Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

Hernández‐Gea, Virginia; Gottardi, Andrea De; Leebeek, Frank W.G.; Rautou, Pierre‐Emmanuel; Salem, Riad; García‐Pagán, Juan Carlos

doi:10.1016/j.jhep.2019.02.015

Cited by 96 publications

(93 citation statements)

References 207 publications

(308 reference statements)

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“…Furthermore, the optimal duration of anticoagulation alone before escalating to salvage therapy is unknown. 30 In our study, the average duration before initiation of our protocol was 4 days. It is our impression that longer intervals would be associated with decreased chance of recanalisation leaving the patient at risk of progressive intestinal ischaemia and long-term sequelae of portal hypertension.…”

Section: Whole Cohort (N = 22)mentioning

confidence: 67%

“…In addition, despite our best efforts at identifying patients at high risk of unfavourable outcome with anticoagulation alone, the literature reports inconsistent poor initial prognostic factors where nonsurgical therapies might still have a role. Furthermore, the optimal duration of anticoagulation alone before escalating to salvage therapy is unknown . In our study, the average duration before initiation of our protocol was 4 days.…”

Section: Discussionmentioning

confidence: 99%

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A stepwise thrombolysis regimen in the management of acute portal vein thrombosis in patients with evidence of intestinal ischaemia

Benmassaoud

Alrubaiy

et al. 2019

Aliment Pharmacol Ther

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Summary Background Anticoagulation alone in acute, extensive portomesenteric vein thrombosis (PVT) does not always result in spontaneous clot lysis, and leaves the patient at risk of complications including intestinal infarction and portal hypertension. Aim To develop a new standard of care for patients with acute PVT and evidence of intestinal ischaemia. Methods We present a case series of patients with acute PVT and evidence of intestinal ischaemia plus ongoing symptoms despite initial systemic anticoagulation, who were treated with a thrombolysis protocol between 2014 and 2019. This stepwise protocol initially uses low‐dose systemic alteplase, and in patients with ongoing abdominal pain, and no evidence of radiological improvement, is followed by local clot dissolution therapy (CDT) through a TIPSS. Outcomes and safety were assessed. Results Twenty‐two patients were included. The mean age was 44.6 (standard deviation [SD] 16.0) years, and 64% had an identifiable prothrombotic risk factor. All patients had intestinal wall oedema and 77% had complete occlusion of all portomesenteric veins. Systemic thrombolysis was started 18.7 (SD 11.2) days after the onset of symptoms. 55% of patients underwent TIPSS insertion for CDT. At the end of treatment, symptoms resolved in 91% of patients and recanalisation in 86%. Only one patient required resection for intestinal ischaemia, and there were no deaths. Major complications occurred in two patients (9%). Conclusions Our stepwise protocol is effective, resulting in good recanalisation rates. It can be commenced early while organising transfer to a centre capable of performing local CDT.

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Section: Whole Cohort (N = 22)mentioning

confidence: 67%

Section: Discussionmentioning

confidence: 99%

A stepwise thrombolysis regimen in the management of acute portal vein thrombosis in patients with evidence of intestinal ischaemia

Benmassaoud

Alrubaiy

et al. 2019

Aliment Pharmacol Ther

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“…In addition to portal hypertension due to cirrhosis (±PVT), TIPS is a well-established treatment option for other vascular diseases of the liver, 25 such as Budd-Chiari syndrome, which have recently been reviewed elsewhere 29 and are not discussed in this review article.…”

Section: Role Of Tips In Patients With Cirrhosismentioning

confidence: 99%

Where does TIPS fit in the management of patients with cirrhosis?

García‐Pagán

Saffo²,

Mandorfer³

et al. 2020

JHEP Reports

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In this review, we summarise the current knowledge on the indications and contraindications of transjugular intrahepatic portosystemic shunt (TIPS) placement for the treatment of the complications of portal hypertension in cirrhosis, specifically variceal haemorrhage and ascites. Moreover, we discuss the role of TIPS for the treatment of portal vein thrombosis (PVT) and the prevention of complications after extrahepatic surgery ('preoperative TIPS') in patients with cirrhosis. The position of TIPS in the treatment hierarchy depends on the clinical setting and on patient characteristics. In acute variceal haemorrhage, preemptive TIPS is indicated in patients at a high risk of failing standard therapy, that is those with a Child-Pugh score of 10-13 points or Child-Pugh B with active bleeding at endoscopy, although the survival benefit in the latter group still remains to be established. Non-preemptive TIPS is a second-line therapy for the prevention of recurrent variceal haemorrhage and for the treatment of ascites. Of note, TIPS may also improve sarcopenia. Contraindications to TIPS placement, independent of clinical setting, include very advanced disease (Child-Pugh >13 points), episodes of recurrent overt hepatic encephalopathy without an identifiable precipitating factor, heart failure, and pulmonary hypertension. In patients with PVT, TIPS placement not only controls complications of portal hypertension, but also promotes portal vein recanalisation. Although the severity of portal hypertension correlates with poor outcomes after extrahepatic surgery, there is no evidence to recommend preoperative TIPS placement.

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“…Budd-Chiari syndrome (BCS), first described by the British physician George Budd in 1845 and the Austrian pathologist Hans Chiari in 1898, is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow, involving small hepatic venules, larger hepatic veins, the entire inferior vein cava (IVC) or all of above[ 1 - 3 ]. Based on the nature of obstruction, BCS is classified as primary if the obstruction is attributed to venous pathology such as thrombosis, webs, or endophlebitis and secondary in cases of extraluminal compression such as tumours, abscess, cysts, and pericardial conditions[ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

Inchingolo¹,

Posa²,

Mariappan³

et al. 2020

WJG

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Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.

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Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

Cited by 96 publications

References 207 publications

A stepwise thrombolysis regimen in the management of acute portal vein thrombosis in patients with evidence of intestinal ischaemia

A stepwise thrombolysis regimen in the management of acute portal vein thrombosis in patients with evidence of intestinal ischaemia

Where does TIPS fit in the management of patients with cirrhosis?

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

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