Portal Hypertension

Related Subjects: |Chronic liver disease |Cirrhosis |Alkaline phosphatase (ALP) |Liver Function Tests |Ascites Assessment and Management |Budd-Chiari syndrome |Autoimmune Hepatitis |Primary Biliary Cirrhosis |Primary Sclerosing Cholangitis |Wilson disease |Hereditary Haemochromatosis |Alpha-1 Antitrypsin (AAT) deficiency |Non alcoholic steatohepatitis (NASH) |Spontaneous Bacterial Peritonitis |Alcoholism and Alcoholic Liver Disease |Liver Transplantation

ℹ️ About Portal Hypertension

• Portal hypertension means abnormally increased pressure within the portal venous system.
• It is usually assessed indirectly using the hepatic venous pressure gradient (HVPG).
• Normal HVPG: ≤5 mmHg.
• Portal hypertension: HVPG ≥6 mmHg.
• Clinically significant portal hypertension (CSPH): HVPG ≥10 mmHg — associated with development of varices, ascites, and hepatic decompensation.
• Variceal bleeding risk: usually rises once HVPG reaches ≥12 mmHg.
• ⚠️ HVPG mainly reflects sinusoidal and post-sinusoidal portal hypertension, so it may be normal in prehepatic or presinusoidal disease despite clinically important portal hypertension.

🫀 Anatomy of the Portal System

• The inferior mesenteric vein usually joins the splenic vein, and the splenic vein joins the superior mesenteric vein to form the portal vein.
• Main collateral sites:
  • 🩸 Oesophageal and gastric varices
  • 🟣 Rectal varices
  • 🌐 Paraumbilical veins / caput medusae

📊 Classification & Causes of Portal Hypertension

Practical point: In UK practice, cirrhosis is by far the commonest cause. In many endemic regions, schistosomiasis remains a major global cause of presinusoidal portal hypertension. :contentReference[oaicite:3]{index=3}

⚙️ Pathophysiology

• Portal pressure broadly follows the rule: Pressure = Flow × Resistance.
• In cirrhosis, portal hypertension reflects:
  • ↑ intrahepatic resistance from fibrosis, regenerative nodules, and endothelial dysfunction
  • ↑ splanchnic blood flow due to vasodilation, especially nitric oxide-mediated
• This leads to:
  • 🩸 Varices and collateral formation
  • 💧 Ascites
  • 🫏 Splenomegaly and hypersplenism
  • 🧠 Hepatic encephalopathy from portosystemic shunting

🩺 Clinical Features

• 🫏 Splenomegaly is common and may cause hypersplenism with thrombocytopenia, leukopenia, and sometimes anaemia.
• 💧 Ascites is a major marker of decompensation.
• 🩸 Varices may be asymptomatic until bleeding occurs.
• 🟣 Rectal varices may occur but should not simply be equated with ordinary haemorrhoids.
• 🌐 Dilated abdominal wall veins or a recanalised paraumbilical vein may be seen.
• 🚨 Decompensation features include:
  • Variceal haemorrhage
  • Ascites
  • Encephalopathy
  • Jaundice

🔎 Investigations

• Blood tests: FBC, LFTs, INR, albumin, renal profile, viral serology, and HCC surveillance where appropriate.
• Ultrasound with Doppler: first-line to assess liver morphology, splenomegaly, portal vein patency, flow direction, and ascites.
• Transient elastography: increasingly important for non-invasive risk stratification in compensated cirrhosis.
• CT or MRI: useful for thrombosis, tumour, collaterals, anatomy, and pre-TIPS work-up.
• Upper GI endoscopy (EGD): gold standard for detecting and grading varices when indicated.
• HVPG: invasive gold standard for sinusoidal portal hypertension; useful for prognosis and treatment response in cirrhosis.

📏 Non-invasive Diagnosis of CSPH

• Baveno VII supports using non-invasive tests in compensated advanced chronic liver disease.
• Rule-out CSPH: liver stiffness ≤15 kPa and platelets ≥150 ×10⁹/L.
• Rule-in CSPH: liver stiffness ≥25 kPa in many patients with viral/alcohol-related cirrhosis or non-obese MASLD/MASH.
• Patients on non-selective beta-blockers for primary prophylaxis do not usually need serial screening endoscopy purely for variceal surveillance.

💊 Management

• 🎯 Treat the cause wherever possible: alcohol abstinence, antiviral therapy, MASLD management, thrombosis treatment in selected cases, etc.
• Primary prevention of decompensation / first bleed
  • In compensated cirrhosis with CSPH, carvedilol is increasingly preferred because it reduces portal pressure and may prevent decompensation.
  • Propranolol is also used.
  • Endoscopic variceal ligation (EVL) is an option when beta-blockers are contraindicated or not tolerated, especially for high-risk varices.
• Acute variceal bleeding
  • 🩸 Resuscitation with restrictive transfusion strategy where appropriate
  • 💉 Start vasoactive therapy (e.g. terlipressin or octreotide analogue depending on setting)
  • 🦠 Give prophylactic antibiotics
  • 🔎 Urgent endoscopy for band ligation
  • ⚠️ Consider pre-emptive TIPS in selected high-risk patients
• Secondary prophylaxis
  • NSBB + EVL is standard after a variceal bleed
  • TIPS if rebleeding occurs or bleeding is difficult to control
• Ascites
  • 🧂 Salt restriction
  • 💊 Spironolactone ± furosemide
  • 💧 Large-volume paracentesis + albumin for tense ascites
  • 🔁 Consider TIPS in refractory cases
• Encephalopathy
  • 🧠 Lactulose ± rifaximin
  • Search for precipitating factors such as bleed, infection, constipation, renal dysfunction
• Advanced disease
  • 🧬 Consider liver transplant assessment in decompensated cirrhosis

Cases - Portal Hypertension Examples

• Case 1 – Variceal bleeding 🩸: A 55-year-old man with alcohol-related cirrhosis presents with haematemesis and hypotension. Endoscopy confirms bleeding oesophageal varices. Management: resuscitation, terlipressin, prophylactic antibiotics, urgent band ligation, and consideration of early TIPS if high risk.
• Case 2 – Ascites 💧: A 60-year-old woman with HCV cirrhosis develops increasing abdominal distension and oedema. Ultrasound shows ascites and portal hypertension. Management: salt restriction, diuretics, diagnostic paracentesis, and therapeutic drainage if tense.
• Case 3 – Hypersplenism 🫏: A 48-year-old man with MASLD cirrhosis is found to have thrombocytopenia and splenomegaly. This likely reflects portal hypertension with hypersplenism. Management: optimise underlying liver disease, assess for CSPH and varices, and continue surveillance.
• Case 4 – Encephalopathy 🧠: A 62-year-old man becomes confused after a recent variceal bleed. He has asterixis and decompensated cirrhosis. Management: lactulose, rifaximin if needed, and treatment of the precipitant.

Teaching Point 🩺:
Portal hypertension is most commonly due to cirrhosis in UK practice. The major complications are varices/bleeding, ascites, encephalopathy, and hypersplenism. HVPG is the haemodynamic gold standard for sinusoidal disease, but elastography + platelet count now allow non-invasive CSPH assessment in many compensated patients. Modern management centres on carvedilol or propranolol, treatment of acute bleeding, management of ascites, and TIPS/transplant when disease progresses.

📚 References

• Baveno VII Consensus – portal hypertension and non-invasive diagnosis
• AASLD Practice Guidance – portal hypertension and varices in cirrhosis
• NICE NG50 / 2023 update – cirrhosis and prevention of decompensation
• BSG guideline – variceal haemorrhage in cirrhosis