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

Management of Portal Hypertension

Portal hypertension management centers on non-selective beta-blockers (NSBBs) as first-line pharmacological therapy, endoscopic intervention for varices, and transjugular intrahepatic portosystemic shunt (TIPS) for refractory cases, with treatment stratified by clinical presentation and severity. 1

Initial Assessment and Risk Stratification

All patients require comprehensive evaluation before initiating therapy:

• Measure hepatic venous pressure gradient (HVPG) when available to stratify risk and guide therapy; clinically significant portal hypertension is defined as HVPG ≥10 mmHg 1, 2
• Perform endoscopic screening if liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) is >20 kPa or platelet count <150×10⁹/L 3
• Obtain cardiac history, examination, 12-lead ECG, and N-Terminal pro-B-type natriuretic peptide (NT-proBNP) in all patients being considered for elective TIPS 4
• Assess for covert hepatic encephalopathy using Psychometric Hepatic Encephalopathy Score (PHES) testing, Stroop testing, or Critical Flicker Frequency, as its presence is a relative contraindication to elective TIPS 4

Primary Pharmacological Management

Carvedilol 12.5 mg/day is the preferred NSBB for clinically significant portal hypertension, demonstrating superior efficacy compared to traditional NSBBs in lowering portal pressure. 1

• Traditional NSBBs (propranolol or nadolol) remain acceptable alternatives when carvedilol is unavailable or not tolerated 1
• The hemodynamic goal is to reduce HVPG to <12 mmHg or achieve ≥10-20% reduction from baseline 1
• NSBBs should be considered to prevent clinical decompensation in patients with compensated cirrhosis and clinically significant portal hypertension 3
• Consider adding simvastatin to reduce portal pressure and decrease liver-related mortality including variceal bleeding deaths 3

Critical caveat: Temporarily suspend beta-blockers in acute bleeding with hypotension (systolic BP <90 mmHg or mean arterial pressure <65 mmHg) 1

Management of Acute Variceal Bleeding

Initiate vasoactive drugs (octreotide, somatostatin analogs, or terlipressin) immediately upon suspicion of variceal hemorrhage, before endoscopy, which reduces mortality by 30 deaths per 1,000 patients. 1, 5

• Administer short-term antibiotic prophylaxis (maximum 7 days); intravenous ceftriaxone 1 g/24h is the antibiotic of choice 1
• Transfuse red blood cells conservatively: start when hemoglobin reaches 7 g/dL with goal of maintaining 7-9 g/dL, as excessive transfusion may paradoxically increase portal pressure 1
• Perform esophagogastroduodenoscopy within 12 hours of presentation once hemodynamically stable 1, 5
• Initiate proton pump inhibitors concurrently to stabilize fibrin clots and stimulate platelet aggregation 5

Early/Pre-emptive TIPS Strategy

Consider early TIPS within 72 hours for high-risk patients (Child-Pugh class C or MELD ≥19 with acute variceal bleeding), which reduces 1-year mortality (RR 0.68) and rebleeding (RR 0.28) compared to pharmacotherapy/band ligation alone. 5

• Use TIPS for recurrent variceal bleeding after medical and endoscopic intervention failure 5
• Emergency TIPS should not be delayed by echocardiogram in acute variceal hemorrhage, as echocardiogram may be inaccurate in this setting 4

Important nuance: The role of early TIPS remains somewhat controversial with conflicting evidence, and patient selection is critical 4

TIPS: Indications and Contraindications

TIPS is strongly recommended in the following clinical situations: 4

1. Acute variceal bleeding unresponsive to endoscopy with banding and drug therapy (patients with very severe liver disease unlikely to benefit)
2. Prevention of rebleeding from varices where patients have bled again despite standard endoscopic and medical therapies
3. Selected patients with refractory ascites or hepatic hydrothorax where recurrent fluid build-up persists despite other treatments (though no clear evidence of better survival)
4. Selected patients with Budd-Chiari syndrome in specialist hospitals offering liver transplantation
5. Portal vein thrombosis in selected patients at specialist centers performing large numbers of TIPS

Absolute contraindications to elective TIPS: 4, 1

• Severe left ventricular dysfunction or severe pulmonary hypertension
• Significant intrinsic renal disease (stage 4/5)
• Bilirubin >50 μmol/L
• Platelets <75×10⁹/L
• Pre-existing encephalopathy
• Active infection
• Severe cardiac failure

