What is decompensated cirrhosis?

Decompensated Cirrhosis

Decompensated cirrhosis is defined as the stage of liver cirrhosis marked by the development of overt clinical signs including ascites, variceal hemorrhage, hepatic encephalopathy, and/or jaundice, representing a critical turning point in disease progression with median survival decreasing to approximately 1-2 years. 1

Definition and Pathophysiology

Decompensated cirrhosis represents the advanced stage of liver cirrhosis when the liver can no longer compensate for extensive damage. The transition from compensated to decompensated cirrhosis is characterized by:

• Progressive portal hypertension (HVPG >10 mmHg)
• Worsening liver function
• Systemic inflammation
• Vasodilatory-hyperdynamic circulatory state
• Decreased effective arterial blood volume 1

This is in stark contrast to compensated cirrhosis, where patients are largely asymptomatic with preserved hepatic function and a median survival exceeding 12 years 2.

Clinical Manifestations

The four major clinical manifestations that define decompensation are:

1. Ascites - Most common first sign of decompensation

   • Requires management with sodium restriction, diuretics, and sometimes paracentesis 1

2. Variceal Hemorrhage - Life-threatening complication

   • Requires prompt intervention with vasoactive drugs and endoscopic therapy
   • Has a 20% 5-year mortality when occurring as isolated complication
   • Mortality exceeds 80% when associated with other complications 1, 3

3. Hepatic Encephalopathy - Neuropsychiatric manifestation

   • Ranges from subtle cognitive changes to coma
   • Often precipitated by infections, GI bleeding, or medications 1

4. Jaundice - Poor prognostic indicator

   • Often requires vasoactive drugs (terlipressin) and albumin 1

Prognostic Implications

The development of decompensation dramatically alters the disease trajectory:

• Median survival drops from >12 years in compensated cirrhosis to only 1-2 years after decompensation 1, 2
• Acute decompensation can be further subdivided into:
  • Stable decompensated cirrhosis (SDC)
  • Unstable decompensated cirrhosis (UDC)
  • Pre-acute-on chronic liver failure (pre-ACLF)
  • Acute-on chronic liver failure (ACLF) - the most severe form with organ failure(s) and high short-term mortality 4

Management Approach

The American Association for the Study of Liver Diseases recommends a comprehensive approach:

1. Treat the underlying cause when possible

   • For example, in HBV-related cirrhosis, antiviral therapy may improve outcomes
   • Interferon-α is contraindicated in decompensated cirrhosis due to risk of serious complications 5, 1

2. Manage specific complications:

   • Ascites: Sodium restriction, diuretics, paracentesis
   • Variceal bleeding: Vasoactive drugs, endoscopic therapy, consideration of TIPS (transjugular intrahepatic portosystemic shunt) in high-risk patients
   • Hepatic encephalopathy: Lactulose and rifaximin are first- and second-line treatments 6
   • Jaundice: Supportive care, treating underlying causes

3. Prevent further decompensation

   • Close monitoring and prompt intervention for complications

4. Evaluate for liver transplantation

   • Patients with decompensated cirrhosis should be managed at centers with expertise in liver disease
   • Liver transplantation should be considered for appropriate candidates 1, 7
   • Even patients with low MELD scores but clinical decompensation may benefit from transplantation evaluation 7

Important Considerations

• Decompensated cirrhosis requires specialized care at centers with expertise in liver disease management
• Regular surveillance for hepatocellular carcinoma is essential
• The dynamic nature of acute decompensation necessitates vigilant monitoring to identify patients at risk for developing more severe complications like ACLF
• Living donor liver transplant has demonstrated significant survival benefit even in patients with lower MELD-Na scores 7

Common Pitfalls

• Failing to recognize the significance of the first decompensation event as a critical prognostic marker
• Delaying transplant evaluation in patients with clinical decompensation despite low MELD scores
• Using interferon-α in decompensated cirrhosis (contraindicated)
• Not addressing modifiable risk factors that could accelerate disease progression
• Inadequate monitoring for the development of hepatocellular carcinoma