What is decompensated cirrhosis?

Definition of 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. 1

Understanding Cirrhosis Progression

Cirrhosis progresses through distinct clinical stages:

1. Compensated Cirrhosis:

   • Asymptomatic phase
   • No overt clinical complications
   • Preserved liver function
   • Median survival exceeds 12 years 1
   • Can be further substaged based on portal hypertension:
     • Mild portal hypertension
     • Clinically significant portal hypertension (HVPG ≥10 mmHg)

2. Decompensated Cirrhosis:

   • Defined by the presence of one or more complications:
     • Ascites (most common first sign)
     • Variceal bleeding
     • Hepatic encephalopathy
     • Jaundice
   • Median survival drops to approximately 2 years 1

3. Further Decompensation:

   • Development of additional complications:
     • Recurrent ascites requiring large-volume paracentesis
     • Recurrent variceal hemorrhage
     • Recurrent hepatic encephalopathy
     • Spontaneous bacterial peritonitis
     • Acute kidney injury/hepatorenal syndrome
     • Jaundice 1

Clinical Significance

The transition from compensated to decompensated cirrhosis represents a critical turning point in disease progression:

• Mortality risk increases substantially after first decompensation event
• Disease progression accelerates
• Patients with decompensated cirrhosis have 1-2 year median survival compared to >12 years in compensated phase 1
• Complications often lead to hospitalization and decreased quality of life

Pathophysiology

Decompensation is primarily driven by:

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

Complications of Decompensated Cirrhosis

1. Ascites

• Most common first sign of decompensation
• Requires management with sodium restriction, diuretics, and sometimes paracentesis
• Can lead to spontaneous bacterial peritonitis

2. Variceal Hemorrhage

• Life-threatening complication
• Requires prompt intervention with vasoactive drugs, endoscopic therapy
• 20% 5-year mortality when occurring as isolated complication, >80% when associated with other complications 1

3. Hepatic Encephalopathy

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

4. Acute Kidney Injury/Hepatorenal Syndrome

• Poor prognostic indicator
• Often requires vasoactive drugs (terlipressin) and albumin 1

5. Bacterial Infections

• Common precipitating factor for decompensation
• Can accelerate disease progression

Management Considerations

Management of decompensated cirrhosis focuses on:

1. Treating the underlying cause of cirrhosis when possible
2. Managing specific complications
3. Preventing further decompensation
4. Evaluating for liver transplantation

Important Caveats

• Patients with decompensated cirrhosis should be managed at centers with expertise in liver disease
• Liver transplantation should be considered for appropriate candidates
• Interferon-α is contraindicated in decompensated cirrhosis due to risk of serious complications 1
• Careful monitoring for development of hepatocellular carcinoma is essential
• Patients with decompensated cirrhosis require close follow-up and may need frequent hospitalizations

The development of decompensation marks a significant prognostic shift in cirrhosis and requires comprehensive management strategies to improve outcomes and quality of life.