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[Pulmonary arterial hypertension in congenital heart disease - Part I]. 2023
Harald Kaemmerer, and
Gerhard Paul Diller, and
Ingo Dähnert, and
Christina A Eichstaedt, and
Andreas Eicken, and
Annika Freiberger, and
Sebastian Freilinger, and
Ralf Geiger, and
Matthias Gorenflo, and
Ekkehard Grünig, and
Alfred Hager, and
Ulrike Herberg, and
Michael Huntgeburth, and
Ann-Sophie Kaemmerer, and
Rainer Kozlik-Feldmann, and
Astrid Lammers, and
Nicole Nagdyman, and
Sebastian Michel, and
Kai Helge Schmidt, and
Anselm Uebing, and
Fabian von Scheidt, and
Christian Apitz
Internationales Zentrum für Erwachsene mit angeborenem Herzfehler, Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, München, Deutschland.
The number of adults with congenital heart disease (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary arterial hypertension (PAH), which may develop early in untreated CHD. Despite timely treatment of CHD, PAH often persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart defects" is addressed only relatively superficially in these guidelines. Therefore, this article addresses the perspective of congenital cardiology in greater depth.
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