Kaemmerer, Harald and Gorenflo, Matthias and Huscher, Dorte and Pittrow, David and Apitz, Christian and Baumgartner, Helmut and Berger, Felix and Bruch, Leonhard and Brunnemer, Eva and Budts, Werner and Claussen, Martin and Coghlan, Gerry and Dahnert, Ingo and D'Alto, Michele and Delcroix, Marion and Distler, Oliver and Dittrich, Sven and Dumitrescu, Daniel and Ewert, Ralf and Faehling, Martin and Germund, Ingo and Ghofrani, Hossein Ardeschir and Grohe, Christian and Grossekreymborg, Karsten and Halank, Michael and Hansmann, Georg and Harzheim, Dominik and Nemes, Attila and Havasi, Kalman and Held, Matthias and Hoeper, Marius M. and Hofbeck, Michael and Hohenfrost-Schmidt, Wolfgang and Jureviciene, Elena and Gumbiene, Lina and Kabitz, Hans-Joachim and Klose, Hans and Koehler, Thomas and Konstantinides, Stavros and Koeestenberger, Martin and Kozlik-Feldmann, Rainer and Kramer, Hans-Heiner and Kropf-Sanchen, Cornelia and Lammers, Astrid and Lange, Tobias and Meyn, Philipp and Miera, Oliver and Milger-Kneidinger, Katrin and Neidenbach, Rhoia and Neurohr, Claus and Opitz, Christian and Perings, Christian and Remppis, Bjoern Andrew and Riemekasten, Gabriele and Scelsi, Laura and Scholtz, Werner and Simkova, Iveta and Skowasch, Dirk and Skride, Andris and Staehler, Gerd and Stiller, Brigitte and Tsangaris, Iraklis and Vizza, Carmine Dario and Noordegraaf, Anton Vonk and Wilkens, Heinrike and Wirtz, Hubert and Diller, Gerhard-Paul and Gruenig, Ekkehard and Rosenkranz, Stephan (2020) Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry. JOURNAL OF CLINICAL MEDICINE, 9 (5): 1456. ISSN , 2077-0383
Full text not available from this repository. (Request a copy)Abstract
Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients' median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naive. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ARTERIAL-HYPERTENSION; EISENMENGER-SYNDROME; SURVIVAL PROSPECTS; SINGLE-CENTER; LONG-TERM; DIAGNOSIS; THERAPY; ANTICOAGULATION; PREDICTORS; GUIDELINES; congenital heart disease; pulmonary hypertension; pulmonary arterial hypertension; adults; observational; survival; targeted therapy |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Innere Medizin II |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 24 Mar 2021 11:57 |
| Last Modified: | 24 Mar 2021 11:57 |
| URI: | https://pred.uni-regensburg.de/id/eprint/44629 |
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