Pulmonale Hypertonie assoziiert mit Lungenerkrankungen

Zusammenfassung

Nicht selten sind Lungenerkrankungen und Hypoventilationssyndrome mit einer pulmonalen Hypertonie (PH) assoziiert. In den meisten Fällen liegt eine nicht schwere PH vor. Diese ist hämodynamisch definiert durch einen pulmonalarteriellen Mitteldruck (PAPm) > 20 mmHg, einen pulmonalarteriellen Verschlussdruck (PAWP) ≤ 15 mmHg und einen pulmonal-vaskulären Widerstand von ≤ 5 Wood-Einheiten (WU). Sowohl die nicht schwere (PVR ≤ 5 WU) als auch deutlich ausgeprägter die schwere PH (PVR > 5 WU) sind prognostisch ungünstig. Bei Verdacht auf eine PH wird empfohlen, primär zu prüfen, ob Risikofaktoren für eine pulmonalarterielle Hypertonie (PAH, Gruppe 1 PH) oder eine chronisch thromboembolische pulmonale Hypertonie (CTEPH, Gruppe 4 PH) vorliegen. Falls Risikofaktoren vorliegen oder bei Lungenkranken der Verdacht auf eine schwere PH besteht, wird eine zeitnahe Vorstellung der Patient*innen in einer PH-Ambulanz empfohlen. Bei Patient*innen mit einer schweren PH assoziiert mit Lungenerkrankungen wird eine personalisierte, individuelle Therapie – möglichst im Rahmen von Therapiestudien – empfohlen. Aktuell sollte bei COPD-Patient*innen nur ein Therapieversuch erwogen werden, wenn die assoziierte PH schwergradig und ein „pulmonalvaskulärer“ Phänotyp (schwere präkapilläre PH, aber typischerweise nur milde bis moderate Atemwegsobstruktion, keine oder milde Hyperkapnie und DLCO < 45 % vom Soll) vorliegt. Bei schwerer PH assoziiert mit einer interstitiellen Lungenerkrankung können entsprechend individueller Abwägung Phosphodiesterase-5-Inhibitoren erwogen werden. Inhaliertes Treprostinil kommt bei diesen Patient*innen auch bei nicht schwerer PH in Betracht.

Abstract

Lung diseases and hypoventilation syndromes are often associated with pulmonary hypertension (PH). In most cases, PH is not severe. This is defined hemodynamically by a mean pulmonary arterial pressure (PAPm) > 20 mmHg, a pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance of ≤ 5 Wood units (WU). Both the non-severe (PVR ≤ 5 WU) and much more the severe PH (PVR > 5 WU) have an unfavorable prognosis.

If PH is suspected, it is recommended to primarily check whether risk factors for pulmonary arterial hypertension (PAH, group 1 PH) or chronic thromboembolic pulmonary hypertension (CTEPH, group 4 PH) are present. If risk factors are present or there is a suspicion of severe PH in lung patients, it is recommended that the patient should be presented to a PH outpatient clinic promptly.

For patients with severe PH associated with lung diseases, personalized, individual therapy is recommended – if possible within the framework of therapy studies. Currently, a therapy attempt with PH specific drugs should only be considered in COPD patients if the associated PH is severe and a “pulmonary vascular” phenotype (severe precapillary PH, but typically only mild to moderate airway obstruction, no or mild hypercapnia and DLCO < 45 % of predicted value) is present. In patients with severe PH associated with interstitial lung disease phosphodiesterase-5-inhibitors may be considered in individual cases. Inhaled treprostinil may be considered also in non-severe PH in this patient population.

Schlüsselwörter

chronisch obstruktive Lungenerkrankung (COPD) - Lungenemphysem - interstitielle Lungenerkrankungen - idiopathische Lungenfibrose - kombinierte pulmonale Fibrose und Emphysem (CPFE) - pulmonale Hypertonie

Keywords

chronic obstructive pulmonary disease (COPD) - emphysema - interstitial lung disease (ILD) - idiopathic pulmonary fibrosis - combined pulmonary fibrosis and emphysema (CPFE) - pulmonary hypertension

Publikationsverlauf

Artikel online veröffentlicht:
14. November 2023

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