Portal hypertension

Before discussing the pathology of the portal circulation we will talk briefly about the
anatomy and physiology of it.

The portal vein which drains the

blood from gastrointestinal
organs and spleen to the liver, is
formed by the union of the
superior mesenteric vein and
splenic vein. Before the splenic
vein joins the superior
mesenteric vein, the inferior
mesenteric vein drains into the
splenic vein.

Keeping in mind that both right

and left gastric veins open
directly into the portal vein.

The portal vein before it enters

the liver, it divides into right and left portal veins.

By going back to the superior mesenteric vein, it drains blood from jejunum, ileum, right
colon and right 1/2 of the transverse colon. While the inferior mesenteric vein drains the
left 1/2 of the transverse colon, left colon, sigmoid colon, upper 1/3 of the rectum. The
Splenic vein drains blood from the spleen. So the blood in all these viscera goes to the
liver, where the detoxification mechanism occurs (emulsification, oxidation, conjugation,…
etc), which is the real function of the liver.

The pressure in the portal system is a low pressure (5-10 mmHg), because it’s a venous
pressure. In spite of that it’s higher than the real pressure in the venous system like the
femoral or the jugular veins, where the pressure is up to 5 mmHg, according to the
inspiration and expiration of the patient, while in the portal circulation the pressure is
between 5-10 mmHg in normal people. Once the patient has the pressure in the portal
system above 12 mmHg, we can say that he has PORTAL HYPERTENSION.

*How we can measure the portal vein pressure?

1. during surgery (laparotomy), we can put a cannula in the portal vein, this cannula is
connected to a mercury column for pressure measurement.

2. we can introduce a cannula percutaneously in the splenic vein.

3. transjugular measurement of portal pressure. Which is the most common used method,
and it’s done by measuring the hepatic venous pressure gradient (HVPG). It’s an indirect
measurement that closely approximates the portal venous pressure.
through the jugular vein we can introduce a cannula reaching to one of the hepatic veins
[venous drainage from the liver : right, middle, and left hepatic veins > inferior vena cava].

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Notes :

1. The capillary system in the body is arterio-venous, which means it’s between arterioles
an venules. While there are two glands in the body that have venous-venous capillary
system, and these glands are: the liver and the pituitary gland.
2. If the portal pressure was above 12 mmHg, this will reflect on the organs drained by
the liver, for example: the spleen will start to get larger “splenomegaly”, due to the
congestion of blood. Also through left gastric vein which drains the gastroesophageal
junction will dilate, and this will cause what’s called “esophageal vertices”.
3. As portal hypertension per se produces no symptoms, it’s usually diagnosed following
presentation with decompensated chronic liver disease, hepatic encephalopathy, ascites,
or variceal bleeding.

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Increased portal pressure is due to blockage of the venous return either:
1. Prehepatic [portal system]
2. Intrahepatic [inside the liver]
3. Posthepatic [hepatic veins and their continuity].

1. Prehepatic:
This is also divided into: intramural and extramural.
 Intramural:
1. Portal vein thrombosis: which is the most common one of this group, and this
thrombosis will occlude the blood flow. The main causes of portal vein
thrombosis is umbilical catheterization in pediatric age group; when the baby is
born a catheter is introduced inside the umbilical vein, so sepsis may occur, or
the catheter may be left for a long period so the thrombus will be formed.
2. Splenic vein thrombosis : mainly due to sepsis or malignancy like lymphoma
affecting the spleen and cause splenomegaly, resulting in splenic vein thrombosis.
3. Tropical splenomegaly: splenomegaly will increase the blood outflow, and this is
by itself will increase the pressure in the splenic vein.
Tropical splenomegaly syndrome, also known as hyperreactive malarial splenomegaly,
occurs due immunological over-stimulation to repeated attacks of malarial infection over a
long period of time... Wiki
4. A/V malformation: due to trauma like pancreatic trauma with an injury to the
splenic vessels resulting later in communication between these vessels, so the
flow from the splenic artery to the splenic vein will increase, and the pressure in
the splenic vein will also increase, due to it’s limited ability to tolerate more
amounts of blood.

<< so the intramural causes include portal vein and its tributaries>>.

