Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry

Ann-Sophie Kaemmerer, Matthias Gorenflo, D. rte Huscher, David Pittrow, Peter Ewert, Christine Pausch, Marion Delcroix, Hossein A. Ghofrani, Marius M. Hoeper, Rainer Kozlik-Feldmann, Andris Skride, Gerd Stähler, Carmine Dario Vizza, Elena Jureviciene, Dovile Jancauskaite, Lina Gumbiene, Ralf Ewert, Ingo Dähnert, Matthias Held, Michael HalankDirk Skowasch, Hans Klose, Heinrike Wilkens, Katrin Milger, Christian Jux, Martin Koestenberger, Laura Scelsi, Eva Brunnemer, Michael Hofbeck, Silvia Ulrich, Anton Vonk Noordegraaf, Tobias J. Lange, Leonhard Bruch, Stavros Konstantinides, Martin Claussen, Judith Löffler-Ragg, Hubert Wirtz, Christian Apitz, Rhoia Neidenbach, Sebastian Freilinger, Attila Nemes, Christian Opitz, Ekkehard Grünig, Stephan Rosenkranz

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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.
Original languageEnglish
Pages (from-to)1255-1268
JournalCardiovascular diagnosis and therapy
Issue number6
Publication statusPublished - 1 Dec 2021

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