3. Definition-An elevation of portal pressure
above 10-12 mm Hg or 30 cm of saline or
intrasplenic pressure>17 mm Hg or
wedged hepatic venous pressure more
than 4 mm Hg above inferior vena caval
pressure.
4. Pathophysiology
• portal system includes all veins,which
carry blood from digestive tract,spleen
pancreas and gall bladder to liver via
portal vein
• portal vein is formed by the union of
superior mesenteric vein and splenic vein
just posterior to neck of pancreas at level
of L 2
6. • portal vein about 6-8 cm long and 12 mm
in diameter
• It dose not contain any valves and hence
the pressure is same in all part of portal
venous system.
• it supplies the liver 80% of blood and 20 %
of oxygen
• the portal system is peculiar in that it
begins as capillaries in mesentery of the
intestine and spleen and end as capillaries
in liver
8. Pre hepatic
PHT
1.Upper GIT bleeding
2.pallor
3.splenomegaly
PRESINUSOIDAL SINUSOIDAL
1.upper GIT bleeding
2.splenomegaly
3.jaundice
4.hepatomegaly
5.ascites
6.hepatic encephalopathy
PORTAL HYPERTENSION
post sinusoidal
post hepatic
abdominal pain with vomiting
massive ascites
tender hepatomegaly
upper GIT bleeding
jaundice
splenomegaly
upper GIT BLEED
the combination of GIT bleed and splenomegaly is pathognomonic of PTH.
left lobe of liver enlarged in intr hepatic PTH and caudate lobe enlarged in
post hepatic PTH
9.
10. PRE HEPATIC PORTAL
HYPERTENSION
block in portal vein occurs before the blood
reaches the liver.
• Patients usually present with upper GIT bleeding,
• normal liver function and, prognosis excellent.
11. AETIOLOGY
1. Developmental defects-Portal vein agenesis, atresia,
stenosis, cavernoma.
2. umbilical sepsis-this is most important cause in developing
countries
umbilical vein
left branch of portal vein
portal vein
infected thrombus
12. 3. trauma
4.Portal vein thrombosis
5.Intra-abdominal infections-like acute appendicitis
and primary peritonitis and pancreatitis.
6.idiopathic-IN 50 % OF children with pre hepatic PTH
etiology can not be found out.
13. CLINCAL FEATURE OF PRE HEPATIC
PTH
UPPER GIT BLEED-
due to ruptured oesophageal varices
Manifestation of upper GI bleed
hematemesis-vomiting of fresh blood
melaenemesis-vomiting of altered blood.
melena-passage of
black,tarry,sticky,offensive,loose stools.
14. On General Examination
• pallor
• splenomegaly
• stunted growth-stunted growth due to the following
factors-
1. massive splenomegaly compresses the stomach.
2.deprivation of hepatotrophic growth factors
• no jaundice
• no hepatomegaly
• no ascites
15. Intra Hepatic PTH
• TYPES-
1.pre sinusoidal
2.sinusoidal
3.post sinusoidal
• the liver functions are usually normal in
pre sinusoidal ,where they are dearrenged
in sinusoidal and post sinusoidal.
17. sinusoidal PTH
this is cause by cirrhosis due to hepatitis B and C
,metabolic disorders like wilson's disease,cholestasis
disorders like biliary atresia,neonatal hepatitis,drugs like
INH,methotrexate.
feature-1.upper GIT bleed
2.splenomegaly
3.jaundice
4.ascites
5.hepatic encephalopathy
6.caput medusa
7.hepatomegaly
18. smith-Howard syndrome:- sometimes the spleen
become impalpable following a massive bleed,which
may cause diagnostic confusion this condition is called
smith-Howard syndrome.
size of spleen dose not correlate well with portal
hypertension.
ascites is a frequent problem in sinusoidal and post
sinusoidal PTH. It is not usually seen in pre hepatic and
pre sinusoid PTH
19. POST SINUSOIDAL PHT
(VENO-OCCLUSIVE disease)
• This is non thrombotic occlusion of terminal hepatic
venous radicles without associated abnormality of
hepatic vein or inferior vena cava.
AETIOLOGY-
TOXINS LIKE aflatoxins
drugs like 6-mercaptopurine,
cyclophosphamide,vincristine
hypervitaminosis A.
