Budd-Chiari syndrome medical therapy: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Budd-Chiari syndrome}} | {{Budd-Chiari syndrome}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}}{{Mazia}} | ||
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==Overview== | ==Overview== | ||
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==Medical Therapy== | ==Medical Therapy== | ||
*The therapy for Budd-Chiari syndrome is aimed at alleviating the [[obstruction]]. Underlying conditions are aggressively treated.<ref name="pmid24923240">{{cite journal |vauthors=Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B |title=Diagnosis and management of Budd Chiari syndrome: an update |journal=Cardiovasc Intervent Radiol |volume=38 |issue=1 |pages=1–12 |year=2015 |pmid=24923240 |doi=10.1007/s00270-014-0919-9 |url=}}</ref><ref name="pmid23742773">{{cite journal |vauthors=Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y |title=Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins |journal=HBPD INT |volume=12 |issue=3 |pages=278–85 |year=2013 |pmid=23742773 |doi= |url=}}</ref> | *The therapy for Budd-Chiari syndrome is aimed at alleviating the [[obstruction]]. Underlying conditions are aggressively treated.<ref name="pmid24923240">{{cite journal |vauthors=Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B |title=Diagnosis and management of Budd Chiari syndrome: an update |journal=Cardiovasc Intervent Radiol |volume=38 |issue=1 |pages=1–12 |year=2015 |pmid=24923240 |doi=10.1007/s00270-014-0919-9 |url=}}</ref><ref name="pmid23742773">{{cite journal |vauthors=Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y |title=Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins |journal=HBPD INT |volume=12 |issue=3 |pages=278–85 |year=2013 |pmid=23742773 |doi= |url=}}</ref> | ||
*Patients with Budd-Chiari syndrome are treated according to the severity of the [[disease]].Treatment options include: | *Patients with Budd-Chiari syndrome are treated according to the severity of the [[disease]]. Treatment options include: | ||
**Initial | **Initial medical therapy | ||
**Endovascular procedure to restore vessel patency include | **[[Endovascular surgery|Endovascular procedure]] to restore [[vessel]] patency include: | ||
***[[Angioplasty]] | ***[[Angioplasty]] | ||
***[[Stenting]] | ***[[Stenting]] | ||
***Local [[thrombolysis]] | ***Local [[thrombolysis]] | ||
**[[Transjugular intrahepatic portosystemic shunts|Transjugular intrahepatic portosystemic shunt]] (TIPS) | **[[Transjugular intrahepatic portosystemic shunts|Transjugular intrahepatic portosystemic shunt]] ([[Transjugular intrahepatic portosystemic shunt|TIPS]]) | ||
**[[Liver transplantation]] | **[[Liver transplantation]] | ||
===Medical | ===Medical therapy=== | ||
*Medical therapy can be used for short-term [[Symptomatic|symptomatic relief]]. However, the use of such medical therapy alone is associated with a high [[Mortality rate|2-year mortality rate]]. | *Medical therapy can be used for short-term [[Symptomatic|symptomatic relief]]. However, the use of such medical therapy alone is associated with a high [[Mortality rate|2-year mortality rate]]. | ||
*A low-sodium diet is recommended for [[patients]] of Budd-Chiari syndrome. This helps in effective control of [[ascites]]. | *A [[Low sodium diet|low-sodium diet]] is recommended for [[patients]] of Budd-Chiari syndrome. This helps in effective control of [[ascites]]. | ||
*[[Symptomatic treatment]] includes [[diuretics]] to control [[ascites]], [[anticoagulants]] such as [[heparin]] and [[warfarin]] for [[Hypercoagulable states|hypercoagulable state]] and [[Antifibrinolytic Agent|antifibrinolytic agents]]. | *[[Symptomatic treatment]] includes [[diuretics]] to control [[ascites]], [[anticoagulants]] such as [[heparin]] and [[warfarin]] for [[Hypercoagulable states|hypercoagulable state]] and [[Antifibrinolytic Agent|antifibrinolytic agents]]. | ||
===Anticoagulation=== | ===Anticoagulation=== | ||
*[[Anticoagulation]] is recommended in all patients of BCS to prevent progression of the [[thrombosis]]. | *[[Anticoagulation]] is recommended in all patients of BCS to [[Prevention|prevent]] progression of the [[thrombosis]]. | ||
*[[Anticoagulation]] with [[Low molecular weight heparin|low molecular weight heparin LMWH]] should be initiated without delay soon after [[diagnosis]]. The risk of associated [[bleeding]] [[complications]] is comparable to [[patients]] with [[anticoagulation therapy]] for other indications. | *[[Anticoagulation]] with [[Low molecular weight heparin|low molecular weight heparin LMWH]] should be initiated without delay soon after [[diagnosis]]. The risk of associated [[bleeding]] [[complications]] is comparable to [[patients]] with [[anticoagulation therapy]] for other indications. | ||
*[[Anticoagulation]] is maintained with a target value of Anti-Xa between 0.5 and 0.8 IU/ml. | *[[Anticoagulation]] is maintained with a target value of Anti-[[Factor Xa|Xa]] between 0.5 and 0.8 IU/ml. | ||
*The goal is to maintain [[INR]] between 2.5 and 3 monitored by regular [[INR]] testing. | *The goal is to maintain [[INR]] between 2.5 and 3 monitored by regular [[INR]] testing. | ||
