HVOTO (Hepatic Venous Outflow Tract Obstruction), also known as Budd-Chiari syndrome, is caused by obstruction of the hepatic veins or inferior vena cava. It has a variable presentation ranging from acute liver failure to chronic liver disease manifestations. Investigations include Doppler ultrasonography, CT/MRI scans, and hepatic venography. Treatment depends on the severity and includes anticoagulation, thrombolysis, TIPS, and liver transplantation in severe cases.

1. HVOTO (Hepatic Venous Outflow Tract Obstruction)
(Budd Chiari syndrome)
Dr Pukar Thapa
DM Resident (Hepatology)
October 9th, 2020

2. Sequence
1. Definition
2. Epidemiology
3. Classification
4. Pathophysiology
5. Etiology
6. Clinical manifestations
7. Investigations
8. Treatment

3. Suspecting HVOTO
Amar M, S K Sarin. Hepatology International 2018

4. Definition
• International expert panels have agreed that “Budd-Chiari syndrome” be used as an
eponym for “hepatic venous outflow tract obstruction” independent of the level or
mechanism of obstruction.
• The obstruction can be located anywhere from the small hepatic venules up to the
entrance of the inferior vena cava (IVC) into the right atrium.
Laurie D. AASLD PRACTICE GUIDELINES. HEPATOLOGY 2009
Not due to cardiac disease, pericardial disease, or sinusoidal
obstruction syndrome (veno-occlusive disease)

5. History
• Lambron in 1842. First case report.
• George Budd in 1845.
– British internist,
– described three cases of hepatic vein thrombosis due to abscess-induced phlebitis.
• Hans Chiari in 1899.
– Austrian pathologist
– added the first pathologic description of a liver with “obliterating endophlebitis of the hepatic veins”.

6. Epidemiology
• Wealthy countries have a lower incidence rate (0.13-0.8 cases per million) compared with
countries such as Nepal (2.5 per million inhabitants).
Duration Place Sample Male/female Incidence
Males/females
Age (median)
10 yrs (hospital
admissions)
Italy 287 132/155
(54% females)
2.0/2.2 per million 50 (36 – 68)
US/Netherlands
/France
237 67% females 35 (13 – 76)
The location of the outflow obstruction was in the hepatic veins (62%), inferior vena cava
(7%), or both (31%), and 34 patients (14%) had associated portal vein thrombosis.
Ageno W. Thromb Haemost 2017

7. Demographic profile ( EAST vs WEST)
FEATURES EAST WEST
OBSTRUCTION Inferior vena cava
(Hepatic vena cava BCS)
Hepatic veins
(Classical BCS)
SEX Males Females
AGE (PRESENTATION) Median age: 45 yrs 3rd or 4th decade
OBSTRUCTION OF IVC Membranous in Japan, Nepal
IVC thrombosis in India
Thrombosis
PRESENTATION Chronic (75-80%) Acute (60-85%)
ASSOCIATION Pregnancy and Infection Oral contraceptives
Myeloproliferative disorders
Plessier A. Semin Liver Dis 2008

8. Classification
• Primary
– when the blockage is intrinsic to the vein (i.e., thrombosis or phlebitis)
• Secondary
– when the obstruction or compression originates external to the vein (i.e., compression or invasion by a
tumor or a benign mass, such as an abscess or cyst).
Type I Type II Type III
IVC with or without
hepatic veins occlusion
Major hepatic veins Small centri-lobular veins

9. Categorization
(Disease duration and severity)
• Acute (fulminant) liver failure
– Characterized by acute liver injury with elevated transaminases, jaundice, hepatic encephalopathy, and an elevated
PT/INR.
• Acute
– Clinical manifestations develop rapidly (over the course of weeks), with intractable ascites and hepatic necrosis.
• Subacute
– Insidious onset, with patients taking up to three months to develop symptoms. Ascites and hepatic necrosis may be
minimal due to decompression of the sinusoids by portal and hepatic venous collaterals.
• Chronic
– Patients present with complications of cirrhosis.

10. Pathophysiology Hepatic Venous outflow
obstruction
Increased sinusoidal and
portal pressure
Extravasation of fluid
from sinusoids
Ascites
Venous stasis and
congestion
Hypoxic damage to
hepatocytes
Hepatocullular necrosis
(centrilobular)
Fibrosis/ Nodular
regenerative hyperplasia
Cirrhosis
Blockage of two or more major
hepatic veins increases the sinusoidal
pressure and reduces sinusoidal blood
flow.
Obstruction of a single hepatic vein is
generally not evident; two veins must
be blocked for clinical disease.