 Extramural :

Exterior compression of the portal vein, which is somehow a rare condition and this
is due to portahepatic lymph node enlargement, in case f some adjacent
malignancies like Antral Gasrtic CA, pancreatic head CA, gall bladder CA,
clolangiocarcinoma, and Klatskin tumor.
 > cholangiocarcinoma is a cancer arising from the common bile duct or the
hepatic duct.
> Klatskin tumor is a cholangiocarcinoma arising from the junction of the two
hepatic ducts, and in this case the patient will come to you having Jaundice
and Ascites.

2. Intrahepatic:
Either presinusoidal or postsinusoidal or mixed.

 presinusoidal:
1. Schistosomiasis
[Bilharzias]: which is
the most common
cause of portal
hypertension
worldwide, the ova of
the Schistosoma will
enter the portal
system causing
blockage over there.
2. Primary biliary cirrhosis [PBC]: cirrhosis affecting females more than
males, in the age of 40-55 years of old. Primary biliary cirrhosis, is
an autoimmune disease of the liver marked by the slow progressive
destruction of the small bile ducts of the liver. When these ducts are
damaged, bile builds up in the liver (cholestasis) and over time damages
the tissue. This can lead to scarring, fibrosis and cirrhosis.
3. Active chronic hepatitis [hepatitis AC]: when there is hepatitis (viral,
alcoholic, drug-induced) the architecture of the liver will be destroyed
and replaced by fibrosis that will block the drainage of the portal
system to the hepatic veins.
4. Srcoidosis.
  sinusoidal:
here the sinusoid itself will be affected and the main causes are: hepatic
cirrhosis, viral or alcoholic hepatitis, metabolic causes like Wilson’s disease
and Haemchromatosis, cytotoxic drugs, vitamin A intoxication.

> Wilson’s disease  abnormal accumulation of copper (Cu) in tissues.

> Haemchromatosis  abnormal accumulation of Fe in the liver.

> if we suspect a pathology in the liver we should ask about the drug intake
history, cause many drugs will damage the function of the liver hepatocytes
leading to an elevation of the levels of AST, ALT, ALP.

3. Posthepatic:
Causes affecting the hepatic veins and inferior vena cava above the liver, the
most common causes are:
1. Budd-Chiari Syndrome: venous occlusion affecting the hepatic veins due to
thrombosis, and this thrombosis is mainly due to thrombophelia.
Most of the patients having this syndrome come to you with severe sudden
abdominal pain with ascites.
Thrombophelia , which is either a congenital or acquired disease (but mostly
it’s congenital). Here the patient has relative deficiency in some coagulation
factors, such as anti-thrombin III, protein C, protein H, factor V. and a
relative deficiency means that when there is some conditions like
Dehydration, malignancies, or immobility, these conditions will exacerbate
the possibility of thrombus formation.
For example : 27 years old pregnant female in the second trimester, has
protein C deficiency, when she reached the 7th month of gestation, she
complained of abdominal pain with ascites that resolved later. One month
later (9th month of gestation) , she was admitted to the hospital complaining
of severe continous abdominal pain, that forced the surgeons to do
caesarian section for her.
 -what was the cause of ascites? Portal hypertension due to Budd-Chiari
sundrome.
-what investigations will you do? Ultrasound.
*the ultrasound showed that there’s a thrombus in the 3 hepatic veins*
-what would you do if she came to you after the first episode? Ultrasound.
And by early diagnoses it will show the thrombus in one hepatic vein only
with two competent hepatic veins, so by giving anticoagulants you will save
the patient and protect the other hepatic veins away from thrombus
formation.
-what is the end stage problem of Budd-Chiari sundrome of the three
hepatic veins and what is the treatment ? the end-stage problem is liver
failure. And the only thing we can do is liver transplant. So imagine how the
early diagnosis can play a role in this case.

2. Veno-occlusive disease.
3. Caval abnormality.
4. Congestive pericarditis : incomplete relax of the right atrium during the
diastole, so this will cause accumulation of blood in the inferior vena cava
downward involving the liver.

