HVOTO: Twenty five years' experience at a tertiary care center

Abstract

Background and Aims: Hepatic venous outflow tract obstruction (HVOTO) is an uncommon disorder characterised by obstruction of the outflow tract of hepatic veins at any level from small hepatic veins to the junction of Inferior vena cava (IVC) and right atrium. The literature regarding this disease is sparse and comprises of case series few in number and limited by small number of patients. We describe a cohort of patients seen over a period of twenty-five years in a tertiary care center. Methods: All consecutive patients diagnosed as HVOTO were followed up. Etiological work up was done, including that for hypercoagulable states. Therapeutic interventions included angioplasty, stenting and TIPS. The patients were followed up for symptom relief and development of immediate and late complications (including HCC). Results: Three hundred and forteen cases of HVOTO were identified. A total of 305(97%) were symptomatic. Mean age was 32.5 ± 12.5 years. The presentation was chronic in most patients -265(87%), and fulminant in none. A specific etiology was found in 63 out of the 183 patients. Protein C deficiency was the most common cause. Isolated Hepatic vein involvement was the most common presentation in 50% cases, followed by combined HV and IVC and isolated IVC involvement in 37% and 13% respectively. Sixty five patients were lost to follow up. Interventions were carried out in 185 patients -20 patients underwent Transjugular intrahepatic portosystemic shunt (TIPS) and the remaining underwent angioplasty/ stenting in IVC/ hepatic veins. Symptomatic improvement occurred in 83% of patients post stenting. Restenosis was seen in 27%, 13% and 10% cases after angioplasty, stenting and TIPS respectively. Conclusions: Most HVOTO patients are young, isolated hepatic vein involvement is seen most commonly. Protein C deficiency is the most common etiology. Therapeutic interventions result in symptomatic improvement in majority of patients.