Hepatic venous outflow tract obstruction: treatment outcomes and development of a new prognostic score

• Published in: Alimentary pharmacology & therapeutics Vol. 43; no. 11; pp. 1154 - 1167
• Main Authors: Shalimar, Kumar, A., Kedia, S., Sharma, H., Gamanagatti, S. R., Gulati, G. S., Nayak, B., Thakur, B., Acharya, S. K.
• Format: Journal Article
• Language: English
• Published: England 01-06-2016
• Subjects: Adolescent; Adult; Budd-Chiari Syndrome - surgery; Child; Child, Preschool; Female; Hepatic Veins; Humans; India; Male; Middle Aged; Portasystemic Shunt, Transjugular Intrahepatic - methods; Prognosis; Retrospective Studies; Stents; Treatment Outcome; Young Adult
• ISSN: 0269-2813; 1365-2036
• DOI: 10.1111/apt.13604

Summary
Background
Results of endovascular interventions in hepatic venous outflow tract obstruction (HVOTO) have been reported from limited studies. Treatment outcomes and prognostic scores need further validation.
Aim
To evaluate treatment outcomes and prognostic scores for hepatic venous outflow tract obstruction in an Indian population.
Methods
Consecutive patients with hepatic venous outflow tract obstruction diagnosed at a tertiary centre were included. Technical success and clinical response after endovascular interventional therapy were documented. Predictors of survival were assessed with Cox‐proportional model. A new score was derived from the factors significant on multivariate analysis and compared with Child–Turcotte–Pugh, model for end‐stage liver disease (MELD), Rotterdam prognostic index (PI) and Budd‐Chiari syndrome‐transjugular intrahepatic portosystemic shunt ( BCS‐TIPSS) PI.
Results
Three hundred and thirty‐four patients (56.6% males), median age 24 (3–62) years were included. Hepatic vein was the commonest site of block‐isolated hepatic vonous block in 48%, combined hepatic venous‐inferior vena cava block in 46%. Endovascular interventional therapy was performed in 233/334 (70%) with 90% technical success. Clinical response was complete in 166 (71.2%), partial in 58 (24.9%) and no response in nine (3.9%). Majority of cases with HV block did not require TIPSS and could be treated with angioplasty (with/without stenting). On Cox‐proportional multivariate analysis, Child class C and response to intervention were independent predictors of outcome and used to derive the All India Institute of Medical Sciences (AIIMS) hepatic venous outflow tract obstruction score. The 5‐year survival was 92% (95% CI, 81–97%) for score ≤3, 79% (95%CI, 63–88%) for score >3 and ≤4, and 39% (95% CI, 21–57%) for score >4. The performance of AIIMS hepatic venous outflow obstruction score was superior to other prognostic indices.
Conclusions
Advanced Child class and no response to intervention are associated with poor outcomes. The All India Institute of Medical Sciences hepatic venous outflow tract obstruction score predicts survival better than other prognostic scores.