Bud chiari syndrome full presentation .pptx

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1. BUDD-CHIARI SYNDROME 9/22/2024 6:30 PM 1

2. OUTLINE INTRODUCTION  PATHOLOGY CLASSIFICATION CLINICAL MANIFESTATION DIAGNOSIS MEDICAL THERAPY INTERVENTIONAL THERAPY – SURGICAL AND  NON SURGICAL 9/22/2024 6:30 PM 2

3. INTRODUCTION Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction, independent of the level or mechanism of obstruction, provided the obstruction is not due to cardiac disease, pericardial disease, or sinusoidal obstruction syndrome ( veno -occlusive disease) BCS is due to occlusion or partial occlusion of one, two, or all three of the major hepatic veins (right, middle, and left) and/or occlusion or partial occlusion of the inferior vein cava Alternate nomenclature is " obliterative hepatocavopathy " Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Okuda K, Kage M, Shrestha SM   Hepatology . 1998;28(5):1191 .  9/22/2024 6:30 PM 3

4. Imperative to distinguish BCS from sinusoidal obstruction syndrome (hepatic  veno -occlusive disease) “Classical BCS” were first described by Budd in 1845 and later by Hans Chiari in 1899 Primary BCS -  due to thrombosis or a primary disease of the venous wall Secondary BCS – due to invasion of hepatic veins/IVC by malignancy (HCC) or other causes  9/22/2024 6:30 PM 4

5. PATHOGENESIS - ETIOLOGY An underlying disorder can be identified in over 80 percent of patients with the Budd-Chiari syndrome. More than one thrombotic risk factor is present in many patients.  Etiology , management, and outcome of the Budd-Chiari syndrome.  Murad et al. Ann Intern Med. 2009;151(3):167.  Many of these disorders are characterized by a hypercoagulable state, which is important to consider when treating Budd-Chiari syndrome  The combination of a myeloproliferative disorder (occult or overt) associated with a hypercoagulable state (or states) is the most common cause of BCS . Polycythemia Vera alone accounts for 10-40 % of these cases.  9/22/2024 6:30 PM 5

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7. ETIOLOGY JAK2 tyrosine kinase (V617F) mutations  have been described in 26 to 59 percent of patients with Budd-Chiari syndrome. This mutation is  present in almost all patients with polycythemia  vera . Nearly  20 percent of cases  of the Budd-Chiari syndrome occur in women who have been on  oral contraceptives for as little as 2 weeks to maximum of 10 years The factor V Leiden mutation , the most frequent cause of hereditary thrombophilia, may be particularly important- often associated with most severe forms of BCS Etiology , management, and outcome of the Budd-Chiari syndrome.  Murad et al. Ann Intern Med. 2009;151(3):167.   9/22/2024 6:30 PM 7

8. MOVC Membranous obstruction (partial or complete) of the inferior vena cava (MOVC) and/or the hepatic veins is an unusual but more common in South Africa, India, and Asia.  Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa. Kew et al. Liver Int 2006 Feb;26(1):1-7 Found just cephalad to the entrance of the right hepatic vein into the inferior vena cava, may be the result of a congenital anomaly MOVC has also been associated with the subsequent development of hepatocellular carcinoma Most common cause of BCS in India, Japan and china 9/22/2024 6:30 PM 8

9. PATHOPHYSIOLOGY Obstruction of the hepatic venous outflow tract results in increased hepatic sinusoidal pressure and portal hypertension – fluid extravasation - ascites The ensuing venous stasis and congestion lead to hypoxic damage to adjacent hepatic parenchymal cells These mechanisms culminate in the development of hepatocyte necrosis in the centrilobular regions, with progressive centrilobular fibrosis, nodular regenerative hyperplasia, and ultimately, cirrhosis of the liver Centrilobular necrosis denotes irreversible step in pathogenesis Blumgart  6th edition  9/22/2024 6:30 PM 9

10. PATHOPHYSIOLOGY Acute BCS -Portal vein becomes draining vein -Increased hepatic arterial flow Chronic BCS -Necrotic area replaced by fibrotic tissue -Intrahepatic collateral formation  -Heterogenous enhancement with peripheral reticular pattern  9/22/2024 6:30 PM 10

