Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Kaemmerer H, Gorenflo M, Huscher D, Pittrow D, Apitz C, Baumgartner H, Berger F, Bruch L, Brunnemer E, Budts W, Claussen M, Coghlan G, Dahnert I, D'Alto M, Delcroix M, Distler O, Dittrich S, Dumitrescu D, Ewert R, Faehling M, Germund I, Ghofrani HA, Grohe C, Grossekreymborg K, Halank M, Hansmann G, Harzheim D, Nemes A, Havasi K, Held M, Hoeper MM, Hofbeck M, Hohenfrost-Schmidt W, Jureviciene E, Gumbiene L, Kabitz HJ, Klose H, Koehler T, Konstantinides S, Koeestenberger M, Kozlik-Feldmann R, Kramer HH, Kropf-Sanchen C, Lammers A, Lange T, Meyn P, Miera O, Milger-Kneidinger K, Neidenbach R, Neurohr C, Opitz C, Perings C, Remppis BA, Riemekasten G, Scelsi L, Scholtz W, Simkova I, Skowasch D, Skride A, Staehler G, Stiller B, Tsangaris I, Vizza CD, Noordegraaf AV, Wilkens H, Wirtz H, Diller GP, Gruenig E, Rosenkranz S (2020)

Publication Type: Journal article

Publication year: 2020

Journal

Book Volume: 9

Journal Issue: 5

DOI: 10.3390/jcm9051456

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.

Authors with CRIS profile

Involved external institutions

Deutsches Herzzentrum Berlin Germany (DE) Evangelische Lungenklinik Berlin gGmbH Germany (DE) Universitätsmedizin der Johannes Gutenberg-Universität Mainz Germany (DE) Steiermärkische Krankenanstaltengesellschaft m. b. H. LKH-Univ. Klinikum Graz Austria (AT) Università degli Studi di Pavia Italy (IT) Ruhr-Universität Bochum (RUB) Germany (DE) Slovak Medical University (SMU) / Slovenská zdravotnícka univerzita v Bratislave Slovakia (SK) Universitätsklinikum Bonn Germany (DE) Pauls Stradiņš Clinical University Hospital / Paula Stradiņa Klīniskā universitātes slimnīca Latvia (LV) Klinik Löwenstein gGmbH Germany (DE) Universitäts-Herzzentrum Freiburg - Bad Krozingen GmbH Germany (DE) National and Kapodistrian University of Athens Greece (GR) Università degli studi "La Sapienza" Italy (IT) Free University Medical Center / VU Medisch Centrum Netherlands (NL) Universitätsklinikum des Saarlandes (UKS) Germany (DE) Universitätsklinikum Leipzig Germany (DE) Universitätsklinikum Münster Germany (DE) Universitätsklinikum Heidelberg Germany (DE) Universitätsklinikum Köln Germany (DE) Technische Universität München (TUM) Germany (DE) Charité - Universitätsmedizin Berlin Germany (DE) Technische Universität Dresden Germany (DE) Universitätsklinikum Ulm Germany (DE) Unfallkrankenhaus Berlin Germany (DE) University Hospital Leuven (UZ) / Universitaire ziekenhuizen Leuven Belgium (BE) LungenClinic Grosshansdorf GmbH Germany (DE) Royal Free Hospital United Kingdom (GB) Herzzentrum Leipzig Germany (DE) Ospedale Monaldi Italy (IT) Universitätsspital Zürich (USZ) Switzerland (CH) Herz- und Diabeteszentrum Nordrhein-Westfalen Germany (DE) Universitätsmedizin Greifswald / Universitätsklinikum Greifswald Germany (DE) Klinikum Esslingen Germany (DE) Universitätsklinikum Gießen und Marburg (UKGM) Germany (DE) Justus-Liebig-Universität Gießen Germany (DE) Medizinische Hochschule Hannover (MHH) / Hannover Medical School Germany (DE) Waldburg-Zeil Kliniken Germany (DE) University of Szeged / József Attila Tudományegyetem Szeged Hungary (HU) Klinikum Würzburg Mitte Germany (DE) Universitätsklinikum Tübingen Germany (DE) REGIOMED-KLINIKEN GmbH Germany (DE) Klinikum Konstanz Germany (DE) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) Universitätsklinikum Freiburg Germany (DE) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) Westfälische Wilhelms-Universität (WWU) Münster Germany (DE) Universitätsklinikum Regensburg Germany (DE) Klinikum der Universität München (Großhadern und Innenstadt) Germany (DE) Robert-Bosch-Krankenhaus Germany (DE) DRK-Kliniken Berlin Germany (DE) St.-Marien-Hospital Lünen Germany (DE) Herz- und Gefäßzentrum Bad Bevensen Germany (DE) Universität zu Lübeck Germany (DE)

How to cite

APA:

Kaemmerer, H., Gorenflo, M., Huscher, D., Pittrow, D., Apitz, C., Baumgartner, H.,... Rosenkranz, S. (2020). Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry. Journal of Clinical Medicine, 9(5). https://doi.org/10.3390/jcm9051456

MLA:

Kaemmerer, Harald, et al. "Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry." Journal of Clinical Medicine 9.5 (2020).

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