Portal Hypertension

Question 1

Define

Answer 1

normally high pressure within the hepatic portal vein.

NOTE: clinically significant portal hypertension is defined as a hepatic venous pressure gradient > 10 mm Hg

Question 2

Causes

Answer 2

Prehepatic: portal/splenic vein thrombosis

Hepatic: cirrhosis (80%), schistosomiasis (commonest worldwide), sarcoid, myeloproliferative disease, congenital hepatic fibrosis

Post-hepatic: Budd–Chiari syndrome (hepatic vein thrombosis), congestive cardiac failure (RH failure), constrictive pericarditis, veno-occlusive disease

Question 3

Symptoms

Answer 3

Consider chronic sings of liver failure

1.  Hematemesis
2.  PR bleeding or melaena
3.  Oesophageal varices leading to a life-threatening upper
4. GI bleed
5.  ±Encephalopathy

Question 4

Signs

Answer 4

Splenomegaly

 Ascites/oedema

 ±Caput medusa (enlarged superficial periumbilical veins)

Question 5

Investigations

Answer 5

Bloods

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

Specific Tests

• Ferritin - haemochromatosis
• Hepatitis serology
• Autoantibodies (e.g. anti-smooth muscle antibodies in autoimmune hepatitis)
• a1-antitrypsin levels
• Caeruloplasmin - Wilson’s disease

Imaging

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

Measure hepatic venous pressure gradient (HVPG)

Liver Biopsy - if indicated

Question 6

Management

Answer 6

Difficult to treat portal hypertension specifically

Treatment is mainly focused on treating the underlying cause where possible

Conservative

• Salt restriction
• 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 7

Complications

Answer 7

1. Bleeding from oesophageal varices
2. Ascites + complications of ascites:
   
   • Spontaneous bacterial peritonitis
   • Hepatorenal syndrome
   • Hepatic hydrothorax
3. Pulmonary complications
   
   • Portopulmonary hypertension
   • Hepatopulmonary syndrome - triad of:
   • Hepatic dysfunction
   • Hypoxaemia
   • Extreme vasodilation (intrapulmonary vascular dilatation)
4. Liver failure
5. Hepatic encephalopathy
6. Cirrhotic cardiomyopathy

Question 8

Prognosis

Answer 8

Depends on the underlying CAUSE

Variceal haemorrhages have a 1-year mortality of 40%