2. DEFINITION
⢠Portal hypertension is present when
portal venous pressure exceeds 10 mm
Hg.
⢠If pressure >12 mmHg chances of variceal
bleeding
⢠Pressure >15mmHg brisk bleeding
⢠Pressure > 20mmHg continuous
bleeding
3. ⢠The portal venous system contributes approximately
75% of the blood and 72% of the oxygen supplied to
the liver. The portal vein is formed by the confluence of
the superior mesenteric vein and the splenic vein.
⢠In the average adult 1000 to 1500 mL/min of portal
venous blood is supplied to the liver. However, this
amount can be significantly increased in the cirrhotic
patient.
⢠The portal venous system is without valves and drains
blood from the spleen, pancreas, gallbladder, and
abdominal portion of the alimentary tract into the liver.
4. ANATOMY
⢠The portal vein arises from the postduodenal
plexus of the embryonic vitelline
veins.Rarely,the preduodenal plexus persists.
giving rise to a portal vein that lies anterior to
the duodenum.
5. ANATOMY
⢠The portal vein trunk is 4.8 to 8.8 cm long with
an average length of 6.4 cm. and 0.6 to1.2 cm
wide with an average width of 0.9 cm.
⢠It is formed behind the neck of the
pancreas,at the level of the second lumbar
vertebra, by the confluence of the superior
mesenteric vein (SMV) and the splenic vein.
7. ANATOMY
⢠The portal vein then passes to the right and cephalad
behind the first portion of the duodenum and into the
hepatoduodenal ligament, which forms the ventral
boundary of the foramen of Winslow.
⢠Within the hepatoduodenal ligament,the portal win
lies posterior to the hepatic artery and common bile
duct, usually slightly to the left of the duct.
⢠It bifurcates in the portahepatis , the right branch,
which supplies the right hepatic lobe, is shorter (0.5 to
1.0 cm Iong).
8. ANATOMY
⢠The more constant and longer left portal vein
with a 4cm average length, supplies the left
hepatic lobe and runs left in the hiiar plate as
the pars transversa until it enters the fissure
for the ligamentum venosum.
9. PORTOSYSTEMIC ANASTOMOSIS
⢠It is important to know the points of
communication between the portal and
systemic (caval, azygous, and hemiazygous)
venous systems
⢠Most important portosystemic anastomoses
are the veins of the proximal stomach and
distal esophagus,which receive flow from the
coronary and short gastric veins and drain into
the superior venacava via the azygous system.
10. ⢠âThe submucosal venous plexus in the rectum
between the superior hemorrhoidal veins (the portal
system) and the middle and inferior hemorrhoidal
veins (the caval system)
⢠The paraumbilical veins, connect the left portal vein
to the epigastric venous network âThis plexus can
become the variceal "caput Medusae¡ in patients
with portal hypertension and produce the
Cruveilhier-Baumgarten syndrome.
⢠Retzius veins: a group of small but numerous
retroperitoneal veins that connect abdominal
viscera. both tubular and solid. with the caval system
via intercostals, phrenic, lumbar and renal veins
12. ⢠Normal portal venous pressure is 5 to 10 mmHg,
and at this pressure very little blood is shunted
from the portal venous system into the systemic
circulation. As portal venous pressure increases,
however, the communications with the systemic
circulation dilate, and a large amount of blood
may be shunted around the liver and into the
systemic circulation. NO is believed to be an
important mediator of this venous dilatation.
13. Overriew of the entire portal venous
system within the abdominal cavity
14. ⢠Definition of the cause is important as
prognosis depends on the underlying liver
disease, and a full evaluation to allow
development of a treatment plan for variceal
bleeding, ascites, or end-stage liver disease is
paramount at initial presentation.
16. ⢠Cirrhosis covers a broad spectrum of disease by
etiology and severity.
⢠Alcoholic liver disease and hepatitis are
⢠the most common etiologies
⢠Others
ďź cholestatic liver diseases of primary biliary cirrhosis
(PBC)
ďź primary sclerosing cholangitis (PSC)
ďź the metabolic liver diseases such as hemochromatosis,
Wilsonâs disease, and 1-antitrypsin deficiency
contributing a small percentage.
17. PATHOPHYSIOLOGY
The sequence of events is as follows:
ď Block to portal flow leads to increased portal
pressure.
ď Splanchnic vascular bed response.
