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Kaemmerer, Ann-Sophie; Gorenflo, Matthias; Huscher, Doerte; Pittrow, David; Ewert, Peter; Pausch, Christine; Delcroix, Marion; Ghofrani, Hossein A.; Hoeper, Marius M.; Kozlik-Feldmann, Rainer; Skride, Andris; Staehler, Gerd; Vizza, Carmine Dario; 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; Brunnemer, Eva; Hofbeck, Michael; 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. In: Cardiovascular Diagnosis and Therapy, Vol. 11, No. 6: pp. 1255-1268

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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.

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