Anatomic Recanalization of Hepatic Vein and Inferior Vena Cava versus Direct Intrahepatic Portosystemic Shunt Creation in Budd-Chiari Syndrome: Overall Outcome and Midterm Transplant-Free Survival
Budd Chiari Syndrome (BCS) is a rare, devastating and potentially life-threatening condition related to significant hepatic venous outflow compromise. There are few studies that have robustly reviewed and compared outcomes of active interventions to reverse or by pass the underlying anatomical manifestation of the problem. Interventional radiology has been the lynch pin of these interventions.
Researchers from Institute of Liver and Biliary Sciences, D-1, Vasant Kunj in New Delhi, India recently published their clinical outcomes data for patients with BCS receiving either hepatic/inferior vena cava venous recanalization or DIPS (Direct intrahepatic porto-systemic shunt).
The retrospective study included 136 patients (92 venous recanalization and 44 DIPS) receiving endovascular treatment option based on a clearly defined clinical algorithm. They objectively evaluated Liver US elastography, biochemical markers, imaging pre and post intervention at defined periods. Patients were all followed up until either liver transplantation, death or last clinical visit prior to the end of the study period. In those patients with symptom recurrence and evidence of in-stent stenosis on imaging there was reintervention.
Both groups demonstrated significant clinical, biochemical and liver stiffness (elastography) improvement with intervention. There were lower number of reinterventions in the recanalization group (4%) vs DIPS group (7%) with the later attributed to mainly poor compliance to anticoagulation therapy.
There were no significant differences in the transplant free survival and overall survival between the two groups. The consistent predictor of death and poor clinical outcome in both groups was initial presentation in acute fulminant liver failure with encephalopathy, regardless of successful outflow restoration. They concluded that although DIPS was successful in flow restoration in that cohort, it needed to be considered carefully due to risk of exacerbating encephalopathy and those patients would be best served with liver transplant.
The study adds to a growing body of evidence of the usefulness and durability of endovascular intervention in managing this rare but debilitating condition – BCS. The authors findings are largely consistent with the findings of other previous publications which had smaller samples hence adding further validity to the role of endovascular intervention.
One of the remarkable aspects about this study is the unique volume of cases (180 in 4 yrs) from this single institution for a generally uncommon condition - which emphasizes the level of experience and expertise with BCS from this institution. We learn from their experience and expertise that recanalization, wherever possible should be the primary endovascular approach vs DIPS/TIPS first approach. Not only does recanalization provide early treatment success; it is durable, has similar survival benefit to DIPS/TIPS and yet less risk of exacerbation or causing encephalopathy which is generally associated with poor outcomes. We also learn that patients presenting in acute fulminant BCS have a very poor prognosis and concerted efforts should be made towards early liver transplantation.
We also learn that biochemical changes and liver elastography changes appear to be closely linked to clinical improvement in the early period (< 3 months) and therefore could be useful surrogate markers of progress during the early post treatment phase. It is interesting to note that the etiology of the prothrombotic state appeared to not have a bearing on the outcomes of endovascular intervention. Although this may be a true finding, the sample may not be adequately powered to demonstrate that difference.
The study has a number of weaknesses which the authors acknowledge and discuss very well: Retrospective, likely operator bias in selecting patients for DIPS vs recanalization, regional variation in disease process hence variation in US elastography measurements and they do not have very long term follow up. Despite these limitations, the study contributes immensely to the growing evidence about the crucial role interventional radiology plays in the management of BCS.
With the other recently published studies on this issue about the role of IR in the management of BCS, it may be an opportunity for a pooled systematic review on this subject.
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Mukund A, Mittal K, Mondal A, Sarin SK. Anatomic Recanalization of Hepatic Vein and Inferior Vena Cava versus Direct Intrahepatic Portosystemic Shunt Creation in Budd-Chiari Syndrome: Overall Outcome and Midterm Transplant-Free Survival. J Vasc Interv Radiol. 2018 Jun;29(6):790-799. doi:10.1016/j.jvir.2018.01.781. Epub 2018 Apr 25. PubMed PMID: 29705227.
Rodrick C Zvavanjanja MD, MSc, FRCR, DABR(VIR/DR)
Department of Diagnostic and Interventional Radiology
University of Texas at Houston McGovern Medical School