Budd-Chiari syndrome due to prothrombotic disorder: mid-term patency and efficacy of endovascular stents

Pelage, Jean-Pierre; Denys, Alban; Valla, Dominique; Sibert, Annie; Sauvanet, Alain; Belghiti, Jacques; Menu, Y.

doi:10.1007/s00330-002-1475-2

Cited by 33 publications

(7 citation statements)

References 31 publications

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“…With respect to HVs, angioplasty 272–274 or stenting 266,275–277 was reported in 14 and 24 patients, respectively, with a follow-up averaging 2 years. The overall technical success rate was unclear.…”

Section: Therapymentioning

confidence: 99%

“…Recent data in a cohort of 39 patients with BCS treated with a surgical shunt followed for a median of 110 months show that maintenance or reestablishment of good shunt function is crucial for long-term survival. 295 Shunt dysfunction may be related to stenosis of intrahepatic IVC (which is amenable to stenting), 275,295,298 to shunt stenosis (amenable to percutaneous transluminal angioplasty and stenting), 273,275,295,299 and to shunt or PVT (amenable to in situ thrombolysis). 299 One risk factor for shunt dysfunction appears to be the use of long prosthetic grafts.…”

Section: Therapymentioning

confidence: 99%

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Vascular disorders of the liver

2008

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Section: Therapymentioning

confidence: 99%

Section: Therapymentioning

confidence: 99%

Vascular disorders of the liver

2008

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“…The technical success rate is high, in the 95% range with an almost instant post‐procedural improvement in signs, symptoms and liver function 1 . Angioplasty of pure hepatic vein block has been less reported 59–64 . The overall success rate is unclear.…”

Section: Diagnostic Workupmentioning

confidence: 99%

“…1 Angioplasty of pure hepatic vein block has been less reported. [59][60][61][62][63][64] The overall success rate is unclear. In general re-obstruction is more common in patients undergoing balloon angioplasty as compared to stenting.…”

Section: Diagnostic Workupmentioning

confidence: 99%

Budd‐Chiari syndrome

Martens

Nevens

2015

UEG Journal

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Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract at any level between the junction of the inferior vena cava with the right atrium and the small hepatic veins. In the West, BCS is a rare hepatic manifestation of one or more underlying prothrombotic risk factors. The most common underlying prothrombotic risk factor is a myeloproliferative disorder, although it is now recognized that almost half of patients have multiple underlying prothrombotic risk factors. Clinical manifestations can be diverse, making BCS a possible differential diagnosis of many acute and chronic liver diseases. The index of suspicion should be very low if there is a known underlying prothrombotic risk factor and new onset of liver disease. Doppler ultrasound is sufficient for confirming the diagnosis, although tomographic imaging (computed tomography (CT) or magnetic resonance imaging (MRI)) is often necessary for further treatment and discussion with a multidisciplinary team. Anticoagulation is the cornerstone of the treatment. Despite the use of anticoagulation, the majority of patients need additional (more invasive) treatment strategies. Algorithms consisting of local angioplasty, TIPS and liver transplantation have been proposed, with treatment choice dictated by a lack of response to a less-invasive treatment regimen. The application of these treatment strategies allows for a five-year survival rate of 90%. In the long term the disease course of BCS can sometimes be complicated by recurrence, progression of the underlying myeloproliferative disorder, or development of post-transplant lymphoma in transplant patients.

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“…Recent data show that maintenance or reestablishment of good shunt function is crucial for long-term survival. 103 Shunt dysfunction may be related to stenosis of intrahepatic IVC (which is amenable to stenting), 80,103,104 to shunt stenosis (amenable to percutaneous transluminal angioplasty and stenting), 78,80,105 and to shunt or portal vein thrombosis (amenable to in situ thrombolysis). 105 Risk factors for shunt dysfunction appear to be the use of long prosthetic grafts.…”

Section: Portosystemic Shuntingmentioning

confidence: 99%

Budd-Chiari Syndrome

2008

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Primary Budd-Chiari syndrome is related to thrombosis of hepatic veins or the terminal portion of the inferior vena cava. This rare disease is usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Half of the patients with primary Budd-Chiari syndrome are affected with a myeloproliferative disease, the recognition of which is largely based on the assessment of V617F Janus tyrosine kinase 2 (JAK2) mutation in peripheral granulocytes. A diagnosis of Budd-Chiari syndrome should be considered in any patient presenting with acute or chronic liver disease, as clinical manifestations are extremely diverse. Spontaneous outcome in symptomatic patients is poor. Diagnosis can be made in most patients noninvasively when imaging shows venous obstruction and/or collaterals. A treatment strategy is recommended where anticoagulation is given first, followed by angioplasty when appropriate, then TIPS in patients not responding to previous measure, and finally liver transplantation. This strategy has achieved 5-year survival rates close to 90%.

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Budd-Chiari syndrome due to prothrombotic disorder: mid-term patency and efficacy of endovascular stents

Cited by 33 publications

References 31 publications

Vascular disorders of the liver

Vascular disorders of the liver

Budd‐Chiari syndrome

Budd-Chiari Syndrome

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