Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry
2020
Online
academicJournal
Zugriff:
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-naïve. 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 ...
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Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry
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Autor/in / Beteiligte Person: | Kaemmerer, Harald ; Gorenflo, Matthias ; Huscher, Dörte ; Pittrow, David ; Apitz, Christian ; Baumgartner, Helmut ; Berger, Felix ; Bruch, Leonhard ; Brunnemer, Eva ; Budts, Werner ; Claussen, Martin ; Coghlan, Gerry ; Dähnert, Ingo ; D’Alto, Michele ; Delcroix, Marion ; Distler, Oliver ; Dittrich, Sven ; Dumitrescu, Daniel ; Ewert, Ralf ; Faehling, Martin ; Germund, Ingo ; Ghofrani, Hossein Ardeschir ; Grohé, Christian ; Grossekreymborg, Karsten ; Halank, Michael ; Hansmann, Georg ; Harzheim, Dominik ; Nemes, Attila ; Havasi, Kalman ; Held, Matthias ; M. Hoeper, Marius ; Hofbeck, Michael ; Hohenfrost-Schmidt, Wolfgang ; Jurevičienė, Elena ; Gumbienè, Lina ; Kabitz, Hans-Joachim ; Klose, Hans ; Köhler, Thomas ; Konstantinides, Stavros ; Köestenberger, Martin ; Kozlik-Feldmann, Rainer ; Kramer, Hans-Heiner ; Kropf-Sanchen, Cornelia ; Lammers, Astrid ; Lange, Tobias ; Meyn, Philipp ; Miera, Oliver ; Milger-Kneidinger, Katrin ; Neidenbach, Rhoia ; Neurohr, Claus ; Opitz, Christian ; Perings, Christian ; Remppis, Bjoern Andrew ; Riemekasten, Gabriele ; Scelsi, Laura ; Scholtz, Werner ; Simkova, Iveta ; Skowasch, Dirk ; Skride, Andris ; Stähler, Gerd ; Stiller, Brigitte ; Tsangaris, Iraklis ; Vizza, Carmine Dario ; Vonk Noordegraaf, Anton ; Wilkens, Heinrike ; Wirtz, Hubert ; Diller, Gerhard-Paul ; Grünig, Ekkehard ; Rosenkranz, Stephan |
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Veröffentlichung: | 2020 |
Medientyp: | academicJournal |
DOI: | 10.3390/jcm9051456 |
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