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.
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
