Hepatopulmonary syndrome

Abstract

Hepatopulmonary syndrome (HPS) is a pulmonary complication observed in patients with chronic liver disease and/or portal hypertension. HPS is attributable to an intrapulmonary vascular dilatation that induces severe hypoxemia. Considering the favorable long-term survival of HPS patients as well as the reversal of the syndrome with a functional liver graft, HPS is now an indication for liver transplantation (LT). Both patients with mild cirrhosis who present with shortness of breath and all patients with end stage liver disease who are candidates for liver transplantation should undergo screening for HPS. Blood gas analysis and contrast-enhanced echocardiography are two main screening tools, together with lung function tests that can also detect additional pulmonary diseases that can contribute to impaired oxygenation. If the partial pressure of oxygen in arterial blood (PaO2) is > 80 mmHg, HPS can be excluded and no other investigation is needed. However, when PaO2 is ≤ 80 mmHg, contrast-enhanced echocardiography should be performed to obtain evidence of or to exclude pulmonary vascular dilatation.