Management of hepatic venous outflow obstruction

Hepatic venous outflow obstruction (HVOO) is a rare cause of portal hypertension and conservative treatment is usually ineffective. A large series of patients gave us an opportunity to devise a management protocol for this disorder. Between 1978 and 1992, we prospectively studied 75 patients with HVOO. The obstruction was in the hepatic vein in 24, in the inferior vena cava (IVC) in 44, and in both in 7. For hepatic vein obstruction proximal splenorenal shunts were done in 7 (2 died postoperatively); 4 shunts blocked and only 1 patient became completely symptom free. In 2 patients with partial obstruction we performed balloon dilatation of the right hepatic veins but within 6 months the obstruction recurred. In the next 6 patients we constructed a side-to-side portocaval shunt; 2 died of encephalopathy after discharge and 4 are alive and well. For IVC obstruction, after surgical procedures had yielded poor results in 14 patients, we changed to balloon angioplasty which was successful in 28 of the 30 other patients; restenosis occurred in 4. Of the 7 patients with a combined block, 3 have had balloon angioplasty followed by a side-to-side portocaval shunt; 1 died, 2 are well, and the remainder have not completed treatment. Of our 75 patients, 22 have died (5 in hospital and 17 after discharge), 7 have not completed treatment, and 2 have been lost to follow-up. However, 44 are symptom free. We did not encounter any case of hepatocellular carcinoma. We suggest that patients with HVOO should be actively managed with a side-to-side portocaval shunt for hepatic vein obstruction, balloon angioplasty for inferior vena caval obstruction, and perhaps both procedures for those with combined obstructions.