Portal Hypertension

Overview 

Normal portal vein pressure is 5–10mmHg. Portal hypertension develops when the portal pressure is greater than 12mmHg.

Mechanism of disease

• Increased Intrahepatic resistance of blood flow through the liver due to cirrhosis
• Increased splanchnic blood flow secondary to vasodilation leading to increased portal flow through liver

Portal Vein Anatomy

• The portal vein drains blood from the GIT so that nutrition absorbed can first enter the liver
• Portal vein is formed from the superior mesenteric vein and splenic vein
• Portal vein ← Superior mesenteric vein + Splenic vein
  • Splenic vein ← inferior mesenteric vein

Complications of Portal Hypertension

• Gastroesophageal varices
• Portal Hypertensive gastropathy
• Splenomegaly
• Ascites

Management

• Treat complications above
• Tranjugular Intrahepatic portosystemic shunt (TIPS)
• Liver transplantation (definitive)

Oesophageal-gastric varices

Overview

>75% of patients with cirrhosis will eventually develop varices. Risk of variceal haemorrhage is 5-15% per year, and is related to variceal size. Risk amplified in progressive CPS and presence of red wale marks. Without secondary prophylaxis, re-bleeding occurs in 60-70%, usually within the first 2 years of index bleed. Screening is important.

Mechanism of disease

Varceal haemorrhage results from rupture of the variceal wall due to excessive wall tension. Tension opposes the variceal transmural pressure, which depends upon portal pressure and vessel size . Once variceal wall rupture occurs, the amount of bleeding is mainly related portal pressure.

Investigations

• Gastroscopy
• CT abdo
• MRI abdo

Management of varices

• Prevention – Screening those at risk with gastroscopy
  • yearly – small varices
  • 2nd yearly – no varices
• If high risk bleeding (Medium and large varices, Any size with red wale markings):
  • Betablockers – Propranolol (to reduce portal venous pressure) OR
  • Endoscopic variceal band ligation

Acute management of variceal bleed – emergency

• Resuscitation
  • Bloods – cross match (aim Hb >70)
  • Monitor
  • Fluid resuscitation
  • Contact gastroenterological/surgical team for emergency gastroscopy
• IV Proton pump inhibitors
• IV Somatostatin OR octreotide
• Consider giving vitamin K and fresh frozen plasma to correct clotting problems
• Antibiotics – oral norfloxacin OR IV ciprofloxacin
• Balloon tamponade – for those who can not get endoscopy immediately
• Endoscopy – first line
  • Repeated sclerotherapy injection
  • Endoscopic variceal band ligation
  • Tranjugular Intrahepatic portosystemic shunt (TIPS)

Ascites

Overview 

Ascites is accumulation of fluid within the peritoneal cavity. Usually 1-2L of fluid in abdomen before patient is aware of abdominal distention.

Clinical features

• Abdominal distention
• Shortness of breath
• Hepatic hydrothorax
• Malnourished
• Muscle wasting
• Fatigue and weakness

Investigation

• Ultrasound
• CT abdomen
• Paracentesis
  • Total protein and albumin
  • FBC
  • Cultures
• Bloods – LFT, FBC, EUC

Serum to albumin gradient (SAAG)

• >11 – portal hypertension
• <11 – malignant or infectious

Management

• Aim 500g of weight loss/day
• Sodium restriction
• Lactulose
• Spironolactone
  • If Urinary K+ >1 = 150mg/day
  • If Urinary K+ <1 can give more.
• Frusemide
• Paracentesis +IV albumin replacement
• TIPS (refractory ascites – not improving with diuretics)

Complication

• Spontaneous bacterial peritonitis
• Spironolactone associated hyperkalaemia

Spontaneous Bacterial Peritonitis (SBP)

• Common complication of ascites
• Usually asymptomatic
• 25% mortality
• E-coli main causative agent
• Paracentesis with high neutrophil count highly suggestive of SBP
• Treatment: cephalosporin or Pip/taz
  • Primary prophylaxis in those with low protein (<10g/L) ascites or bilirubin >50 with impaired renal function

Splenomegaly

Clinical features

• Enlarged spleen
• Thrombocytopaenia and leukopaenia

Complications

• Hypersplenism

Click here for more information on splenomegaly 

Hepatorenal syndrome

Overview

• Renal failure without renal pathology due to renal vasoconstriction
• Renal vasoconstriction leads to RAAS activation which further causes systemic vasoconstriction
• Vasodilatation in splanchnic system

Diagnosis

• Large Ascites
• High creatinine

Types of Hepatorenal syndrome

• Type I – decrease eGFR and increase in creatinine within 1-2 weeks of presentation
• Type II – decrease eGFR with increase in creatinine level but stable

Management

• Midodrine
• α-agonist
• Octreotide
• IV albumin
• Liver transplantation

Hepatic encephalopathy

Overview

• Alteration in mental status and cognitive function due to liver failure
• Clinical diagnosis

Precipitating events

• Hypokalaemia
• Infection
• Increased dietary protein load
• Electrolyte disturbances

Clinical features

• Personality change within weeks to months
• Violent OR
• Sleepy
• Ascites
• Asterixis

Mechanism of condition

• Gut-derived neurotoxins bypass liver
• Neurotoxins reach the brain

Investigations

• Ammonia (does not correlate with severity)

Management

• Identify precipitating factor and correct it
• Lactulose OR antibiotics (for those where lactulose is no good) – to promote 2-3 soft stools a day and minimise ammonia production by gut bacteria
• Zinc supplementation

Other complications

Bone disease due to Vitamin D deficiency

• The liver plays  a role in the conversion of cholecalciferol to calcidiol
  • Calicidiol then goes to kidneys to become calcitriol the active form of vitamin D
• Liver failure means the conversion from cholecalciferol to calcidiol does not occur so there is vitmain D deficiency
• Vitamin D deficiency leads to hypercalcaemia

Liver Transplantation

Indications for chronic liver disease

• Life threatening complications
  • MELD score >15
• 2 year mortality of 50% without transplant
• Diuretic resistant ascites
• Recurrent hepatin encephalopathy
• Recurrent CBP
• Recurrent or persistent gastrointestinal haemorrhage
• Intractable cholnagitis
• Hepatorenal syndrome

Stratifies severity of end-stage liver disease, for transplant planning.

Acute liver disease (King’s College Hospital Criteria)

• Paracetamol induced liver failure
  • arterial pH <7.3 OR
  • all three of the following: INR >6.5, Serum creatinine >300 and Grade III/IV encepalopathy
• Non-paracetamol induced acute liver failure
  • INR >6.5 OR
  • Three of the following five: age <11 or >40, serum bilirubin >300, jaundice to coma time of >7 days, INR >3.5 and drug toxicity

Liver Transplantation of hepatocellular carcinoma (Milan Mazzaferro criteria)

• A single nodule <5cm OR
• 3 nodules the largest of which is <3cm
• no evidence of gross vascular invasion
• No metastasis
• 4 year survival of 75%