What is the treatment for cirrhosis with portal hypertension?

Treatment of Cirrhosis with Portal Hypertension

The cornerstone of managing cirrhosis with portal hypertension is treating the underlying liver disease, implementing non-selective beta-blockers (NSBBs) for patients with clinically significant portal hypertension and varices, and using transjugular intrahepatic portosystemic shunt (TIPS) for refractory complications. 1, 2

Stage-Based Treatment Algorithm

Stage 1: Mild Portal Hypertension (HVPG 5-10 mmHg)

• Focus exclusively on treating the underlying etiology - this is the only effective intervention at this stage 1, 3
• Remove causative factors: alcohol cessation, antiviral therapy for hepatitis B/C, weight loss for NAFLD, immunosuppression for autoimmune hepatitis 1
• Do NOT initiate NSBBs - they are ineffective because the hyperdynamic circulatory state has not yet developed and increase adverse events without benefit 1, 2, 4
• Perform screening endoscopy to establish baseline variceal status 1, 2

Stage 2: Clinically Significant Portal Hypertension Without Varices (HVPG ≥10 mmHg)

• Continue aggressive etiologic treatment - this remains the primary intervention 1
• NSBBs are NOT recommended for preventing varix formation - a large placebo-controlled trial showed no benefit and 18% vs 6% serious adverse events with timolol 1, 2
• The treatment goal shifts from preventing varices to preventing clinical decompensation (ascites, encephalopathy, bleeding) 1, 2
• Repeat endoscopy every 2-3 years in compensated cirrhosis, every 1-2 years if decompensated 1

Stage 3: Compensated Cirrhosis with Small Varices (F1)

• Initiate NSBBs ONLY if high-risk features present: Child-Pugh B/C disease OR red color signs on varices 2
• If no high-risk features: continue surveillance endoscopy annually (ongoing liver injury) or every 2 years (quiescent disease) 1
• Carvedilol is superior to traditional NSBBs (propranolol/nadolol) - achieves hemodynamic response in 50-75% vs 46%, target dose 12.5 mg/day 2, 4
• Carvedilol reduces portal pressure through both beta-blockade (decreased cardiac output) and alpha-1 blockade (decreased intrahepatic resistance) 1, 2

Stage 4: Compensated Cirrhosis with Large Varices (F2/F3) or High-Risk Small Varices

• Initiate NSBBs immediately - this is the standard of care for primary prophylaxis 1, 2
• Carvedilol 12.5 mg/day is first-line; propranolol or nadolol are acceptable alternatives if carvedilol unavailable or not tolerated 2, 4
• Target hemodynamic response: HVPG reduction ≥20% from baseline or to <12 mmHg 1, 5
• Hemodynamic responders have dramatically improved outcomes: 95% vs 52% 8-year survival, reduced risk of rebleeding, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and encephalopathy 5
• Continue NSBBs indefinitely unless contraindications develop 1, 2

Stage 5: Decompensated Cirrhosis with Complications

For Acute Variceal Bleeding:

• Initiate vasoactive drugs immediately when variceal hemorrhage suspected - before endoscopy 2, 4
• Options: octreotide, somatostatin, or terlipressin (terlipressin more effective with longer half-life) 4
• Perform endoscopy within 12 hours once hemodynamically stable, with endoscopic variceal ligation (EVL) if varices confirmed 2
• Administer prophylactic antibiotics (maximum 7 days) - ceftriaxone 1g IV daily is first choice - reduces mortality, infections, and rebleeding 2, 4
• Transfuse red blood cells conservatively: start at hemoglobin 7 g/dL, maintain 7-9 g/dL - excessive transfusion paradoxically increases portal pressure 2, 4
• Continue vasoactive drugs for 2-5 days post-endoscopy, then transition to oral NSBBs 2
• For secondary prophylaxis: combine NSBBs + EVL - significantly superior to monotherapy 4

For Refractory Variceal Bleeding:

• TIPS is strongly recommended when bleeding fails endoscopic and medical therapy 2, 4, 6
• Consider early/preemptive TIPS within 72 hours in high-risk patients: Child-Pugh C or MELD ≥19 2, 4, 6

For Refractory Ascites:

• TIPS is recommended for selected patients with refractory or recurrent ascites 4, 6
• Long-term albumin administration and norfloxacin prophylaxis may reduce further decompensation 7
• Diuretics with sodium restriction remain first-line for uncomplicated ascites 7

For Hepatic Hydrothorax:

• TIPS may be considered, though further comparative studies needed 4

Critical Safety Considerations and Contraindications

NSBB Safety:

• Temporarily suspend NSBBs in acute bleeding with systolic BP <90 mmHg or MAP <65 mmHg 2
• Use extreme caution or suspend in refractory ascites with hypotension 2
• Absolute contraindications: severe bradycardia, heart block, severe asthma, decompensated heart failure 2

TIPS Contraindications:

• Bilirubin >50 μmol/L, platelets <75×10⁹, pre-existing encephalopathy, active infection, severe cardiac failure, severe pulmonary hypertension 4
• Hepatic encephalopathy occurs in approximately one-third post-TIPS - most respond to medical therapy, but severe cases may require TIPS reduction or occlusion 4

Coagulation Management:

• Do NOT routinely correct coagulation abnormalities before prophylactic band ligation in stable cirrhotic patients - provides no benefit 2
• Do NOT use tranexamic acid in active variceal bleeding - contraindicated 2

Monitoring and Surveillance

• HVPG monitoring is not routinely recommended outside clinical trials - changes in heart rate and noninvasive tests do not correlate with HVPG changes 1
• When HVPG available, target reduction to ≤12 mmHg or ≥20% from baseline 4, 5
• Endoscopic surveillance intervals: every 2-3 years (compensated, quiescent disease), every 1-2 years (decompensated or ongoing injury) 1

Definitive Treatment

• Liver transplantation should be considered in all patients with decompensated cirrhosis - it is the most effective approach to reduce portal pressure and improve survival 2, 3