11. • Compensatory mechanisms to HVOTO take place rapidly and comprise:
–Re-distribution of portal perfusion towards the unobstructed areas
–Increased portal pressure
–Increased arterial inflow
–Development of the most efficient hepatic venous or caval collaterals.
Pathophysiology
The development of such extensive collaterals explains why BCS may be
asymptomatic in up to 20% of patients.

12. • Regions of the liver with a preserved blood supply undergo hypertrophy, as is commonly
the case for segment I (Spiegel lobe), for which the venous drainage is preserved because its drainage is
independent of the major hepatic veins.
• Areas deprived of portal venous inflow but with enhanced arterial inflow undergo
regenerative changes, which can be microscopic (regenerative foci or nodular regenerative hyperplasia)
or macroscopic (regenerative macronodules or focal nodular hyperplasia).
Pathophysiology

13. Etiology - Prevalence of Thrombotic Risk Factors for BCS
In up to 90% of patients, at least
one prothrombotic risk factor is
identified, and in up to 46% of
patients multiple concurrent risk
factors exist.

14. Etiology
10%
Idiopathic – 20%

15. Membranous webs
• Membranous obstruction (partial or complete) of the inferior vena cava (MOVC) and/or the hepatic
veins is an unusual but potentially treatable cause of the BCS and is much more common in South Africa,
India, and Asia.
• These web-like lesions, which usually are found just cephalad to the entrance of the right hepatic vein into
the inferior vena cava, may be the result of a congenital anomaly. However, they are more often attributable
to an acquired thrombotic process such as a myeloproliferative disease. (ORGANIZED THROMBUS).
• It is important to diagnose MOVC or so-called short-length hepatic vein stenoses because of the
availability of treatment options (eg, anticoagulation, angioplasty, or stenting) that are usually not germane
to other causes of the BCS.
Wang ZG. Asian J Surg 1996

16. Clinical manifestations
• HIGHLY VARIABLE (fever, abdominal pain, abdominal distension (from ascites), lower extremity
edema, jaundice, gastrointestinal bleeding (from varices or portal hypertensive gastropathy), and/or hepatic
encephalopathy.)
• Because the presentation of BCS is highly variable, clinicians should consider it in the differential
diagnosis of patients presenting with ALF, acute hepatitis, or CLD.
Asymptomatic BCS (20%) – Subacute or chronic BCS ( incidental finding)
Often associated with hepatic venous collaterals.
In IVC obstruction, patients may develop a subcutaneous collateral circulation across the abdomen,
chest, and back. Although this finding is uncommon, it is very specific for IVC block.

17. Clinical manifestations

18. • Severity of symptoms will depend on the extent of the thrombosis, the time-course over which the
obstruction develops, and the duration of untreated disease.
• Acute liver failure (5%): elevated transaminases, jaundice, hepatic encephalopathy, and an elevated
PT/INR.
• Acute BCS (20%): clinical manifestations develop rapidly (over the course of weeks), patients usually
present with severe right upper quadrant pain and hepatomegaly, jaundice and ascites may not be apparent
initially but often develop rapidly.
Clinical manifestations

19. • Subacute BCS: may be asymptomatic or minimally symptomatic until the disease progresses,
symptoms develop over the course of months, may report a history of vague discomfort in the mid
epigastrium or right upper quadrant.
• Ascites and hepatic necrosis may be minimal due to decompression of the sinusoids by portal and hepatic
venous collaterals.
• The hypertrophied caudate lobe can cause compression of the intrahepatic portion of the inferior vena
cava, leading to further outflow obstruction and portal hypertension.
• Venous collaterals may be seen on the anterior abdominal wall.
Clinical manifestations

20. • Chronic BCS: symptoms develop once the patient has cirrhosis (stigmata of chronic liver disease).
• Signs of portal hypertension such as ascites and esophageal varices.
Clinical manifestations

21. Benign regenerative nodules in BCS
• Up to 80% of BCS patients have benign regenerative nodules (result of heterogeneous perfusion).
• These are generally multiple, small, hyper-vascularized, and disseminated throughout the liver, and they
may increase in number and in size over time.