(a) Initial increased vasoconstrictor and decreased
vasodilator response increases intrahepatic
resistance.
(b) Secondarily, the vasodilator response
dominates, with increase in splanchnic inflow.
18. ď Collaterals develop between the portal
circulation and the systemic circulation.
ďPlasma volume expansion occurs with the
development of a systemic hyperdynamic
circulation.
The vascular response leads to increased flow in
the gut and thus major clinical manifestations of
collaterals in multiple locations: at the umbilicus,
in the retroperitoneum,hemorrhoidal, and at the
gastroesophageal junction.
19. EVALUATION
These are the essential components, and
should be done at initial presentation:
⢠Assessment of the liver disease
⢠Assessment of the portal circulation
⢠Upper gastrointestinal (GI) endoscopy
20. ⢠In the United States and Europe,
approximately 90% of patients with portal
hypertension have cirrhosis, with a small
percentage having portal vein thrombosis
(PVT)or hepatic Fibrosis.
⢠Schistosomiasis characterized by fibrosis of
the terminal portal venules, and in the
absence of concurrent hepatitis, these
patients have normal liver function.
21. COMPLICATIONS
Major complications of portal hypertension are
as follows:
⢠Variceal bleeding
⢠Ascites
⢠End-stage liver disease
⢠Hepatopulmonary syndromes
22. Assessment of the liver disease
⢠Liver evaluation is based on clinical findings and
laboratory studies.
Clinical
⢠Jaundice, ascites, encephalopathy, and malnutrition
define a patient with end-stage liver disease. Some
patients with variceal bleeding do not show these
clinical signs and have well-preserved liver function.
Laboratory tests
⢠add objectivity, the most useful indicators being serum
bilirubin,albumin, prothrombin time, and creatinine.
23. ASSESSMENT
⢠Specific Serologic Markers - Viral Hepatitis,
⢠Antimitochondrial Antibody â Primary Biliary
Cirrohosis
⢠Iron Studies - Heamocromatosis
⢠1-antitrypsin â Cirrhosis Of Liver
⢠Ceruloplasmin Levels â Wilsonâs Disease
⢠Alpha-fetoprotein (AFP) should be measured in
all such patients as a screening test for
hepatoma.
25. Quantitative liver testing with studies such as
⢠Indocyanine green clearance
⢠Galactose elimination capacity
⢠Monoethylglycinexylidide (MEGX)
26. ⢠Ultrasound to show overall liver morphology to
pick up focal lesions suggestive of hepatoma.
⢠Contrast-enhanced computed tomography (CT)
scan or magnetic resonance imaging (MRI) alone
or in combination with CT, MRI angiography for
morphologic assessment.
⢠Liver biopsy may be required to confirm
cirrhosis, and in focal lesions, to differentiate
hepatocellular carcinoma from regenerative
nodules.
27. Doppler ultrasound.
⢠Doppler ultrasound can assess the hepatic artery,
portal and hepatic veins(vascular anatomy)
⢠Documenting size, directional flow, velocities, and
wave-form patterns of the portal and hepatic veins is a
standard procedure.
⢠Tributaries to the portal veinâthe SMV and splenic
veins, and large collaterals such as the coronary and
umbilical vein may also be readily defined.
⢠most important information that the clinician needs to
know is the patency (or thrombosis) of the portal vein.
Hepatic artery patency, course, and resistive index can
be assessed with Doppler ultrasound.
28. ANGIOGRAPHY
⢠Portal pressure is measured indirectly from
the hepatic veins by measuring wedged and
free hepatic vein pressures, difference is
hepatic venous pressure gradient(HVPG).
⢠The HVPG represents the pressure in the
hepatic sinusoids and portal vein and is a
measure of portal venous pressure.
29. ⢠Done using a balloon occlusion technique akin
to a Swan-Ganz catheter measurement in the
pulmonary circulation.
⢠Normal HVPG is 6â8 mm Hg, and in cirrhosis
it will be greater than 10 mm Hg.
⢠Direct portal pressure measurement also can
be done by the transjugular, transhepatic
route.
30. UPPER GI ENDOSCOPY
⢠All patients with cirrhosis should have an
upper endoscopy.
1. 30% patients with cirrhosis develop portal
hypertension.
2. 30% patients with portal hypertension will
bleed from varices within 2 years.
3. The rate of development of varices in
patients with cirrhosis approximately 8% per
year.
