Portal Hypertension

Definition

Causes / Classification

1. Prehepatic (Extrahepatic)

2. Intrahepatic

• Schistosomiasis (S. mansoni, S. japonicum) — eggs deposit in presinusoidal portal venules → granulomatous fibrosis; common in sub-Saharan Africa, Middle East, Asia
• Congenital hepatic fibrosis
• Nodular regenerative hyperplasia
• Idiopathic portal hypertension / hepatoportal sclerosis (more common in India, Japan)
• Sarcoidosis, myeloproliferative disorders, graft-versus-host disease
• Primary biliary cholangitis (early stage — presinusoidal; cirrhotic stage — sinusoidal)

• Cirrhosis (all causes) — the leading cause worldwide
  • Alcohol-associated liver disease
  • Viral hepatitis (HBV, HCV)
  • NAFLD/MASLD
  • Primary biliary cholangitis (late stage)
  • Autoimmune hepatitis
  • Primary sclerosing cholangitis, haemochromatosis
• Infiltrative liver diseases

• Sinusoidal obstruction syndrome (hepatic veno-occlusive disease)

3. Posthepatic (Extrahepatic)

• Budd-Chiari syndrome (hepatic vein obstruction)
• Inferior vena cava web or thrombosis
• Congestive heart failure / constrictive pericarditis → cardiac cirrhosis
• Severe tricuspid regurgitation

Most common cause in the United States and Europe: Cirrhosis (intrahepatic sinusoidal).

Pathophysiology

1. Increased intrahepatic resistance — structural (fibrosis, nodule formation distorting sinusoidal architecture) + dynamic (hepatic stellate cell contraction, reduced intrahepatic nitric oxide)
2. Increased portal blood flow — splanchnic arteriolar vasodilation (mediated by nitric oxide, prostacyclin, glucagon) → hyperdynamic circulation → further raises portal pressure

• Portosystemic collateral formation (varices)
• Splanchnic vasodilation → activation of RAAS + SNS → renal sodium retention → ascites
• Systemic inflammation via bacterial translocation (PAMPs/DAMPs)

Clinical Features

Consequences of Portosystemic Collateral Formation

• Gastroesophageal varices — ~30% of compensated and ~60% of decompensated cirrhotics; supplied mainly by the left gastric (coronary) vein
• Anorectal varices — present in ~45% of cirrhotics (distinguish from haemorrhoids, which do not communicate with portal system)
• Caput medusae — recanalization and dilation of the umbilical vein → visible periumbilical collaterals
• Splenorenal and mesocaval shunts — spontaneous but ineffective at decompression

Splenomegaly & Hypersplenism

• Enlarged, tortuous splenic vessels
• Functional hypersplenism: leukopenia, thrombocytopenia, anaemia (pancytopenia)

Ascites

• Combination of portal hypertension + hepatocyte dysfunction
• Sodium retention (RAAS/SNS activation) drives fluid accumulation

Systemic Complications (Full Spectrum)

Management

A. Primary Prevention of Variceal Bleeding (Prophylaxis)

• Non-selective β-blockers (propranolol, nadolol, carvedilol) — reduce portal pressure; meta-analyses show ~45% reduction in index variceal bleed and ~50% decrease in bleeding mortality
• ~20% of patients do not respond; ~20% cannot tolerate

• Variceal band ligation (VBL) — recommended for medium-to-large varices; performed every 1–2 weeks until obliteration, then EGD at 1–3 months, then every 6 months surveillance

