Portal hypertension

Question 1

Define portal hypertension

Answer 1

Abnormally high pressure within the hepatic portal vein

Clinically significant defined as hepatic venous pressure gradient >10mmHg

Question 2

Aetiology of portal hypertension

most common + 3

Answer 2

Cirrhosis
Pre hepatic
Hepatic
Post hepatic

Question 3

Aetiology of portal hypertension - pre hepatic

4

Answer 3

(blockage of portal vein before the liver)
Congenital stenosis
Portal vein thrombosis
Splenic vein thrombosis 
Extrinsic compression

Question 4

Aetiology of portal hypertension - hepatic

4

Answer 4

Cirrhosis
Chronic hepatitis
Schistosomiasis
Myeloproliferative disease

Question 5

Aetiology of portal hypertension - post hepatic

3

Answer 5

(blockage of hepatic veins or venules)
Budd-Chiari syndrome (hepatic vein obstruction)
Constrictive pericarditis
Right heart failure

Question 6

Epidemiology of portal hypertension

Answer 6

Common consequence of cirrhosis

Question 7

Presenting symptoms of portal hypertension - features of liver disease
(4)

Answer 7

Jaundice
History of alcohol abuse
Risk factors for viral hepatitis
FH (e.g. haemochromatosis)

Question 8

Presenting symptoms of portal hypertension - complications of portal hypertension
(5)

Answer 8

Haematemesis of melaena
Lethargy, irritability, changes in sleep - hepatic encephalopathy 
Abdominal distention - ascites
Abdominal pain & fever - SBP
Pulmonary involvement

Question 9

Signs of portal hypertension on physical examination

3

Answer 9

Caput medusae
Splenomegaly
Ascites

Question 10

Signs of liver failure on physical examination

9

Answer 10

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

Question 11

Investigations for portal hypertension

5

Answer 11

Bloods
Specific tests
Imaging 
HVPG (hepatic venous pressure gradient)
Liver biopsy

Question 12

Investigations for portal hypertension - bloods

5

Answer 12

LFTs
U&Es
Blood glucose
FBC
Clotting screen - prolongation of PT is one of earliest signs of liver failure

Question 13

Investigations for portal hypertension - specific tests

5

Answer 13

Ferritin - haemochromatosis 
Hepatitis serology
Autoantibodies (e.g. anti smooth muscle antibodies in autoimmune hepatitis) 
α1-antitrypsin levels 
Caeruloplasmin - Wilson’s disease

Question 14

Investigations for portal hypertension - imaging

4

Answer 14

Abdo US - check liver & spleen size & assess portal blood flow
Doppler US - assess direction of flow in blood vessels
CT/MRI - if others inconclusive
Endoscopy - check oesophageal varices

Question 15

Investigations for portal hypertension - liver biopsy

Answer 15

If indicated

Question 16

Management of portal hypertension

general + 6

Answer 16

Difficult to treat portal hypertension specifically, so focussed on underlying cause where possible

Conservative - salt restriction & diuretics
Treatment of oesophageal varices if [resent
Non selective β blockers - reduces portal pressure & risk of variceal bleeding
Terlipressin - reduce portal venous pressure
TIPS - surgical shunt between hepatic portal vein & hepatic vein to ease congestion
Liver transplant

Question 17

Complications of portal hypertension

6

Answer 17

Bleeding from oesophageal varices

Ascites -
SBP
hepatorenal syndrome
hepatic hydrothorax

Pulmonary complications -
portopulmonary hypertension
hepatopulmonary syndrome

Liver failure

Hepatic encephalopathy

Cirrhotic cardiomyopathy

Question 18

Define hepatorenal syndrome

Answer 18

Life threatening condition w/ rapid deterioration in kidney function in individuals w/ cirrhosis or fulminant liver failure

Question 19

Define hepatic hydrothorax

Answer 19

Transudative pleural effusion in patients w/ portal hypertension without any underlying primary cardiopulmonary cause

Question 20

Hepatopulmonary syndrome is a triad of:

Answer 20

hepatic dysfunction 
hypoxaemia 
extreme vasodilation (intrapulmonary vascular dilatation)

Question 21

Prognosis of portal hypertension

2

Answer 21

Depends on underlying cause

Variceal haemorrhages hae 40% 1 yr mortality