Pulmonale Hypertonie bei Linksherzerkrankungen

Zusammenfassung

Linksherzerkrankungen (LHD) sind die häufigste Ursache einer pulmonalen Hypertonie (PH), die weiter in isolierte postkapilläre PH (IpcPH) und kombinierte prä- und postkapilläre PH (CpcPH) unterteilt wird.

Mit dem Ziel Behandlungsansätzen näherzukommen, setzte die PH-LHD-Arbeitsgruppe des 7. Welt-Symposiums zur pulmonalen Hypertonie einen stärkeren Fokus auf die Phänotypisierung, Subtypisierung und die ausdifferenziertere Betrachtungsweise des breiten Spektrums der PH-LHD. Mit dieser Intention wurde ein 4-stufiges PH-LHD-System erarbeitet, in dem jede Stufe (A = Risiko, B = strukturelle Herzerkrankung, C = symptomatische Herzerkrankung, D = fortgeschritten) durch spezifische pathophysiologische, hämodynamische, klinische und letztendlich auch therapeutische Strategien gekennzeichnet ist. In diesem Kontext wird zudem auf zur PH-LHD führende kardiologische Krankheitsbilder mit spezifischen Therapieansätzen, wie die Mitral- und Aortenklappenstenose sowie ausgewählte Kardiomyopathien, ein Augenmerk gerichtet. Ebenso wird die Überschneidung mit anderen PH-Gruppen wie der CTEPH und der PH bei Lungenerkrankungen betont und konkretisiert. Der therapeutische Einsatz von PAH-Medikamenten, die einen pulmonalvaskulären Ansatz haben, kann anhand von Ergebnissen randomisierter klinischer Studien weiterhin außerhalb klinischer Studien nicht empfohlen werden.

Abstract

Left heart disease (LHD) is the most common cause of pulmonary hypertension (PH), which is further categorised into isolated post-capillary PH (IpcPH) and combined pre- and post-capillary PH (CpcPH).

With the aim of developing treatment strategies, the PH-LHD working group of the 7^th World Symposium on Pulmonary Hypertension placed a stronger focus on phenotyping, subtyping and a more differentiated approach to the broad spectrum of PH-LHD. With this intention, a four-stage PH-LHD system was developed in which each stage (A = risk, B = structural heart disease, C = symptomatic heart disease, D = advanced) is characterised by specific pathophysiological, haemodynamic, clinical and ultimately therapeutic strategies. In this context, attention is also paid to cardiac diseases leading to PH-LHD with specific therapeutic approaches, such as mitral and aortic valve stenosis and selected cardiomyopathies. The overlap with other PH groups such as CTEPH and PH in lung diseases is also emphasised and specified. The therapeutic use of PAH drugs that have a pulmonary vascular approach can still not be recommended outside of clinical trials.

Schlüsselwörter

Linksherzerkrankung - Stenose - Ejektionsfraktion - postkapilläre pulmonale Hypertonie

Keywords

left heart disease - stenosis - ejection fraction - postcapillary pulmonary hypertension

^* geteilte Letztautorenschaft

Publication History

Article published online:
06 October 2025

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