Portal hypertension

Question 1

Define:

Answer 1

• Abnormally high pressure within the hepatic portal vein.

Clinically significant if the hepatic venous pressure gradient is > 10mmHg

Question 2

Main cause:

Answer 2

Most common cause is Cirrhosis. There is deposition of collagen and fibrosis. Retention of vasoactive substances such as NO and sodium retention all leading to an increased pressure.

Question 3

Pre-hepatic causes:

Answer 3

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

Question 4

Post-hepatic causes:

Answer 4

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

Question 5

hepatic causes:

Answer 5

CIRRHOSIS
•	Chronic hepatitis 
•	Schistosomiasis
•	Granulomata 
•	Myeloproliferative disease

Question 6

epidemiology:

Answer 6

common complication of cirrhosis

Question 7

Symptoms:

Answer 7

•	Features of Liver Disease
o	Jaundice 
o	History of alcohol abuse 
o	Risk factors for viral hepatitis (e.g. tattoos, unprotected sex, IV drug use, travel abroad and blood transfusion) 
o	Family history (e.g. haemochromatosis)

Complications of Portal Hypertension
o Haematemesis or melaena
o Lethargy, irritability, changes in sleep (hepatic encephalopathy)
o Abdominal distension (ascites)
o Abdominal pain and fever (spontaneous bacterial peritonitis)

Question 8

Signs:

Answer 8

Specific to portal hypertension:

• Caput medusa
• Splenomegaly
• Ascites

Other signs of chronic liver disease:
o	Jaundice 
o	Spider naevi
o	Palmar erythema
o	Confusion 
o	Asterixis 
o	Fetor hepaticus 
o	Enlarged or small liver 
o	Gynaecomastia 
o	Testicular atrophy

Question 9

Investigations:

Answer 9

Bloods - FBC, U+E’s, LFTs, glucose, clotting screen

Hepatitis serology

Ferritin for haemochromatosis

Autoantibodies (e.g. anti-smooth muscle antibodies in autoimmune hepatitis

Caeruloplasmin - Wilson’s disease

A1-antitrypsin levels

Imaging - Abdo US, Doppler US, CT/MRI, endoscopy for varices

Hepatic venous pressure gradient

Liver biopsy

Question 10

management:

Answer 10

• Immediate treatment: terlipressin (ADH agonist) and prophylactic antibiotics
• Endoscopy is done within 12h to diagnose and treat using band ligation or injection sclerotherapy
• If insufficient: TIPS
Transjugular Intrahepatic Portosystemic Shunt (TIPS) - surgical shunt placed between the hepatic portal vein and the hepatic vein to ease congestion in the portal vein
• Liver transplant
• Beta blockers used for prophylaxis of variceal bleed

Question 11

complications:

Answer 11

•	Bleeding from oesophageal varices 
•	Ascites + complications of ascites:
o	Spontaneous bacterial peritonitis 
o	Hepatorenal syndrome 
•	DEFINITION: a life-threatening condition that consists of a rapid deterioration in kidney function in individuals with cirrhosis or fulminant liver failure
o	Hepatic hydrothorax
•	DEFINITION: a transudative pleural effusion in patients with portal hypertension without any underlying primary cardiopulmonary cause
•	Pulmonary complications 
o	Portopulmonary hypertension
o	Hepatopulmonary syndrome - triad of:
•	Hepatic dysfunction
•	Hypoxaemia
•	Extreme vasodilation (intrapulmonary vascular dilatation)
•	Liver failure 
•	Hepatic encephalopathy
•	Cirrhotic cardiomyopathy

Question 12

prognosis:

Answer 12

depends on the cause

if bleeding oesophageal varices (usually form at porto-systemic anastamoses) then 1 year mortality of 40%