B. Acute Variceal Haemorrhage

1. Resuscitation — ICU admission; target Hb ~8 g/dL (avoid over-transfusion — worsens portal pressure and rebleeding); cautious use of FFP and platelets for severe coagulopathy
2. Antibiotic prophylaxis — ceftriaxone 1 g/day IV; reduces bacterial infection risk and improves survival (SBP accounts for ~50% of infections in this setting)
3. Vasoactive drugs (initiate immediately on clinical suspicion):
   • Octreotide (somatostatin analogue) — preferred agent; can be administered for ≥5 days; reduces splanchnic blood flow
   • Terlipressin (where available) — most potent vasoconstrictor
   • Note: vasopressin limited by systemic side effects (hypertension, MI, arrhythmias, ischaemia)
4. Endoscopy (EGD) — as soon as feasible; VBL is first-line; combination of pharmacologic + endoscopic therapy improves 5-day haemostasis
5. Balloon tamponade (Sengstaken-Blakemore tube) — for refractory bleeding when endoscopy fails; controls bleeding in up to 90%; limited to ≤36 hours due to risk of aspiration, airway obstruction, and oesophageal perforation
6. TIPSS — for refractory or recurrent bleeding not controlled by endoscopic/medical therapy (see below)

C. Secondary Prevention (Post-Bleed)

• If left untreated, 70% of patients rebleed within 2 years
• Combination of non-selective β-blocker + repeated VBL is standard
• TIPSS — superior to endoscopic/pharmacologic therapy for secondary prevention; rebleeding occurs in only 5–15% post-TIPS (usually due to stent occlusion)

D. Ascites Management

• Low-sodium diet + diuretics (spironolactone ± furosemide)
• Large-volume paracentesis (LVP) with albumin infusion for refractory ascites
• TIPSS — for refractory ascites (see below)

TIPSS (Transjugular Intrahepatic Portosystemic Shunt/Stent)

What It Is

Technique

1. Access via the right internal jugular vein under fluoroscopic guidance
2. Guidewire and sheath advanced through SVC → IVC → hepatic vein
3. Contrast injected to locate the portal vein; needle advanced through liver parenchyma to create a transhepatic tract
4. Angioplasty balloon dilates the tract
5. PTFE (polytetrafluoroethylene)-covered stent deployed — now the current standard (higher patency vs. uncovered stents)

Indications

Contraindications

• Congestive heart failure
• Severe tricuspid regurgitation
• Severe pulmonary hypertension (mean pulmonary pressure >45 mmHg)
• Uncontrolled systemic sepsis
• Severe pre-existing hepatic encephalopathy
• Multiple hepatic cysts / unrelieved biliary obstruction
• Primary prevention of variceal bleeding (not indicated in asymptomatic patients)

• MELD score ≥18 (significantly higher post-procedure mortality; some centres use ≥25 as the threshold)
• Serum bilirubin >3 mg/dL
• Serum sodium ≤130 mEq/L
• Portal or hepatic vein thrombosis
• Centrally located hepatic mass
• Moderate pulmonary hypertension
• Pre-existing hepatic encephalopathy (relative — predictor of mortality)

Outcomes & Patency

• 1-year patency: ~93%, 3-year patency: ~75% with PTFE-covered stents
• Rebleeding in 5–15% post-TIPS; most due to stent stenosis or thrombosis
• Ascites recurrence: 42% TIPS vs. 89% paracentesis
• Transplant-free survival at 6/12/24/36 months with TIPS: 75%/63%/49%/38% vs. 65%/53%/35%/29% with paracentesis
• Earlier TIPS placement (before waiting for transplant) associated with improved 1-year transplant survival (93% vs. 53%)

Complications

TIPSS vs. Surgical Shunts

• TIPS can be completely removed with the explanted liver at transplantation — advantage over surgical shunts, which require ligation/revision intraoperatively
• In noncirrhotic portal hypertension or well-compensated cirrhosis where TIPS is not feasible, surgical portosystemic shunts remain effective (>90% success in controlling variceal bleeding)
• Parallel TIPS (double TIPS) has been described for TIPS occlusion or Budd-Chiari syndrome refractory to single shunting

Stent Size

• Smaller diameter stents (8–10 mm) are associated with lower incidence of hepatic encephalopathy post-TIPSS without reducing ascites clearance — a useful approach in patients at high encephalopathy risk

Summary: TIPSS at a Glance