Portal Hypertension

Question 1

What is portal hypertension? When is this defined as clinically significant?

Answer 1

Abnormally high pressure within the hepatic portal vein.
Hepatic venous pressure gradient > 10 mm Hg
(NORMAL: < 5mmHg)

Question 2

What is the most common cause of portal hypertension? Describe the mechanism of this

Answer 2

CIRRHOSIS
Collagen deposition + fibrosis.
+
Sodium retention + vasoactive substances e.g. NO (due to accumulation of toxic metabolites) increase plasma volume + splanchnic vasodilation, maintaining portal HTN.

Question 3

How can other causes of portal hypertension be classified?

Answer 3

Pre-Hepatic: blockage of the portal vein before the liver
Hepatic
Post-Hepatic: blockage of hepatic veins or venules

Question 4

Describe the epidemiology of portal hypertension

Answer 4

Common consequence of cirrhosis

Question 5

List symptoms of portal hypertension

Answer 5

Features of Liver Disease (likely to coexist with portal HTN)
Jaundice

Question 6

List 3 signs of portal hypertension

Answer 6

Caput medusae
Splenomegaly
Ascites

Question 7

What rhyme can be used to remember signs of portal hypertension?

Answer 7

Butt: Rectal varices
Gut: Splenomegaly + Oesophageal varices
Caput: Caput medusa

Question 8

List 9 signs of liver failure that may be seen in portal hypertension

Answer 8

Asterixis  
Confusion  
Palmar erythema 
Enlarged or small liver
Fetor hepaticus  
Gynaecomastia  
Jaundice  
Spider naevi 
 Testicular atrophy

Question 9

List 3 common features in the history of patients with portal hypertension

Answer 9

Hx of alcohol abuse
RF for viral hepatitis (e.g. tattoos, unprotected sex, IV drug use, travel abroad + blood transfusion)
FH (e.g. haemochromatosis)

Question 10

List 5 hepatic causes of portal hypertension

Answer 10

CIRRHOSIS 
Chronic hepatitis  
Schistosomiasis 
Granulomata  
Myeloproliferative disease

Question 11

List 4 pre-hepatic causes of portal hypertension

Answer 11

Congenital stenosis
Portal vein thrombosis
Splenic vein thrombosis
Extrinsic compression

Question 12

List 3 post-hepatic causes of portal hypertension

Answer 12

Budd-Chiari syndrome
Constrictive pericarditis
Right heart failure

Question 13

What is Budd-Chiari syndrome?

Answer 13

Sudden-onset ascites with tender hepatomegaly in the absence of jaundice (hepatic vein obstruction)
Triad: Abdo pain, ascites + hepatomegaly

Question 14

What bloods are taken in portal hypertension?

Answer 14

LFTs  
U+Es 
Blood glucose  
FBC 
Clotting screen (prolongation of PT = early sign of liver failure)

Question 15

What specific tests are requested in portal hypertension to determine cause?

Answer 15

Ferritin: haemochromatosis  
Hepatitis serology  
Autoantibodies (e.g. ASMA in AI hepatitis)   
Alpha1-antitrypsin levels  
Caeruloplasmin: Wilson's disease

Question 16

What imaging is performed in portal hypertension?

Answer 16

Abdominal US: check liver + spleen size + assess portal blood flow
Doppler US: assess direction of blood flow in vessels
CT/MRI: if other imaging methods are inconclusive

Question 17

What invasive tests may be performed in portal hypertension?

Answer 17

Endoscopy: identify oesophageal varices + measure hepatic venous pressure gradient (HVPG)
Liver Biopsy

Question 18

What is the immediate treatment for oesophageal varices in portal hypertension?

Answer 18

Terlipressin: ADH agonist, causes splanchnic vasoconstriction reducing portal pressure
+ prophylactic abx
Endoscopy within 12h to diagnose + treat using band ligation or injection sclerotherapy

Question 19

What conservative management approaches can be taken in portal hypertension?

Answer 19

Diuretics

Reduce salt intake

Question 20

Describe surgical management in portal hypertension

Answer 20

Transjugular Intrahepatic Portosystemic Shunt (TIPSS) - surgical shunt placed between hepatic portal vein + hepatic vein to ease congestion in the portal vein

Question 21

List 6 complications of portal hypertension

Answer 21

Bleeding from oesophageal varices: Haematemesis 
Ascites + complications of ascites
Pulmonary complications  
Liver failure  
Hepatic encephalopathy 
Cirrhotic cardiomyopathy

Question 22

What is Spontaneous bacterial peritonitis?

Answer 22

infection of ascitic fluid that can’t be attributed to any intra-abdominal, ongoing inflammatory, or surgically correctable condition

Question 23

What is hepatorenal syndrome?

Answer 23

Rapid deterioration in kidney function in individuals with cirrhosis or fulminant liver failure
Life threatening

Question 24

What is hepatic hydrothorax?

Answer 24

Transudative pleural effusion in pts with portal HTN without any underlying primary cardiopulmonary cause

Question 25

What is hepatopulmonary syndrome?

Answer 25

Triad of:
Hepatic dysfunction
Hypoxaemia
Extreme vasodilation (intrapulmonary vascular dilatation)

Question 26

List 3 complications of ascites developing in portal hypertension

Answer 26

Spontaneous bacterial peritonitis
Hepatorenal syndrome
Hepatic hydrothorax

Question 27

List 2 pulmonary complications that may develop in portal hypertension

Answer 27

Portopulmonary HTN

Hepatopulmonary syndrome

Question 28

What is the prognosis in portal HTN?

Answer 28

Depends on the underlying CAUSE

Variceal haemorrhages have a 1-year mortality of 40%

Question 29

Why are non selective B blockers a first line drug used in management of portal HTN? Give an example

Answer 29

e.g. carvedilol
Reduce portal pressure (inhibit B2 receptors in GIT, cause splanchnic vasoconstriction, reduce portal blood flow)
Reduce rates of bleeding + re-bleeding in patients with oesophageal varices.
May protect against SBP (increase intestinal transit).

Question 30

Give 3 indications for a TIPSS procedure

Answer 30

Persistent, recurrent or tx resistant oesophageal varices
Gastric varices
Refractory ascites