Relentless Progression of Venous Obstruction in a Case of Budd-Chiari Syndrome Related to Heterozygous Protein C Deficiency

A 28-year-old man with heterozygous protein C deficiency presented with Budd-Chiari syndrome resulting from hepatic vein obstruction. Over the next 40 months, standard oral anticoagulant therapy and multiple percutaneous interventions aimed at relieving hepatic vein obstruction could not prevent progression of the disease ultimately to cirrhosis and death. Serial angiography provided unique documentation of the relentless progression of hepatic venous obstruction, which was related to the disease and to iatrogenic factors. Operative findings obtained during unsuccessful mesocaval shunt surgery revealed that venous disease in protein C deficiency can be far more extensive than is clinically anticipated. The ineffectiveness of therapy in this patient may be related to standard oral anticoagulant therapy being insufficient to offset the risk of recurrent thrombosis and progression to an advanced stage of vascular damage.