Abstract
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two distinct pulmonary complications of liver disease, which seem pathogenetically linked to the presence of portal hypertension. The most common presenting symptom of both syndromes is dyspnoea, but HPS is more prevalent in 5-30% of patients. The diagnosis of HPS requires the documentation of arterial hypoxemia and intrapulmonary vascular dilatation with anatomical shunting. In contrast, POPH has a prevalence of 2-16% and is only considered proven, if other causes of the pulmonary hypertension than the high portal pressure are excluded. Moderate to severe HPS or POPH carry a poor prognosis and liver transplantation remains the only curative treatment, although POPH in particular is associated with high perioperative mortality.
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