Budd-Chiari Syndrome Medication
- Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD more...
Medications commonly used in patients with Budd-Chiari syndrome include diuretics, anticoagulants, and thrombolytics. The therapeutic interventions used (medical or otherwise) must be tailored to each patient's condition. The use of thrombolytics should be reserved for experts familiar with the special circumstances in which they may be appropriate. The use of anticoagulants should be directed towards therapy of an underlying coagulopathy.
These agents prevent recurrent or ongoing thromboembolic occlusion.
Warfarin interferes with hepatic synthesis of vitamin K–dependent coagulation factors. It is used for the prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders. Tailor the dose to maintain an international normalized ratio (INR) in the range of 2-3.
Fibrinolytic drugs are used to dissolve a pathologic intraluminal thrombus or embolus that has not been dissolved by the endogenous fibrinolytic system. They are also used for the prevention of recurrent thrombus formation and for the rapid restoration of hemodynamic disturbances.
Urokinase is a direct plasminogen activator that acts on the endogenous fibrinolytic system and converts plasminogen to the enzyme plasmin, which, in turn, degrades fibrin clots, fibrinogen, and other plasma proteins. It is most often used for local fibrinolysis of thrombosed catheters and superficial vessels. An advantage to this agent is that it is nonantigenic. However, urokinase is more expensive than streptokinase, limiting its use. When it is used for local fibrinolysis, urokinase is given as a local infusion directly into the area of the thrombus, with no bolus given. The dose should be adjusted to achieve clot lysis or patency of the affected vessel.
Alteplase is a tissue plasminogen activator used in the management of acute myocardial infarction, acute ischemic stroke, and pulmonary embolism. Its safety and efficacy with concomitant administration of heparin or aspirin during the first 24 hours after symptom onset have not been investigated.
Diuretics can be useful to reduce the amount of ascites, providing symptomatic relief and reducing the need for paracentesis.
Spironolactone is a potassium-sparing diuretic. It competes with aldosterone for receptor sites in the distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.
This agent is often preferred because of its potassium-sparing effects, particularly in a clinical setting that includes secondary hyperaldosteronism.
Furosemide increases the excretion of water by interfering with the chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and the distal renal tubule.
Torsemide increases excretion of water by interfering with the chloride-binding co-transport system, which, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule. It increases excretion of water, sodium, chloride, magnesium, and calcium. If a switch is made from intravenous to oral administration, an equivalent oral dose should be used. Doses vary depending on the patient's clinical condition.
A thiazide diuretic, chlorothiazide inhibits sodium-chloride symport, blocking sodium reabsorption in the distal convoluted tubule.
Hydrochlorothiazide acts on the distal nephron to impair sodium reabsorption, enhancing sodium excretion. It has been in use for more than 40 years and is generally an important agent for the treatment of essential hypertension.
Khuroo MS, Al-Suhabani H, Al-Sebayel M, et al. Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt. J Gastroenterol Hepatol. 2005 Oct. 20(10):1494-502. [Medline].
Montano-Loza AJ, Tandon P, Kneteman N, Bailey R, Bain VG. Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival. Aliment Pharmacol Ther. 2009 Nov 15. 30(10):1060-9. [Medline].
Valla DC. Primary Budd-Chiari syndrome. J Hepatol. 2009 Jan. 50(1):195-203. [Medline].
Segev DL, Nguyen GC, Locke JE, et al. Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl. 2007 Sep. 13(9):1285-94. [Medline].
Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007 May 21. 13(19):2693-6. [Medline].
Darwish Murad S, Plessier A, Hernandez-Guerra M,et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009 Aug 4. 151(3):167-75. [Medline].
Chaudhuri M, Jayaranganath M, Chandra VS. Percutaneous recanalization of an occluded hepatic vein in a difficult subset of pediatric Budd-Chiari syndrome. Pediatr Cardiol. 2012 Jun. 33(5):806-10. [Medline].
Paradis V, Bieche I, Dargere D, et al. Quantitative gene expression in Budd-Chiari syndrome: a molecular approach to the pathogenesis of the disease. Gut. 2005 Dec. 54(12):1776-81. [Medline]. [Full Text].
Tian ZL, Jia GL, Xi HL, Feng S, Wang XK, Li R. Investigation on etiology of hepatic venous obstruction Budd-Chiari syndrome. Cell Biochem Biophys. 2014 Dec. 70(3):1803-6. [Medline].
Rautou PE, Angermayr B, Garcia-Pagan JC, et al. Pregnancy in women with known and treated Budd-Chiari syndrome: maternal and fetal outcomes. J Hepatol. 2009 Jul. 51(1):47-54. [Medline].
Moucari R, Rautou PE, Cazals-Hatem D, et al. Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. Gut. 2008 Jun. 57(6):828-35. [Medline].
Rajani R, Melin T, Bjornsson E, et al. Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience. Liver Int. 2009 Feb. 29(2):253-9. [Medline].
Ki M, Choi HY, Kim KA, Kim BH, Jang ES, Jeong SH. Incidence, prevalence, and complications of Budd-Chiari syndrome in South Korea: a nationwide, population-based study. Liver Int. 2015 Nov 12. [Medline].
Qi X, Ren W, Wang Y, Guo X, Fan D. Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies. Expert Rev Gastroenterol Hepatol. 2015 Jun. 9 (6):865-75. [Medline].
Liu FY, Wang MQ, Duan F, Fan QS, Song P, Wang Y. Hepatocellular carcinoma associated with Budd-Chiari syndrome: imaging features and transcatheter arterial chemoembolization. BMC Gastroenterol. 2013 Jun 24. 13:105. [Medline]. [Full Text].
