Controversies in the Management of Budd–Chiari Syndrome
The flowchart of Budd–Chiari Syndrome (BCS) management is not evidence-based and relies to experts’ opinion. The aim of this chapter is to enlighten the controversies about BCS management.
Guidelines about BCS management suggest following a step-wise strategy where sole medical therapy is the initial treatment, revascularization or TIPS the second step, and liver transplant the rescue therapy. However, sole medical therapy generally results in bad long-term outcome. The main debated issue of guidelines is that further intervention is suggested only when hemodynamic consequences of portal hypertension become evident. However, as a theory recently stated, liver fibrosis could be the final result of chronic micro-vascular ischemia. Consequently, in the context of BCS, impaired venous hepatic outflow could result in portal hypertension development so triggering hepatic fibrosis and liver failure through chronic liver ischemic injury. Moreover, treatment induced liver congestion relief might preserve liver function avoiding BCS complications development. Recently, early TIPS was suggested to possibly improve the outcome of BCS.
Future studies should be designed with the aim of evaluating whether the outcome of BCS could be improved with early intervention versus step-wise strategy. Furthermore, researchers should explore, using non-invasive tools, which subgroup of patients on only medical therapy would mostly benefit from early intervention.
KeywordsBudd–Chiari syndrome TIPS Liver transplant Outcome
- 12.Gwon D 2nd, Ko GY, Yoon HK, Sung KB, Kim JH, Lee SS, Lee JM, Ohm JY, Shin JH, Song HY. Hepatocellular carcinoma associated with membranous obstruction of the inferior vena cava: incidence, characteristics, and risk factors and clinical efficacy of TACE. Radiology. 2010;254:617–26.PubMedCrossRefPubMedCentralGoogle Scholar
- 21.Simonetto DH, Yang HY, Yin M, et al. Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces. Hepatology. 2015;61:649–59.Google Scholar
- 26.De Gottardi A, Trebicka J, Klinger C, Plessier A, Seijo S, Terziroli B, Magenta L, Semela D, Buscarini E, Langlet P, Görtzen J, Puente A, Müllhaupt B, Navascuès C, Nery F, Deltenre P, Turon F, Engelmann C, Arya R, Caca K, Peck-Radosavljevic M, Leebeek FWG, Valla D, Garcia-Pagan JC, Investigators VALDIG. Antithrombotic treatment with direct-acting oral anticoagulants in patients with splanchnic vein thrombosis and cirrhosis. Liver Int. 2017;37:694–9.PubMedCrossRefPubMedCentralGoogle Scholar
- 53.Yoon YI, Lee SG, Moon DB, Ahn CS, Hwang S, Kim KH, Ha TY, Song GW, Jung DH, Park GC, Kim DS, Choo SJ. Surgical techniques and long-term outcomes of living-donor liver transplantation with inferior vena cava replacement using atriocaval synthetic interposition graft for Budd-Chiari syndrome. Ann Surg. 2019;269(4):e43–5.PubMedCrossRefGoogle Scholar