Kaemmerer, Harald, Gorenflo, Matthias, Huscher, Dorte ORCID: 0000-0001-9070-0761, Pittrow, David, Apitz, Christian, Baumgartner, Helmut, Berger, Felix, Bruch, Leonhard, Brunnemer, Eva, Budts, Werner, Claussen, Martin, Coghlan, Gerry, Dahnert, Ingo, D'Alto, Michele, Delcroix, Marion, Distler, Oliver, Dittrich, Sven ORCID: 0000-0002-8455-3416, Dumitrescu, Daniel, Ewert, Ralf, Faehling, Martin, Germund, Ingo, Ghofrani, Hossein Ardeschir, Grohe, Christian, Grossekreymborg, Karsten, Halank, Michael, Hansmann, Georg ORCID: 0000-0003-0709-3935, Harzheim, Dominik, Nemes, Attila, Havasi, Kalman, Held, Matthias, Hoeper, Marius M., Hofbeck, Michael, Hohenfrost-Schmidt, Wolfgang, Jureviciene, Elena, Gumbiene, Lina, Kabitz, Hans-Joachim, Klose, Hans, Koehler, Thomas, Konstantinides, Stavros, Koeestenberger, Martin, Kozlik-Feldmann, Rainer, Kramer, Hans-Heiner, Kropf-Sanchen, Cornelia, Lammers, Astrid, Lange, Tobias, Meyn, Philipp, Miera, Oliver, Milger-Kneidinger, Katrin, Neidenbach, Rhoia, Neurohr, Claus, Opitz, Christian, Perings, Christian, Remppis, Bjoern Andrew, Riemekasten, Gabriele, Scelsi, Laura, Scholtz, Werner, Simkova, Iveta, Skowasch, Dirk, Skride, Andris, Staehler, Gerd, Stiller, Brigitte, Tsangaris, Iraklis, Vizza, Carmine Dario ORCID: 0000-0002-3540-4983, Noordegraaf, Anton Vonk, Wilkens, Heinrike ORCID: 0000-0002-7789-8563, Wirtz, Hubert, Diller, Gerhard-Paul, Gruenig, Ekkehard and Rosenkranz, Stephan (2020). Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry. J. Clin. Med., 9 (5). BASEL: MDPI. ISSN 2077-0383

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Abstract

Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients' median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naive. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Kaemmerer, HaraldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorenflo, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huscher, DorteUNSPECIFIEDorcid.org/0000-0001-9070-0761UNSPECIFIED
Pittrow, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Apitz, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Baumgartner, HelmutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Berger, FelixUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bruch, LeonhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Brunnemer, EvaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Budts, WernerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Claussen, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Coghlan, GerryUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dahnert, IngoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
D'Alto, MicheleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Delcroix, MarionUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Distler, OliverUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dittrich, SvenUNSPECIFIEDorcid.org/0000-0002-8455-3416UNSPECIFIED
Dumitrescu, DanielUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ewert, RalfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Faehling, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Germund, IngoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ghofrani, Hossein ArdeschirUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Grohe, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Grossekreymborg, KarstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Halank, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hansmann, GeorgUNSPECIFIEDorcid.org/0000-0003-0709-3935UNSPECIFIED
Harzheim, DominikUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nemes, AttilaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Havasi, KalmanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Held, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoeper, Marius M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hofbeck, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hohenfrost-Schmidt, WolfgangUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jureviciene, ElenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gumbiene, LinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kabitz, Hans-JoachimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klose, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koehler, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Konstantinides, StavrosUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koeestenberger, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kozlik-Feldmann, RainerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kramer, Hans-HeinerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kropf-Sanchen, CorneliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lammers, AstridUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, TobiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meyn, PhilippUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Miera, OliverUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Milger-Kneidinger, KatrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neidenbach, RhoiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neurohr, ClausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Opitz, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Perings, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Remppis, Bjoern AndrewUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Riemekasten, GabrieleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scelsi, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scholtz, WernerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Simkova, IvetaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skowasch, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skride, AndrisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Staehler, GerdUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stiller, BrigitteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tsangaris, IraklisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vizza, Carmine DarioUNSPECIFIEDorcid.org/0000-0002-3540-4983UNSPECIFIED
Noordegraaf, Anton VonkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wilkens, HeinrikeUNSPECIFIEDorcid.org/0000-0002-7789-8563UNSPECIFIED
Wirtz, HubertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Diller, Gerhard-PaulUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gruenig, EkkehardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rosenkranz, StephanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-334886
DOI: 10.3390/jcm9051456
Journal or Publication Title: J. Clin. Med.
Volume: 9
Number: 5
Date: 2020
Publisher: MDPI
Place of Publication: BASEL
ISSN: 2077-0383
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
ARTERIAL-HYPERTENSION; EISENMENGER-SYNDROME; SURVIVAL PROSPECTS; SINGLE-CENTER; LONG-TERM; DIAGNOSIS; THERAPY; ANTICOAGULATION; PREDICTORS; GUIDELINESMultiple languages
Medicine, General & InternalMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/33488

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