Cirrhosis and Portal Hypertension

Cirrhosis Fundamentals - Liver's Last Stand

• Definition: Irreversible end-stage liver disease; diffuse fibrosis, regenerative nodule formation, disrupting liver architecture & vascular integrity.
• Etiology (India):
  • Alcohol (most common)
  • Viral Hepatitis (HBV, HCV)
  • NAFLD/NASH
  • Others: Autoimmune, Biliary (PBC, PSC), Metabolic (Wilson's, Hemochromatosis).
• Pathophysiology: Chronic injury → hepatocyte damage → stellate cell (Ito cell) activation → ↑collagen & ECM deposition → bridging fibrosis, regenerative nodules → impaired liver function (synthetic, detoxification), portal hypertension.

⭐ Hepatic stellate cells (Ito cells), in space of Disse, are key drivers of fibrosis, producing excess collagen.

Portal Hypertension - Pressure Cooker Inside

• Elevated portal pressure; normal Hepatic Venous Pressure Gradient (HVPG) 1-5 mmHg.
• PHT diagnosed if HVPG > 5 mmHg.
  • HVPG = Wedged Hepatic Venous Pressure (WHVP) - Free Hepatic Venous Pressure (FHVP).
  • Clinically Significant (CSPH): HVPG ≥ 10 mmHg; predicts varices, decompensation.
  • Severe: HVPG ≥ 12 mmHg; predicts variceal bleeding.
• Pathophysiology: ↑ Intrahepatic resistance (structural: fibrosis, nodules; dynamic: ↓NO, ↑Endothelin-1) + ↑ Splanchnic inflow.
• Most common cause: Cirrhosis (sinusoidal PHT).

⭐ HVPG measurement is crucial: values ≥ 10 mmHg indicate clinically significant portal hypertension, a threshold for varices development and other complications.

Complications Unveiled - When It All Goes South

Decompensated cirrhosis presents with life-threatening issues:

• Variceal Hemorrhage: Rupture of esophageal/gastric varices. High mortality.
  • Acute Mgmt: ABCs, octreotide, antibiotics, urgent endoscopy (banding). TIPS for refractory.
  • Prophylaxis: NSBBs, EBL.
• Ascites: Pathologic fluid in peritoneal cavity. SAAG > 1.1 g/dL (portal HTN).
  • Mgmt: Na+ restriction, diuretics (spironolactone ± furosemide), paracentesis (+ albumin if LVP >5L).
  • Spontaneous Bacterial Peritonitis (SBP): Infection of ascitic fluid.
    • Dx: Ascitic fluid PMN > 250/mm³; culture. Often subtle signs.
    • Mgmt: Empiric 3rd gen cephalosporins (e.g., cefotaxime).

    ⭐ SBP prophylaxis (e.g., norfloxacin) for high-risk: prior SBP, ascitic protein < 1.5 g/dL, GI bleed, Child-Pugh ≥ 9 & bilirubin > 3 mg/dL.

• Hepatic Encephalopathy (HE): Reversible neuropsychiatric syndrome (↑ammonia).
  • Precip: Infection, GI bleed, constipation.
  • Mgmt: Lactulose, rifaximin.
• Hepatorenal Syndrome (HRS): Functional acute kidney injury.
  • Type 1: Rapid (Cr ↑ > 2.5 mg/dL or CrCl ↓ < 20 mL/min in <2 wks).
  • Mgmt: Vasoconstrictors + IV albumin. Transplant definitive.
• Other: Hepatopulmonary Syndrome (HPS - platypnea-orthodeoxia), Portopulmonary HTN (PoPH).

Diagnosis & Staging - Sizing Up the Damage

• Clinical: Stigmata (spider naevi, ascites), history (alcohol, viral).
• Labs: ↓Platelets (early sign), ↑AST/ALT, ↓Albumin, ↑INR, ↑Bilirubin. Viral markers.
• Imaging:
  • USG: Nodular liver, splenomegaly, ascites, portal vein >13mm.
  • FibroScan: Liver stiffness >12.5-14 kPa (cirrhosis).
• Endoscopy (OGD): Detects varices.
• Liver Biopsy: Gold standard (if diagnosis unclear).
• HVPG: Gold standard for PHT. >5 mmHg (PHT); >10 mmHg (CSPH); >12 mmHg (bleeding risk).
• Prognostic Scores:
  • Child-Pugh (CPS): (📌 BEAPA: Bilirubin, Encephalopathy, Ascites, PT/INR, Albumin). Classes: A (5-6), B (7-9), C (10-15).
  • MELD: Bilirubin, INR, Creatinine. For transplant allocation.

⭐ Thrombocytopenia is often the earliest laboratory abnormality in cirrhosis.

High‑Yield Points - ⚡ Biggest Takeaways

• Cirrhosis: Irreversible liver fibrosis and nodule formation, impairing liver function.
• Portal Hypertension (PHT): Portal gradient >5 mmHg (clinically significant >10 mmHg), drives complications.
• Ascites: Most common complication; manage with Na+ restriction, diuretics (spironolactone, furosemide).
• Variceal Bleeding: Most lethal PHT complication; beta-blockers for primary prophylaxis.
• Hepatic Encephalopathy (HE): Neuropsychiatric syndrome; treat with lactulose, rifaximin.
• Prognosis: Child-Pugh and MELD scores assess severity and guide transplant decisions.