11. Caudate lobe hypertrophy – 50% Concomitant Portal vein obstruction 10-20% Isolated IVC web – 10% Hepatic vein obstruction –66% (obstruction in 2 veins for clinically significant disease) Shin N, Kim YH, Xu H, Shi HB, Zhang QQ, Colon Pons JP, Kim D, Xu Y, Wu FY, Han S, Lee BB, Li LS. Redefining Budd-Chiari syndrome: A systematic review. World J Hepatol 2016; 8(16): 691-702 [PMID: 27326316 DOI: 10.4254/wjh.v8.i16.691 ] 9/22/2024 6:30 PM 11

12. EPIDEMIOLOGY 0.2 - 2 per million in general population  7-9% of all portal hypertension patients in india   Non asian countries -  more common in women ; Thrombotic causes more common            -Site of obstruction – Pure Hepatic Vein  Asian countries - There is a slight predominance of men, with a median age of 45 years at          presentation         -Pure inferior vena cava or combined inferior vena cava and hepatic vein           blockage predominate Budd-Chiari syndrome.  Plessier A, Valla DC.  Semin Liver Dis. 2008 Aug;28(3):259-69 9/22/2024 6:30 PM 12

13. CLASSIFICATION Based on site of obstruction  Based on duration of symptoms Based on precipitating factor in acute on chronic BCS 9/22/2024 6:30 PM 13

14. Bansal V, Gupta P, Sinha S, Dhaka N, Kalra N,  Vijayvergiya  R, et al. Budd-Chiari syndrome: imaging review. Br J  Radiol  2018; 91: 20180441. 9/22/2024 6:30 PM 14

15. Duration of symptoms Budd-Chiari syndrome.   Ferral H, Behrens G, Lopera AJR Am J Roentgenol . 2012 Oct;199(4):737-45.  9/22/2024 6:30 PM 15

16. TYPE 1 Acute forms 7% BEST PROGNOSIS TYPE 2 Chronic forms; sequelae of remote injury 45% TYPE 3 Acute on Chronic forms 45 WORST PROGNOSIS 9/22/2024 6:30 PM 16

17. Acute on Chronic BCS Shukla A, Shreshtha A, Mukund A, Bihari C, Eapen CE, Han G, Deshmukh H, et al. Budd-Chiari syndrome: consensus guidance of the Asian Pacific Association for the study of the liver (APASL). Hepatol Int. 2021;15(3):531-567. 9/22/2024 6:30 PM 17

18. CLINICAL FEATURES SYMPTOMS    Abdominal pain Distension  Anorexia  Jaundice  SIGNS Ascites (almost every patient at some point) Hepatomegaly  Collaterals – Esophageal varices (20%)  Splenomegaly  Pedal edema Shin N et al. BCS review World J Hepatol 2016 9/22/2024 6:30 PM 18

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20. APASL DIAGNOSTIC SCORE 9/22/2024 6:30 PM 20

21. USG - Doppler First Investigation of choice  Sensitivity and specificity >85% Findings : Inability to visualize the junction of the major hepatic veins with the inferior vena cava Thickening, irregularity, stenosis, or dilation of the walls of the hepatic veins A spider-web appearance in the vicinity of the hepatic vein ostia coupled with the absence of a normal hepatic vein Caudate hypertrophy ( mean AP dia >35mm) Doppler  large hepatic vein that appears void of flow-signal or has reversed or turbulent flow (MOST DIAGNOSTIC) Large intrahepatic or subcapsular collaterals  hepatic wave form that is flat or lacks fluttering Bansal V, Gupta P, Sinha S, Dhaka N, Kalra N,  Vijayvergiya  R, et al. Budd-Chiari syndrome: imaging review. Br J  Radiol  2018; 91: 20180441. 9/22/2024 6:30 PM 21

22. CECT It delineates the vascular abnormality, evaluates the hepatic morphological changes as well as allows mapping of the vascular anatomy prior to endovascular interventions or surgery Acute Phase   Liver - Patchy, decreased peripheral enhancement, size and surface normal  Lack of contrast enhancement in hepatic veins at both venous and delayed phases Compression of the intrahepatic IVC Ascites 9/22/2024 6:30 PM 22

