BCS is defined as obstruction of hepatic venous outflow from venules to the entrance of the IVC. It can be primary (without external compression) or secondary. Epidemiology shows higher rates in Asia. Pathogenesis involves hepatic congestion from obstruction of two or more veins. Evaluation involves Doppler ultrasound, CT or MRI, and liver biopsy. Treatment involves anticoagulation long term with medical management or procedures like TIPS or transplant for severe cases. Prognosis depends on severity and treatment response.

2. BCS is defined as the obstruction of hepatic venous
outflow that can be located from small hepatic
venules up to the entrance of IVC into Right atrium.
Hepatic outflow obstruction related to cardiac
diseases, pericardial disease or sinusoidal
obstruction syndrome has been excluded from this
definition.(EASL 2015)
It can be divided into –
 Primary-Caused by thrombosis with absence of
outside compression, malignant invasion or parasitic
infection
 Secondary-

3. Epidemiology-
 Vascular disorder of liver-5/10,000(MC-Ischemic
hepatitis)
 BCS-1.4 -5.3 /million, may be higher in asian
population
 F>M ,in western higher ratio is there for female:male
 3rd -4th decade, median age-37 yrs
Pathogenesis-
Hepatic congestion
 Severity of congestion ,severity of its onset, duration
of congestion defines the course and presentation of
the disease.

4.  It has been seen that generally obstruction of two
veins are needed to present symptomatically
,although single vein rapid and complete blockage not
leading to formation of collaterals can rarely present
symptomatically.
 In acute and fulminant presentation ascites is there as
a consequence of rapid obstruction leading to leaking
from liver and hepatocyte necrosis cheifly starting
from centrilobular zones .
 In chronic presentation ascites is a sequale of portal
hypertension.

6. Etiology-

8.  In European countries,Myeloproliferative neoplasm account for
40-45% of all thrombophilias. So Evaluation with JAKV617F and
Calreticulin is needed ,even if blood count is normal, as 1st
manifestation of MPL neoplasm may be hepatic vein
thrombosis.(It has been proposed that there is some specific
interaction between abnormal RBCs of MPL neoplasm with
endothelium of hepatic veins.)
 Enlarged spleen with Platelet count 200gm/L is a specific
marker although not highly sensitive.
 Indian data also supports the presence of at least one
coagulation abnormality in about 85%, 2 coagulation
abnormality in about 45%.
 Data from 50 BCS from india –showed APLA (14%),idiopathic (15-
20%),MPL/Protein c/s/Antithrombin
/homocystenemia/malignant and local factors each acounted
for 3-6%(D amprapurkar et al) .

9.  Previous the pattern of venous outflow obstruction
predominant in india/Asia was assumed to be Membranous
obstruction of IVC , quite different from site from western
countries ie hepatic venous thrombosis.
 Recent study from India and other asian countries states same
pattern of obstruction as western countries, and has been
proposed that membranous obstruction previously thought to be
the mc common cause is the late manifestation of hepatic
vein/IVC thrombosis.
 Data from D Amrapurkar,published in WJG on consecutive data
from 49 BCS patients from india, showed in
 60%-Hepatic venous thrombosis(Group 2)
 20%-Hepatic and IVC thrombosis(Group1)
 16%-Isolated IVC thrombosis
 4%- Membranous obstruction of IVC

10. Asymptomati
c
Fulminant Acute Sabacute Chronic
5-20% 5% 20%
<1month
duration
Along with
chronic
accounts for
60%
</= 3months
duration
6 months
Asymptomatic
hepatomegaly
may need
evaluation
with doppler
USG
Presentation
as ALF a/w
Abdominal
pain,fever
AST/ALT >5
times
Jaundice
Hepatomegaly
Ascites(HIGH
SAAG HIGH
PROTEIN)
Fever
Abdominal pain
Insidious
course
Trace ascites
/jaundice/
settled
transimanse
Clinically
evident signs
of Portal
Hypertension
(HIGH SAAG
LOW PROTEIN
)

11.  In a multicentre prospective study of a large cohort of patients with
BCS at diagnosis, ascites were present in 83% of patients,
hepatomegaly in 67%, abdominal pain in 61%, esophageal varices in
58% and gastrointestinal bleeding in 5% . In approximately 15% of
cases, BCS and PVT occur simultaneously. Back vein prominence
shows obstruction at IVC level.