21. POST HEPATIC PTH
(Budd- chiari syndrome)
thrombosis of hepatic vein.
etiology-
A.Thrombosis complicating-
1.abdominal trauma
2.polycythemia rubra vera
3.neoplasms
4.S.L.E
5.cirrhosis
6.sickle cell anaemia
B.membranous obstruction of supra hepatic vena
cava
22. • acute budd chiari syndrome-
1. sever abdominal pain with vomiting
2. mild jaundice
3.ascites
chronic budd chiari syndrome
1.massive ascites
2.hepatomegaly
3.upper GI bleed
4.hypertrophy of the caudate lobe.
5.compression of the intrahepatic portion of the IVC, leading to
lower extremity edema
24. INVESTIGATION
• CBC
• LFT-dearrenged in sinusoidal,post sinusoidal and post hepatic portal
hypertension.normal in pre sinusoidal and pre hepatic PHT..
USG
• portal vein diameter >17mm
• portal vein not increases in diameter during inspiration.
• demonstration of collaterals
• splenomegaly
color Doppler-assess the blood flow within the portal vein
27. primary prophylaxis
propranolol-
dose -:1-2 mg /kg/day
mechanism of action-it lowers the venous
pressure and portal venous pressure by
decreasing the heart rate by 25% and the
blood pressure by 15 mm Hg.It also
causes splanchnic vasoconstriction.
28. Treatment of bleeding
esophageal varices
• fluid resuscitation- i.v. fluids and blood
transfusion to correct hypovolemia in a patient
with massive upper GIT bleeding.
• The first step is to assess the degree of blood
loss by the following methods:-
1.disproportionate tachycardia,suggest that the
patient has lost about 20 % of blood volume.
29. 2.TILT TEST-the basal pulse rate and BP are
measured.The head end of the patient is then tilted
or raised to 45 degree, so as to produce postural
hypertension.
if the pulse rate rises by more than 20/min or if the
diastolic BP falls by more than 10 mm of mercury,the
patient has suffered about 25% blood loss.
3. Capillary refill sign
In a normal person if the nail bed is compressed
and the pressure released, the blood column comes
back almost immediately. But if the patient has lost
the blood 25% blood volume it take more than 5 sec
to come back.
4. If the patient is in shock, he has lost about 40%
blood volume.
30. • use of Nasogastric tube to confirm active
bleeding
• normal saline at room temperature is best
for gastric lavage
• chilled or iced saline gastric wash does not
stop bleed and may cause central
hypothermia.
31. Pharmacological therapy
1.vasopressin
0.33 u/kg in 20 min than iv infusion of
0.33U/kg/hr
2.octreotide
administered by continuous intravenous
infusion of 1.0-5.0 µg/kg/hr
H2 blocker/PPI
I.V. vit k
32. Endoscopic therapy
variceal band
ligation-it is gold
standard treatment
for esophageal
varices.less
bleeding,fewer
complication and
requires fewer
treatment sessions
to achieve variceal
eradication than
sclerotherapy.
33. B.variceal sclerotherapy-via endoscope
sclerosing agent is injected in lumen of
each varix,causing thrombosed.endoscopy
is usually repeated with in 48 hr than
weekly interval.
• sclerosant used in esophageal varices-
1.ethalonamide oleate
2.absolute alcohol
3.phenol
4.butyl cyanoacrylate
34. Disadvantage of sclerotherapy
• chances of re bleeding is high (>25%)
hence repeated injections are necessary
• multiple esophageal ulceration
• esophageal stricture
• paraplegia
36. • TIPSS is percutaneous technique creates
communication between the right hepatic
vein and the right or left branch of the
portal vein
• performed if failed medical and
endoscopic management
37. Surgical procedure
• Surgical porto-systemic shunts (often
spleno-renal)
• De vascularization surgery
• Liver transplantation
38. • creation of an anastomosis between portal
and systemic venous system.
• most common anastomosis between
splenic vein and left renal vein.
Surgical porto-systemic shunts
40. child-pugh scorechild-pugh score
features 1 2 3
bilirubin(mg%) 1-2 2-3 >3
ascites absent slight moderate
prothrombin time <4 sec
4-6 sec >6 sec
serum albumin >3.5g 2.8-3.5g <2.8g
encephalopathy
(stage)
none 1-2 3-4
child's criteria are useful guide for deciding the suitability
of a patient for shunt surgery and prognosis
41. grading points prognosis
grade A 5-6
liver function is maintained.<10%
mortality in surgical procedure
grade B 7-9 intermediate
grade C 10-15
poor.