Relative contraindications:

• Age >65 years (increases risk of encephalopathy but not absolute contraindication) 4
• Covert hepatic encephalopathy 4

TIPS Procedural Standards

All TIPS procedures must use PTFE-covered stents (8-10 mm diameter) as they are associated with better patency rates than bare stents. 4, 3

Pre-Procedure Requirements:

• Discuss all patients in multidisciplinary team with hepatology and interventional radiology review 4
• Obtain cross-sectional imaging prior to TIPS where possible 4
• Perform under general anaesthetic or deep sedation using propofol 4
• Conduct detailed nutritional and functional assessment for elective cases 4

Intra-Procedure Standards:

• Measure portal pressure gradient between portal pressure and IVC, pre- and post-stent deployment 4
• Reduce portal pressure gradient to <12 mmHg or by ≥20% of baseline for variceal bleeding 4, 3
• For other indications, balance efficacy with risk of hepatic encephalopathy and individualize pressure reduction 4
• Base coagulopathy correction decisions on thromboelastography since INR is unreliable in liver disease 4, 5
• Consider platelet transfusion if platelet count <50×10⁵/L 4

Antibiotic Prophylaxis:

• Routine prophylactic antibiotics are NOT recommended except for TIPS performed for variceal bleeding, complex procedures, or previous biliary instrumentation 4, 5

Critical pitfall: TIPS carries enhanced risk of infection during passage through infected bile ducts and may be contraindicated with dilated intrahepatic ducts in the TIPS tract 4

Post-TIPS Monitoring

Perform Doppler ultrasound at 1 week after TIPS implantation in patients with prothrombotic conditions or suspected TIPS dysfunction. 4, 3, 5

• Routine Doppler ultrasound at 6-12 monthly intervals, or 6-monthly in patients undergoing HCC surveillance 4, 3
• Patients with prothrombotic conditions (e.g., Budd-Chiari syndrome) require careful consideration for TIPS venography as determined by interventional radiologist 4
• No indication for routine venography in other patients 4

Post-TIPS Hepatic Encephalopathy:

Post-TIPS hepatic encephalopathy occurs in up to 55% of cases, with risk factors including age >65 years, Child-Pugh score ≥10, previous encephalopathy history, larger diameter stents, and diabetes mellitus. 5

• Treat with lactulose titrated to 2-3 soft bowel movements daily as first-line therapy 3
• Add rifaximin if lactulose alone fails to control symptoms 3
• If encephalopathy continues despite standard management, consider shunt reduction, embolization, or occlusion 4

Management of Refractory Ascites

Medical management with diuretics is first-line treatment for ascites. 1

• TIPS is recommended for selected patients with refractory or recurrent ascites, provided there are no contraindications 1
• Although TIPS is effective in reducing fluid build-up, there is no clear evidence of better survival and impact on quality of life is not clear 4

Special Populations

Extrahepatic Portal Vein Thrombosis:

• Treat portal hypertension according to recommendations for cirrhotic patients with NSBBs and endoscopic therapy 1
• TIPS can be considered in selected patients at specialist centers 4

Primary Sclerosing Cholangitis (PSC):

• Management of portal hypertension-related complications should follow standard guidelines 4
• NSBBs should be used to prevent portal hypertension-related decompensation 4
• TIPS may be contraindicated with dilated intrahepatic ducts in the TIPS tract 4

Peristomal Varices:

• Develop in up to 26% of patients with ileostomies, particularly those with PSC post-colectomy 3
• Manage with local sclerotherapy, cyanoacrylate injection, microcoil embolization, or TIPS 3
• Patients requiring colectomy should preferably undergo ileal pouch-anal anastomosis rather than terminal ileostomy to prevent peristomal varix development 3

Treatment of Underlying Liver Disease

Remove the etiological factor (particularly alcohol cessation, hepatitis B or C treatment) to decrease the risk of decompensation and increase survival. 1

• Treatment of the underlying cause should be optimized but should not delay initiation of portal hypertension therapy 1
• Any effective treatment of the underlying liver disease should result in improvement of portal hypertension 4

Center Volume Requirements

Centers should perform a minimum of 10 TIPS cases annually, with complex cases reserved for centers performing ≥20 cases yearly. 3