14.  USG doppler-(75% sensitive and specific)
 Caudate lobe hypertrophy
 HV/PV/IVC thrombosis
 Character of thrombus
 A large vein with absent, reversed or turbulent flow- Large intrahepatic or
subcapsular collaterals with continuous flow
 Spider web appearance located at the vicinity of the hepatic vein ostea
with absence of hepatic vein flow in that area
 Absent or flat hepatic vein wave form without fluttering
 A hyperechoic cord replacing a normal vein
 Triple phase CT
 Additional sensitivity and specificity
 Rule out Secondary causes.
 Nodular enhancement in Arterial phase ,get isointense in portal phase(Size is
mostly <3cm,>3 nodules),need to get differentiated from HCC.
 MRI-T1 hyperintense /T2 hypointense

15.  Venography is recommended if the diagnosis remains
uncertain or for the characterization of anatomy prior to
treatment.
 Liver biopsy-
 Sinusoidal dilation with congestion,fibrosis and liver cell loss in
absence of inflammation,predominating in the centrilobular
area.
 The observation of thrombosed central hepatic vein is rare and
non specific.

17. Rx:

19.  Medical management-
 Heparin(LMWH) (5-7 days)as a bridge therapy followed by
warfarin,to maintain INR2-3. In case of recurrent thrombosis
2.5-3.5.(pharmacological or endoscopic management of varices
is needed before starting anticoagulants)
 Before stopping LMWH, twice monitoring of INR between 2-3 is
needed.
 Life long anticoagulation has been favoured in most of studies
even if thrombophilia work up is negative.
 Familial screening for thrombophilia of the patient is yet not
approved.
 Management of concomitant disorders.
 Newer DAA have not been approved(All studies excluded CLD).
 As per largest European survey data of step up approach
management-about 25% had their disease controlled without
any intervention.

21. Some criteria have been proposed:
---- clinical failure to therapy (treatment failure) is considered when criteria
for complete or ongoing response were lacking . Complete response was
considered when all of the following 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 (spironolactone 75 mg/d or furosemide 40 mg/d) and moderate
NaCl intake.
(2) increase in coagulation factor V to a level above 40% of normal value.
(3) decrease in conjugated serum bilirubin to a level below 15 micromol/L.
(4) absence of first or recurrent portal hypertension related bleeding while on
primary or secondary prophylaxis with non-selective beta blockers or with
endoscopic therapy
(5) no occurrence of spontaneous bacterial infection; and
(6) BMI>20 kg/m2 after subtraction of ascites and edema.

22. Ongoing response –
was considered when all of the following three criteria were
met on a 2-weekly evaluation basis:
(1) in the presence of ascites, a negative sodium and water
balance was achieved using low dose diuretics and moderate
sodium intake, together with normal serum sodium and
creatinine levels, or with increasing serum sodium if initially
low and decreasing serum creatinine levels if initially high.
(2) factor V level was increasing if initially low.
(3) serum conjugated bilirubin level was decreasing if initially
high.

23.  Angioplasty with stenting is the first intervention modality to think off as a
stepup approach after medical therapy. This is particularly more feasible in
Indian population since short segmant stenosis (<4cm) are more prevalent in
comparison to western population. Around 5% could stop their disease
progression with it.
 Thrombolysis with rTPA has been done in small studies in Acute BCS, need
not be done in subacute or chronic BCS.
 TIPS-
 Success has been comparable to OLT.(80% survival around 5 yrs)
 Covered stent- Median patency has been seen more than 22 months. 20%
restenosis in one year.(In uncovered-around 4.4 months)
 In cases of complete thrombosis of hepatic veins, Transcaval approach is
taken (around 45%),ie direct puncture from intrahepatic part of IVC.
 35%of the patients had disease control by it.
 No mortality benefits have been found with surgical shunts in recently
conducted large scale trials. Although mesocaval shunts with PTFE stents
or autologus jugular venous interposition are preferred to side to side
shunts. Many studies claim that worse outcome in surgical shunts in BCS
is due to fact that,the patients profile going surgical shunts are much
worse.

25.  OLT:
 In 15%cases, patients had to go for OLT for survival.
 1yr survival was around 88% and 5yr was around 78%.
After all these post intervention ,patients have to
continue anticoagulants for preventing
BCS.(except a few thrombophilia that can be
corrected by liver transplantation.)

26.  Paucity of large studies due to being a rare disease.
 Hypercoaguable state of pregnancy must create a bigger risk along
with other causative factors .
 Prothrombin gene mutation presence has been associated with
worst outcomes.
 BCS patients ,who are in compensated state on treatment for
it,maternal outcome have been good.
 High incidence of CS in course of more placental abnormalities
 Females had been suffering from PH and increased ICP in different
studies.
 Fetal outcomes particularly reaching 20 weeks are better, even
though 76% are born preterm.

27.  General supportive care alone had a survival of about 10% at 3 yrs.
 ACLF presentation of BCS bears the worst prognosis.
 At a median duration of 5yrs,around 4% develops HCC. In different studies,2-12% risk
of developing HCC in BCS have been reported.
 Primary IVC obstruction has been associated with particularly high risk of HCC.
 Rotterdom criteria-It shows high prediction for intervention free periods.
 BCS TIPS score>7 predicts highest mortality and fulminant course, with the
emergency need for OLT. It also came out as better predictor of long term survival.
 New clinchy score,in the european studies correlated best with the survival in BCS
patients.