Pathophysiology of portal hypertension

Increased portal pressure reduces portal venous flow. so there will be:

 a high risk of hypoxia to the liver, because the blood supply to the liver
is mainly via the portal vein (70%), and hepatic artery contributes just
by 30%.
 development of porto-systemic anastomosis. The six potential routes of
porto-systemic anastomosis :
1. When there is increment of the pressure in the splenic vein , the blood will enter
the short gastric vessels that drain the fundus of the stomach, so varices will be
formed.
2. The left gastric vein which
comes from the lesser
curvature and the
gastroesophageal junction,
drains directly into the
portal vein, so when there’s
a high venous pressure
there will be backflow
(reflux) , and this will form
anastomosis with the
Hemiazygous system (which
is from the superior vena
cava), causing what is called
esophageal varices.
3. Caput Medusa: dilation of
the umbilical veins and it’s
branches around the
umbilicus.
4. Haemoorrhoids or anorectal
varices: superior
haemorrhoidal vein (which
normally drains into the inferior mesenteric vein) backing up into the middle and
inferior haemorrhoidal veins (systemic veins).
5. In the retroperiotoneal space around the kidney , there are small mesenteric
veins called retroperitoneal veins of Retzius, draining retroperitonealy into
lumbar veins.
6. Peri-hepatic veins of Sappy (Diaphragmatic veins) , which are very dangerous
veins in the RUQ. During operations like cholecyctectomy, if these veins are
injured this is going to be a catastrophic problem, so good surgeon must be
familiar with them.

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 The most common cause of portal hypertension is cirrhosis. Cirrhosis means the
destruction of the liver architecture and replacement by fibrosis. The most
common cause of cirrhosis worldwide is schitosomiasis.
Cirrhosis gives features of:
 Liver failure.
 Portal hypertension, which by itself has features of:
- Bleeding from varices.
- Ascites.
- Encephalopathy.

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As we said before, portal hypertension per se has no symptoms, the symptoms

develop as a complication of portal hypertension. The main clinical presentations
of portal hypertension are:
 Bleeding presentation: upper GI bleeding ( either due to rupture of
esophageal varices or rupture of gastric varices), bleeding from
haemorrhoids.
 Non-bleednig presentation: 1. Caput medusa 2. Encephalopathy
3.ascites.

>> The mechanism of ascites formation:

1. Transudate leakage of the portal system and this represents only about 20%
of ascites.
2. Hepatocellular failure which represents 80% . how ?
 It causes hypoproteinemia in general and mostly hypoalbuminemia. And
because of the oncotic pressure, the patient will have fluids anywhere
inside his/her body . The main site of fluid accumulation is the potential
spaces (pritonel and pleural cavities).
 Hypoalbuminemia will increase the formation of Angiotensin Converting
Enzymes [ACE] “from lungs as we know” , and that will affect the kidneys
to decrease the excretion of fluids from the body , so by this the
kidneys will participate in the formation of ascites.
<< so, if the patient has ascites with liver failure, you expect to find the following :
1.hypoprotienemia due to the failure of hepatocytes’ function 2.poor excretion of
the kidneys due to the intervention of ACE. 3.mechanical presentation which is
transudate leakage of the portal system.>>

Any patient comes with upper GI bleeding due to rupture of Esophageal varices
with ascites, you have to think about liver failure. And the most common cause of
liver failure differs geographically, for example in Europe the most common cause
is Postalcoholic, whie in Jordan and the middle east is postviral, and in Egypt is
schistosomiasis.
What if the patient comes to you without ascites -only with upper GI bleeding-
that means the hepatocytes are still functioning, an example about that: Abd El-
Haleem Hafiz, who died of schistosomiasis without developing ascites.

>>>> 90% of patients with liver cirrhosis will deveope esophageal varices.
>>>> 30% of those 90% will have bleeding.

Dr Hussain showed a picture of a patient having severe dilation of the abdominal veins, and the question
: how can we differentiate between these veins?
the answer : from the sites of these veins.
1. Centrally located around the umbilicus, forming sunrays shape, this is caput medusa.
2. Peripherally located, these veins are tortuously dilated due to caval obstruction mainly the IVC.

