Outcome of patients with primary hepatic venous obstruction treated with anticoagulants alone

Background Outcome of patients with hepatic venous outflow tract obstruction (HVOTO) has improved with newer treatments, including anticoagulants, radiological interventions and liver transplant. In India, however, liver transplant and radiological interventions are costly and have limited availability. Hence, patients often opt for anticoagulation alone. We followed up a group of such patients to determine the clinical outcome with such treatment. Methods Consecutive patients with HVOTO, treated with oral anticoagulation and supportive medical therapy but no radiological or surgical intervention, were followed up for at least 12 months. Diagnosis of HVOTO was based on color Doppler, and either angiography or magnetic resonance venography. Warfarin dose was adjusted to maintain international normalized ratio (INR) between 2.0 and 3.0. Patients with secondary HVOTO and those with baseline INR >= 2.0 were excluded. Response was defined as absence of ascites and/or encephalopathy, normal AST/ALT, bilirubin < 1.5 mg/dL, and no portal hypertension related bleed after starting therapy. Results Of 43 patients (mean [SD] age=28.7 [8.4] years; 20 men), 26 (61%) had a response during a median follow up of 23 (range 15-33) months. The response first appeared within 2 months of the start of treatment in 18 patients and between 2 and 5 months from the start of treatment in eight patients. Seven patients died of progressive liver failure (6 patients) or GI bleed (1 patient). Nine patients had anticoagulation-related complications. On univariate analysis, short duration of symptoms, high serum albumin, low baseline INR, and low baseline Child-Pugh's (CP) or Clichy scores predicted response. Presence of hepatic encephalopathy, portal vein thrombosis, obstruction of all hepatic veins, low albumin, high INR, high serum bilirubin, high baseline CP score, Murad score and adverse Clichy index were associated with higher mortality rate. However, on multivariate analysis, only low CP score was associated with response, and no factor was found to predict death. Conclusions More than half of patients with HVOTO show response with only supportive medical therapy and anticoagulants. This occurs more often in patients with low CP score. Some patients may have delayed response.