What is the management approach for a patient with portal hypertension?

Management of Portal Hypertension

Portal hypertension management requires a systematic, risk-stratified approach prioritizing non-selective beta-blockers (NSBBs) as first-line pharmacological therapy, with transjugular intrahepatic portosystemic shunt (TIPS) reserved for specific indications including refractory variceal bleeding and refractory ascites. 1

Initial Assessment and Risk Stratification

Diagnostic Evaluation

• Perform hepatic venous pressure gradient (HVPG) measurement when available to quantify portal pressure, with clinically significant portal hypertension (CSPH) defined as HVPG ≥10 mmHg and high-risk threshold at ≥12 mmHg 1, 2
• Screen for varices via endoscopy if liver stiffness measurement (LSM) >20 kPa or platelet count <150×10⁹/L 1
• Obtain cross-sectional imaging to assess portal vein patency, collateral circulation, and hepatic architecture 3

Pre-Treatment Workup

Before initiating therapy, assess:

• Cardiac function: 12-lead ECG and NT-proBNP in all patients; proceed to echocardiogram if abnormal 3
• Hepatic encephalopathy risk: Use psychometric hepatic encephalopathy score (PHES), Stroop testing, or Critical Flicker Frequency 3
• Renal function: Stage 4/5 chronic kidney disease is a contraindication to elective TIPS 3
• Nutritional status: Detailed assessment recommended before elective procedures 3

Primary Pharmacological Management

Non-Selective Beta-Blockers (First-Line)

Carvedilol 12.5 mg/day is the preferred NSBB for CSPH, demonstrating superior portal pressure reduction compared to traditional NSBBs 1

Alternative NSBBs if carvedilol unavailable or not tolerated:

• Propranolol (titrated to heart rate reduction of 25% or resting heart rate 55-60 bpm) 1, 4
• Nadolol 1, 2

Hemodynamic goal: Reduce HVPG to <12 mmHg or achieve ≥10-20% reduction from baseline 1

Adjunctive Pharmacotherapy

• Consider simvastatin to reduce portal pressure and decrease liver-related mortality including variceal bleeding deaths 1
• Treat underlying liver disease aggressively (alcohol cessation, antiviral therapy for hepatitis B/C) as this reduces decompensation risk 1

Management by Clinical Presentation

Acute Variceal Bleeding (Emergency)

Immediate interventions (before endoscopy):

• Start vasoactive drugs immediately upon suspicion: octreotide, somatostatin analogs, or terlipressin, which reduces mortality by 30 deaths per 1,000 patients 1, 5
• Administer IV ceftriaxone 1 g/24h (maximum 7 days) for antibiotic prophylaxis 1
• Initiate proton pump inhibitors to stabilize fibrin clots 5
• Transfuse red blood cells conservatively: start at hemoglobin 7 g/dL with goal 7-9 g/dL, as excessive transfusion paradoxically increases portal pressure 1

Endoscopic management:

• Perform esophagogastroduodenoscopy within 12 hours once hemodynamically stable 1, 5
• Endoscopic band ligation (EBL) or sclerotherapy controls bleeding in up to 85% of patients 6

Rescue therapy for refractory bleeding:

• Consider early/pre-emptive TIPS within 72 hours for high-risk patients (Child-Pugh class C, MELD ≥19, or active bleeding at endoscopy), which reduces 1-year mortality (RR 0.68) and rebleeding (RR 0.28) 5
• Balloon tamponade as temporary bridge if endoscopy fails 4

Secondary Prevention of Variceal Rebleeding

Combination therapy is superior to monotherapy:

• NSBBs plus endoscopic band ligation for patients with prior variceal bleeding 3
• Elective TIPS for treatment failures or intolerance to combined medical/endoscopic therapy 3

Refractory Ascites

• First-line: Medical management with diuretics (spironolactone with or without furosemide) 1
• TIPS for selected patients with refractory or recurrent ascites who fail diuretic therapy and repeated large-volume paracentesis, though survival benefit remains unclear 3, 1

Special Populations

Budd-Chiari syndrome (BCS):

• TIPS in specialist centers offering liver transplantation 3
• Requires close Doppler ultrasound surveillance due to prothrombotic state 3

Portal vein thrombosis:

