, Russia; 5Hotel Dieu de France, Beirut, Lebanon; 6Prince Sultan Military Medical City, Riyadh, Saudi Arabia; 7State Budgetary Healthcare Institutio, Primorskaya Regional Clinical Hospital №1, Vladivostok, Russia; 8Ege University, Faculty of Medicine, Izmir, Turkey; 9Regional State Budgetary Healthcare Institution, Regional Clinical Hospital №1, Khabarovsk, Russia; 10Federal State Budget Institution, National Medical Center of Cardiology, Moscow, Russia; 11National Research Cardiac Surgery Center, Astana, Kazakhstan; 12Marmara University, Faculty of Medicine, Istanbul, Turkey; 13Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey; 14Institute of Cardiology in Almaty, Almaty, Lebanon; 15Ankara University, Faculty of Medicine, Ankara, Turkey; 16King Fahad Medical City, Riyadh, Saudi Arabia; 17State Budgetary Healthcare Institution, Pirogov City Clinical Hospital №1 of Moscow City, Moscow, Russia; 18Federal State Budgetary Institution, North-Western Federal Medical Research Center Almazov, Saint Petersburg, Russia; 19National Center of Cardiology and Internal Medicine, Bishkek, Kyrgyzstan; 20Hacettepe University Faculty of Medicine, Ankara, Turkey; 21State Budgetary Institution of the Republic of Sakha (Yakutia), Republican Hospital №1, Yakutsk, Russia; 22Osmangazi University Faculty of Medicine, Eskisehir, Turkey; 23State Budgetary Healthcare Institution, City Clinical Hospital № 51 of Moscow Health Department, Moscow, Russia; 24Istitut Dr. Schuaerte, Munich, Germany; 25Bayer AG, Berlin, Germany; 26Department of Organs Transplant, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.

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