Goel RM, Johnston EL, Patel KV, Wong T. Budd-Chiari syndrome: investigation, treatment and outcomes. Postgrad Med J. 2015 Dec. 91 (1082):692-7. [Medline].
[Guideline] Lee WM, Larson AM, Stravitz T. AASLD position paper: the management of acute liver failure: update 2011. National Guideline Clearinghouse. September 2011. Available at http://guideline.gov/content.aspx?id=36894&search=liver+failure. Accessed: Nov 3, 2014.
Hwang HJ, Kim KW, Jeong WK, et al. Hepatic outflow obstruction at middle hepatic vein tributaries or inferior right hepatic veins after living donor liver transplantation with modified right lobe graft: comparison of CT and Doppler ultrasound. AJR Am J Roentgenol. 2009 Sep. 193(3):745-51. [Medline].
Song HP, Yu M, Zhang M, et al. Diagnosis of active hemorrhage from the liver with contrast-enhanced ultrasonography after percutaneous transhepatic angioplasty and stent placement for Budd-Chiari syndrome. J Ultrasound Med. 2009 Jul. 28(7):955-8. [Medline].
Virmani V, Khandelwal N, Kang M, Gulati M, Chawla Y. MDCT venography in the evaluation of inferior vena cava in Budd-Chiari syndrome. Indian J Gastroenterol. 2009 Jan-Feb. 28(1):17-23. [Medline].
Cheng D, Xu H, Hua R, et al. Comparative study of MRI manifestations of acute and chronic Budd-Chiari syndrome. Abdom Imaging. 2015 Jan. 40(1):76-84. [Medline].
Garcia-Pagan JC, Heydtmann M, Raffa S, et al. TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology. 2008 Sep. 135(3):808-15. [Medline].
Hasija RP, Nagral A, Marar S, Bavdekar AR. Transjugular intrahepatic portosystemic shunt (TIPSS) for Budd Chiari syndrome. Indian Pediatr. 2010 Jun. 47(6):527-8. [Medline].
Fitsiori K, Tsitskari M, Kelekis A, Filippiadis D, Triantafyllou K, Brountzos E. Transjugular intrahepatic portosystemic shunt for the treatment of Budd-Chiari syndrome patients: results from a single center. Cardiovasc Intervent Radiol. 2014 Jun. 37(3):691-7. [Medline].
Van Brusselen D, Janssen CE, Scott C, et al. Budd-Chiari syndrome as presenting symptom of hepatic sarcoidosis in a child, with recurrence after liver transplantation. Pediatr Transplant. 2012 Mar. 16(2):E58-62. [Medline].
Tang TJ, Batts KP, de Groen PC, et al. The prognostic value of histology in the assessment of patients with Budd-Chiari syndrome. J Hepatol. 2001 Sep. 35(3):338-43. [Medline].
Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y. Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins. Hepatobiliary Pancreat Dis Int. 2013 Jun. 12(3):278-85. [Medline].
Horton JD, San Miguel FL, Ortiz JA. Budd-Chiari syndrome: illustrated review of current management. Liver Int. 2008 Apr. 28(4):455-66. [Medline].
Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B. Diagnosis and management of Budd Chiari syndrome: an update. Cardiovasc Intervent Radiol. 2015 Feb. 38 (1):1-12. [Medline].
Mancuso A. An update on the management of Budd-Chiari syndrome: the issues of timing and choice of treatment. Eur J Gastroenterol Hepatol. 2015 Mar. 27 (3):200-3. [Medline].
Hermeziu B, Franchi-Abella S, Plessier A, et al. Budd-Chiari syndrome and essential thrombocythemia in a child: favorable outcome after transjugular intrahepatic portosystemic shunt. J Pediatr Gastroenterol Nutr. 2008 Mar. 46(3):334-7. [Medline].
Murad SD, Luong TK, Pattynama PM, et al. Long-term outcome of a covered vs. uncovered transjugular intrahepatic portosystemic shunt in Budd-Chiari syndrome. Liver Int. 2008 Feb. 28(2):249-56. [Medline].
Panagiotou I, Kelekis DA, Karatza C, et al. Treatment of Budd-Chiari syndrome by transjugular intrahepatic portosystemic shunt. Hepatogastroenterology. 2007 Sep. 54(78):1813-6. [Medline].
Wu X, Ding W, Cao J, Han J, Li J. Modified transjugular intrahepatic portosystemic shunt in the treatment of Budd-Chiari syndrome. Int J Clin Pract. 2010 Mar. 64(4):460-4. [Medline].
Ulrich F, Pratschke J, Neumann U, et al. Eighteen years of liver transplantation experience in patients with advanced Budd-Chiari syndrome. Liver Transpl. 2008 Feb. 14(2):144-50. [Medline].
Oldakowska-Jedynak U, Ziarkiewicz M, Ziarkiewicz-Wroblewska B, et al. Myeloproliferative neoplasms and recurrent thrombotic events in patients undergoing liver transplantation for Budd-Chiari syndrome: a single-center experience. Ann Transplant. 2014 Nov 14. 19:591-7. [Medline].
Cura M, Haskal Z, Lopera J. Diagnostic and interventional radiology for Budd-Chiari syndrome. Radiographics. 2009 May-Jun. 29(3):669-81. [Medline].
Ren W, Qi X, Yang Z, Han G, Fan D. Prevalence and risk factors of hepatocellular carcinoma in Budd-Chiari syndrome: a systematic review. Eur J Gastroenterol Hepatol. 2013 Jul. 25(7):830-41. [Medline].
Li T, Zhai S, Pang Z, et al. Feasibility and midterm outcomes of percutaneous transhepatic balloon angioplasty for symptomatic Budd-Chiari syndrome secondary to hepatic venous obstruction. J Vasc Surg. 2009 Nov. 50(5):1079-84. [Medline].