title: Paradoxical arterial hypoxemia in a left-to-right shunt congenital heart disease.
abstract: 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.
authors.forename: Efrén
authors.initials: E
authors.lastname: Martínez-Quintana
authors.forename: Fayna
authors.initials: F
authors.lastname: Rodríguez-González
mesh_terms: D000328:Adult; D001401:Azygos Vein / Q000473:pathology*; D004108:Dilatation, Pathologic / Q000209:etiology; D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D006439:Hemodynamics; D020065:Hepatopulmonary Syndrome / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000175:diagnosis; D000860:Hypoxia / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D011169:Portal Vein / Q000473:pathology*; D011379:Prognosis; D011667:Pulmonary Veins / Q000473:pathology