• Manage according to cirrhotic portal hypertension guidelines with NSBBs and endoscopic therapy 1
• TIPS in selected patients at high-volume specialist centers 3

Primary sclerosing cholangitis (PSC):

• May develop "non-cirrhotic" CSPH with pre-sinusoidal component 3
• HVPG may underestimate portal hypertension severity; varices can occur with HVPG <10 mmHg 3
• TIPS contraindicated if dilated intrahepatic ducts in the TIPS tract due to infection risk 3

TIPS: Indications and Technical Considerations

Established Indications (Strong Evidence)

1. Acute variceal bleeding unresponsive to endoscopic/medical therapy (rescue TIPS) 3
2. Prevention of variceal rebleeding after failure of standard therapy 3
3. Refractory ascites or hepatic hydrothorax despite optimal medical management 3
4. Budd-Chiari syndrome in transplant centers 3

Uncertain/Investigational Indications

• Early/pre-emptive TIPS within 72 hours of first variceal bleed in stable patients (conflicting data on patient selection) 3
• Idiopathic non-cirrhotic portal hypertension 3
• Prophylaxis before non-hepatic surgery 3

Absolute Contraindications

• Severe left ventricular dysfunction 3
• Severe pulmonary hypertension 3
• Stage 4/5 chronic kidney disease (for elective TIPS) 3
• Bilirubin >50 μmol/L 1
• Platelets <75×10⁹/L 1
• Pre-existing hepatic encephalopathy (relative contraindication) 3, 1
• Active infection 1

Pre-Procedure Requirements

Multidisciplinary team discussion mandatory involving hepatology and interventional radiology 3

Covert hepatic encephalopathy screening is a relative contraindication to elective TIPS 3

Age >65 years increases encephalopathy risk and should factor into eligibility decisions 3

Technical Specifications

• Use PTFE-covered stents exclusively (8-10 mm diameter) for superior patency versus bare metal stents 3, 7
• Measure portal pressure gradient pre- and post-deployment between portal vein and IVC 3
• Target portal pressure gradient <12 mmHg or ≥20% reduction from baseline for variceal bleeding 3, 7
• General anesthesia or deep sedation with propofol recommended 3

Procedural Considerations

• Prophylactic antibiotics only for: variceal bleeding indication, complex procedures, or prior biliary instrumentation 3, 5
• Base coagulopathy correction on thromboelastography, not INR (INR unreliable in liver disease) 3, 5
• Consider platelet transfusion if count <50×10⁹/L 3
• Variceal embolization role unclear; individualize based on imaging showing large varices where pressure reduction alone may be insufficient 3, 7

Post-TIPS Monitoring and Complications

Surveillance Protocol

• Doppler ultrasound at 1 week post-procedure in patients with prothrombotic conditions or suspected dysfunction 3, 7, 5
• Routine Doppler ultrasound at 6-12 month intervals (6-monthly if undergoing HCC surveillance) 3, 1, 7
• No indication for routine venography except in prothrombotic conditions like BCS 3

Hepatic Encephalopathy Management

Post-TIPS encephalopathy occurs in up to 55% of patients 5

Risk factors include:

• Age >65 years 5
• Child-Pugh score ≥10 5
• Previous encephalopathy history 5
• Larger diameter stents 5
• Diabetes mellitus 5

Treatment algorithm:

1. Lactulose titrated to 2-3 soft bowel movements daily (first-line) 7
2. Add rifaximin if lactulose fails 7
3. Consider shunt reduction, embolization, or occlusion for refractory encephalopathy 3

Other Complications

Acute complications (<5% incidence): bleeding, infection, heart failure, liver failure, kidney failure, vascular/bile duct injury 3

Critical Safety Considerations

When to Withhold Beta-Blockers

Temporarily suspend NSBBs in acute bleeding with hypotension (systolic BP <90 mmHg or MAP <65 mmHg) 1

Patients Unlikely to Benefit from TIPS

• Very severe liver disease (unable to tolerate procedural insult) 3
• Dilated intrahepatic bile ducts in TIPS tract (PSC patients) 3

Volume Center Requirements

Centers should perform minimum 10 TIPS cases annually, with complex cases reserved for centers performing ≥20 cases yearly 7

Endoscopic Surveillance Schedule

• Every 2-3 years in compensated cirrhosis 1
• Every 1-2 years in decompensated cirrhosis 1