22. BCS and HCC
• BCS patients are also at risk of developing HCC. Guidelines for non-invasive diagnosis of HCC in
cirrhosis are not applicable to BCS patients.
• A French study showed that an α-fetoprotein (AFP) level greater than 15 ng/mL had a positive
predictive value of 100% and a negative predictive value of 90% for the diagnosis of HCC in BCS
patients.
• The same study suggested that biopsy should be considered in patients with three nodules or less,
nodules with a diameter of 3 cm or more, heterogeneity or washout on the venous phase, changes in two
consecutive imaging studies, or increase in AFP levels
Moucari R. Gut 2008

23. BCS and PVT
• Portal vein thrombosis (PVT) occurs in approximately 15% of BCS patients.
• These patients have a higher prevalence of multiple risks factors and their prognosis is worse.
• Therapeutic options are limited and prognosis tends to be worse in BCS-PVT patients with splenic and/or
superior mesenteric vein thrombosis compared with those without.

24. BCS and pregnancy
• Nearly 20 % of cases of the BCS occur in women who have been on oral contraceptives (for as little as
two weeks), are pregnant, or have delivered a child within the previous two months.
• Pregnancies reaching week 20 of gestation are associated with an acceptable fetal prognosis even when
preterm delivery can occur (76%).
• VKA are associated with a high risk of miscarriage and congenital malformations. Therefore, a pregnancy
test must be done as early as possible, if positive mothers should switch to LMWH with periodic
monitoring of anti-Xa activity.
• Liver transplantation has been used as a lifesaving measure.
Fickert P. Gastroenterology 1996

25. Investigations and Treatment
• Considering Budd Chiari syndrome
– Radiographic findings - Doppler Ultrasonography
– Cross sectional imaging-CT, MRI
– Venography
– Liver biopsy
• Treatment
– Anticoagulation
– Thrombolysis/Stenting
– TIPS/DIPS
– Surgical therapy
– Liver transplantation

26. Radiographic findings - Ultrasonography
• Nonspecific ultrasonographic findings include hepatomegaly, splenomegaly, ascites, intra-
abdominal collaterals, caudate lobe hypertrophy, atrophy of other hepatic lobes, and
compression or narrowing of the inferior vena cava.
• Findings that are more specific for hepatic venous outflow obstruction include:
 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.
 A hyperechoic cord replacing a normal vein.

27. Radiographic findings - Ultrasonography

28. Radiographic findings - Ultrasonography

29. Radiographic findings - Ultrasonography

30. • Abnormal flow patterns seen on Doppler imaging:
– A large hepatic vein that appears void of flow-signal or has reversed or turbulent flow.
– Large intrahepatic or subcapsular collaterals with continuous flow connecting the hepatic veins or the
diaphragmatic or intercostal veins.
– A hepatic wave form that is flat or lacks fluttering.
– Sensitivity (87%)
Radiographic findings - Doppler Ultrasonography

31. • Ultrasound images (A-D) and color Doppler images (E, F). A: Inferior vena cava web with intra-luminal floating
thrombus; B: Partial thrombus within the middle hepatic vein and osteal narrowing of the right hepatic vein; C:
Fibrosed right hepatic vein; D: Comma-shaped intrahepatic collaterals; E, F: Web at hepatic vein ostium and
intrahepatic collaterals.

32. • A: Normal tri-phasic waveform of patent hepatic vein.
• B: Monophasic waveform in hepatic venous occlusion.

33. Limitations of sonography
• Restriction from body habitus.
• Intestinal gas or excessive ascites.
• Failure to detect fresh thrombus in veins.
• Failure to demonstrate patent veins within congested or conversely, shrunken cirrhotic
liver.
• Failure to demonstrate retroperitoneal collaterals unless they are hugely dilated.
• Operator dependency.

34. Cross-sectional imaging
(CT Scan)
• Specific findings on CT scan that suggest BCS include:
• Delayed or absent filling of the three major hepatic veins (which are usually visible within 40 to 60
seconds after rapid intravenous contrast).
• A patchy, flea-bitten appearance of the liver due to increased central contrast enhancement
relative to the periphery.
• Rapid clearance of contrast from the caudate lobe.
• Narrowing and/or lack of opacification of the inferior vena cava.

