Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting

Fisher, Neil; McCafferty, Ian; Dolapci, Mete; Wali, M; Buckels, J. A. C.; Olliff, Simon; Elias, Elwyn

doi:10.1136/gut.44.4.568

Cited by 152 publications

(97 citation statements)

References 21 publications

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“…Radiological interventions for both IVC and HV achieved technical success rates of more than 90% and long-term patency rates of more than 80%; also radiological re-interventions are usually successful [1,69,[77][78][79][80] . In our series, radiological intervention protocols according to morphology of the obstruction yielded good results.…”

Section: Discussionmentioning

confidence: 99%

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Amarapurkar¹,

Punamiya²,

Patel³

2008

WJG

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AIM:To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India. METHODS:Forty-nine consecutive cases of BuddChiari syndrome (BCS) were prospectively evaluated. All patients with refractory ascites or deteriorating liver function were, depending on morphology of inferior vena cava (IVC) and/or hepatic vein (HV) obstruction, triaged for radiological intervention, in addition to anticoagulation therapy. Asymptomatic patients, patients with diuretic-responsive ascites and stable liver function, and patients unwilling for surgical intervention were treated symptomatically with anticoagulation. RESULTS:Mean duration of symptoms was 41.5 ± 11.2 (range = 1-240) mo. HV thrombosis (HVT) was present in 29 (59.1%), IVC thrombosis in eight (16.3%), membranous obstruction of IVC in two (4%) and both IVC-HV thrombosis in 10 (20.4%) cases. Of 35 cases tested for hypercoagulability, 27 (77.1%) were positive for one or more hypercoagulable states. Radiological intervention was technically successful in 37/38 (97.3%): IVC stenting in seven (18.9%), IVC balloon angioplasty in two (5.4%), combined IVC-HV stenting in two (5.4%), HV stenting in 11 (29.7%), transjugular intrahepatic portosystemic shunt (TIPS) in 13 (35.1%) and combined TIPS-IVC stenting in two (5.4%). Complications encountered in follow-up: death in five, re-stenosis of the stent in five (17.1%), hepatic encephalopathy in two and hepatocellular carcinoma in one patient. Of nine patients treated medically, two showed complete resolution of HVT. CONCLUSION:In our series, HVT was the predominant cause of BCS. In the last five years with the availability of sophisticated tests for hypercoagulability, etiologies were defined in 85.7% of cases. Non-surgical management was successful in most cases.

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Section: Discussionmentioning

confidence: 99%

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Amarapurkar¹,

Punamiya²,

Patel³

2008

WJG

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“…13 Other treatment methods are thrombolysis 14 and percutaneous hepatic vein balloon angioplasty. 15 Several studies have been published on the cause, clinical manifestations, prognosis, and interventions in BCS. 4 -6,9,10,14,16 -20 However, the results vary widely, and unequivocal conclusions cannot be drawn.…”

Section: Bcsmentioning

confidence: 99%

Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome

et al. 2004

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Budd-Chiari syndrome (BCS) is a rare disorder that is characterized by hepatic venous outflow obstruction. The aim of this study was to assess determinants of survival and to evaluate the effect of portosystemic shunting. In this international multicenter study, 237 patients with BCS, diagnosed between 1984 and 2001, were investigated. Univariate, multivariate, and time-dependent Cox regression analyses were performed. Overall survival at 1, 5, and 10 years was 82% (95% CI, 77%-87%), 69% (95% CI, 62%-76%), and 62% (95% CI, 54%-70%), respectively. Encephalopathy, ascites, prothrombin time, and bilirubin were independent determinants of survival. A prognostic classification combining these factors could identify three classes of patients (classes I-III). The 5-year survival rate was 89% (95% CI, 79%-99%) for class I, 74% (95% CI, 65%-83%) for class II, and 42% (95% CI, 28%-56%) for class III. Anticoagulants were administered to 72%; only for patients in class I was this associated with a trend toward improved survival (relative risk [

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“…In some experiences, short-length stenoses have been treated with angioplasty or stenting, with a good medium term outcome [19][20] .…”

Section: Treatments For Budd-chiari Syndromementioning

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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Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting

Cited by 152 publications

References 21 publications

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome

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

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