Portal Hypertension

BASICS

Definition: Portal venous pressure >5 mm Hg, associated with splanchnic vasodilation, portosystemic collateral formation, and hyperdynamic circulation.
Progressive course—risk of variceal bleeding, ascites, hepatic encephalopathy, portopulmonary syndrome, hepatorenal syndrome.
Most common cause: Elevated hepatic venous pressure gradient (HVPG).

Classified by site:
Prehepatic: Portal vein thrombosis, obstruction
Intrahepatic: Cirrhosis (90%—viral, alcohol, NAFLD, schistosomiasis, Wilson, hemochromatosis, PBC, sarcoidosis)
Posthepatic: Hepatic vein thrombosis (Budd-Chiari), right heart failure
Mechanism: Resistance to portal flow → ↑ HVPG → collateral formation (esophagus, stomach, rectum, umbilicus), splanchnic vasodilation, angiogenesis
Pediatric: Portal vein thrombosis most common extrahepatic cause; intrahepatic causes include biliary atresia, viral hepatitis, metabolic liver disease

General: Pallor, icterus, clubbing, palmar erythema, splenomegaly, spider angioma, gynecomastia, testicular atrophy, caput medusae, abdominal bruit, hemorrhoids
Complications: Gastroesophageal varices, hypotension, tachycardia, ascites (fluid wave, shifting dullness), hepatic encephalopathy (confusion, asterixis, hyperreflexia)

Gold Standard: HVPG measurement (wedged hepatic vein - free hepatic vein pressure)
Key thresholds: HVPG >10 mm Hg—esophageal varices; >12 mm Hg—variceal bleeding risk
Labs: Anemia, leukopenia, thrombocytopenia (hypersplenism), hypoalbuminemia, hyperbilirubinemia, elevated LFTs, abnormal coagulation, elevated ammonia, iron deficiency anemia
Imaging: US, CT, MRI—detect cirrhosis, splenomegaly, ascites, varices. Doppler for portal flow, thrombosis.
Elastography: Assesses fibrosis/cirrhosis noninvasively.
Paracentesis: SAAG >1.1 g/dL suggests portal hypertensive ascites.
Endoscopy: Screen for esophageal/gastric varices.
Child-Pugh classification: Estimates hepatic reserve.

Primary/secondary prophylaxis for variceal bleeding:
Nonselective β-blockers: Nadolol 20–40 mg PO daily; propranolol 20–40 mg PO BID–TID; carvedilol 6.25 mg PO daily. Titrate to HR 55–60 bpm.
Acute variceal bleed:
Octreotide: 50 mcg IV bolus, then 50 mcg/hr infusion (pediatric: 1 mcg/kg bolus & infusion)
Vasopressin: 0.2–0.8 U/min IV (pediatric: 0.002–0.005 U/kg/min; max 0.01 U/kg/min)
Ascites:
Furosemide: 20–40 mg/day PO (pediatric: 1–2 mg/kg/dose)
Spironolactone: 50–100 mg/day PO (pediatric: 1–3 mg/kg/day)

Varices (no bleed): Endoscopic variceal ligation (EVL) for large varices
Variceal hemorrhage: EVL or sclerotherapy within 12 hours
Refractory ascites: Large-volume paracentesis, peritoneovenous shunt, TIPS (contraindicated in recurrent encephalopathy), liver transplant for advanced disease
Other: Balloon tamponade (rare), portacaval shunt

Acute GI bleed: Resuscitate, type and cross, correct coagulopathy, urgent endoscopy
Encephalopathy: Monitor for mental status, avoid sedatives, restrict protein if needed
Ascites/edema: Sodium and fluid restriction
Alcohol withdrawal: Monitor and treat per protocol

Diet: Sodium <2 g/day, alcohol abstinence
Monitoring: Hemoglobin, hematocrit, vitals (bleeding); mental status (encephalopathy)
Discharge criteria: No bleeding for 24h, stable H/H, hemodynamically stable, resolved encephalopathy

Variceal bleeding: 1/3 will bleed, 50% rebleed within 2 years, 15–20% mortality
Ascites: 50% 1-year survival without transplant (vs. 90% for cirrhotics without ascites)
Complications: Recurrent ascites, encephalopathy

Portal hypertension often diagnosed on physical exam with cirrhosis risk factors.
Endoscopic treatment successful for acute variceal hemorrhage in 85% of cases.
Prognosis for patients with ascites is poor without transplant (50% 1-year survival).