Medical treatment of pulmonary hypertension in adults with congenital heart disease updated and extended results from the International COMPERA-CHD Registry /
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...
Elmentve itt :
| Szerzők: | |
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| Dokumentumtípus: | Cikk |
| Megjelent: |
2021
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| Sorozat: | CARDIOVASCULAR DIAGNOSIS AND THERAPY
11 No. 6 |
| Tárgyszavak: | |
| doi: | 10.21037/cdt-21-351 |
| mtmt: | 32201288 |
| Online Access: | http://publicatio.bibl.u-szeged.hu/37317 |
| LEADER | 04377nab a2200577 i 4500 | ||
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| 024 | 7 | |a 10.21037/cdt-21-351 |2 doi | |
| 024 | 7 | |a 32201288 |2 mtmt | |
| 040 | |a SZTE Publicatio Repozitórium |b hun | ||
| 041 | |a eng | ||
| 100 | 1 | |a Kaemmerer Ann-Sophie | |
| 245 | 1 | 0 | |a Medical treatment of pulmonary hypertension in adults with congenital heart disease |h [elektronikus dokumentum] : |b updated and extended results from the International COMPERA-CHD Registry / |c Kaemmerer Ann-Sophie |
| 260 | |c 2021 | ||
| 300 | |a 1255-1268 | ||
| 490 | 0 | |a CARDIOVASCULAR DIAGNOSIS AND THERAPY |v 11 No. 6 | |
| 520 | 3 | |a 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. | |
| 650 | 4 | |a Klinikai orvostan | |
| 700 | 0 | 1 | |a Gorenflo Matthias |e aut |
| 700 | 0 | 1 | |a Huscher Doerte |e aut |
| 700 | 0 | 1 | |a Pittrow David |e aut |
| 700 | 0 | 1 | |a Ewert Peter |e aut |
| 700 | 0 | 1 | |a Pausch Christine |e aut |
| 700 | 0 | 1 | |a Delcroix Marion |e aut |
| 700 | 0 | 1 | |a Ghofrani Hossein A. |e aut |
| 700 | 0 | 1 | |a Hoeper Marius M. |e aut |
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| 700 | 0 | 2 | |a Daehnert Ingo |e aut |
| 700 | 0 | 2 | |a Held Matthias |e aut |
| 700 | 0 | 2 | |a Halank Michael |e aut |
| 700 | 0 | 2 | |a Skowasch Dirk |e aut |
| 700 | 0 | 2 | |a Klose Hans |e aut |
| 700 | 0 | 2 | |a Wilkens Heinrike |e aut |
| 700 | 0 | 2 | |a Milger Katrin |e aut |
| 700 | 0 | 2 | |a Jux Christian |e aut |
| 700 | 0 | 2 | |a Koestenberger Martin |e aut |
| 700 | 0 | 2 | |a Scelsi Laura |e aut |
| 700 | 0 | 2 | |a Brunnemer Eva |e aut |
| 700 | 0 | 2 | |a Hofbeck Michael |e aut |
| 700 | 0 | 2 | |a Ulrich Silvia |e aut |
| 700 | 0 | 2 | |a Nemes Attila |e aut |
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