To define the severity of liver cirrhosis, we can use a type of classification, which
is called Child-Pugh classification, which represents the function of the
hepatocytes:

Variable/points 1 point 2 points 3 points

Encephaopathy Absent Mild/moderate Severe or coma
Bilirubin (µmol/L) < 34 34-51 > 51
Albumin (g/L) > 3.5 2.8-3.5 < 2.8
Prothrombin Time 1-4 4-6 >6
  score 5-6  class A : which has the best prognosis with a morbidity rate
< 50%
 score 7-9  morbidity rate up to 35%
 score > 10  morbidity rate > 55%
 encephalopathy is caused by the inability of the hepatocytes to
detoxificate the toxic material (mainly the ammonium) , and those toxins
will affect the brain.
 Mild/moderate encephalopathy; the patient has confused thinking,
sometimes he may forget the name of his children, the time or the place
he exists in. fortunately he will remember these things after a while.
But the problem is if he is comatose or having severe encephalopathy
that lets him to have permanent confusion, so he will not be able to
recognize anything around him.
 Normal bilirubin is up to 17 µmol/L
 Bilirubin, PT, and albumin reflect the real function of the liver.

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Management
Unfortunately, most cases of portal hypertension cannot be treated. Instead
treatment focuses on prevention or managing the complications.

1) The best treatment of the liver with metabolic disorder like wilson’s diease
and haemchromatosis is liver transplant. Because he will have portal
hypertension till death (portal hypertension is irreversible), so the only way
for treatment is liver transplant.
the only problem that we face after liver transplant is the chronic active
hepatitis (hepatitis AC), while all the metabolic disorders will disappear.
>Indications of liver transplant: 1. Primry Biliary Cirrhosis, which is the most
common indiction. 2.Budd-Chiri syndrome.
 Question from one of the students: does liver transplant occur in Jordan ?
yes, many operations were successfully done with assistance from German
surgeons. Dr Hussain told a story about one of his patients , 23 years old
female has primary biliary cirrhosis and metabolic disorders. “I tried my
best for her to do Liver transplant, but the problem that there was no living
donor, I kept her for one year waiting for a donor, after this year she
developed ascites with bleeding and hyoalbuminemia , so I did a bypass
surgery for her to convert the blood from the portal vein to the venous
system (IVC)”.

2) Management of bleeding varices:

** in patient with known varices, bleeding can be prevented by β-blockers,
as they reduce the systolic (arterial) blood pressure and this will reduce the
venous pressure.
** also we can prevent the bleeding of varices by Endoscopic
Sclerotherapy, by the usage of sclerizing agents such as Ethanol, Amine or
Tetra decay sulfate.
** TIPS: Transjugular Intraheptic Porto-systemic shunt: by introducing a
catheter through the internal jugular vein we go downward to the superior
vena cava, to the right atrium, to the Inferior vena cava, then we can enter
the portal vein through winslow’s foramen (it’s a foramen between the portal
vein and IVC, and it’s the only way to enter the lesser sac that is behind the
stomach). So by doing TIPS we shunt the blood from the portal vein directly
to IVC.
Question: what about the toxins? 25% of patients with TIPS will develop
Encephalopathy.
so the role of TIPS: 1. Temporary shunting of the blood from the portal vein
to the venous system, till presence of a living donor. 2. Avoid destroying
the normal anatomy of the liver and the adjacent structures by doing an
opened surgery before the liver transplant, because it’s not a good idea to
expose the patient to two surgeries while we can do TIPS without
laparotomy.
 ** surgical shunt : which is the porto-caval shunt , and from anatomical
point of view it’s called Truncal shunt if it’s done between the portl vein and
IVC. And there’s also Peripheral shunt , which is done between the splenic
vein and the renal vein.

3) Encephalopathy management
waril’s selective spleno-renal shunt : is done to decrease the encephalopathy.
short gastric vein  splenic vein anastomosis with the left renal vein  IVC.
Question: how does waril’s shunt decrease the Encephalopathy? By the
diversion of the portal circulation directly to the venous system , what we
are doing is just diluting the toxins in the blood, in fact the toxins are not
detoxified or reduced but they are diluted only, and this the aim of this
shunt.

4) Ascites management:
-the initial treatment is to restrict additional salt intake and commence
diuretics using either spironolactone or fursamide.
this should be combined with advice on avoiding any precipitating factor for
impaired live function such as alcohol intake in patient with alcoholic
cirrhosis.
-abdominal paracentisis.
-peritoneo-venous shunting.
-TIPS

THE END