Pulmonale Hypertonie im Zusammenhang mit Linksherzerkrankungen (Gruppe 2)

Patienten mit Linksherzerkrankungen (LHD) weisen häufig eine pulmonale Hypertonie (PH) auf, die Einfluss auf Morbidität und Letalität hat. Dieser Beitrag bietet einen Überblick über aktuelle Entwicklungen im Kontext der PH-LHD inkl. diagnostischer Abklärung und Behandlungsstrategien. Darüber hinaus wird der aktuelle Kenntnisstand zu den pathophysiologischen Zusammenhängen bei PH-LHD, der prognostischen Relevanz hämodynamischer Parameter sowie Therapiestrategien beleuchtet. Der Artikel untermauert die Notwendigkeit für eine präzise diagnostische Abklärung und individualisierte Behandlungsstrategien bei Patienten mit PH-LHD.

Abstract

Patients with left heart disease (LHD) often display pulmonary hypertension (PH), which impacts morbidity and mortality. The pathophysiology of PH is complex and entails pulmonary congestion due to elevated left-sided filling pressures, pulmonary vasoconstriction as well as vascular remodeling. The recent ESC/ERS Guidelines on pulmonary hypertension updated the hemodynamic definitions of pulmonary hypertension in general, and the subclassification of post-capillary PH. This review summarizes recent advances in the diagnostic work-up and management strategies of PH associated with LHD. Specifically, we summarize revisited hemodynamic definitions and the characteristics of isolated post-capillary PH (IpcPH) and combined post- and pre-capillary PH (CpcPH). Furthermore, we review the current knowledge on the pathogenesis of PH-LHD, the prognostic relevance of hemodynamic parameters, and the management strategies, differentiating between treatment of the underlying left heart disease and therapies targeting the pulmonary circulation. The article emphasises the need for precise diagnostic work-up and individualized treatment strategies in patients with PH-LHD.

Kernaussagen

• Bei Patienten mit LHD lässt sich häufig eine PH beobachten, welche erheblichen Einfluss auf Morbidität und Letalität besitzt.

• Insbesondere Patienten mit erhöhtem PVR weisen eine schlechtere Prognose sowie eine stärkere Beeinträchtigung der Belastungstoleranz auf.

• Dies unterstreicht die klinische Relevanz hämodynamischer Charakteristika und die Notwendigkeit einer detaillierten diagnostischen Aufarbeitung und Phänotypisierung.

• Wichtig ist die Erkenntnis, dass bei Patienten mit LHD die primäre Ursache für eine PH in der Regel eine Erhöhung der linksseitigen Füllungsdrucke ist; dennoch können weitere Komponenten wie pulmonale Vasokonstriktion und vaskuläres Remodeling zur Erhöhung des PAP beitragen und das Ausmaß einer PH mit determinieren.

• In Abhängigkeit des Ausmaßes dieser einzelnen Komponenten können unterschiedliche hämodynamische Phänotypen existieren.

• Primäre Behandlungsstrategie ist eine Verminderung bzw. Normalisierung der linksseitigen Füllungsdrucke. Jedoch kann bei Patienten mit weiterhin deutlich erhöhtem PVR (d.h. > 5 WE) ein individuelles Therapiekonzept erforderlich sein.

• Bei der Interpretation von Studienergebnissen sind die präzise Berücksichtigung der Charakteristika der eingeschlossenen Patienten (Art der LHD, Vorhandensein einer PH, Unterscheidung zwischen IpcPH und CpcPH) sowie der untersuchten Medikamentenklasse/Substanz und des beeinflussten Signalweges essenziell.

• Weitere Studien sind notwendig, um das therapeutische Potenzial und die Sicherheit von gezielten PH-Therapien bei einzelnen Formen von Linksherzerkrankungen zu evaluieren.

Schlüsselwörter

pulmonale Hypertonie - Linksherzerkrankung - Klappenvitium - isoliert postkapilläre pulmonale Hypertonie (IpcPH) - kombiniert post- und präkapilläre pulmonale Hypertonie (CpcPH)

Keywords

pulmonary hypertension - left heart disease - valvular heart disease - isolated post-capillary PH (IpcPH) - combined post- and pre-capillary PH (CpcPH)

Publication History

Article published online:
10 November 2023

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