Please use this identifier to cite or link to this item: doi:10.22028/D291-31056
Title: Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry
Author(s): Kaemmerer, Harald
Gorenflo, Matthias
Huscher, Dörte
Pittrow, David
Apitz, Christian
Baumgartner, Helmut
Berger, Felix
Bruch, Leonhard
Brunnemer, Eva
Budts, Werner
Claussen, Martin
Coghlan, Gerry
Dähnert, Ingo
D’Alto, Michele
Delcroix, Marion
Distler, Oliver
Dittrich, Sven
Dumitrescu, Daniel
Ewert, Ralf
Faehling, Martin
Germund, Ingo
Ghofrani, Hossein Ardeschir
Grohé, Christian
Grossekreymborg, Karsten
Halank, Michael
Hansmann, Georg
Harzheim, Dominik
Nemes, Attila
Havasi, Kalman
Held, Matthias
Hoeper, Marius M.
Hofbeck, Michael
Hohenfrost-Schmidt, Wolfgang
Jurevičienė, Elena
Gumbienè, Lina
Kabitz, Hans-Joachim
Klose, Hans
Köhler, Thomas
Konstantinides, Stavros
Köestenberger, 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
Stähler, Gerd
Stiller, Brigitte
Tsangaris, Iraklis
Vizza, Carmine Dario
Vonk Noordegraaf, Anton
Wilkens, Heinrike
Wirtz, Hubert
Diller, Gerhard-Paul
Grünig, Ekkehard
Rosenkranz, Stephan
Language: English
Title: Journal of Clinical Medicine
Volume: 9
Issue: 5
Publisher/Platform: MDPI
Year of Publication: 2020
Free key words: congenital heart disease
pulmonary hypertension
pulmonary arterial hypertension
targeted therapy
DDC notations: 610 Medicine and health
Publikation type: Journal Article
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-naïve. 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.
DOI of the first publication: 10.3390/jcm9051456
Link to this record: urn:nbn:de:bsz:291--ds-310563
ISSN: 2077-0383
Date of registration: 22-Dec-2020
Faculty: M - Medizinische Fakultät
Department: M - Innere Medizin
Professorship: M - Keiner Professur zugeordnet
Collections:SciDok - Der Wissenschaftsserver der Universität des Saarlandes

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