*[[Prothrombin time (PT)|Prothrombin time]] and [[activated partial thromboplastin time]] are measured once [[anticoagulation]] is started and should be maintained within the [[therapeutic range]]. | *[[Prothrombin time (PT)|Prothrombin time]] and [[activated partial thromboplastin time]] are measured once [[anticoagulation]] is started and should be maintained within the [[therapeutic range]]. | ||
*Before switching from [[LMWH]] to [[Anticoagulants|oral anticoagulants]], all [[contraindications]] | *Before switching from [[low molecular weight heparin]] ([[LMWH]]) to [[Anticoagulants|oral anticoagulants]], all [[contraindications]] have to be ruled out and a complete [[diagnostic]] workup has to be completed. | ||
===Thrombolysis=== | ===Thrombolysis=== | ||
*[[Thrombolytic|Thrombolytic agents]] include [[streptokinase]], [[urokinase]], [[Rt-PA|recombinant tissue-type plasminogen activator (rt-PA)]]. | *[[Thrombolytic|Thrombolytic agents]] include [[streptokinase]], [[urokinase]], [[Rt-PA|recombinant tissue-type plasminogen activator (rt-PA)]]. | ||
*[[Thrombolysis|Local thrombolysis]] performed by an [[interventional radiologist]] is preferable over [[Thrombolysis|systemic thrombolysis]]. | *[[Thrombolysis|Local thrombolysis]] performed by an [[interventional radiologist]] is preferable over [[Thrombolysis|systemic thrombolysis]]. | ||
*[[Systemic]] or intra-arterial [[thrombolysis]] in BCS has to be administered locally into the [[hepatic vein]], [[inferior vena cava]], and [[Transjugular intrahepatic portosystemic shunt|TIPS]] in case of [[Thrombosis|acute thrombosis]]. | *[[Systemic]] or intra-[[arterial]] [[thrombolysis]] in BCS has to be administered locally into the [[hepatic vein]], [[inferior vena cava]], and [[Transjugular intrahepatic portosystemic shunt|TIPS]] in case of [[Thrombosis|acute thrombosis]]. | ||
==References== | ==References== | ||
Latest revision as of 19:04, 27 December 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]
Overview
Medical therapy can be used for short-term symptomatic relief in Budd-Chiari syndrome. However, the use of such medical therapy alone is associated with a high 2-year mortality rate. A low-sodium diet is recommended for patients with Budd-Chiari syndrome. This helps in effective control of ascites. Symptomatic treatment includes diuretics to control ascites, anticoagulants such as heparin and warfarin for hypercoagulable state and antifibrinolytic agents.
Medical Therapy
- The therapy for Budd-Chiari syndrome is aimed at alleviating the obstruction. Underlying conditions are aggressively treated.[1][2]
- Patients with Budd-Chiari syndrome are treated according to the severity of the disease. Treatment options include:
- Initial medical therapy
- Endovascular procedure to restore vessel patency include:
- Angioplasty
- Stenting
- Local thrombolysis
- Transjugular intrahepatic portosystemic shunt (TIPS)
- Liver transplantation
Medical therapy
- Medical therapy can be used for short-term symptomatic relief. However, the use of such medical therapy alone is associated with a high 2-year mortality rate.
- A low-sodium diet is recommended for patients of Budd-Chiari syndrome. This helps in effective control of ascites.
- Symptomatic treatment includes diuretics to control ascites, anticoagulants such as heparin and warfarin for hypercoagulable state and antifibrinolytic agents.
Anticoagulation
- Anticoagulation is recommended in all patients of BCS to prevent progression of the thrombosis.
- Anticoagulation with low molecular weight heparin LMWH should be initiated without delay soon after diagnosis. The risk of associated bleeding complications is comparable to patients with anticoagulation therapy for other indications.
- Anticoagulation is maintained with a target value of Anti-Xa between 0.5 and 0.8 IU/ml.
- The goal is to maintain INR between 2.5 and 3 monitored by regular INR testing.
- Prothrombin time and activated partial thromboplastin time are measured once anticoagulation is started and should be maintained within the therapeutic range.
- Before switching from low molecular weight heparin (LMWH) to oral anticoagulants, all contraindications have to be ruled out and a complete diagnostic workup has to be completed.
Thrombolysis
- Thrombolytic agents include streptokinase, urokinase, recombinant tissue-type plasminogen activator (rt-PA).
- Local thrombolysis performed by an interventional radiologist is preferable over systemic thrombolysis.
- Systemic or intra-arterial thrombolysis in BCS has to be administered locally into the hepatic vein, inferior vena cava, and TIPS in case of acute thrombosis.
References
- ↑ Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B (2015). "Diagnosis and management of Budd Chiari syndrome: an update". Cardiovasc Intervent Radiol. 38 (1): 1–12. doi:10.1007/s00270-014-0919-9. PMID 24923240.
- ↑ Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y (2013). "Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins". HBPD INT. 12 (3): 278–85. PMID 23742773.