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

Kaemmerer, Ann-Sophie ; Gorenflo, Matthias ; Huscher, Dörte ; Pittrow, David ; Ewert, Peter ; Pausch, Christine ; Delcroix, Marion

; Ghofrani, Hossein A. ; Hoeper, Marius M. ; Kozlik-Feldmann, Rainer ; Skride, Andris ; Stähler, Gerd ; Vizza, Carmine Dario

; Jureviciene, Elena ; Jancauskaite, Dovile ; Gumbiene, Lina ; Ewert, Ralf ; Dähnert, 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 ; Löffler-Ragg, Judith ; Wirtz, Hubert ; Apitz, Christian ; Neidenbach, Rhoia ; Freilinger, Sebastian ; Nemes, Attila ; Opitz, Christian ; Grünig, Ekkehard ; Rosenkranz, Stephan

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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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