23. Subacute phase   Heterogeneous enhancement of the peripheral liver in the early contrast enhanced phase with homogeneous attenuation in delayed phases Homogeneous enhancement and enlarged caudate lobe Collateral vessel development is frequently seen with intrahepatic shunting   Chronic phase  Regenerative nodules are multiple, ranging in size from 0.5 to 4 cm Nodules are homogeneous; Show marked arterial phase enhancement. Enhancement persists in portovenous phase The diameter of the hepatic artery is enlarged compared to the splenic artery Multiple comma shaped collaterals  9/22/2024 6:30 PM 23

24. MRI MR venography is preferred over CT venography for the initial evaluation of patients suspected to have BCS- Second line of investigation Helps in delineating liver nodules which are sequelae of BCS Acute Phase:  T1W-decreased signal intensity within liver periphery and preservation of more normal liver T2W-heterogeneously increased signal intensity in the peripheral portion of the liver and caudate lobe Abnormal appearance of hepatic veins on T2W images 9/22/2024 6:30 PM 24

25. Subacute phase   Post-contrast enhancement of caudate lobe is less prominent than the heterogeneously increased enhancement observed in the peripheral liver. Overall enhancement pattern becomes more homogeneous on more delayed imaging sequence.   Chronic phase  Changes of fibrosis and chronic liver parenchymal disease.  Collaterals more prominently seen Regenerative nodules: T1 bright T2 isointense to hypointense Post-contrast – hypervascular in arterial phase, no washout in the venous phase 9/22/2024 6:30 PM 25

26. ANGIOGRAPHY AND VENOGRAPHY CONVENTIONAL ANGIOGRAPHY Gold standard Pressure profile Anatomy of block IVC VENOGRAPHY  Gradient >15mm Hg  - Mesoatrial shunt HEPATIC VENOGRAPHY/SMA VENOGRAPGY Occluded / narrow hepatic veins Spider web pattern of venous collaterals Wedge hepatic venous- IVC gradient >10mm Hg  Budd-Chiari syndrome.   Ferral H, Behrens G, Lopera AJR Am J Roentgenol . 2012 Oct;199(4):737-45.  9/22/2024 6:30 PM 26

27. IVC Compression RHV Thrombosed Spider web appearance 9/22/2024 6:30 PM 27

28. LIVER BIOPSY Liver biopsy is rarely required In selected patients with a subacute presentation, when the presence of cirrhosis is not apparent by noninvasive studies, and the finding of significant fibrosis/cirrhosis or severe centrizonal congestion would indicate that the patient could benefit from transjugular intrahepatic or surgical portosystemic shunting. Distribution of the typical pathologic findings may be focal or patchy Liver biopsies are often obtained via the transjugular route during performance of hepatic venography since the majority of patients have ascites and will require anticoagulation 9/22/2024 6:30 PM 28

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30. MANAGEMENT Factors 9/22/2024 6:30 PM 30

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32. MEDICAL THERAPY BCS is associated with a prothrombotic state in up to 80% in the west and hence requires life-long anticoagulation. The aim of anticoagulation therapy is to avoid the extension of thrombosis Around 10–20% of patients respond to anticoagulation without any need for additional therapy Trial of medical therapy with low molecular weight heparin and general symptomatic measures may be given for 2 weeks to 2 months to patients. (EASL guidelines) VKA are preferred anticoagulants for long-term anticoagulation   (EASL guidelines) VALDIG trial – Usefulness of Direct Xa Inhibitors in BCS. Still not approved as standard of care  Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger M-H, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology. 2006;44:1308–1316. 9/22/2024 6:30 PM 32

33. Management of ascites          – Sodium restriction (<2gm/day)     – Diuretic therapy     – Therapeutic paracentesis Complete response is considered  six criteria were met and stable:   (1) absence of clinically detectable ascites, with normal serum sodium and creatinine levels, in the absence of diuretic therapy, or on low-dose diuretics   (2) increase in coagulation factor V to a level above 40% of normal value  (3) decrease in conjugated serum bilirubin to a level below 15mol/L   (4) absence of first or recurrent portal hypertension–related bleeding on primary or secondary prophylaxis with nonselective betablockers  or with endoscopic therapy;   (5) absence of spontaneous bacterial infection  (6) BMI20 kg/m2 after substraction of ascites and edema 9/22/2024 6:30 PM 33

