PORTAL

HYPERTENSION &
HEPATIC
ENCEPHALOPATHY
By
Dr. Preethi Subramanian,
Associate Prof.,
Pediatrics, MIMS
 ANATOMY
PHYSIOLOGY of PHT & COLLATERALS
ETIOLOGY of PHT
HEMODYNAMICS OF PHT
CONTENT CLINICAL MANIFESTATIONS
HEPATIC ENCEPHALOPATHY
INVESTIGATIONS
TREATMENT & PROPHYLAXIS
ANATOMY
• Normal Pressure in PV:
5mmHg
• PHT: 10-12mmHg
• >10mmhg: Collateral form.
• >12mmHg: Variceal bleed
SITES OF PORTO
SYSTEMIC
SHUNT
• Group I
• Group II
• Group III
• Group IV
PATHOPHYSIOLOGY OF PHT
ETIOLOGY OF
PHT
ETIOLOGY OF
PHT
ETIOLOGY OF
PHT
 ic
yna m
HEMODYNAMI a l haem ased
o d
ent re
CS OF PHT e
a m i n
fund ity is an l flow
c
Th al rta
or m p o
abn ance to
t
resis
 CLINICAL
MANIFESTATIONS
 GI Hemorrhages

• Hematemesis & Malena

• Age at first bleed depends on underlying etiology.
• Development of EHPVO was strictly associated with a neonatal disorder
including history of prematurity, neonatal illness, and umbilical venous
catheter.
• Since splenomegaly is a very common sign detected in children with PH at
the time of GI bleeding, the association between GI bleeding and
splenomegaly should be suggestive of PH until proven otherwise.
• Variceal bleeding in children with chronic liver disease often follows an acute
upper respiratory tract infection, with the contribution of several factors such
as
• the increased abdominal pressure during coughing or sneezing,
• the increased cardiac output due to fever,
• the erosive effect of nonsteroidal anti-inflammatory drugs used to treat
the fever.
• Gastroesophageal reflux is another factor which may contribute to erosions of
varices leading to its rupture and bleeding
Splenomegaly/
Hypersplenism
• Splenomegaly is due to PH which causes at the beginning
only spleen congestion and eventually tissue hyperplasia
and fibrosis.
• The increase in spleen size is followed by an increase in
splenic blood flow, which participates in PH actively
congesting the portal system.
• Liver function tests and Doppler ultrasound are mandatory
in healthy children with splenomegaly and hypersplenism to
exclude the presence of EHPVO and avoid worthless
procedures.
• Platelet count and splenomegaly are usually considered the
most reliable parameters to predict the presence di EV.
 • The serum ascites albumin gradient (SAAG) is used to classify ascites into portal and non-portal
hypertensive etiologies.

Ascites • The SAAG is calculated by subtracting the ascitic fluid albumin level value from the serum albumin
value, and the result correlates directly with portal pressure.
• Thus, a high gradient (SAAG ≥1.1 g/dl) indicates that the ascites is due to PH, whereas a low
gradient (SAAG ≤1.1 g/dl) indicates that ascites is not associated with increased portal pressure.
Hepatic
Encephalopathy
HE is defined as a metabolically induced, potentially reversible, functional
disturbance of the brain that may occur in acute or CLD.

Three Types: Type A: ALF; Type B: HE in Portosystemic shunting without

intrinsic hepatocellular failure; Type C: in CLD.

Type C could be further subclassified into minimal, episodic, or persistent.

HE is called minimal hepatic encephalopathy (MHE), when diagnosis could be

made only on psychometric analysis in an apparently normal person with CLD.

Episodic encephalopathy in CLD coincides with episodes of high protein

intake, gastrointestinal bleed, infection, etc.

Usually, these episodes resolve with the treatment of the precipitating factors,
but sometimes they persist and termed as persistent HE.
PATHOPHYSIOLOGY OF HE
• Under healthy condition, around 80–90 % of ammonia is
either converted to urea by periportal hepatocytes or
glutamine by perivenous hepatocytes.
• Excess ammonia is converted to glutamine by glial cells.
This glutamine increases intracellular osmotic pressure,
leading on to swelling of astrocytes, cerebral edema and
can competitively bind to glutamate receptors and inhibit
them.
• Oxidative stress triggered by ammonia toxicity in the
astrocyte resulting in mitochondrial dysfunction.
• Enhanced cytokine activity and impaired intracellular
signalling.
• Altered Fischer’s ratio (BCAA: AAA). There is decrease
in Fischer ratio in liver failure, due to preferential usage of
BCAA by muscles and decreased clearance of AAA by
liver. Elevated serum AAA can cross the blood–brain
barrier into the brain and results in synthesis of false
neurotransmitter such as octopamine and synephrine.
Decreased muscle
mass is a risk factor
for HE

Alkalosis prevents
excretion of
ammonium ion in
exchange for
hydrogen. So
alkalosis is to be
avoided.
Diagnosis of HE

WESCHLER’S INTELLIGENCE CRITICAL FLICKER EEG

SCALE FREQUENCY IN > 8YEARS OLD
Markers of
CLD
Growth Retardation

• Failure to thrive in children with

EHPVO depends on duration of PH and
declines further with age despite
appropriate energy intake.
• The pathogenetic mechanisms may
include the reduced portal blood supply
to the liver and the consequent
deprivation of hepatotropic factors, the
poor substrate utilization associated with
the malabsorption due to portal
hypertensive enteropathy, as well as
growth hormone resistance.
Portal Hypertensive Biliopathy
• PHB is a disorder characterized by anatomical and functional abnormalities of the intrahepatic, extrahepatic,
and pancreatic ducts occurring most commonly in patients with non-cirrhotic PH.
• The pathogenesis is mainly related to long-standing portal cavernoma in the biliary and peribiliary region,
causing compressive and ischemic changes of the biliary tree, and more frequently in the left hepatic duct.
• only 20–30 % develop clinical signs of cholestasis.
• Magnetic resonance cholangiopancreatography (MRCP) is the first-choice tool to diagnose PHB in children.
Complications
• Hepatopulmonary syndrome (HPS)
• Porto pulmonary Hypertension (PPH)
• Hepatorenal Syndrome (HRS)
CLINICAL ASSESSMENT
HEPATIC VENOUS PRESSURES
MANAGEMENT
• Acute Variceal Bleeding
General Measures
Vasoactive drugs
Sengstaken blake more tube
Endoscopic ligation
Sclerosing Agents
TIPS
Variceal Bleed:
Vasoactive
Drugs

Vasopressin/ Terlipressin
Octreotide
Vasoactive Drugs
Shunt Surgeries
THANK YOU