>90% mortality in surgical
procedure
42. Devascularization surgery
• transthoracic para-esophageal
devascularization is combined with
esophageal transection, splenectomy,
esophagogastric devascularization, truncal
vagotomy and pyeloplasty.
43. Liver transplantation
• Orthotopic liver transplantation represents
a much better therapy for portal
hypertension resulting from intrahepatic
disease and cirrhosis
44. portal hypertension
PRIMARY Prophylaxis
propranolol-dose 1 mg /kg/day
Bleeding
esophageal varices
iv fluids and blood transfusion
vit k
H2 blocker or PPI
after bleeding episode
or bleeding is not stop
pharmacological drugs
sengstaken-blackemore tube
surgical procedure
de vascularization
porto-systemic shunt
liver transplantation
bleeding persists
TIPSS
EST/ EVL
45. Pathophysiology Of Ascites In Portal
Hypertension
portal hypertension
hypovolaemia
activation of renal
angiotensin-aldosterone
system(RAAS)
increase Na and water
reabsorption from DCT
renin
Ascitesexpansion of plasma
increase pressure in sinusoid
protein rich fluid from sinusoids
into hepatic lymphatics
causing increased hepatic lymph
excess lymph enter into the
peritoneal cavity
46. Management OF Ascites
• In 80% of cases ,ascites is caused by cirrhosis
• in portal hypertension transudate ascites occur.
• Serum -Ascites Albumin Gradient (SAAG) is useful
for distinguishing ascites caused by portal
hypertension from non-portal hypertension
ascites.
• SAAG=(ascites albumin -serum albumin).
• SAAG>1.1g/dl :- presence of portal hypertension.
47. SAAG
>1.1 g/dl
Ascitic protein <2.5g/dl
cirrhosis
late budd-chiari syndrome
massive metastases
Ascitic protein_>2.5g/dl
congestive heart failure/constrictive
pericarditis
early budd-chiari syndrome
IVC obstruction
post sinusoidal PTH
<1.1g/dl
biliary leak
nephrotic syndrome
pancreatitis
peritoneal carcinoma
tuberculosis
48. Management of ascites-
Bed Rest
Bed rest :
• Upright posture activates sodium retaining
mechanisms , impairs renal perfusion and
sodium excretion.
49. Sodium Restriction
Sodium restriction :
A negative Na balance can attained by
restricting oral intake of salt 2 g/day.
50. WATER RESTRICTION
• Central hypovolaemia - > stimulates ADH receptors
- > decreases free water clearance - > dilutional
hyponatraemia.
• Restriction of water worse> central hypovolaemia.
51. DIURETICS
• potassium -sparing diuretics is considered the diuretic of
choice in portal hypertension.
• hyper aldosteronism promotes sodium retention in distal
tubules and collecting ducts in portal hypertension
• this action is inhibited by spironolactone.
• loop diuretics act on thick ascending limb of henle by
inhibiting Na-k-2Cl symport,preventing the reabsorption of
Na and water.there is hperaldosteronism In portal
hypertension.so Na and water absorbed from the DCT.so
loop along is not effective.
• spironolactone is not a powerful natriuretic agent .As mono
therapy,it takes several days to induce weight loss.hence it
is preferable to use a combination of spironolactone and
furosemide.
52. • Dose of spironolactone-start with 1mg/kg and
increase at rate of 1mg/kg/day to a maximum of 6
mg/kg/day.
Assess response to diuretics
• Weight loss of 0.5kg/day in absence of edema and
1kg/day when edema present.
• Use Spironolactone & Furosemide 100mg/40mg
ratio
53. Refractory ascites
• Definition-ascites does not respond to
salt restriction and diuretics.
• Therapeutic options in refractory ascites-:
1.Serial Paracentesis
2.Peritoneovenous Shunts
3.TIPSS
4.Liver Transplantation
54.
55. Peritoneo-venous shunt
• ascitic fluid is shunted from the high
pressure peritoneal cavity to the low
pressure superior vena cava by
1.Le veen shunt
2.denver shunt