35. BCS on CT Scan
CT scan through the liver of a 17-year-old male
with new onset abdominal distension shows a
mottled appearance to the underperfused liver with
collapsed portal veins, ascites (small arrows), and
extensive retroperitoneal varices (large arrow).
Note also the enlarged caudate lobe of the liver (large arrowhead)
and the collapsed small inferior vena cava (small arrowhead).

36. Gd-enhanced T1-weighted MR image. Hyperintense structures represent portal venules (arrows), which are visible because
of postsinusoidal portal hypertension.
Gadolinium-enhanced T1-weighted MR image shows enlarged caudate lobe that has pseudotumor appearance. Direct
venous drainage (arrow) of caudate lobe into inferior vena cava is evident.
MRI (Magnetic Resonance Imaging)

37. Venography
• Gold standard test - which is performed by accessing the hepatic venous circulation.
• Venography should be performed if noninvasive tests are negative or nondiagnostic, but there is strong
clinical suspicion for the disease. It can also be used to direct subsequent therapy by clearly defining which
vessels are involved.
Venography---Useful in directing therapy
• Noninvasive studies may not accurately define the extent or characteristics of the hepatic venous flow.
• In particular, compression or occlusion of the intrahepatic vena cava leads to sluggish flow in hepatic
veins. As a result, hepatic veins that are patent and amenable to therapy may be undetectable on Doppler
imaging.

38. Computed tomography angiography shows segmental occlusion of the IVC. Blood flow within the IVC reverses to the
IRHV and then continues through the CVs to the MHV and then to the RA.
IVC, inferior vena cava; HV, heptatic vein; IRHV, inferior right HV; CV, communicating vein; MHV, middle HV; RA, right
atrium.

40. Contemporary role of venography
• Platform for interventional radiology procedures.
• Tips placement.
• Catheter directed thrombolysis.
• Mechanical thrombectomy.
• Balloon angioplasty.
• Recanalization of an occluded hepatic vein or vena cava with stent placement.
• Transjugular liver biopsy.
• Essential guide and road map for surgical therapy in BCS.

41. Liver biopsy
• The thrombotic process in BCS may not involve all the hepatic veins. Thus, the distribution of the typical
pathologic findings may be focal or patchy. As a result, some patients require biopsy of both the right and the
left lobes of the liver. A laparoscopic approach may be better suited for this purpose.
• Indications
• There is confusion regarding the diagnosis (an uncommon situation given the multitude of available
imaging tests).
• 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.
Only method that establishes a diagnosis of BCS of the small
intrahepatic veins.

42. Liver biopsy findings
• Liver biopsy findings in acute BCS include centrilobular and sometimes midlobular sinusoidal congestion, acute
hemorrhage, and hepatocyte ischemia or drop out.
• Chronic outflow obstruction leads to hepatocyte dropout, bridging fibrosis between central veins with sparing of
the portal tracts, nodular regenerative hyperplasia, and ultimately cirrhosis.
This low-power photomicrograph
shows the centrizonal congestion,
hemorrhage, and hepatocyte
necrosis typical of the acute type
of BCS.
Masson trichrome stain.
Sinusoidal dilatation and congestion

43. Nutmeg liver
Gross appearance Microscopic appearance

44. Evaluating for predisposing conditions
• An underlying disorder can be identified in over 80 % of patients with BCS.
• Many of these disorders are characterized by a hypercoagulable state.
• More than one thrombotic risk factor is present in a quarter of patients.

45. Diagnosis of MPN
• Typical peripheral blood changes may be masked by plasma volume expansion, hypersplenism, and iron
deficiency anemia related to portal hypertension.
• Genetic testing for the JAK2 V617F mutation.
• Greater than 90% of patients with PV and approximately 50% of patients with ET and IMF carry the
JAK2 V617F mutation.
• Recently, somatic calreticulin mutations have been described in up to 67% and 88% of the JAK2 V617F–
negative ET and IMF, respectively.
Nangalia J. N Engl J Med 2013

46. Biochemistry in acute BCS
• Elevation of serum aminotransferases occurs because vascular congestion results in ischemic hepatocellular
damage. (Can range from 100 to 200 IU/L to more than 600 IU/L.)
• Serum bilirubin levels are usually less than 7 mg/dL at the time of presentation, though they may
subsequently increase.
• Ascitic fluid in patients with BCS has a high serum-to-ascites protein gradient (>1.1), reflecting elevated
portal pressures.
• The protein concentration is variable (ranging from 1.5 to 4.9 g/dL) and is directly related to the serum
protein and inversely related to portal pressure.

