Zusammenfassung
Das hepatopulmonale Syndrom (HPS) ist charakterisiert durch die Kombination einer Hepatopathie und der Störung des pulmonalen Gasaustausches mit arterieller Hypoxämie, intrapulmonaler Gefäßdilatation und Shuntbildung ohne zugrunde liegende kardiale oder pulmonale Erkrankung. Der Verlauf ist in der Regel progredient und das Mortalitätsrisiko in Abhängigkeit vom pulmonalarteriellen Shuntvolumen und dem Ausmaß der pO2-Erniedrigung bei Raumluft zunehmend erhöht. Die Pathophysiologie des HPS ist bis dato nur partiell geklärt. Favorisiert wird eine multifaktorielle Genese. Neben funktionellen intrapulmonalen arteriovenösen Shunts, welche die wichtigste Ursache für das HPS darstellen, führen ein Ventilations-Perfusions-Ungleichgewicht sowie eine eingeschränkte Sauerstoffdiffusion zur Entwicklung einer arteriellen Hypoxämie. Klinisch gilt es zwischen pulmonalen und hepatischen Symptomen zu differenzieren. Diagnostisch ist vor allem die Kontrastmittelechokardiographie das Verfahren der Wahl. Symptomatisch kann die für das HPS charakteristische Hypoxämie durch Sauerstoffgabe gelindert werden. Der einzige in größeren Patientenkollektiven belegte erfolgreiche Behandlungsansatz des HPS ist die Lebertransplantation. Ziel der folgenden Übersicht ist deshalb eine Standortbestimmung.
Abstract
The hepatopulmonary syndrome (HPS) is characterized by a combination of liver disease and pulmonary gas exchange abnormalities with arterial hypoxemia, intrapulmonary vasodilatation and arteriovenous shunting in the absence of intrinsic cardiopulmonary disease. The course of the disease is typically progressive. The mortality rate correlates with the pulmonary shunt volume and the degree of hypoxemia at room air. While the pathyphysiology of HPS is still not fully understood, a multifactorial etiology is favored. Apart from functional intrapulmonary arteriovenous shunts which appear to represent a major factor in the development of HPS, both ventilation-perfusion mismatch and limited oxygen diffusion contribute to the HPS. Regarding its clinical appearance, pulmonary and hepatic symptoms have to be distinguished. Contrast echocardiography is the primary diagnostic tool. Symptomatically, hypoxemia can be treated with oxygen. So far, the only successful treatment approach which has been tested in larger patient groups, is liver transplantation. Given this background, the aim of this review is to critically discuss current concepts of this serious complication of liver diseases.
Schlüsselwörter
Hepatopathie - hepatopulmonales Syndrom - Hypoxämie - NO - pulmonale Vasodilatation
Key words
Hepatopathy - hepatopulmonary syndrome - hypoxemia - NO - pulmonary vasodilatation
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PD Dr. med. Jürgen M. Gschossmann
Klinik für Gastroenterologie, Inselspital/Universität Bern
3010 Bern, Schweiz
Phone: ++ 41/31/6 32 02 91
Email: juergen.gschossmann@insel.ch
