An unusual cause of refractory hypoxemia in cirrhosis

Pulmonary gas exchange abnormalities and refractory hypoxemia cause myriad difficulties in patients with chronic liver disease. In addition to intrinsic cardiopulmonary diseases and hepatopulmonary syndrome, some unusual pathophysiological mechanisms in patients with portosystemic collaterals might contribute to hypoxemia. We report the clinical presentation of an unusual portosystemic anatomic shunt that permits venous admixture with oxygenated blood, causing hypoxemia that is refractory to the administration of supplemental oxygen, and recurrent hepatic encephalopathy. There has been no such report in the published literature. This case highlights the importance of keeping direct portopulmonary venous anastomosis in the differential diagnosis of oxygen-refractory hypoxemia and recurrent hepatic encephalopathy in patients with cirrhosis in the appropriate clinical context.