Kaemmerer, Ann-Sophie, Gorenflo, Matthias, Huscher, Doerte, Pittrow, David, Ewert, Peter, Pausch, Christine, Delcroix, Marion ORCID: 0000-0001-8394-9809, Ghofrani, Hossein A., Hoeper, Marius M., Kozlik-Feldmann, Rainer, Skride, Andris, Staehler, Gerd, Vizza, Carmine Dario ORCID: 0000-0002-3540-4983, Jureviciene, Elena, Jancauskaite, Dovile, Gumbiene, Lina, Ewert, Ralf, Daehnert, Ingo, Held, Matthias, Halank, Michael, Skowasch, Dirk, Klose, Hans, Wilkens, Heinrike, Milger, Katrin, Jux, Christian, Koestenberger, Martin, Scelsi, Laura ORCID: 0000-0001-9409-691X, Brunnemer, Eva, Hofbeck, Michael ORCID: 0000-0001-7034-1132, Ulrich, Silvia, Noordegraaf, Anton Vonk, Lange, Tobias J., Bruch, Leonhard, Konstantinides, Stavros, Claussen, Martin, Loeffler-Ragg, Judith, Wirtz, Hubert, Apitz, Christian, Neidenbach, Rhoia, Freilinger, Sebastian, Nemes, Attila, Opitz, Christian, Gruenig, Ekkehard and Rosenkranz, Stephan (2021). Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry. Cardiovisc. Diagn. Ther., 11 (6). S. 1255 - 1269. SHATIN: AME PUBL CO. ISSN 2223-3660

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Abstract

Background: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. Methods: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD Results: A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3 +/- 16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369 +/- 121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of followup, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of Non-Eisenmenger PAH patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. Conclusions: Analyzing real life data from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the Non-Eisenmenger PAH group and to patients with complex CHD, including Fontan patients.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Kaemmerer, Ann-SophieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorenflo, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huscher, DoerteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pittrow, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ewert, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pausch, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Delcroix, MarionUNSPECIFIEDorcid.org/0000-0001-8394-9809UNSPECIFIED
Ghofrani, Hossein A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoeper, Marius M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kozlik-Feldmann, RainerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skride, AndrisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Staehler, GerdUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vizza, Carmine DarioUNSPECIFIEDorcid.org/0000-0002-3540-4983UNSPECIFIED
Jureviciene, ElenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jancauskaite, DovileUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gumbiene, LinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ewert, RalfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Daehnert, IngoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Held, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Halank, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skowasch, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klose, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wilkens, HeinrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Milger, KatrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jux, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koestenberger, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scelsi, LauraUNSPECIFIEDorcid.org/0000-0001-9409-691XUNSPECIFIED
Brunnemer, EvaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hofbeck, MichaelUNSPECIFIEDorcid.org/0000-0001-7034-1132UNSPECIFIED
Ulrich, SilviaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Noordegraaf, Anton VonkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, Tobias J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bruch, LeonhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Konstantinides, StavrosUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Claussen, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Loeffler-Ragg, JudithUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wirtz, HubertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Apitz, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neidenbach, RhoiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Freilinger, SebastianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nemes, AttilaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Opitz, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gruenig, EkkehardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rosenkranz, StephanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-607709
DOI: 10.21037/cdt-21-351
Journal or Publication Title: Cardiovisc. Diagn. Ther.
Volume: 11
Number: 6
Page Range: S. 1255 - 1269
Date: 2021
Publisher: AME PUBL CO
Place of Publication: SHATIN
ISSN: 2223-3660
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
EISENMENGER SYNDROME; ARTERIAL-HYPERTENSION; CLINICAL-ASPECTS; SINGLE-CENTER; ANTICOAGULATION; EPIDEMIOLOGY; PREVALENCE; GUIDELINES; MANAGEMENT; THERAPIESMultiple languages
Cardiac & Cardiovascular SystemsMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/60770

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