Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Grzegorz Kopeć, Olga Dzikowska-Diduch, Ewa Mroczek, Tatiana Mularek-Kubzdela, Łukasz Chrzanowski, Ilona Skoczylas, Michał Tomaszewski, Małgorzata Peregud-Pogorzelska, Danuta Karasek, Ewa Lewicka, Wojciech Jacheć, Zbigniew Gąsior, Piotr Błaszczak, Katarzyna Ptaszyńska-Kopczyńska, Katarzyna Mizia-Stec, Andrzej Biederman, Dariusz Zieliński, Roman Przybylski, Piotr Kędzierski, Marcin Waligóra, Marek Roik, Marek Grabka, Joanna Orłowska, Aleksander Araszkiewicz, Marta Banaszkiewicz, Sylwia Sławek-Szmyt, Szymon Darocha, Wojciech Magoń, Alicja Dąbrowska-Kugacka, Jakub Stępniewski, Kamil Jonas, Karol Kamiński, Jarosław D Kasprzak, Piotr Podolec, Piotr Pruszczyk, Adam Torbicki, Marcin Kurzyna, Grzegorz Kopeć, Olga Dzikowska-Diduch, Ewa Mroczek, Tatiana Mularek-Kubzdela, Łukasz Chrzanowski, Ilona Skoczylas, Michał Tomaszewski, Małgorzata Peregud-Pogorzelska, Danuta Karasek, Ewa Lewicka, Wojciech Jacheć, Zbigniew Gąsior, Piotr Błaszczak, Katarzyna Ptaszyńska-Kopczyńska, Katarzyna Mizia-Stec, Andrzej Biederman, Dariusz Zieliński, Roman Przybylski, Piotr Kędzierski, Marcin Waligóra, Marek Roik, Marek Grabka, Joanna Orłowska, Aleksander Araszkiewicz, Marta Banaszkiewicz, Sylwia Sławek-Szmyt, Szymon Darocha, Wojciech Magoń, Alicja Dąbrowska-Kugacka, Jakub Stępniewski, Kamil Jonas, Karol Kamiński, Jarosław D Kasprzak, Piotr Podolec, Piotr Pruszczyk, Adam Torbicki, Marcin Kurzyna

Abstract

Background: Significant achievements in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) have provided effective therapeutic options for most patients. However, the true impact of the changed landscape of CTEPH therapies on patients' management and outcomes is poorly known. We aimed to characterize the incidence, clinical characteristics, and outcomes of CTEPH patients in the modern era of CTEPH therapies.

Methods: We analyzed the data of CTEPH adults enrolled in the prospective multicenter registry.

Results: We enrolled 516 patients aged 63.8 ± 15.4 years. The incidence rate of CTEPH was 3.96 per million adults per year. The group was burdened with several comorbidities. New oral anticoagulants (n = 301; 58.3%) were preferred over vitamin K antagonists (n = 159; 30.8%). Pulmonary endarterectomy (PEA) was performed in 120 (23.3%) patients and balloon pulmonary angioplasty (BPA) in 258 (50%) patients. PEA was pretreated with targeted pharmacotherapy in 19 (15.8%) patients, and BPA in 124 (48.1%) patients. Persistent CTEPH was present in 46% of PEA patients and in 65% of patients after completion of BPA. Persistent CTEPH after PEA was treated with targeted pharmacotherapy in 72% and with BPA in 27.7% of patients. At a mean time period of 14.3 ± 5.8 months, 26 patients had died. The use of PEA or BPA was associated with better survival than the use of solely medical treatment.

Conclusions: The modern population of CTEPH patients comprises mostly elderly people significantly burdened with comorbid conditions. This calls for treatment decisions that are tailored individually for every patient. The combination of two or three methods is currently a frequent approach in the treatment of CTEPH.

Clinical trial registration: clinicaltrials.gov/ct2/show/NCT03959748.

Keywords: chronic thromboembolic pulmonary hypertension; epidemiology; registry.

Conflict of interest statement

Conflict of interest statement: Grzegorz Kopeć reports grants and personal fees from Actelion Pharma, personal fees from Janssen-Cilag Polska, personal fees from AOP Orphan, personal fees from MSD. Olga Dzikowska-Diduch has nothing to declare. Ewa Mroczek has nothing to declare. Tatiana Mularek-Kubzdela reports fees for lectures from MSD, Bayer, Jansen, AOP Orphan, Beringher, Pfizer, grants from MSD, Bayer, Jansen, AOP Orphan, Beringher. Łukasz Chrzanowski reports fees for lectures from MSD, non-financial support from MSD. Ilona Skoczylas has nothing to declare. Michał Tomaszewski has nothing to declare. Małgorzata Peregud-Pogorzelska has nothing to declare. Danuta Karasek has nothing to declare. Ewa Lewicka reports fees for lectures from Bayer, MSD, AOP Orphan, Janssen-Cilag, Pfizer and Boehringer-Ingelheim. Wojciech Jacheć has nothing to declare. Zbigniew Gąsior has nothing to declare. Piotr Błaszczak has nothing to declare. Katarzyna Ptaszyńska-Kopczyńska has nothing to declare. Katarzyna Mizia-Stec has nothing to declare. Andrzej Biederman has nothing to declare. Dariusz Zieliński has nothing to declare. Roman Przybylski has nothing to declare. Piotr Kędzierski has nothing to declare. Marcin Waligóra has nothing to declare. Marek Roik has nothing to declare. Marek Grabka has nothing to declare. Joanna Orłowska has nothing to declare. Aleksander Araszkiewicz has nothing to declare. Marta Banaszkiewicz has nothing to declare. Sylwia Sławek-Szmyt has nothing to declare. Szymon Darocha reports grants and personal fees from Janssen-Cilag, MSD, Bayer, and AOP Orphan. Wojciech Magoń has nothing to declare. Alicja Dąbrowska-Kugacka reports fees for lectures from MSD, Actelion, AOP Orphan and Bayer. Jakub Stępniewski has nothing to declare. Kamil Jonas has nothing to declare. Karol Kamiński has nothing to declare. Jarosław D. Kasprzak reports fees for lectures from Janssen/Actelion, AOP Orphan. Piotr Podolec reports personal fees from Actelion, personal fees from Jansen, personal fees from AOP ORPHAN, personal fees from Bayer. Piotr Pruszczyk reports fees for lectures from Merck. Adam Torbicki reports grants and personal fees from Actelion Pharma, personal fees from Janssen-Cilag Polska, personal fees from AOP Orphan, personal fees from MSD, grants and personal fees from Bayer Healthcare and the Chairperson of the Foundation on Pulmonary Hypertension. The foundation receives donations including those from industry. The chairmanship is an honorary function and is unrelated to financial or other benefits. The funders had no role in the design of the study, in the collection, analyses, or interpretation of data, in the writing of the manuscript, or in the decision to publish the results. Marcin Kurzyna reports fees for lectures from MSD, AOP Orphan and Janssen-Cilag.

© The Author(s), 2021.

Figures

Figure 1.
Figure 1.
Assessment of operability by the chronic thromboembolic pulmonary hypertension (CTEPH) teams and final treatment of the study group.
Figure 2.
Figure 2.
Kaplan–Meier survival curves for patients with chronic thromboembolic pulmonary hypertension treated with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA) (invasive treatment) and with only pharmacotherapy including targeted treatment.

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