34. Local Thrombolytic therapy Catheter directed Delivered just proximal or within thrombus Overall success rate low Risk of bleeding Useful in patients with short history of thrombosis (acute disease) Sharma S, Texeira A, Texeira P, Elias E, Wilde J, Olliff SP. Pharmacological thrombolysis in Budd Chiari syndrome: a single centre experience and review of the literature. J Hepatol. 2004 Jan;40(1):172-80. doi : 10.1016/j.jhep.2003.09.028. PMID: 14672630. 9/22/2024 6:30 PM 34

35. Transluminal angioplasty Recanalization with balloon angioplasty is recommended as the preferable invasive intervention and is particularly effective for symptomatic BCS with membranous or segmental obstruction Favorable long-term outcomes after recanalization have been reported in large series from Asia 5-year survival rate after recanalization alone is 88.6% Frequent restenosis of recanalized veins in up to 50% patients at first year. Stenting advised along with angioplasty Zhang F, Wang C, Li Y. The outcomes of interventional treatment for Budd-Chiari syndrome: systematic review and meta-analysis. Abdom Imaging. 2015 Mar;40(3):601-8. doi : 10.1007/s00261-014-0240-8. PMID: 25248791. 9/22/2024 6:30 PM 35

36. Stenting in all patients ? 9/22/2024 6:30 PM 36

37. APASL guidelines Angioplasty is the first-line interventional treatment for patients with BCS Step-wise approach with initial balloon dilatation followed by stents in cases with residual stenosis or significant pressure gradient across stenosis Balloon angioplasty alone has high recanalization success rates for membranous type of obstruction For long-segment IVC or HV stenosis or occlusion, balloon dilatation with primary stenting is preferred Uncovered metallic stents are used for HV and IVC stenting. SEMS of size 20-25 mm for IVC and 10-15mm for HV Long-term oral anticoagulants (warfarin) to maintain INR 2-3 Periodic Doppler ultrasound imaging at 1,3,6 months and thereafter 6-monthly follow-up is recommended for all patients TIPS placement is done if endovascular attempts to reopen the occluded segment fail. 9/22/2024 6:30 PM 37

38. TIPS TIPS the next step for patients not responding to medical therapy, with failed or unsuitable angioplasty/stenting of venous outflow stenosis Early TIPS only in patients with signs of portal hypertension; Otherwise follow stepup approach CECT is most valuable preop due to its accuracy and ability to perform multiplanar reconstructions, often required when planning complex interventions TIPS is absolutely contraindicated in patients with right heart failure and severe pulmonary hypertension DIPS – Technical modification. PV accessed from IVC through caudate lobe 9/22/2024 6:30 PM 38

39. Technical aspects After accessing, IVC is ideally punctured at the level of hepatic veins and is usually within 6 cm of its insertion into the right atrium Accessing the portal vein is the most difficult part of the procedure In case there is co-existing narrowing or occlusion of the inferior vena cava near or above the intended entry puncture site, the IVC would need to be stented before PTFE or PET covered stents are used to prevent intimal hyperplasia The size of the TIPS shunt is directly proportional to the degree of portal decompression Most series achieved a post-TIPS portosystemic gradient of 12 mm Hg using 10 mm diameter stents, with low risk of hepatic decompensation or encephalopathy 9/22/2024 6:30 PM 39

40. 9/22/2024 6:30 PM 40 Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol . 2011 Jul 28;3(7):169-77. doi : 10.4329/wjr.v3.i7.169. PMID: 21860712; PMCID: PMC3158894.

