Portal hypertension

Pathologically elevated pressure in the portal venous system (normal portal pressure ~5 mm Hg; clinically significant ≥10–12 mm Hg) usually due to increased resistance to portal blood flow (most often cirrhosis). It leads to diversion of blood through portosystemic collaterals (e.g., esophageal varices) and causes splenomegaly, ascites, and other complications.

• Portal hypertension underlies most major complications of cirrhosis. Variceal hemorrhage is one of the most lethal events in cirrhosis, and portal hypertensive complications (ascites, encephalopathy, etc.) account for frequent hospitalizations and mortality in liver disease. Recognizing and managing portal hypertension is crucial to improving outcomes in these patients and is a high-yield exam topic.

• Typically seen in patients with cirrhosis (e.g., due to chronic alcohol use, viral hepatitis, NASH). Key findings include ascites (abdominal fluid buildup), splenomegaly (enlarged spleen with low platelets), and dilated venous collaterals such as esophageal varices (which can bleed) and caput medusae (umbilical vein recanalization).
• Cirrhosis is by far the most common cause in the US, but think of other etiologies in the right context. Prehepatic causes: portal vein thrombosis (e.g., hypercoagulable states). Intrahepatic causes: schistosomiasis or congenital hepatic fibrosis (presinusoidal), cirrhosis (sinusoidal), veno-occlusive disease (postsinusoidal). Posthepatic causes: Budd-Chiari syndrome (hepatic vein thrombosis), severe right-sided heart failure or constrictive pericarditis (causing hepatic congestion).
• Clues: Thrombocytopenia is often an early lab finding (from hypersplenism). Imaging (ultrasound/CT) may show a nodular liver, enlarged spleen, and collateral veins (e.g., recanalized paraumbilical vein). Endoscopy in cirrhosis often reveals esophageal or gastric varices. Patients may also have signs of chronic liver disease (jaundice, spider angiomas) if cirrhosis is the cause.

1. Suspect portal hypertension in any patient with cirrhosis or unexplained splenomegaly/ascites. Obtain a Doppler ultrasound of the abdomen to assess liver morphology and portal blood flow (detects portal vein thrombosis or flow reversal).
2. Cirrhotic patients with portal hypertension should undergo endoscopic screening for varices. Noninvasive markers (e.g., liver stiffness via elastography with platelet count) can stratify risk of varices when endoscopy is deferred.
3. Hepatic venous pressure gradient (HVPG) measurement is the gold standard for quantifying portal pressure (≥6 mm Hg = portal HTN, ≥10 mm Hg = clinically significant), but it’s invasive—used selectively for diagnosis or to guide therapy efficacy.
4. If an acute variceal bleed occurs: secure the airway, stabilize circulation (IV fluids/blood), start IV octreotide (vasoconstrictor to reduce portal flow) and prophylactic antibiotics, and perform urgent endoscopic variceal ligation. If bleeding cannot be controlled endoscopically, prepare for TIPS or balloon tamponade as a bridge.
5. Identify and manage precipitating factors for decompensation: e.g., treat infections promptly (to prevent worsening portal HTN and encephalopathy), avoid NSAIDs (can precipitate renal failure in ascites), and abstain from alcohol.

1. Treat the underlying cause whenever possible: e.g., antiviral therapy for hepatitis, alcohol cessation, anticoagulation for Budd-Chiari (hepatic vein thrombosis), or antiparasitic therapy for schistosomiasis.
2. Primary prophylaxis of variceal bleeding (in cirrhosis with significant varices): nonselective β-blockers (e.g., propranolol or carvedilol) or periodic endoscopic variceal ligation (banding) to prevent first bleed.
3. Ascites management: salt restriction and oral diuretics (usually spironolactone ± furosemide). Perform large-volume paracentesis for tense or refractory ascites (with IV albumin support). Avoid NSAIDs and ACE-Is which can worsen renal perfusion in ascites.
4. TIPS (transjugular intrahepatic portosystemic shunt) is indicated for refractory ascites or recurrent variceal hemorrhage unresponsive to medical/endoscopic therapy. It creates a channel between portal and hepatic veins to reduce portal pressure (but can precipitate encephalopathy).
5. Definitive treatment for end-stage liver disease is liver transplantation, which cures the portal hypertension by replacing the diseased liver.

• Remember the classic sites of portal-caval anastomoses: "Gut, butt, and caput"—esophageal varices (gut), hemorrhoids (butt), and caput medusae (paraumbilical veins).
• In cirrhosis, platelets drop first due to splenic sequestration from portal hypertension—unexplained thrombocytopenia may be an early clue to chronic liver disease.
• Nonselective β-blockers (e.g., propranolol, nadolol) prevent variceal hemorrhage by reducing portal inflow (β1 blockade lowers cardiac output; β2 blockade causes splanchnic vasoconstriction).

• Bright red hematemesis in a known liver disease patient → suspect ruptured esophageal varices (portal hypertensive bleeding), a life-threatening emergency requiring immediate resuscitation and endoscopic intervention.
• Worsening renal function in a cirrhotic with ascites (oliguria, rising creatinine) → consider hepatorenal syndrome (renal failure due to severe portal hypertension/cirrhosis); often precipitated by infections or over-diuresis, and carries high mortality (requires specialist management and consideration for transplant).

1. Cirrhosis diagnosed → evaluate for portal hypertension complications: obtain baseline endoscopy (or noninvasive assessment) to check for varices.
2. If no varices or only small varices → periodic surveillance endoscopy as recommended. If large varices are found → initiate nonselective β-blocker therapy or schedule endoscopic variceal ligation for primary prophylaxis.
3. Manage ascites with dietary sodium restriction and diuretics (spironolactone ± furosemide). If ascites is refractory → perform repeated paracenteses; consider TIPS to control ascites if frequent paracentesis is needed.
4. Acute variceal bleed → ICU care: protect airway, volume resuscitation, IV octreotide, IV antibiotics, and emergent endoscopic ligation. Persistent bleeding despite endoscopy → perform rescue TIPS.
5. Refer patients with decompensated cirrhosis (recurrent ascites, variceal bleeds, encephalopathy) for liver transplant evaluation, as this is the only curative option for advanced portal hypertensive disease.

• Middle-aged alcoholic with ascites, caput medusae, and episodes of hematemesis/melena → Portal hypertension from cirrhosis (esophageal variceal hemorrhage).
• Young adult from an endemic region (Egypt) with massive splenomegaly, variceal bleeding, but preserved liver function tests → Schistosomiasis causing presinusoidal portal hypertension.