Hepatopulmonary syndrome K76.8

Functional abnormalities of pulmonary circulation with intrapulmonary vascular dilatations and consecutive arterial hypoxemia. Specific liver diseases predisposing to HPS or particular laboratory chemical abnormalities that would be predictive of HPS are not known (Koch DG et al. 2014).

The prevalence of hepatopulmonary syndrome occurs in 5 to 29% of patients with advanced liver disease.

Arterial hypoxaemins, the genesis of which involves the interaction of several pathomechanisms, some of which are not yet fully understood (Wagner Th et al. 2002):

functional intrapulmonary arteriovenous shunts

Ventilation-perfusion imbalances

Restriction of oxygen diffusion

The formation of functional intrapulmonary shunts with a diameter of 15 to 160 µm is considered the most important cause of severe hypoxemia and is the characteristic finding in HPS (Berthelot P et al. 1966). Various factors are held responsible for intrapulmonary vasodilation: nitric oxide (NO) seems to play a leading role (Rolla G et al. 1997). The up-regulation of endothelial nitric oxide synthetase (eNOS) in the intraloberal pulmonary arteries and the NO-mediated impairment of vasoconstriction coincides in animal models with the development of HPS . It was reported that the NO concentration in the exhaled air is increased in patients with HPS or liver cirrhosis. Circulating endothelin-1 (ET-1) has been suggested to be responsible for increased pulmonary eNOS expression and NO synthesis in liver cirrhosis (Wolfe JD et al 1977).

Triad of liver disease, disturbance of pulmonary gas exchange with arterial hypoxemia and intrapulmonary vascular dilatation without underlying cardiac or pulmonary disease (Aboussouan LS et al. 2000).

A specific therapy for hepatopulmonary syndrome is not known. The hypoxemia can be improved by oxygenation on demand or by continuous administration of oxygen. In individual cases, an improvement in HPS has been described in connection with the recovery of liver function, for example after surviving hepatitis A.

In individual cases an improvement of the HPS after reduction of portal hypertension has been described by application of a transjugular intrahepatic portosystemic shunt (TIPSS). A TIPSS system can also lead to an improvement in oxygenation in these patients and is therefore suitable in individual cases to bridge the waiting time until transplantation (Lange PA et al. 1996). Ultimately, liver transplantation represents the only curative approach (Wagner Th et al. 2002).

The mortality rate of 41% over a period of 3 years is very high regardless of liver function.

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