41. Indications Bridge to transplant in fulminant Acute form BCS   Sub acute form BCS  with Porta- caval pressure gradient >10mmHg Menon et al, NEJM 2004 Complications of TIPS  Procedure related mortality – 1-2% Worsening of encephalopathy- 13-44% Shunt dysfunction (Portal pressure gradient > 12mm Hg, decreased luminal shunt diameter) –  18-78% Mostly in 1 st year Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol . 2011 Jul 28;3(7):169-77. doi : 10.4329/wjr.v3.i7.169. PMID: 21860712; PMCID: PMC3158894. 9/22/2024 6:30 PM 41

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43. SURGICAL SHUNTS Pre-requisite – Reversible liver injury Indications – Technically difficult TIPS (massive thrombosis) – Porta- caval pressure gradient > 10 mm Hg 9/22/2024 6:30 PM 43

44. Decision Making in Shunt Surgery Documentation of ongoing necrosis Demonstration of intact lobular architecture Status of infrahepatic IVC Status of PV - 20% have associated PVT Status of Caudate lobe DAMAGED LIVER WITH NO   LOBULAR COLLAPSE :   SHUNT SX SEVERELY DAMAGED LIVER WITH LOBULAR COLLAPSE AND FIBROSIS : LT 9/22/2024 6:30 PM 44

45. Shunts in Normal IVC Side to side portacaval Shunt (SSPCS) Mesocaval shunt (MCS) Splenocaval shunt (SCS) Proximal splenorenal shunt (PSRS) Distal splenorenal shunt (DSRS) 9/22/2024 6:30 PM 45

46. Shunts in Thrombosed IVC – Meso-atrial shunts ( MAS ) – Cavo-atrial shunt with SSPCS – IVC stenting before PCS/ MCS – Cavocaval transflow – Splenoatrial shunt – Splenojugular shunt – Mesojugular shunt 9/22/2024 6:30 PM 46

47. SSPCS Described by Prof. Marshall Orloff Valveless PV can act as an outflow tract for sinusoidal decompression Shunt patency depends on the pressure gradient between the high pressure portal system &  the low pressure IVC  Best patency rates   Shunt blockage rate is -- 3% at 13 years Difficult in presence of caudate hypertrophy 9/22/2024 6:30 PM 47

48. Problems with PCS Portal vein thrombosis is an obvious contraindication Dissection at porta- subsequent technical difficulty at transplant Fulminant liver failure and decompensated cirrhosis- Liver transplant 9/22/2024 6:30 PM 48

49. 1. Long-term Shunt Patency In 97-100% 2. Hepatic Sinusoidal Decompression Was Maintained 3. No Ascites Or Need For Diuretics 4. Liver Function And Size  Returned To Normal 5. Variceal Bleeding Decreased 6.Encephalopathy Did Not Occur When Shunt Was Patent 7.10 Year Survival Was ≥ 91% 9/22/2024 6:30 PM 49

50. BLEEDING BEST MANAGED WITH PRESSURE DECOMPRESS PORTAL SYSTEM AS FAST AS POSSIBLE RESECTION OF THE ENLARGED CAUDATE LOBE IS HAZARDOUS PRESSURE GRADIENT AT THE END OF SURGERY OF > 50 MM OF SALINE IS UNACCEPTABLE AND  REVISION MAY BE REQUIRED 9/22/2024 6:30 PM 50

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52. 9/22/2024 6:30 PM 52 75 patients (Between 1978 and 1992). HV OBSTRUCTION IN 24, IVC OBSTRUCTION in 44, BOTH in 7. HV OCCLUSION ( 24) 7 PATIENTS : PSRS 2 deaths postoperatively); 4 shunts blocked and only 1 patient became completely symptom free. 2 patients ( partial obstruction): balloon dilatation of the right HV but within 6 months the obstruction recurred. 6 patients : SSPCS 2 died of HE after discharge and 4 are alive and well. IVC OCCLUSION (44) 14 pts : Surgical procedures yielding poor results. 30 patients : Balloon angioplasty : successful in 28 ; restenosis occurred in 4. Of the 7 patients with a combined block, 3 have had balloon angioplasty followed by a SSPCS; 1 died, 2 are well, and the remainder have not completed treatment. Patients with HVOO should be actively managed with a side-to-side portocaval shunt for hepatic vein obstruction, balloon angioplasty for inferior vena caval obstruction, and perhaps both procedures for those with combined obstructions.

