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Pulmonary hypertension in adults with congenital heart defects (ACHDs) - in light of the 2022 ESC PAH guidelines - part I: definition, epidemiology, classification, diagnostics, genetics, risk stratification and follow-up, gender aspects : review article

The number of adults with congenital heart defects (ACHDs) is steadily increasing and is about 360,000 in Germany. Congenital heart defect (CHD) is often associated with pulmonary hypertension (PH), which sometimes develops early in untreated CHD. Despite timely treatment of CHD, PH not infrequently...

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Main Authors: Kaemmerer, Harald (Author) , Diller, Gerhard Paul (Author) , Dähnert, Ingo (Author) , Achenbach, Stephan (Author) , Eichstaedt, Christina (Author) , Eicken, Andreas (Author) , Freiberger, Annika (Author) , Freilinger, Sebastian (Author) , Geiger, Ralf (Author) , Gorenflo, Matthias (Author) , Grünig, Ekkehard (Author) , Hager, Alfred (Author) , Huntgeburth, Michael (Author) , Kaemmerer-Suleiman, Ann-Sophie (Author) , Kozlik-Feldmann, Rainer (Author) , Lammers, Astrid E. (Author) , Nagdyman, Nicole (Author) , Michel, Sebastian (Author) , Schmidt, Kai Helge (Author) , Suleiman, Mathieu (Author) , Uebing, Anselm (Author) , Scheidt, Fabian von (Author) , Herberg, Ulrike (Author) , Apitz, Christian (Author)
Format: Article (Journal)
Language:English
Published: October 31, 2024
In: Cardiovascular Diagnosis and Therapy
Year: 2024, Volume: 14, Issue: 5, Pages: 935-948
ISSN:2223-3660
DOI:10.21037/cdt-24-148
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.21037/cdt-24-148
Verlag, lizenzpflichtig, Volltext: https://cdt.amegroups.org/article/view/130029
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Author Notes:Harald Kaemmerer, Gerhard Paul Diller, Ingo Dähnert, Stephan Achenbach, Christina A. Eichstaedt, Andreas Eicken, Annika Freiberger, Sebastian Freilinger, Ralf Geiger, Matthias Gorenflo, Ekkehard Grünig, Alfred Hager, Michael Huntgeburth, Ann-Sophie Kaemmerer-Suleiman, Rainer Kozlik-Feldmann, Astrid E. Lammers, Nicole Nagdyman, Sebastian Michel, Kai Helge Schmidt, Mathieu Suleiman, Anselm Uebing, Fabian von Scheidt, Ulrike Herberg, Christian Apitz
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Summary:The number of adults with congenital heart defects (ACHDs) is steadily increasing and is about 360,000 in Germany. Congenital heart defect (CHD) is often associated with pulmonary hypertension (PH), which sometimes develops early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists, redevelops in older age, and is associated with significant morbidity and lethality. The revised European Society of Cardiology (ESC)/European Respiratory Society (ERS) 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 treated only relatively superficially in this context. In the present article, part I, therefore, this topic is commented on in detail from the perspective of congenital cardiology with a special focus on definition, epidemiology, classification, diagnostics, genetics, risk stratification and follow-up and gender aspects of PH in ACHDs. This paper consists of two parts. Part II will provide comments on the topics of supportive therapy, special situations like pregnancy, contraception, and non-cardiac surgery, targeted pharmacotherapy, organ transplantation, special management like shunt lesion, left ventricular disease, and univentricular hearts, interventions, intensive care, ACHDs follow-up and future perspective on PH in ACHDs. By examining these aspects in detail, this article aims to fill the gaps in the existing guidelines and provide a more thorough understanding from the perspective of congenital cardiology.
Item Description:Gesehen am 06.08.2025
Physical Description:Online Resource
ISSN:2223-3660
DOI:10.21037/cdt-24-148

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