Pulmonary hypertension in adults with congenital heart defects (ACHDs) in light of the 2022 ESC PAH guidelines-part II: supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesion, left ventricular disease, univentricular hearts), interventions, intensive care, ACHD follow-up, future perspective.

Autor:	Kaemmerer H; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Diller GP; Department of Cardiology III: Congenital Heart Malformations (ACHD) and Valve Diseases, University Hospital Münster, Münster, Germany., Dähnert I; University Clinic for Pediatric Cardiology, Heart Center Leipzig, Leipzig, Germany., Achenbach S; University Hospital Erlangen, Medizinische Klinik 2 - Kardiologie und Angiologie, Erlangen, Germany., Eichstaedt CA; Center for Pulmonary Hypertension, Thorax Clinic Heidelberg at Heidelberg University Hospital, Heidelberg, Germany.; Institute of Human Genetics, Heidelberg University, Heidelberg, Germany.; Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Germany., Eicken A; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Freiberger A; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Freilinger S; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Geiger R; Department of Pediatrics III, Cardiology, Pneumology, Allergology, Cystic Fibrosis, Innsbruck, Austria., Gorenflo M; Department of Pediatric Cardiology and Congenital Heart Defects, Heidelberg University Hospital, Heidelberg, Germany., Grünig E; Center for Pulmonary Hypertension, Thorax Clinic Heidelberg at Heidelberg University Hospital, Heidelberg, Germany.; Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Germany., Hager A; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Huntgeburth M; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Kaemmerer-Suleiman AS; Department of Cardiac Surgery, Erlangen University Hospital, Erlangen, Germany., Kozlik-Feldmann R; Clinic and Polyclinic for Pediatric Cardiology, Hamburg University Heart and Vascular Center, Clinic and Polyclinic for Pediatric Heart Medicine and Adults with Congenital Heart Defects, Hamburg, Germany., Lammers AE; Department of Pediatric Cardiology, University Hospital Münster, Münster, Germany., Nagdyman N; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Michel S; Division for Congenital and Pediatric Heart Surgery, Department of Cardiac Surgery, Ludwig Maximilian University Munich, Campus Großhadern, Munich, Germany.; Comprehensive Pneumology Center Munich, German Center for Lung Research (DZL), Munich, Germany., Schmidt KH; University Medical Center Mainz, Center for Cardiology - Cardiology I, Johannes Gutenberg University Mainz, Mainz, Germany., Suleiman M; Department of Cardiac Surgery, Erlangen University Hospital, Erlangen, Germany., Uebing A; University Hospital Schleswig-Holstein, Clinic for Congenital Heart Defects and Pediatric Cardiology, Kiel, Germany.; German Centre for Cardiovascular Research (DZHK), Partner Site Kiel, Kiel, Germany., von Scheidt F; International Center for Adults with Congenital Heart Defects, Clinic for Congenital Heart Defects and Pediatric Cardiology, Deutsches Herzzentrum München, TUM University Hospital, Munich, Germany., Herberg U; Department of Pediatric Cardiology (U.H.), Medical Faculty, Rheinisch-Westfälische Technische Hochschule Aachen University, Aachen, Germany., Apitz C; Department of Pediatric Cardiology, University Hospital for Pediatrics and Adolescent Medicine Ulm, Ulm, Germany.
Jazyk:	angličtina
Zdroj:	Cardiovascular diagnosis and therapy [Cardiovasc Diagn Ther] 2024 Oct 31; Vol. 14 (5), pp. 921-934. Date of Electronic Publication: 2024 Oct 22.
DOI:	10.21037/cdt-24-167
Abstrakt:	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 mortality. 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. After the first part commenting on a broad range of topics like definition, epidemiology, classification, diagnostics, genetics, risk stratification and follow-up, and gender aspects, the second part focuses on supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management [shunt lesion, left ventricular (LV) disease, univentricular hearts], interventions, intensive care, ACHD follow-up, and future perspective. In the present article, therefore, this topic is commented on from the perspective of congenital cardiology. 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. Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cdt.amegroups.com/article/view/10.21037/cdt-24-167/coif). The series “Current Management Aspects of Adult Congenital Heart Disease (ACHD): Part VI” was commissioned by the editorial office without any funding or sponsorship. H.K. served as the unpaid Guest Editor of the series. H.K. received sponsorship and honoraria from Janssen/Jonson & Johnson, and Bristol Myers Squibb, and participated in the steering board of COMPERA International. G.P.D. has received honoraria and consulting fees from Janssen Pharmaceuticals. I.D. serves as an unpaid board member for Treasurer of the German Society for Pediatric Cardiology and Congenital Heart Disease. S.A. serves as board member for European Society of Cardiology, and Deutsche Herzstiftung. C.A.E. received honoraria for lectures and presentations from OMT and MSD, consulting fees from MSD. C.A.E. is co-inventor of the issued European patent “Gene panel specific for pulmonary hypertension and its uses” (EP3507380). E.G. has received research grants outside the submitted work from Actelion, Janssen, Bayer, MSD, Merck, Ferrer; research grants to the institution outside the submitted work from Acceleron, Actelion, Bayer, MSD, Janssen, Liquidia, United Therapeutics, OMT; consultancy fees outside the submitted work from Actelion, Janssen, Bayer, MSD, Merck, Ferrer; Speaker honoraria outside the submitted work from Actelion, Bayer/MSD, GSK, AOP, Janssen, phev, OMT, GEBRO, Ferrer, GWT; participation in AdBoards from MSD and Ferrer; unpaid borard member for A DUE Steering committee and patient organization phev. M.H. received consulting fees, honoraria, and travel support from Janssen. S.M. received Research Grant from German Center for Lung Research (DZL). A.U. received consulting fees from Medtronic. U.H. serves as an unpaid board member for Deutsche Gesellschaft für Kinderkardiologie und Angeborene Herzfehler, and Deutsche Gesellschaft für Kinder- und Jugendmedizin. C.A. received lecture and consulting fees from Janssen. The authors have no other conflicts of interest to declare. (2024 AME Publishing Company. All rights reserved.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu