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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.

In every patient with dyspnea, this must be ruled out. Screening can be done using echocardiography. This examination usually overestimates the pulmonary pressures. The diagnosis is made using cardiac catheterization, to examine the right side of the heart. Failure to recognize this can lead to sudden death during a surgical procedure. The cause of death in these patients is sudden death.

The differential diagnosis must be made with pulmonary hypertension due to left heart failure and other forms of pulmonary arterial hypertension, such as systemic diseases and drug-induced forms. The patients are eligible for pulmonary arterial hypertension-lowering medication.

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