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

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

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