Pulmonale arterielle Hypertonie bei Erwachsenen mit angeborenen Herzfehlern – Teil I

Zusammenfassung

Die Zahl der Erwachsenen mit angeborenen Herzfehlern (AHF) steigt ständig und liegt in Deutschland aktuell bei etwa 360.000. AHF sind häufig mit einer pulmonalarteriellen Hypertonie (PAH) assoziiert, die sich bei unbehandelten AHF teils schon frühzeitig entwickelt. Trotz einer zeitgerechten Behandlung des AHF persistiert eine PAH nicht selten bzw. entwickelt sich im höheren Lebensalter neu und ist mit erheblicher Morbidität und Letalität behaftet.

Die überarbeiteten Leitlinien der European Society of Cardiology/European Respiratory Society 2022 für die Diagnose und Behandlung der PAH stellen einen wesentlichen Beitrag zur optimierten Versorgung der Betroffenen dar. Im vorliegenden Artikel soll der Abschnitt zur PAH assoziiert mit AHF aus den Leitlinien zusammengefasst und kommentiert werden.

Abstract

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.

Schlüsselwörter

pulmonalarterielle Hypertonie - angeborene Herzfehler - Eisenmenger-Syndrom

Keywords

pulmonary arterial hypertension - congenital heart disease - Eisenmenger syndrome

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