Pulmonary hypertension in adults with congenital heart disease : real-world data from the international COMPERA-CHD registry
Creators: name: Kaemmerer, Harald
name: Gorenflo, Matthias
name: Huscher, Dörte
name: Pittrow, David
name: Apitz, Christian
name: Baumgartner, Helmut
name: Berger, Felix
name: Bruch, Leonhard
name: Brunnemer, Eva
name: Budts, Werner
name: Claussen, Martin
name: Coghlan, Gerry
name: Dähnert, Ingo
name: D’Alto, Michele
name: Delcroix, Marion
name: Distler, Oliver
name: Dittrich, Sven
name: Dumitrescu, Daniel
name: Ewert, Ralf
name: Faehling, Martin
name: Germund, Ingo
name: Ghofrani, Hossein Ardeschir
name: Grohé, Christian
name: Grossekreymborg, Karsten
name: Halank, Michael
name: Hansmann, Georg
name: Harzheim, Dominik
name: Nemes, Attila
name: Havasi, Kalman
name: Held, Matthias
name: M. Hoeper, Marius
name: Hofbeck, Michael
name: Hohenfrost-Schmidt, Wolfgang
name: Jurevičienė, Elena
name: Gumbienè, Lina
name: Kabitz, Hans-Joachim
name: Klose, Hans
name: Köhler, Thomas
name: Konstantinides, Stavros
name: Köestenberger, Martin
name: Kozlik-Feldmann, Rainer
name: Kramer, Hans-Heiner
name: Kropf-Sanchen, Cornelia
name: Lammers, Astrid
name: Lange, Tobias
name: Meyn, Philipp
name: Miera, Oliver
name: Milger-Kneidinger, Katrin
name: Neidenbach, Rhoia
name: Neurohr, Claus
name: Opitz, Christian
name: Perings, Christian
name: Remppis, Bjoern Andrew
name: Riemekasten, Gabriele
name: Scelsi, Laura
name: Scholtz, Werner
name: Simkova, Iveta
name: Skowasch, Dirk
name: Skride, Andris
name: Stähler, Gerd
name: Stiller, Brigitte
name: Tsangaris, Iraklis
name: Vizza, Carmine Dario
name: Vonk Noordegraaf, Anton
name: Wilkens, Heinrike
name: Wirtz, Hubert
name: Diller, Gerhard-Paul
name: Grünig, Ekkehard
name: Rosenkranz, Stephan
Publisher: name: Universität Ulm
Year: 2020
Subjects: subject: congenital heart disease
subject: observational
subject: survival
subject: targeted therapy
subject: DDC 610 / Medicine &amp; health
subject: Pulmonary arterial hypertension
subject: Heart defects, Congenital
subject: Adult
subject: Herzkrankheit
subject: Pulmonale Hypertonie
subject: Erwachsener

description: 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, &gt;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 CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p &lt; 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.