Case report of hepatopulmonary syndrome in patients with non-cirrhotic portal hypertension

We report the case of a female patient diagnosed with hepatopulmonary syndrome (HPS) which occurred in the context of chronic thrombosis of the portal and mesenteric veins resulting from primary thrombophilia (protein C and protein S deficiencies). This case of HPS was diagnosed when echocardiography with agitated saline contrast showed the patient was positive for extra cardiac shunt. Scintigraphy using albumin macro- aggregates showed evidence of uptake in the brain and persistent hypoxemia with elevated alveolar-arterial gradient for the age of the patient.