31. UPPER GI ENDOSCOPY
⢠Varices-size, extent, and tortuosity; and the presence
or absence of red color signs. Large varices with red
color signs are at greater risk of bleeding.
⢠In patients with cirrhosis and upper GI bleeding,
endoscopy may be both diagnostic and therapeutic
with banding.
⢠Following an acute variceal bleed, the extent of varices
should be assessed after stabilization.
⢠Finally, the gastric mucosa should be evaluated for
evidence of portal hypertensive gastropathy with
congestion and cobble stoning.
32. EndoscopyâGrading of varices:
⢠I. Minimal varices without luminal prolapse
⢠II. Moderate varices, with luminal prolapse
and with minimal obscuring of O-G junction
⢠III. Large varices, with luminal prolapse and
with moderate obscuring of O-G junction
⢠IV. Very large varices, with luminal prolapse
and with complete obscuring of O-G junction
35. Pharmacologic therapy
ďPreventing initial bleed,
ďmanaging the acute variceal bleed,
ď and as 1st-line treatment in preventing
rebleeding.
Noncardioselective beta-blockers - propranolol
or nadolol
36. ⢠Pharmacologic therapy for acute variceal bleeding was
initially with vasopressin. Vasopressin is a potent
vasocontrictor and has been the most extensively used
drug for the initial control of variceal haemorrhage, but
it can cause myocardial ischaemia, arrhythmias,
mesenteric and limb ischaemia It has been largely
replaced by terlipressin, or somatostatin or one of its
analogue.
⢠These drugs effectively reduce portal pressure and flow
in the patient with acute variceal bleeding.
37. VARICEAL TAMPONADE
⢠Sengstaken- Blakemore tube is used
Advantage :
⢠Immediate cessation of bleeding in more than 85%
of patients
⢠widespread availability of this device
Disadvantages
⢠Cannot be used in the gastric varices
⢠Recurrent hemorrhage in up to 50% of patients
after balloon deflation
⢠Discomfort for the patient
⢠High incidence of serious complications when used
incorrectly by an inexperienced health care provider.
38. ⢠If the rate of blood loss prohibits endoscopic
evaluation, a SengstakenâBlakemore tube may
be inserted to provide temporary
haemostasis.
40. ⢠An x-ray is used to confirm the position of the
tube. The balloons should be temporarily
deflated after 12 hours to prevent pressure
necrosis of the oesophagus.
⢠Aspiration pneumonia and oesophageal
ulceration are other complications.
⢠Balloon tamponade is very effective in stopping
bleeding and once the patient is stabilised, a
more definitive treatment can be carried out.
41. ENDOSCOPIC THERAPY
ENDOSCOPIC BANDING
⢠The current standard for endoscopic therapy for
esophageal varices is endoscopic banding.
ADVANTAGES- fewer side effects, obliterates varices faster,
and can be easily applied. Multiband ligators allow the firing
of six to eight bands in a spiral fashion
⢠A course of bandingâusually two to three sessionsâis
then applied over the next month to 6 weeks
ENDOSCOPIC SCLEROTHERAPY
⢠Endoscopic sclerotherapy with injection of a sclerosing
solution,polidocanol (1â3 per cent) or ethanolamine (5 per
⢠cent), into or around the varix, may be a useful adjunct to
complete the obliteration of smaller varices that cannot be
banded.
43. Complications
⢠Fever, retrosternal pain, massive bleed,
perforation,
⢠mediastinitis, stricture oesophagus
⢠further bleeding episode after two injection
treatments during a single hospital admission
is defined as failure of emergency endoscopic
therapy
44. ⢠The emergency management of variceal
haemorrhage has been revolutionised by the
introduction of transjugular intrahepatic
portosystemic stent shunts (TIPSS) in 1988.
⢠Over a short period, it has become the main
treatment of variceal haemorrhage that has not
responded to drug treatment and endoscopic
therapy.
⢠The shunts are inserted under local anaesthetic,
analgesia and sedation using fluoroscopic
guidance and ultrasonography.
45. TRANSJUGULAR INTRAHEPATIC
PORTOSYSTEMIC SHUNT (TIPS)
⢠Direct puncture of the internal jugular vein (IJV),
⢠passage of a catheter through the right atrium into one of
the major hepatic veins followed by a trans-parenchymal
puncture of the liver to cannulate the portal vein.
