What are the treatment options for portal vein thrombosis or hypertension?

Treatment Options for Portal Vein Thrombosis and Portal Hypertension

Portal Vein Thrombosis in Cirrhosis

For recent (<6 months) portal vein thrombosis with >50% occlusion or involvement of the main portal vein/mesenteric vessels in cirrhotic patients, anticoagulation should be initiated unless there is active intestinal ischemia or bleeding. 1

Anticoagulation Decision Framework

Acute PVT (<6 months) with minimal obstruction (<50%):

• Monitor with serial cross-sectional imaging given high spontaneous recanalization rates 1
• Consider anticoagulation if: symptomatic PVT, worsening portal hypertension, awaiting liver transplant, or thrombus progression on imaging 1

Acute PVT (<6 months) with significant obstruction (≥50%):

• Initiate anticoagulation for partial or complete obstruction and/or involvement of multiple vascular beds 1
• Increased benefit in: involvement of >1 vascular bed, thrombus progression, liver transplant candidates, inherited thrombophilia 1
• Anticoagulation increases recanalization with odds ratio of 4.8 (95% CI, 2.7–8.7) 1

Chronic PVT (>6 months) with cavernous transformation:

• Do not anticoagulate - complete occlusion with collateralization is not responsive to anticoagulation 1

Anticoagulation Regimens

For Child-Pugh A or B cirrhosis:

• Use either DOAC or LMWH with/without VKA based on patient preference 1
• DOACs show 87% recanalization vs 44% with VKA, with major bleeding RR 0.29 (95% CI, 0.08–1.01) 1

For Child-Pugh C cirrhosis:

• Use LMWH alone (or as bridge to VKA in patients with normal baseline INR) 1

Thrombocytopenia considerations:

• Do not withhold anticoagulation for moderate thrombocytopenia 1
• Case-by-case decision when platelet count <50 × 10⁹/L based on thrombus extent, progression risk, patient preference, and active bleeding risk 1

Duration:

• Continue anticoagulation for at least 3-6 months 2
• Lifelong if underlying permanent pro-coagulant condition or thrombosis extending to mesenteric veins 2

Bleeding Risk Management

Critical point: Portal hypertension complications (varices, ascites), when adequately treated, are NOT contraindications to anticoagulation 1

• Perform prophylaxis with band ligation if high-risk varices present before anticoagulation 1
• Traditional anticoagulants show portal hypertension bleeding 9.3% vs 13.9% without treatment (P=0.12) 1
• Mortality hazard ratio with anticoagulation: 0.59 (95% CI, 0.49–0.70) 1

TIPS for PVT

PVT should not be an absolute contraindication to TIPS, though cavernoma has high failure rates 1

• Technical success rate: 86.7% (95% CI, 78.6%-92.1%) 1
• Portal vein recanalization after TIPS: 84.4% (95% CI, 78.4%-89.0%) 1
• Mean portosystemic gradient reduction: 14.5 mmHg 1
• Hepatic encephalopathy rate: 41% 1

For acute extensive PVT with bowel ischemia:

• Consider TIPS combined with local thrombolysis 3
• In non-cirrhotic patients, 3 of 4 treated with this approach survived >6 years 3

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Portal Hypertension Management

Primary Prophylaxis of Variceal Bleeding

Non-selective beta-blockers (NSBBs) are first-line for primary prophylaxis, preferred over endoscopic band ligation 4

• NSBBs reduce portal pressure and prevent other portal hypertension complications 4
• Target HVPG reduction to ≤12 mmHg or ≥20% reduction from baseline 4
• NSBBs are ineffective in compensated cirrhosis with mild portal hypertension (HVPG <10 mmHg) 4

Endoscopy screening criteria:

• Perform endoscopy if liver stiffness >20 kPa or platelet count <150 × 10⁹/L 1

Acute Variceal Bleeding

Immediate vasoactive agents followed by endoscopic therapy is mandatory 4

1. Start vasoactive agent immediately (octreotide, terlipressin, or somatostatin) 1, 4

   • Terlipressin is more effective than octreotide with longer half-life and fewer adverse effects 4
   • Combination therapy achieves 77% 5-day hemostasis vs 58% with endoscopy alone 1, 4

2. Add prophylactic antibiotics - reduces mortality (RR 0.73), bacterial infections (RR 0.40), and rebleeding (RR 0.53) 1

3. Perform endoscopic therapy (EVL or sclerotherapy) once patient stabilized 1

   • EVL and sclerotherapy equally efficacious with 85-90% initial control 1

Secondary Prophylaxis (Prevention of Rebleeding)

Combined NSBBs plus endoscopic band ligation significantly decreases rebleeding compared to monotherapy 4

TIPS Indications

Early/pre-emptive TIPS within 72 hours for high-risk patients:

• Child-Pugh C or MELD ≥19 4
• MELD >18 has 80.9% sensitivity and 69.4% specificity for predicting 90-day mortality 1

TIPS for refractory complications:

• Gastroesophageal variceal bleeding refractory to endoscopic and drug therapy 4
• Refractory or recurrent ascites 4
• Hepatic hydrothorax (selected patients) 1, 4

TIPS contraindications:

• Bilirubin >50 μmol/L 4
• Platelets <75 × 10⁹/L 4
• Pre-existing encephalopathy 4
• Active infection 4
• Severe cardiac failure or severe pulmonary hypertension 4

Pre-TIPS evaluation:

• Screen for covert and overt encephalopathy using ≥2 of: PHES testing, Stroop testing, Critical Flicker Frequency, Spectral Enhanced/quantitative EEG 1
• Cardiac history, examination, 12-lead ECG, and NT-proBNP mandatory 1
• Age >65 increases encephalopathy risk but not absolute contraindication 1

Post-TIPS management:

• Hepatic encephalopathy affects approximately one-third of patients 4
• If severe encephalopathy persists, consider shunt reduction, embolization, or occlusion 1, 4

Special Considerations

Budd-Chiari Syndrome:

• Initiate anticoagulation immediately and continue indefinitely 5
• LMWH for 5-7 days, then VKA targeting INR 2-3 5
• Angioplasty/stenting for focal stenoses 5
• TIPS after failure of medical treatment or when angioplasty ineffective 5

Idiopathic Non-Cirrhotic Portal Hypertension:

• TIPS indications similar to cirrhosis, use covered stents 1
• Selection criteria similar to cirrhosis with attention to encephalopathy risk factors 1