Portal Hypertension

Question 1

Definition

Answer 1

Abnormally high pressure within the hepatic portal vein.
o NOTE: clinically significant portal hypertension is defined as a hepatic venous
pressure gradient > 10 mm Hg

Question 2

Aetiology (pre-hepatic)

Answer 2

Pre-Hepatic - blockage of the portal vein before the liver

• Congenital stenosis
• Portal vein thrombosis
• Splenic vein thrombosis
• Extrinsic compression

Question 3

Aetiology (hepatic)

Answer 3

Hepatic

• CIRRHOSIS
• Chronic hepatitis
• Schistosomiasis
• Myeloproliferative disease

Question 4

Aetiology (post-hepatic)

Answer 4

Post-Hepatic - blockage of hepatic veins or venules

• Budd-Chiari syndrome (hepatic vein obstruction)
• Constrictive pericarditis
• Right heart failure

Question 5

Epidemiology

Answer 5

A common consequence of cirrhosis

CIRRHOSIS is the most common cause

Question 6

Presenting symptoms (features of liver disease)

Answer 6

Likely to coexist with portal hypertension

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)

Question 7

Presenting symptoms (complications from portal hypertension)

Answer 7

o Haematemesis or melaena
o Lethargy, irritability, changes in sleep (hepatic encephalopathy)
o Abdominal distension (ascites)
o Abdominal pain and fever (spontaneous bacterial peritonitis)
o Pulmonary involvement

Question 8

Signs on physical examination (portal hypertension)

Answer 8

o Caput medusae
o Splenomegaly
o Ascites

Important to remember these 3

Question 9

Signs on physical examination (liver failure)

Answer 9

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 10

Investigations (bloods)

Answer 10

o LFTs
o U&Es
o Blood glucose
o FBC
o Clotting screen (prolongation of PT is one of the earliest signs of liver failure)

Question 11

Investigations (specific)

Answer 11

o Ferritin - haemochromatosis
o Hepatitis serology
o Autoantibodies (e.g. anti-smooth muscle antibodies in autoimmune hepatitis)
o 1-antitrypsin levels
o Caeruloplasmin - Wilson's disease

Question 12

Investigations (general)

Answer 12

• Imaging
o Abdominal ultrasound - check liver and spleen size and assess portal blood flow
o Doppler ultrasound - assess direction of blood flow in vessels
o CT/MRI - if other imaging methods are inconclusive
o Endoscopy - to check for oesophageal varices

• Measure hepatic venous pressure gradient (HVPG)
• Liver Biopsy - if indicated

Question 13

Management plan

Answer 13

• Difficult to treat portal hypertension specifically
• Treatment is mainly focused on treating the underlying cause where possible

• Conservative
o Salt restriction
o Diuretics

• Treatment of oesophageal varices if present

• Non-selective beta-blockers - reduces portal pressure and
reduces risk of variceal bleeding

• Terlipressin - can reduce portal venous pressure

• 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

Question 14

Possible complications

Answer 14

• Bleeding from oesophageal varices
• Liver failure
• Hepatic encephalopathy
• Cirrhotic cardiomyopathy

Question 15

Possible complications (ascites)

Answer 15

• 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

Question 16

Possible complications (pulmonary)

Answer 16

o Portopulmonary hypertension

o Hepatopulmonary syndrome - triad of:
• Hepatic dysfunction
• Hypoxaemia
• Extreme vasodilation (intrapulmonary vascular dilatation)

Question 17

Prognosis

Answer 17

• Depends on the underlying CAUSE

* Variceal haemorrhages have a 1-year mortality of 40%