Portopulmonale Hypertonie

Zusammenfassung

Häufig liegen pulmonale Erkrankungen bei Patienten mit einer portalen Hypertension vor und können sich unterschiedlich manifestieren. Veränderungen des pulmonalarteriellen Widerstandes verursachen entweder das Bild eines hepatopulmonalen Syndroms oder einer portopulmonalen Hypertonie (PPHT). Definitionsgemäß liegt eine PPHT bei Vorliegen eines erhöhten pulmonalarteriellen Drucks, eines erhöhten pulmonalvaskulären Widerstands, eines normalen, pulmonalkapillären Wedge-Drucks und bestehender portaler Hypertonie vor, wenn andere bekannte Ursachen der pulmonalen Hypertonie ausgeschlossen sind. Ätiologisch werden eine Hyperzirkulation, eine Volumenüberladung und zirkulierende vasokonstriktorische Mediatoren unter Umgehung des Leberkreislaufs diskutiert. Die Prognose der schweren PPHT ist, aufgrund eines drohenden Rechtsherzversagens deutlich eingeschränkt. Die Sterblichkeit bei Lebertransplantationen ist bei Patienten mit einer moderaten bis schweren PPHT deutlich erhöht. Klinisch kann sich die PPHT vergleichbar mit der idopathisch pulmonalarteriellen Hypertonie mit Dyspnoe als Hauptsymptom und vergleichbaren histopathologischen Veränderungen äußern. Zurzeit wird bei der schweren PPHT die kontinuierliche intravenöse Prostazyklingabe als die medikamentöse Therapie der Wahl angesehen. Für inhalativ appliziertes Prostazyklin oder dessen Analogon Iloprost und für den Endothelinrezeptor-Antagonisten Bosentan, der per os appliziert wird, ist die Datenlage noch zu begrenzt, so dass zurzeit deren Stellenwert in der Therapie der PPHT ungeklärt ist.

Abstract

Patients with portal hypertension may develop pulmonary complications such as hepatopulmonary syndrome (HPS) or portopulmonary hypertension (PPHT). PPHT is defined as elevated pulmonary pressure, elevated pulmonary vascular resistance, a normal pulmonary capillary wedge pressure, and portal hypertension in the absence of other known causes pulmonary hypertension. Various factors such as hyperdynamic circulation, volume overload, and circulating vasoactive mediators are suspected to be involved in the pathogenesis of PPHT. The prognosis of patients with severe PPHT is significantly reduced due to the risk of right heart failure. In patients with moderate to severe PPHT liver transplantation is associated with a significantly increased mortality. The chief symptom of PPHT may be dyspnoe in the presence of typical histomorphological alterations comparable with idiopathic pulmonary hypertension. Continuous intravenous application of prostacyclin is currently regarded as the treatment of choice for patients with severe PPHT. Inhaled prostacyclin or its analogue iloprost or oral treatment with the endothelin-receptor antagonist bosentan may be promising alternatives which should be further investigated in randomized controlled trials.

Schlüsselwörter

Leberzirrhose - portale Hypertonie - pulmonale Hypertonie - portopulmonale Hypertonie - Endothelin

Key words

Liver cirrhosis - portal hypertension - pulmonary hypertension - portopulmonary hypertension - endothelin

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