What are the symptoms and management of portal hypertension?

Symptoms and Management of Portal Hypertension

Portal hypertension primarily manifests as gastrointestinal bleeding, ascites, reduced blood cell counts, gastroesophageal varices, portal hypertensive gastropathy, and portosystemic collaterals. 1

Definition and Pathophysiology

• Portal hypertension is defined as a pathological increase in portal pressure with hepatic venous pressure gradient (HVPG) >5 mmHg, with clinically significant portal hypertension (CSPH) occurring at HVPG ≥10 mmHg 2
• Portal hypertension develops through increased intrahepatic resistance and increased portal blood flow, following the hydraulic principle where "Pressure = Resistance × Flow" 2
• The gold standard for diagnosing and quantifying portal hypertension is measurement of the HVPG 2

Clinical Manifestations

Common Symptoms and Signs

• Gastrointestinal bleeding related to portal hypertension (most frequent complication) 1
• Ascites (most common complication, reduces 5-year survival from 80% to 50%) 2
• Splenomegaly and hypersplenism leading to reduced blood cell counts 1
• Gastroesophageal varices and portal hypertensive gastropathy 1
• Portosystemic collaterals detected on abdominal imaging 1
• Post-prandial abdominal pain or features of incomplete bowel obstruction related to ischemic stenosis 1

Less Common Manifestations

• Biliary symptoms (biliary pain, pancreatitis, cholecystitis) related to portal cholangiopathy 1
• Progressive cholestatic disease or recurrent bacterial cholangitis (rare) 1
• Hepatic encephalopathy (uncommon except following gastrointestinal bleeding) 1
• Subclinical encephalopathy (more common than previously suspected) 1
• Hepatic hydrothorax/pleural effusion (occurs in 0%-8% of cases) 1

Diagnosis

• HVPG measurement is the gold standard (normal: 1-5 mmHg, portal hypertension: >5 mmHg, CSPH: ≥10 mmHg) 2
• Non-invasive assessment methods include:
  • Imaging-based tests: transient elastography (sensitivity 90-96%, specificity 48-50% at 15 kPa) 2
  • Blood-based tests: APRI (56% sensitivity, 68% specificity) 2
• Diagnostic paracentesis with measurement of ascitic fluid albumin/protein, neutrophil count, culture, and amylase when ascites is present 1
• Abdominal ultrasound to evaluate liver appearance, splenomegaly, and portosystemic collaterals 1

Management Based on Disease Stage

Mild Portal Hypertension (HVPG >5 but <10 mmHg)

• Focus on treating the underlying cause of liver disease 3
• Healthy lifestyle modifications (abstain from alcohol, avoid/correct obesity) 3
• Non-selective beta-blockers (NSBBs) are ineffective at this stage 3

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

• Consider non-selective beta-blockers (NSBBs), including carvedilol 3
• Implement screening program for hepatocellular carcinoma 3
• Focus on preventing clinical decompensation 2

Management of Specific Complications

Variceal Bleeding

• Acute management: Initiate vasoactive agents and perform endoscopic therapy 2
• Combination of endoscopic therapy and pharmacological treatment improves hemostasis and reduces mortality 3
• For prevention of first bleeding:
  • NSBBs are mandatory if moderate/large varices are present 3
  • If contraindicated or intolerant to NSBBs, endoscopic band ligation is recommended 1
• For prevention of rebleeding (secondary prophylaxis):
  • Combined therapy with NSBBs plus endoscopic band ligation 1
  • TIPS (transjugular intrahepatic portosystemic shunt) for treatment failures/intolerance 1

Ascites

• Grade 1 (mild): Detectable only by ultrasound 1
• Grade 2 (moderate): Causing moderate symmetrical distension of abdomen 1
• Grade 3 (large): Causing marked abdominal distension 1
• Refractory ascites: Cannot be mobilized or recurs early despite therapy 1
  • Diuretic resistant: Unresponsive to sodium restriction and intensive diuretic treatment 1
  • Diuretic intractable: Complications preclude effective diuretic dosage 1
• TIPS is indicated for refractory ascites 3

Special Considerations

• HVPG ≥16 mmHg is independently associated with increased mortality in both compensated and decompensated cirrhosis 3
• Aggravation of esophageal varices is a major long-term problem after BRTO (balloon-occluded retrograde transvenous obliteration) for gastric varices, with rates of 27-35% at 1 year, 45-66% at 2 years, and 45-91% at 3 years 1
• Portal hypertension can occur in the absence of cirrhosis (e.g., extrahepatic portal vein obstruction, nodular regenerative hyperplasia) 1
• Liver transplantation should be considered for all patients with decompensated cirrhosis as definitive treatment 3, 2

Prognostic Factors

• Previous gastrointestinal bleeding and size of esophageal varices predict future gastrointestinal bleeding 1
• Presence of underlying prothrombotic condition predicts recurrent thrombosis 1
• Age, ascites, extension to superior mesenteric vein, and severity of underlying conditions predict mortality 1
• HVPG reduction of ≥10% after therapy is associated with decreased risk of first variceal hemorrhage 2