⢠The catheter is passed into the portal vein
⢠The intraparenchymal track is then dilated and the track
stented with an expandable metal stent in the 10- to 12-
mm-diameter range.
⢠The technical success rate is high (>90%) with a low
procedural morbidity and mortality (<10%).
⢠Patients are usually in the hospital for 1â2 days and the
shunt patency should be documented the day after the
procedure with a Doppler ultrasound.
46.
47. TIPS
⢠It is a temporary procedure
⢠main contraindication to TIPSS is portal vein occlusion.
⢠Advantages:
⢠Useful in patients waiting for the transplant
⢠Useful in advanced hepatic functional decompensation
who are unlikely to survive for longer duration
⢠Useful for the treatment of medically intractable
ascites.
48. DISADVANTAGES
⢠The main early complication of this technique
is perforation of the liver capsule, which can
be associated with fatal intraperitoneal
haemorrhage.
⢠Post-shunt encephalopathy is the confusional
state caused by the portal blood bypassing the
detoxification of the liver. It occurs in about 40
per cent of patients, a similar incidence to that
found after surgical shunts
49. DISADVANTAGES
⢠Shunt stenosis, Shunt thrombosis . Seen within the first
year. Covered TIPS stents have reduced thrombosis and
stenosis rates.
⢠Reintervention rates to maintain patency are high with
uncovered stents, ranging from 40 to 80% and 20% with
covered stents.
⢠Rebleeding rates for TIPS are around 20%, and this was
reduced to 13% in the covered stent .Shunt stenosis,
which is usually secondary to neointimal hyperplasia, is
more common than thrombosis and can often be
resolved by balloon dilation of the TIPS or, in some cases,
by placement of a second shunt.
50. Decompressive Shunts
Considered 2nd line treatment and reserved for those
who rebleed after drug therapy or endoscopic or at
high risk.
Total Shunts
1. End-to-side portacaval shunt
2. Side to side portocaval shunt (diameter >10mm)
Partial shunts
1. Side to side portocaval shunt(diameter <8mm)
Selective shunts
1. Distal splenorenal shunt
51. End-to-side portacaval shunt
⢠In end to side portocaval shunt (Eck fistula)
portal vein is divided close to the hilus of the
liver and the splanchnic end anastomosed to
the side of the vena cava.
⢠It does not relieve ascites but will control
variceal bleeding.
⢠Complications : portosystemic
encephalopathy and accelerated hepatic
failure.
52.
53. Side to side portocaval shunt
⢠side-to-side portacaval shunts with direct vein-to-vein
or a short interposition graft, or the other inter-
position shunts such as mesocaval or mesorenal
⢠These shunts need to be at least 10 mm in diameter,
usually being 12â15 mm, to fully decompress portal
hypertension.
⢠shunts differ from the end-to-side portacaval shunt in
that the intact upper end of the portal vein serves as a
decompressive outflow from the high-pressureâ
obstructed liver sinusoids.
⢠Hence, in addition to controlling variceal bleeding,
these shunts also control ascites.
54.
55. Partial Shunts
⢠Partial shunts are side-to-side shunts whose
diameter is reduced to 8 mm.
⢠90% control of variceal bleeding
⢠polytetrafluoroethylene (PTFE) graft is
approximately 2â3 cm long, and bevelled at
each end to give a larger anastomosis.
56.
57. Splenorenal shunt
⢠The conventional splenorenal shunt consists
of anastomosis of the proximal splenic vein to
the renal vein.
⢠Splenectomy is performed.
⢠Complications : shunt thrombosis
58.
59. ⢠The only indication for a total portal
systemic shunt at present is for patients
with acute Budd-Chiari syndrome
60. Selective Shunts
⢠Selective shunts are most commonly the distal
splenorenal shunt (DSRS)
⢠Divide the splenic vein at its junction with the superior
mesenteric vein, and anastomoses the splenic vein to
the left renal vein.
⢠This selectively decompresses gastroesophageal
varices.
⢠Control of bleeding has been at 94%, with good portal
perfusion maintained in 90% of patients initially.
⢠The overall incidence of encephalopathy has been
around 15% following this operation.
61.
62. Devascularization Procedures
⢠Sugiura operation
⢠These operations approach the problem of variceal
bleeding by interrupting inflow to the varices.
⢠The components are splenectomy, gastric and esophageal
devascularization, and possibly esophageal transection
⢠The advantage of these procedures is that portal
hypertension is maintained with portal flow to the cirrhotic
liver.
