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Presents as cyanosis in a patient with advanced liver disease. This is a functional right-left shunt due to dilation of the vascular bed, due to the increased concentrations of nitric oxide in the blood. Clinically, the central cyanosis and tachypnea are noticeable. The patient may develop clubbing of the fingers.

Severe hepatopulmonary syndrome occurs when the PaO2 < 70 mm Hg. The diagnosis is made with echocardiography with contrast. In this case, the bubbles appear immediately in the left half of the heart, after three beats. The only good treatment is liver transplantation.

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