Diagnosis and Treatment Patterns of Chronic Thromboembolic Pulmonary Hypertension in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia: A Registry Study

Öngen H.G. Akdeniz B. Düzenli M.A. Chernyavsky A. Dabar G. Idrees M. Khludeeva E. Kültürsay H. Lukianchikova V. Martynyuk T. Moğulkoç N. Mukarov M.A. Mutlu B. Okumuş G. Omarov A. Önen Z.P. Sakkijha H. Shostak N. Simakova M. Tokgözoğlu L. Tomskaya T. Yildirim H. Zateyshchikov D. Hechenbichler K. Kessner S. Schauerte I. Turgut N. Vogtländer K. Aldalaan A.
March 2024 Adis

Drugs - Real World Outcomes
2024 #11 Issue 1 149 - 165 pp.

Background: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) in countries with limited resources have, to date, been poorly represented in registries. Objective: This work assesses the epidemiology, diagnosis, hemodynamic and functional parameters, and treatment of CTEPH in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia. Methods: A prospective, cohort, phase IV, observational registry with 3-year follow-up (n = 212) in patients aged ≥ 18 years diagnosed with CTEPH was created. Clinical, hemodynamic, and functional parameters were obtained at an initial visit, follow-up visits, and a final visit at the end of 3 years’ observation or end of follow-up. Data were recorded on electronic case report forms. Parameters evaluated included 6-minute walking distance (6MWD), use of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), pulmonary hypertension (PH)-targeted therapy, and survival. All statistical analyses were exploratory and descriptive, and were performed in the overall population. Results: The most common symptoms were typical of those expected for CTEPH. Almost 90% of patients underwent right heart catheterization at diagnosis or initial study visit. In total, 66 patients (31%) underwent PEA before the initial visit; 95 patients (45%) were considered operable, 115 (54%) were inoperable, and two (1%) had no operability data. Only 26 patients (12%) had been assessed for BPA at their initial visit. PH-targeted therapy was documented at diagnosis for 77 patients (36%), most commonly a phosphodiesterase type 5 inhibitor (23%). Use of PH-targeted therapy increased to 142 patients (67%) at the initial visit, remaining similar after 3 years. Use of riociguat increased from 6% of patients at diagnosis to 38% at 3 years. Between baseline and end of observation, results for patients with paired data showed an increase in 6MWD. Survival at the end of observation was 88%. Conclusions: These data highlight the current diagnosis and management of CTEPH in the participating countries. They show that early CTEPH diagnosis remains challenging, and use of off-label PH-targeted therapy is common. ClinicalTrials.gov: NCT02637050; registered December 2015. Graphical Abstract: (Figure presented.)

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Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, 34098, Turkey
Faculty of Medicine, Dokuz Eylül University, Izmir, Turkey
Meram Faculty of Medicine, Necmettin Erbakan University, Konya, Turkey
E. Meshalkin National Medical Research Center of the Ministry of Health of the Russian Federation, Moscow, Russian Federation
Hotel Dieu de France, Beirut, Lebanon
Prince Sultan Military Medical City, Riyadh, Saudi Arabia
State Budgetary Healthcare Institution, Primorskaya Regional Clinical Hospital No. 1, Vladivostok, Russian Federation
Faculty of Medicine, Ege University, Izmir, Turkey
Regional State Budgetary Healthcare Institution, Regional Clinical Hospital No. 1, Khabarovsk, Russian Federation
Federal State Budget Institution, National Medical Center of Cardiology, Moscow, Russian Federation
National Research Cardiac Surgery Center, Astana, Kazakhstan
Faculty of Medicine, Marmara University, Istanbul, Turkey
Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey
Institute of Cardiology in Almaty, Almaty, Kazakhstan
Faculty of Medicine, Ankara University, Ankara, Turkey
King Fahad Medical City, Riyadh, Saudi Arabia
State Budgetary Healthcare Institution, Pirogov City Clinical Hospital No. 1 of Moscow City, Moscow, Russian Federation
Federal State Budgetary Institution, Almazov National Medical Research Center, Saint Petersburg, Russian Federation
Faculty of Medicine, Hacettepe University, Ankara, Turkey
State Budgetary Institution of the Republic of Sakha (Yakutia), Republican Hospital No. 1, Yakutsk, Russian Federation
Faculty of Medicine, Osmangazi University, Eskisehir, Turkey
State Budgetary Healthcare Institution, City Clinical Hospital No. 51 of Moscow Health Department, Moscow, Russian Federation
Institut Dr. Schauerte, Munich, Germany
Bayer AG, Berlin, Germany
Bayer AG, Wuppertal, Germany
Department of Pulmonary Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

Cerrahpasa Faculty of Medicine
Faculty of Medicine
Meram Faculty of Medicine
E. Meshalkin National Medical Research Center of the Ministry of Health of the Russian Federation
Hotel Dieu de France
Prince Sultan Military Medical City
State Budgetary Healthcare Institution
Faculty of Medicine
Regional State Budgetary Healthcare Institution
Federal State Budget Institution
National Research Cardiac Surgery Center
Faculty of Medicine
Istanbul Faculty of Medicine
Institute of Cardiology in Almaty
Faculty of Medicine
King Fahad Medical City
State Budgetary Healthcare Institution
Federal State Budgetary Institution
Faculty of Medicine
State Budgetary Institution of the Republic of Sakha (Yakutia)
Faculty of Medicine
State Budgetary Healthcare Institution
Institut Dr. Schauerte
Bayer AG
Bayer AG
Department of Pulmonary Medicine

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