⢠Devascularization can be useful when patients have
extensive portal and splenic venous thrombosis and there
are no other operative or radiologic options.
63. ⢠Postoperative management requires attention
to detail to minimize the risk of ascites as
these patients still have portal hypertension.
Follow-up endoscopy around 6 months is
often helpful to document if there are any
residual varices, treat them endoscopically at
that time, or document the complete- ness of
the devascularization procedure.
64. Liver transplant
⢠Liver transplant is the most commonly used
operation for patients with portal
hypertension at the present time.
⢠The major issues are
1. Patient Selection
2. Timing Of Transplant
3. Expanding The Donor Pool
4. Outcomes.
65. Patient selection
⢠Standards for patient listing have been set by the
United Network for Organ Sharing (UNOS)
⢠The indication for transplant is end-stage liver
disease
⢠Variceal bleeding ,ascites and encephalopathy
are clinical indicators of end-stage liver disease.
⢠comorbid medical conditions and a psychosocial
suitability for transplant particularly in the
alcoholic.
⢠Hepatitis C population with hepatoma
66. Timing
⢠The timing of transplant is dictated by the
severity of the underlying liver disease.
⢠Prioritization for organs occurs on the basis of
MELD scores
⢠Sickest patients receiving cadaveric organs first
based on bilirubin, prothrombin time, and serum
creatinine.
⢠Timing is dictated by these objective criteria
rather than individual physician decisions in day-
to- day patient management
70. MANAGEMENT STRATEGIES
⢠Variceal Bleeding
treatment options just described need to be
used appropriately in treatment strategies for
⢠Prophylaxis
⢠Acute variceal bleeding
⢠Prevention of rebleeding
71. Prophylaxis
Beta-blockers should be used for all patients
with medium or large varices to reduce their
risk of an initial bleed.
Bleeding risk can be reduced from 30% to 18â
20% with beta blocker treatment.
72. ⢠Endoscopic therapy for prophylaxis should
only be used for large varices and patients
intolerant to beta-blockers.
⢠Surgical therapy or radiologic shunts are not
indicated in prophylaxis prior to an initial
variceal bleed.
73. Prevention of Rebleeding
⢠First-line treatment for prevention of
rebleeding is a course of variceal banding and
concomitant pharmacologic therapy with a
beta-blocker no matter what the underlying
cause is.
⢠first elective banding episode should be
performed within 3 or 4 days of the acute
bleeding episode and subsequently and beta
blockers
74. Failure of 1st-line treatment can occur in several
scenarios.
main reasons are
(1) the patient may have a further acute variceal bleed.
(2) the patient may have recurring small bleeding
episodes that are not transfusion-requiring
(3)the patient may fail to have the varices obliterated
and continue to have large varices with risk factors.
.
75. Patients with any of the above scenarios, who
also have advanced liver disease, are
candidates for liver transplantation, possibly
using TIPS as a bridge.
Any of the above occurring in patients with well
compensated liver disease (Childâs class A)
may lead to variceal decompression with DSRS
or TIPS
76. Ascites
⢠The management of ascites is primarily medical with
dietary salt restriction and diuretics (spironolactone
and furosemide).
⢠When ascites becomes refractory to such a
regimen,large-volume paracentesis or TIPS may be
considered, but these are a bridge to transplant.
⢠Refractory ascites is one of the major clinical signs of
end-stage liver disease.
⢠surgeonâs role in treating patients with ascites is now
limited to liver transplant which is the best therapy for
patients with intractable ascites.
77.
78. THE MULTIDISCIPLINARY TEAM
Hepatologists are in the front line for diagnosing and
directing the management for many of the clinical
presentations.
Endoscopists play an important role diagnostically and in
primary therapy for managing variceal bleeding.
Endoscopic banding requires significant expertise.
Radiologists, both imaging and interventional, play roles
in diagnosis, directed biopsy, and procedural (TIPS)
manage- ment of these patients.
Surgeons play a major role in liver transplant but may
also have a role in shunting good-risk patients with
refractory variceal bleeding.
79. ⢠Pathologists with an interest in liver pathology
are important in the accurate diagnosis and
staging of disease severity.
⢠Critical care physicians and anesthesiologists are
vital team members when patients with portal
hypertension have acute events and in their
perioperative management.
⢠Nephrologists, cardiologists, and pulmonologists
all play a role in the management
