Key Facts

Portal hypertension is the main driver of complications in cirrhosis. It results from increased resistance to portal blood flow and increased splanchnic blood flow. Management focuses on reducing portal pressure and preventing/treating complications.

Epidemiology

Portal hypertension is present in virtually all patients with cirrhosis. Clinically significant portal hypertension (HVPG >10 mmHg) develops progressively. 50% of cirrhotics have varices at diagnosis. Annual rate of variceal bleeding is ~15% for large varices.

Aetiology

Pre-hepatic (portal vein thrombosis — PVT):

• Myeloproliferative disorders (JAK2 mutation), thrombophilia, pancreatitis, cirrhosis, pylephlebitis, umbilical vein catheter (neonatal)

Intrahepatic (~90%):

• Pre-sinusoidal: schistosomiasis, sarcoidosis, congenital hepatic fibrosis
• Sinusoidal: cirrhosis (ANY cause) — most common
• Post-sinusoidal: veno-occlusive disease (SOS — post-HSCT)

Post-hepatic:

• Budd-Chiari syndrome (hepatic vein thrombosis)
• Right heart failure, constrictive pericarditis
• IVC obstruction

Pathophysiology

Portal hypertension in cirrhosis has two components:

1. Increased intrahepatic resistance: structural (fibrosis, regenerative nodules, sinusoidal capillarisation) and dynamic (activated stellate cells, endothelial dysfunction with reduced NO, increased endothelin-1)
2. Increased portal blood flow: splanchnic vasodilation (excess NO in splanchnic circulation — paradox) → increased mesenteric blood flow → increased portal inflow

The resulting portal hypertension drives formation of portosystemic collaterals (varices) at sites of portal-systemic anastomosis, and contributes to ascites through splanchnic vasodilation → effective hypovolaemia → RAAS activation → sodium/water retention.

Varices

• Oesophageal varices (most important clinical consequence)
• Gastric varices (GOV1/2, IGV1 — Sarin classification)
• Rectal varices (distinct from haemorrhoids)
• Caput medusae (periumbilical collaterals — recanalized umbilical vein)
• Anorectal varices

Ascites

• Most common decompensation event
• Abdominal distension, shifting dullness, fluid wave

Splenomegaly

• Due to splenic congestion
• Hypersplenism: thrombocytopaenia, leucopaenia, anaemia

Portosystemic Encephalopathy

• Confusion, sleep-wake reversal, asterixis
• Due to portosystemic shunting of ammonia and other toxins

Red Flags

• Massive haematemesis (variceal bleed)
• Rapidly increasing ascites (portal vein thrombosis, HCC, SBP)
• New encephalopathy (infection, GI bleed, electrolyte disturbance)
• Abdominal pain with ascites (SBP, Budd-Chiari)

Bedside

• Clinical assessment: ascites, splenomegaly, caput medusae, variceal stigmata

Bloods

• FBC: thrombocytopaenia (<150 suggests CSPH), leucopaenia
• LFTs, albumin, INR: liver synthetic function
• Ascitic tap: cell count, albumin (SAAG ≥11 g/L = portal hypertension), culture, cytology

Imaging

• Doppler USS: portal vein flow direction and velocity, splenic size, ascites, PVT
• CT abdomen (portal venous phase): portal vein/hepatic vein patency, varices, HCC
• Transient elastography: liver stiffness as surrogate for CSPH (>20-25 kPa)

Special Tests

• OGD: variceal screening — grade size, identify red signs (high-risk stigmata)
• HVPG measurement: gold standard (interventional radiology); >10 mmHg = CSPH; >12 mmHg = variceal bleeding risk
• Baveno VII criteria: LSM <20 kPa AND platelets >150 = CSPH unlikely → can avoid screening OGD
• Thrombophilia screen + JAK2: if PVT suspected
• Echocardiography: if post-hepatic cause suspected

Non-pharmacological

• Variceal screening OGD: at diagnosis of cirrhosis
• Salt restriction (<5 g/day): for ascites management
• Alcohol abstinence: reduces portal pressure

Pharmacological

• Primary variceal prophylaxis (medium/large varices, no prior bleed):
  • Non-selective beta-blocker (NSBB): propranolol 40 mg BD (titrate to HR 55-60); OR carvedilol 6.25-12.5 mg OD (preferred by many — also reduces intrahepatic resistance)
  • OR variceal band ligation if NSBB intolerant/contraindicated
• Acute variceal bleed: terlipressin + antibiotics + urgent OGD band ligation (see oesophageal varices entry)
• Secondary prophylaxis: NSBB + VBL (combination most effective)
• Ascites: spironolactone ± furosemide

Surgical/Interventional

• TIPSS: creates portosystemic shunt within the liver (radiological procedure); reduces HVPG; indicated for:
  • Refractory ascites (not responsive to diuretics)
  • Recurrent variceal bleeding despite endoscopic + pharmacological therapy
  • Early TIPSS (within 72 hours) in high-risk variceal bleed (Child C 10-13 or Child B with active bleed)
  • Complications: encephalopathy (~30%), shunt dysfunction
• Surgical shunts (portocaval, splenorenal): rarely performed; TIPSS has largely replaced
• Liver transplantation: definitive cure for portal hypertension

Referral Criteria

• Hepatology referral for all patients with cirrhosis/portal hypertension
• Interventional radiology for TIPSS
• Transplant assessment if decompensated

Portal hypertension is the main determinant of prognosis in cirrhosis. Variceal bleeding mortality is ~15-20% per episode. CSPH (HVPG >10) is the threshold for decompensation risk. Reduction of HVPG to <12 mmHg (or >20% reduction) with beta-blockers virtually eliminates variceal bleeding risk. TIPSS controls refractory ascites in ~70% and variceal bleeding in ~90% but may worsen encephalopathy. Liver transplantation cures portal hypertension.

Classification of Portal Hypertension

Baveno VII Key Recommendations