[Hepatopulmonary syndrome and portal hypertension]

The hepatopulmonary syndrome is known by the association of chronic hepatopathy and refractory hypoxemia linked to pulmonary vasodilatation. The hepatopathy may be an hepatic cirrhosis, a congenital porto-case shunt, a porte cavernous angioma or a portal high blood pressure.

Report new cases

We report the observation of a girl followed from the age of 5 years for type I auto-immune hepatitis complicated of portal high blood pressure, in whom the hepatopulmonary syndrome appears 6 years later and the diagnosis was established in front of the presence of clinical signs (cyanosis and fingers clubbing) associated to a severe hypoxia at 43 mmHg without heart attack and in front of the results of scintigraphy use with albumine micro-agregat marked to technetium 99 m which objected an increase of perfusion at the lungs and an extra pulmonary fixation (cerebral, thyroïdien and renal). In front of the severity of hypoxia and the intensity of the extra pulmonary fixation which corresponds the importance of the shunts, the hepatic transplantation shouldn't be realized and the child followed only a salt-free diet associated to a martial addition and to treatment by propanolol.

The hepatopulmonary syndrome is a diagnosis to evocate in front of all hypoxia happening during the evolution of a chronic hepatopathy with portal high blood pressure. Its prognosis is severe in the absence of a hepatic transplantation.