47. Biochemistry in subacute/chronic BCS
• Patients with subacute or chronic BCS may present with normal or mild to moderate elevations of serum
aminotransferases, alkaline phosphatase, and serum bilirubin.
• They may also have hypoalbuminemia.
• As in patients with acute BCS, ascitic fluid in patients with subacute or chronic BCS has a high serum-to-
ascites protein gradient (>1.1).

48. Management – Treatment aims
• Prevent the propagation of the clot
– Anticoagulation
• Restore patency of thrombosed veins
– Thrombolysis
– Angioplasty/stenting
• Decompress the congested liver
– Transjugular intrahepatic portosystemic shunt (TIPS) placement
– Surgical shunting
• Prevent or manage complications
– Treatment of portal hypertension
– Liver transplantation

49. Drawback of BCS management
• The current drawback of BCS management is the lack of well-defined criteria for “treatment
failure” to step-up in treatment.
• Previously reported criteria for treatment failure were the occurrence of any of the following that cannot
be explained by other cause after at least 2 weeks of adequate medical treatment:
– Patients with ascites requiring paracentesis,
– development of spontaneous bacterial peritonitis,
– progressive renal failure,
– an episode of variceal bleeding or of hepatic encephalopathy
– further deterioration in liver function and/or persistent transaminases greater than 300 IU/mL.

50. Prevent propagation of the clot (Anticoagulation)
• Anticoagulation should be initiated immediately in most patients to prevent propagation of the clot,
provided there are no contraindications.
• However, the risk of anticoagulation should be considered, especially in patients who present with
bleeding complications or who have varices.
• LMWH is preferred initially. (Target anti Xa activity to 0.5-0.8 IU/ml). Bridging with Warfarin is done with
INR between 2-3.
• Anticoagulation is continued indefinitely unless a major contraindication is present, a complication occurs,
or the obstruction is due to an anatomic cause that has been corrected.
AASLD 2009 and EASL 2015

51. • Thrombolytic therapy is an option for treating patients with a clot that is known to be recent (less than three
to four weeks old) and is well-defined on venography, provided there are no contraindications to the use of
thrombolytic agents.
Restore patency of thrombosed veins
(Thrombolytic therapy)
Contraindication to
thrombolytics
Extensive clot involving the intrahepatic vena cava and hepatic veins (because of technical difficultly
performing thrombolysis in such patients) or who have a clot of unknown age.
Should not be used in the treatment of the chronic form of BCS. Clots in such patients have matured and are
unlikely to be recanalized by thrombolytic agents. Furthermore, because such patients may have developed
portal hypertension, treatment can be associated with disastrous bleeding complications.

52. Angioplasty and stenting
• Radiologically-guided treatment, including angioplasty and stenting, can be used to treat patients with
acute or subacute BCS who are symptomatic, provided a venous obstruction amenable to percutaneous
angioplasty and stenting is visualized radiologically (eg, on magnetic resonance venography or
percutaneous venography).
• Angioplasty with or without stenting should be considered in patients with short-length stenosis.
• These stenoses are present in 60% of patients with IVC obstruction, and 25% to 30% of those with HV
obstruction.
• Angioplasty can be combined with thrombolytic therapy in patients in whom there is recent
thrombosis of a single hepatic vein.
DRAWBACKS
Re-occlusion of the affected vessel is common.
Once inserted, the stent cannot be removed.

53. Decompress the liver - TIPS
• The rationale behind TIPS placement in BCS is to decompress congested segments in the liver by creating
an alternative venous outflow tract.
• However, TIPS placement is not technically feasible in many patients, may only drain a small portion of
the liver, presence of thrombosis and is associated with a high rates of shunt occlusion.
(polytetrafluoroethylene-covered stents have a reduced incidence of TIPS dysfunction).
• TIPS placement is reserved for patients who have failed treatment with anticoagulation,
thrombolysis, and/or angioplasty (with or without stenting). - RESCUE THERAPY
• TIPS may have a role as a temporizing measure to treat complications of portal hypertension (eg, variceal
bleeding) prior to liver transplantation in patients with acute liver failure.
Seijo S. Hepatology 2013
• Overall survival
• (Greater than 88% and 72% at 1 and 5 years, respectively)
• LT-free survival
• (Greater than 85% and 72% at 1 and 5 years, respectively)

