Etiology, Management, and Outcome of the Budd-Chiari Syndrome

Murad, Sarwa Darwish; Plessier, Aurélie; Hernández‐Guerra, Manuel; Fabris, F; Eapen, C E; Bahr, Matthias J.; Trebicka, Jonel; Morard, Isabelle; Lasser, Luc; Heller, J.; Hadengue, Antoine; Langlet, Philippe; Miranda, Helena; Primignani, Massimo; Elias, Elwyn; Leebeek, Frank W.G.; Rosendaal, Frits R.; García‐Pagán, Juan Carlos; Valla, Dominique; Janssen, Harry

doi:10.7326/0003-4819-151-3-200908040-00004

Cited by 454 publications

(343 citation statements)

References 26 publications

(34 reference statements)

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“…Apart from LTx, surgical treatment is generally not contemplated in the BCS management by recent guidelines [1,2] . In fact, the most used treatment for BCS non responsive to medical therapy is TIPS, LTx is used as a rescue therapy, while surgical treatments are limited to a strict minority of patients [3] . TIPS is surely the mostly used treatment for BCS when medical therapy fails [3] .…”

Section: Discussionmentioning

confidence: 99%

“…In fact, the most used treatment for BCS non responsive to medical therapy is TIPS, LTx is used as a rescue therapy, while surgical treatments are limited to a strict minority of patients [3] . TIPS is surely the mostly used treatment for BCS when medical therapy fails [3] . In early experiences, TIPS has proved effective as BCS treatment, also in the technically difficult case of extension of thrombosis to the por-tal vein tree [10,11] .…”

Section: Discussionmentioning

confidence: 99%

“…However, apart from liver transplantation, surgical approach is performed in a minority of patients [3] . This is probably the consequence of the absence of a well established surgical strategy for BCS, the huge difference in the outcome reported after surgery and the good response homogeneously reported after interventional approaches [4][5][6][7][8][9][10][11][12][13] .…”

Section: Case Reportmentioning

confidence: 99%

“…This is probably the consequence of the absence of a well established surgical strategy for BCS, the huge difference in the outcome reported after surgery and the good response homogeneously reported after interventional approaches [4][5][6][7][8][9][10][11][12][13] . In fact, TIPS has recently become the standard treatment of BCS non responsive to medical therapy [3] . However, a subgroup of patients with BCS, namely those with inferior vena cava (IVC) obstruction proximal to the atrium, do not benefit from TIPS and a surgical approach is mandatory.…”

Section: Case Reportmentioning

confidence: 99%

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A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Mancuso

Martinelli²,

Carlis³

et al. 2013

WJH

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Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavoatrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft. Key words: Budd-Chiari syndrome; Inferior vena cava; Occlusion; Surgery; Liver transplantation Core tip: We describe a novel surgical approach using a fresh caval homograft for inferior vena cava (IVC) obstruction proximal to the atrium. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for Budd-Chiari syndrome. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

See 2 more Smart Citations

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Mancuso

Martinelli²,

Carlis³

et al. 2013

WJH

View full text Add to dashboard Cite

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“…The 24-month survival was 82%. Importantly, about one-third of the patients remained on medical therapy only, reporting a worse outcome [45] . However, the follow-up was not long enough to eventually show the consequences of a slowly progressing disease, possibly prevented by early recanalization or decompression, and to draw any definitive conclusion about the exact timing of treatment.…”

Section: Early Versus Delayed Interventional Treatment Of Bcsmentioning

confidence: 99%

Timing of transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: An Italian hepatologist’s perspective

Mancuso

2017

Journal of Translational Internal Medicine

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Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident. Recent data support that in BCS liver fibrosis could arise from chronic microvascular ischemia. A reasoning model of BCS physiopathology is that impaired hepatic vein outflow has hemodynamic consequences on portal hypertension development and causes hepatic fibrosis and liver failure through chronic ischemic damage. On this assumption is the concept that relieving liver congestion could ameliorate liver function and prevent development of BCS complications. Recently, early interventional treatment with TIPS for BCS has been reported to be effective. Early TIPS seems to be the best option for BCS management. Future multicenter controlled studies should compare the outcome of BCS treated with early interventional treatment compared with stepwise strategy.

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Long-term Clinical Outcomes of Splanchnic Vein Thrombosis

et al. 2015

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IMPORTANCE Little information is available on the long-term clinical outcome of patients with splanchnic vein thrombosis (SVT).OBJECTIVE To assess the incidence rates of bleeding, thrombotic events, and mortality in a large international cohort of patients with SVT. DESIGN, SETTING, AND PARTICIPANTSA prospective cohort study was conducted beginning May 2, 2008, and completed January 30, 2014, at hospital-based centers specialized in the management of thromboembolic disorders; a 2-year follow-up period was completed January 30, 2014, and data analysis was conducted from July 1, 2014, to February 28, 2015. Participants included 604 consecutive patients with objectively diagnosed SVT; there were no exclusion critieria. Information was gathered on baseline characteristics, risk factors, and antithrombotic treatment. Clinical outcomes during the follow-up period were documented and reviewed by a central adjudication committee. MAIN OUTCOMES AND MEASURESMajor bleeding, defined according to the International Society on Thrombosis and Hemostasis; bleeding requiring hospitalization; thrombotic events, including venous and arterial thrombosis; and all-cause mortality. RESULTSOf the 604 patients (median age, 54 years; 62.6% males), 21 (3.5%) did not complete follow-up. The most common risk factors for SVT were liver cirrhosis (167 of 600 patients [27.8%]) and solid cancer (136 of 600 [22.7%]); the most common sites of thrombosis were the portal vein (465 of 604 [77.0%]) and the mesenteric veins (266 of 604 [44.0%]). Anticoagulation was administered to 465 patients in the entire cohort (77.0%) with a mean duration of 13.9 months; 175 of the anticoagulant group (37.6%) received parenteral treatment only, and 290 patients (62.4%) were receiving vitamin K antagonists. The incidence rates (reported with 95% CIs) were 3.8 per 100 patient-years (2.7-5.2) for major bleeding, 7.3 per 100 patient-years (5.8-9.3) for thrombotic events, and 10.3 per 100 patient-years (8.5-12.5) for all-cause mortality. During anticoagulant treatment, these rates were 3.9 per 100 patient-years (2.6-6.0) for major bleeding and 5.6 per 100 patient-years (3.9-8.0) for thrombotic events. After treatment discontinuation, rates were 1.0 per 100 patient-years (0.3-4.2) and 10.5 per 100 patient-years (6.8-16.3), respectively. The highest rates of major bleeding and thrombotic events during the whole study period were observed in patients with cirrhosis (10.0 per 100 patient-years [6.6-15.1] and 11.3 per 100 patient-years [7.7-16.8], respectively); the lowest rates were in patients with SVT secondary to transient risk factors (0.5 per 100 patient-years [0.1-3.7] and 3.2 per 100 patient-years [1.4-7.0], respectively). CONCLUSIONS AND RELEVANCEMost patients with SVT have a substantial long-term risk of thrombotic events. In patients with cirrhosis, this risk must be balanced against a similarly high risk of major bleeding. Anticoagulant treatment appears to be safe and effective in most patients with SVT.

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Etiology, Management, and Outcome of the Budd-Chiari Syndrome

Abstract: Fifth Framework Programme of the European Commission.

Cited by 454 publications

References 26 publications

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Timing of transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: An Italian hepatologist’s perspective

Long-term Clinical Outcomes of Splanchnic Vein Thrombosis

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