Pulmonale Hypertonie bei Erwachsenen mit angeborenen Herzfehlern (EMAH) – neue Aspekte vom 7. Weltsymposium zu Pulmonaler Hypertonie 2024

Zusammenfassung

Die Zahl Erwachsener mit angeborenen Herzfehlern (EMAH, AHF) steigt stetig an und liegt in Deutschland bei mehr als 360 000, weltweit bei 50 Millionen. Bei vielen AHF tritt die pulmonale Hypertonie (PH) in Form einer pulmonalarteriellen Hypertonie (P[A]H-AHF) auf. Sie kann frühzeitig auftreten oder sich im Langzeitverlauf auch Jahrzehnte nach einer chirurgischen oder interventionellen Behandlung entwickeln und ist mit erheblicher Morbidität und Letalität behaftet. Aufgrund der anatomischen und hämodynamischen Komplexität bedarf eine P(A)H gerade bei EMAH einer differenzierten Betrachtung, Diagnostik und Therapieabwägungen als andere PH-Formen. Die bestehenden Leitlinien der Europäischen Gesellschaft für Kardiologie (ESC) und (ERS) zur PH bieten allgemein gehaltene Diagnostik- und Behandlungsansätze, die bei Weitem nicht alle für EMAH relevanten Aspekte abdecken. Darüber hinausgehend liefert das 7. Weltsymposium zur Pulmonalen Hypertonie (7. WSPH, Barcelona 2024) einen wesentlichen Beitrag zur optimierten Versorgung der Betroffenen, kommentiert die P(A)H-AHF im Kindes- und Jugendalter, aber die P(A)H-AHF bei EMAH wird weitestgehend ignoriert. Im vorliegenden Artikel werden spezifische EMAH-Aspekte aus allen Themenbereichen des 7. WSPH, im Kontext der bestehenden Leitlinien, aber auch hinsichtlich Empfehlungslücken und Zukunftsperspektiven, aus Sicht der kongenitalen Kardiologie kommentiert.

Abstract

The number of adults with congenital heart defects (ACHD, CHD) is constantly rising, with more than 360,000 affected individuals in Germany and 50 million worldwide. Many patients with congenital heart defects (CHD) develop pulmonary hypertension (PH), often as pulmonary arterial hypertension (PAH) associated with congenital heart defects (P[A]H-CHD). P(A)H-CHD may occur early in life or develop long-term, even decades after repair, which is associated with significant morbidity and mortality. Due to the anatomical and hemodynamic complexity of CHD, PH in this population, especially among ACHD, often requires a more differentiated nuanced approach to diagnosis and treatment compared to most forms of PH. The European society of Cardiology (ESC) guidelines provide diagnostic and treatment approaches to P(A)H-CHD, but do not cover all aspects. The current contributions from the 7^th World Symposium on Pulmonary Hypertension (7^th WSPH, Barcelona 2024) provides an update of the diagnosis and an overview of the optimization of PH treatment to improve care of affected individuals, but primarily focuses on children and adolescents with CHD, while recommendations for adults with P(A)H-CHD are scarce. This article comments on specific aspects covering all contents of the 7^th WSPH, in the context of existing guidelines, as well as regarding gaps in recommendations and future perspectives from the standpoint of congenital cardiology for ACHD.

Schlüsselwörter

pulmonale Hypertonie - pulmonal-arterielle Hypertonie - angeborene Herzfehler - Erwachsene mit angeborenen Herzfehlern - EMAH - Eisenmenger-Syndrom - WSPH

Keywords

pulmonary hypertension - pulmonary arterial hypertension - congenital heart disease - adult congenital heart disease - ACHD - Eisenmenger syndrome - WSPH

Publication History

Article published online:
06 October 2025

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