53. Mesoatrial Shunt Introduced by Prof.Cameron et al in 1978 Synthetic Dacron or Gore-Tex grafts ranging from 14 to 20 mm in diameter Using autologous internal jugular vein IN MCS ; Patency equal to PCS Easier to manage during future LT Technically easier in caudate hypertrophy In case with infrahepatic IVC obstruction       Higher thrombosis rate : 33% at 5 years Cameron JL, Maddrey WC. Mesoatrial shunt: a new treatment for the Budd–Chiari syndrome. Ann Surg. 1978;187:402–06 9/22/2024 6:30 PM 53

54. AUTHOR CASES F/U ( MONTHS SURVIVAL GRAFT PATENCY % STRINGER (1989) 5 9-16 100 100% WANG ( CHINA – 89) 32 2-66 88 ? HENDERNESS - 90 9 1-47 67 33 KLEIN - 90 16 2-80 37 50 ORLOFF 98 8 12-120 37 37 KHANNA (PGI CHANDIGARH – 1992) 13 2- 7.5 YEARS 62% BEHERA (PGI CHANDIGARH -2001) 10 6-71 MONTHS 90% 100% 8 cm external silicone rubber sleeve around a 16 mm ring-reinforced Gore-Tex prosthesis to prevent compression of the graft by the sternum.  9/22/2024 6:30 PM 54 61

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56. Combined PCS and MAS Orloff and Colleagues 1992 9/22/2024 6:30 PM 56

57. Shunt Results Success rates of portal decompression in BCS  after direct SSPCS : 85% to 97%   ;           Hemming et al, 1996 ; ; Orloff et al, 1992 67% success rate after splenorenal shunt            Ahn et al, 1987 ; McCarthy et al, 1985 Mesocaval or portacaval interposition grafts with autologous internal jugular vein : success rate of 89%                          Bismuth & Sherlock, 1991 ; Panis et al, 1994   Mesocaval or portocaval interposition grafts using synthetic materials, such as Dacron or PTFE (Gore-Tex ), have been successful in approximately 52% of 39 patients with BCS.                Hemming et al, 1996 ; Henderson et al, 1990   9/22/2024 6:30 PM 57

58. Non Shunt Surgical options Transcardiac membranotomy : More than 125 cases have been described with success rate if 70-90% after brief follow up ( 2-84 months) Splenopneumopexy - Portopulmonary shunt ( induce collateral circulation between the portal system and the pulmonary veins) - It involves performing a parenchymatous anastomosis between the amputated superior pole of the spleen and the exposed pulmonary venous structures in the left lower lobe Foley’s tube dilatation Endovenotomy : Radical membrane excision-  12 attempts, 5 success, 7 failures, 3 deaths Kawashima and colleagues 1997 9/22/2024 6:30 PM 58

59. Senning Operation Direct method of removing obstruction of the IVC and HV in patients with chronic BCS.  Reconstructing the hepatic outflow tract by suturing the RA to the liver capsule 17 patients: 6 deaths within 2 weeks of operation & 4 later. Actuarial 1 year and 3 year survival rates were 76% and 57%, respectively;  During follow-up of 7 months to 11 years, 10 (67%) of the 15 early survivors had prolonged relief of BCS.                                                                                                                                     Senning 1987 9/22/2024 6:30 PM 59

60. LIVER TRANSPLANTATION First Successful Liver transplant for BCS – 1974 by Putnam et al (Starzl group- Denver) It is useful in far advanced, decompensated liver disease, when liver dysfunction has progressed beyond a salvageable state by other portal decompression procedures More Than 1000 Patients With BCS Have Undergone Liver Transplantation Among them 30 Patients With BCS Underwent LDLT; All Were From Asian Countries Akamatsu N, Sugawara Y, Kokudo N. Budd-Chiari syndrome and liver transplantation. Intractable & Rare Diseases Research. 2015;4(1):24-32. doi:10.5582/irdr.2014.01031 9/22/2024 6:30 PM 60

61. Indications Fulminant Liver failure -  rare  Chronic and progressive liver disease ( Reasonable prediction that the patient will die within 1 year—the most common indication for  LT and the same used widely in other liver diseases ) Decompensated cirrhosis Decompensation after shunt procedures Shunt failure Unshuntable portal hypertension Curative in protein C, S, antithrombin III deficiency and factor V leiden mutation Blumgart 6th Ed 9/22/2024 6:30 PM 61

