| Contributors: |
Kaemmerer, A. -S.; Gorenflo, M.; Huscher, D.; Pittrow, D.; Ewert, P.; Pausch, C.; Delcroix, M.; Ghofrani, H. A.; Hoeper, M. M.; Kozlik-Feldmann, R.; Skride, A.; Stahler, G.; Vizza, C. D.; Jureviciene, E.; Jancauskaite, D.; Gumbiene, L.; Ewert, R.; Dahnert, I.; Held, M.; Halank, M.; Skowasch, D.; Klose, H.; Wilkens, H.; Milger, K.; Jux, C.; Koestenberger, M.; Scelsi, L.; Brunnemer, E.; Hofbeck, M.; Ulrich, S.; Noordegraaf, A. V.; Lange, T. J.; Bruch, L.; Konstantinides, S.; Claussen, M.; Loffler-Ragg, J.; Wirtz, H.; Apitz, C.; Neidenbach, R.; Freilinger, S.; Nemes, A.; Opitz, C.; Grunig, E.; Rosenkranz, S. |
| Description: |
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 ... |