54. • DIPS is a modification of the TIPS procedure, using intravascular ultrasound-guidance, combined with
fluoroscopy.
• The DIPS procedure involves intravascular ultrasound-guided puncture from the inferior vena cava to the
portal vein through the caudate lobe of the liver. The shunt is completed with a polytetrafluoroethylene-
covered stent graft.
• When there is complete thrombosis (45% of the cases) of the hepatic veins, instead of TIPS it may be
necessary to do DIPS.
DIPS (Direct Intrahepatic Portocaval Shunt)

55. Surgical therapy
• The goal of surgical therapy for BCS is to restore hepatic venous drainage and thereby decompress the
liver. This is usually done by the creation of a surgical shunt.
• Surgical decompression is unlikely to be beneficial in patients who have cirrhosis or biochemical
evidence of advanced liver dysfunction (elevated prothrombin time and serum bilirubin level,
decreased serum albumin). Such patients are best managed with liver transplantation.
• Side-to-side portacaval, splenorenal, and mesocaval shunts all are feasible only if the inferior vena
cava is patent and without a significant pressure gradient between its infrahepatic and suprahepatic
portions.
• It has been suggested that the infrahepatic pressure in the inferior vena cava should be at least 10 mmHg
lower than the portal pressure.

56. • Synthetic shunts can be constructed from the portal-mesenteric system to the right atrium (meso-atrial
shunt), which can bypass the inferior vena cava if it is occluded or there is a significant pressure gradient.
• However, shunts that require artificial graft material are more likely to be complicated by thrombosis.
Orloff MJ. Ann Surg 2000
Surgical shunts
• Five-year survival following shunt surgery depends on the extent of liver damage prior to surgery and
the continued patency of the shunt.
• Five-year survival rates as high as 90 percent have been reported in patients who underwent shunt
surgery prior to the development of cirrhosis, and whose shunts remained patent.

57. Liver transplantation
• Liver transplantation may be the only option for patients with BCS who are not candidates for or fail other
treatments, or patients who have decompensated cirrhosis., or those with fulminat liver failure. (AASLD
2009)
• Patients who developed BCS as a result of protein S, protein C, or antithrombin III deficiency may also be
cured of their clotting tendency by liver transplantation since the transplanted liver produces normal
amounts of these enzymes.
• Anticoagulation needs to be continued in most BCS patients after LT. (EASL 2015)
• Survival after liver transplantation
• Survival following LT depends on the underlying cause of the BCS and the patient's condition at the time
of the transplantation.
• One of the largest series (510 patients) compared survival in patients with BCS who underwent liver
transplantation after adoption of the MELD score with the pre-MELD era. Three-year graft survival (81
versus 65 percent) and patient survival (85 versus 73 percent) were significantly better following
adoption of the MELD score.
Segev DL. Liver Transpl 2007

58. Manage complications
Complications of cirrhosis and portal hypertension
• Ascites
• Hepatic encephalopathy
• Variceal hemorrhage
• Spontaneous bacterial peritonitis
• Hepatorenal syndrome
• Portal hypertensive gastropathy
• Hepatic hydrothorax
• Hepatopulmonary syndrome
• Portopulmonary hypertension
• Cirrhotic cardiomyopathy

59. BCS-specific prognostic index

60. Follow up and monitoring
• Standard laboratory studies (liver transaminases, bilirubin, albumin, INR, CBC) every three months.
• Serial UGIE (looking for varices) and noninvasive methods for detecting hepatic fibrosis (eg, ultrasound-
based transient elastography annually, decreasing the interval to every two to three years if laboratory,
endoscopic, and histologic findings are stable.
• Patients receiving long-term anticoagulation need close follow-up and monitoring of coagulation status,
especially during invasive procedures. (AASLD 2009)
• Patients with compensated cirrhosis due to BCS should be monitored for the development of
complications from portal hypertension. In addition, patients with cirrhosis should be monitored for the
development of HCC.

61. Algorithm for the management of BCS/HVOTO
Amar M, S K Sarin. Hepatology International 2018