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Pneumologie 2024; 78(08): 566-577
DOI: 10.1055/a-2274-1025
Übersicht

Pulmonale Hypertonie bei Erwachsenen mit angeborenen Herzfehlern im Lichte der 2022-ESC-PAH-Leitlinien

Part II: Supportive Therapie, Sondersituationen (Schwangerschaft, Kontrazeption, nicht-kardiale Operationen), zielgerichtete Pharmakotherapie, Organtransplantation, spezielles Management (Shuntvitien, Linksherzerkrankungen, univentrikuläre Herzen), Interventionen, Intensivmedizin, Nachsorge, ZukunftsperspektivePulmonary hypertension in adults with congenital heart disease in light of the 2022-ESC-PAH guidelinesPart II: Supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesions, left ventricular disorders, univentricular hearts), interventions, intensive care, follow-up, future perspectives
Harald Kaemmerer
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Gerhard Paul Diller
 2   Klinik für Kardiologie III: angeborene Herzfehler (EMAH) und Klappenerkrankungen, Universitätsklinikum Münster, Münster, Deutschland
,
Stephan Achenbach
 3   Universitätsklinik Erlangen, Medizinische Klinik 2 – Kardiologie und Angiologie, Erlangen, Deutschland
,
Ingo Dähnert
 4   Universitätsklinik für Kinderkardiologie, Herzzentrum Leipzig, Leipzig, Deutschland
,
Christina A. Eichstaedt
 5   Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg am Universitätsklinikum Heidelberg, Heidelberg, Deutschland; Institut für Humangenetik, Universität Heidelberg, INF 366, TLRC am DZL Heidelberg, Deutschland
,
Andreas Eicken
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Annika Freiberger
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Sebastian Freilinger
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Ralf Geiger
 6   Univ.-Klinik für Pädiatrie III, Kardiologie, Pneumologie, Allergologie, Cystische Fibrose, Innsbruck, Österreich
,
Matthias Gorenflo
 7   Klinik für Kinderkardiologie und angeborene Herzfehler, Universitätsklinikum Heidelberg, Heidelberg, Deutschland
,
Ekkehard Grünig
 5   Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg am Universitätsklinikum Heidelberg, Heidelberg, Deutschland; Institut für Humangenetik, Universität Heidelberg, INF 366, TLRC am DZL Heidelberg, Deutschland
,
Alfred Hager
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Michael Huntgeburth
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Ann-Sophie Kaemmerer-Suleiman
 8   Herzchirurgische Klinik, Universitätsklinikum Erlangen, Erlangen, Deutschland
,
Rainer Kozlik-Feldmann
 9   Klinik und Poliklinik für Kinderkardiologie, Universitäres Herz- und Gefäßzentrum Hamburg, Klinik und Poliklinik für Kinderherzmedizin und Erwachsene mit angeborenen Herzfehlern, Hamburg, Deutschland
,
Astrid E. Lammers
10   Klinik für Pädiatrische Kardiologie, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, Münster, Deutschland
,
Nicole Nagdyman
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Sebastian Michel
11   LMU Klinikum, Herzchirurgische Klinik und Poliklinik, Sektion für Chirurgie angeborener Herzfehler und Kinderherzchirurgie, Campus Großhadern, München, Deutschland
,
Kai Helge Schmidt
12   Universitätsmedizin Mainz, Zentrum für Kardiologie – Kardiologie I, Johannes Gutenberg-Universität Mainz, Mainz, Deutschland
,
Anselm Uebing
13   Universitätsklinikum Schleswig-Holstein, Klinik für angeborene Herzfehler und Kinderkardiologie, Kiel, Deutschland
,
Fabian von Scheidt
 1   Internationales Zentrum für Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Deutschland
,
Christian Apitz
14   Sektion Pädiatrische Kardiologie, Universitätsklinik für Kinder- und Jugendmedizin Ulm, Ulm, Deutschland
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Zusammenfassung

Die Zahl der Erwachsenen mit angeborenen Herzfehlern (AHF) steigt ständig und liegt in Deutschland bei etwa 360 000. AHF sind häufig mit einer pulmonalen Hypertonie (PH) assoziiert, die sich bei unbehandelten AHF teils schon frühzeitig entwickeln. Trotz einer zeitgerechten Behandlung des AHF persistiert eine PH häufig oder entwickelt sich im höheren Lebensalter neu und ist mit erheblicher Morbidität und Letalität behaftet.

Die überarbeiteten Leitlinien (LL) der European Society of Cardiology/European Respiratory Society 2022 für die Diagnostik und Behandlung der PH stellen einen wesentlichen Beitrag zur optimierten Versorgung der Betroffenen dar. Der Themenbereich „Erwachsene mit angeborenen Herzfehlern“ wird in diesem Zusammenhang allerdings nur relativ oberflächlich behandelt. In dem vorliegenden Artikel wird diese Thematik daher detailliert aus Sicht der kongenitalen Kardiologie kommentiert.

Abstract

The number of adults with congenital heart defects (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary hypertension (PH), which may develop early in untreated CHD. Despite timely treatment of CHD, PH not infrequently 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 disease” is addressed only relatively superficial in these guidelines. Therefore, in the present article, this topic is commented in detail from the perspective of congenital cardiology.

Schlüsselwörter

pulmonalarterielle Hypertonie - angeborene Herzfehler - Eisenmenger-Syndrom

Keywords

pulmonary arterial hypertension - congenital heart disease - Eisenmenger syndrome


Publication History

Article published online:
24 May 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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