Paradoxical arterial hypoxemia in a left-to-right shunt congenital heart disease

The hepatopulmonary syndrome is a rare complication of different types of chronic hepatic diseases with associated portal venous hypertension, resulting in pulmonary vascular dilatation, predominantly in the lower lung fields, and leading to ventilation-perfusion mismatch, arterial hypoxemia and a poor prognosis. We present the case of 42-year-old male patient with an anomalous drainage of the right superior pulmonary vein into the azygos vein and a portal vein cavernomatosis with associated portal venous hypertension who presented severe oxygen desaturation, during exercise, in the context of a hepatopulmonary syndrome.