Pulmonale Hypertonie bei Lungenerkrankungen und/oder Hypoxie

Basierend auf den Vorschlägen des 6. PH-Weltsymposiums 2018 wurden 2022 die aktualisierten Leitlinien zur Diagnostik und Therapie der pulmonalen Hypertonie (PH) der Europäischen Fachgesellschaften für Kardiologie und Pneumologie veröffentlicht [1]. Dieser Beitrag gibt ein Überblick über die Pathophysiologie, Diagnose, Prognose und Therapie der PH bei Lungenerkrankungen und/oder Hypoxie (PH associated with chronic Lung Disease, PH-CLD).

Abstract

The pathophysiology of pulmonary hypertension associated with chronic lung disease (PH-CLD) is complex, multifactorial, and not consistent among pulmonary diseases. However, pulmonary vasculopathy triggered by various factors, such as chronic alveolar hypoxia or cigarette smoking, seems to play a central role in the pathogenesis of PH-CLD. While the initial workup of PH-CLD is usually complicated by an overlap of symptoms of PH and the underlying lung disease, PH-CLD should be considered when there is a discrepancy between symptoms (especially exertional dyspnea) and pulmonary function tests. Clinical suspicion of PH-CLD can be strengthened by noninvasive diagnostic tools such as transthoracic echocardiography (TTE) or N-terminal pro-B-type natriuretic peptide (NT-pro-BNP). However, a right heart catheterization should only be performed in specialized centers to establish the diagnosis if therapeutic consequences for the patient were expected.

The basic treatment of PH-CLD is optimal management of the underlying lung disease. Among the existing interventional and registry-based studies, only a small number of data suggests favorable outcomes when treating PH-CLD patients with PAH-specific medications. Some publications even suggest negative effects. Nevertheless, recent data on inhaled vasoactive therapy in PH-CLD showed positive results for inhaled Treprostinil, although long-term data for this therapeutic approach are still lacking. Treatment of PH-CLD patients with PAH-specific drugs should only be performed in specialized centers and preferably in the context of clinical trials.

Kernaussagen

• Die pathophysiologischen Mechanismen der PH-CLD sind multifaktoriell und nicht einheitlich über die verschiedenen Lungenerkrankungen und Phänotypen hinweg.

• Die pulmonale Vaskulopathie, die durch verschiedene Faktoren wie z.B. die chronische alveoläre Hypoxie oder das Zigarettenrauchen getriggert wird, scheint eine zentrale Rolle bei der Pathogenese der PH-CLD zu spielen.

• Die PH-CLD geht mit einer zusätzlichen Beeinträchtigung der Belastbarkeit, Lebensqualität und Prognose im Vergleich zu CLD-Patienten ohne PH einher.

• Während die Diagnose der PH-CLD in der Regel durch die Überschneidung der Symptome von PH und Lungenerkrankung erschwert wird, sollte die PH-CLD in Betracht gezogen werden, wenn eine Diskrepanz zwischen der Einschränkung der klinischen Belastbarkeit und der Lungenfunktion besteht.

• Der klinische Verdacht auf PH-CLD kann durch nicht invasive Diagnostik wie TTE oder NT-pro-BNP erhärtet werden, allerdings sollte eine invasive RHK-Untersuchung nur dann in erfahrenen Zentren zur Diagnosestellung durchgeführt werden, wenn sich hieraus für den Patienten therapeutische Konsequenzen ergeben.

• Die Behandlung von PH-CLD-Patienten mit PAH-spezifischen Medikamenten sollte nur in spezialisierten Zentren und möglichst im Rahmen klinischer Studien durchgeführt werden.

Schlüsselwörter

pulmonale Hypertonie - chronische Lungenerkrankung - pulmonale Vaskulopathie - COPD - interstitielle Lungenerkrankung

Keywords

pulmonary hypertension - chronic lung disease - pulmonary vasculopathy - chronic obstructive pulmonary disease - interstitial lung disease

Publication History

Article published online:
10 November 2023

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