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63. 3 time periods : historical (1987–1997), pre-MELD (1998–2002) and MELD (2002–2006 ),   100 patient from 2002-2006 (MELD ERA) Better graft & patient survival Independent factors for Graft loss & death  - Life support -Prior transplantation -Prolonged ischemia time – 3 years survival – 85% 9/22/2024 6:30 PM 63

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65. Issues with LT Allocation problem Shortage of donors Long wait times Unpredictable availability of donors Inaccurate MELD score- INR while on warfarin Technical  Haemostasis Large caudate lobe- difficult mobilization Diffuse retroperitoneal fibrotic reaction – difficult IVC control Thick, narrow, thrombosed portal vein 9/22/2024 6:30 PM 65

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69. Post LT - Management Lifelong  anticoagulation  - Initially heparin followed by warfarin Heparin 7500-30,000 U/day followed by warfarin In Myeloproloferative diseases – Hydroxyurea and aspirin to be continued  Rebleed rate – 11-44% Mentha et al J.of Hepatology 2006 Recurrence – 13-27% May require Interventional procedure / re transplant Usually in early postop period (25% in 1st week)  Substantial incidence of posttransplant thrombosis in other splanchnic blood vessels , particularly in the PV (range, 9%-40%) 9/22/2024 6:30 PM 69

70. BCS following LT with the piggyback technique is a well-described complication Technical factors for recurrence :  inadequate graft size and use of two hepatic veins for the venous anastomosis The rate of BCS after construction of the venous anastomosis with two veins is 2.3% while the use of three veins decreases the rate to 0.7% Conventional piggy back technique vs the side-to-side variation of the piggyback technique : 2.4% vs. 0.7% respectively  incidence of BCS Horton et al Liver International ISSN 1478-3223 , 2007 9/22/2024 6:30 PM 70

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73. Prognosis in BCS Prognosis depends upon Age Severity of liver failure Presence of refractory ascites Serum creatinine Good prognosis Younger patients with low CTP score Absent or easily controlled ascites Low serum creatinine Valla DC et al, Semin Liv Dis 2002 9/22/2024 6:30 PM 73

74. Langlet Murad 9/22/2024 6:30 PM 74

75. Which is the best surgery for Budd- Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. Ringe B , Lang H , Klinik fur Abdominal- und Transplantations, Hennover , Germany.; Published in Hepatology - 1995 50 patients treated between 1981 and 1993 was retrospectively analyzed . 12 pts : PSS or local decompressive procedures, 43 cases(38+5 ) : LT , including 5 with previous conventional surgery The overall mortality of 18 of 50 was concentrated within the early postoperative period, with no patient lost after 1 year . In the venous decompression group, the success rate was only 29%, and treatment failure was closely related to the finding of cirrhosis or technical problems like vascular thrombosis . After LT, early complications were rejection, primary non function, or graft necrosis, and contributed significantly to the risk of sepsis. 30 of 43 liver recipients are currently alive, including 4 rescued after failed decompressive surgery, with 1- and 10-year survival of 69% , and excellent recurrence-free rehabilitation. Conclusion: Patient selection plays a dominant prognostic role in the treatment of BCS. 9/22/2024 6:30 PM 75

76. CONCLUSIONS - SURGICAL 9/22/2024 6:30 PM 76

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78. APASL TREATMENT ALGORITHM 9/22/2024 6:30 PM 78

79. TAKE HOME Difference in Management of BCS based on site of block and anatomy of block Medical Management first step in asymptomatic patients  Shunt, TIPS and liver transplant - Complimentary modalities Shunts first line for non cirrhotic liver HV disease – TIPS,DIPS  replacing Surgical Shunts Early decompression is the key  Liver transplantation is indicated for rapidly progressive (Acute fulminant) BCS and in failure of conventional treatment for chronic BCS Outflow reconstruction is critical from the technical aspects of view in liver transplantation for BCS cases 9/22/2024 6:30 PM 79