Cirrhotic Cardiomyopathy (CCM)

1️⃣ Definition

Cirrhotic cardiomyopathy (CCM) is a chronic cardiac dysfunction seen in patients with advanced liver cirrhosis, characterized by:

  • Impaired cardiac contractile response to stress
  • Diastolic dysfunction
  • Electrophysiological abnormalities (especially QT prolongation)
  • In the absence of intrinsic heart disease

It is a functional, stress-induced cardiomyopathy that becomes clinically apparent during:

  • Sepsis
  • TIPS insertion
  • Liver transplantation
  • Major surgery
  • GI bleeding


2️⃣ Why It Matters in ICU

For you (critical care focus), CCM is important because:

  • Cirrhotics may appear hemodynamically stable at rest
  • Under stress acute LV failure
  • Major cause of:
    • Post-TIPS heart failure
    • Post-liver transplant cardiovascular collapse
    • Hepatorenal syndrome worsening
    • Poor septic shock outcomes


3️⃣ Pathophysiology 

Cirrhosis hyperdynamic circulation chronic neurohormonal stimulation myocardial remodeling.

Stepwise Mechanism

1. Hyperdynamic Circulation

  • SVR (splanchnic vasodilation via NO)
  • CO at rest
  • Effective arterial blood volume

2. Chronic Neurohormonal Activation

  • RAAS activation
  • Sympathetic overactivity
  • Vasopressin release

3. Myocardial Changes

  • β-adrenergic receptor downregulation
  • Impaired calcium signaling


4️⃣ Structural & Functional Changes

A. Systolic Dysfunction (Stress-Induced)

  • EF may be normal at rest
  • Blunted response to:
    • Dobutamine
    • Exercise
    • Surgery

B. Diastolic Dysfunction (Early Finding)

  • Impaired relaxation
  • Increased LV stiffness
  • E/A reversal
  • Prolonged deceleration time

C. Electrophysiological Abnormalities

  • QT prolongation (30–60% cases)
  • Chronotropic incompetence
  • Atrial arrhythmias
  • Rare torsades


5️⃣ Diagnostic Criteria 

Updated criteria (2019 consensus):

A. Systolic Dysfunction

One of:

  • EF < 50%
  • Global longitudinal strain (GLS) < 18%
  • Blunted contractile response to stress

B. Diastolic Dysfunction

≥ 3 of:

  • E/e′ > 14
  • e′ < 7 cm/s
  • LA volume index > 34 mL/m²
  • TR velocity > 2.8 m/s

C. Supportive Findings

  • QTc prolongation
  • Elevated BNP
  • Elevated troponin


Why Does QT Prolongation Occur in Cirrhotic Cardiomyopathy?

 First – What Does QT Represent?

QT interval = total duration of ventricular depolarization + repolarization

  • QRS depolarization
  • ST + T repolarization
  • QTc (corrected QT) normally:
    • Men < 440 ms
    • Women < 460 ms
    • Dangerous ≥ 500 ms

QT prolongation = delayed ventricular repolarization

Repolarization depends mainly on potassium efflux currents (especially IKr and IKs).

In cirrhosis these potassium currents are impaired action potential duration increases QT prolongs.


6️⃣ Clinical Presentation

Often Silent at Rest, but all these factors precipitate it therefore do screening ECHO in all these

Trigger

Presentation

Sepsis

Sudden LV failure

TIPS

Pulmonary edema

Liver transplant

Intraoperative shock

GI bleed

Acute pulmonary edema

Large fluid resuscitation

Decompensation


7️⃣ Biomarkers in CCM

Marker

Significance

BNP

Elevated even without HF

Troponin

Mild elevation common

NT-proBNP

Correlates with severity

QTc

Prognostic marker


8️⃣ Hemodynamic Pattern

Typical cirrhotic patient:

  • High CO
  • Low SVR
  • Low MAP
  • Normal/low filling pressures

When CCM manifests:

  • Cardiac output under stress
  • LVEDP
  • Pulmonary edema


9️⃣ CCM vs Alcoholic Cardiomyopathy

Feature

CCM

Alcoholic CM

Etiology

Cirrhosis-related

Direct ethanol toxicity

EF at rest

Usually normal

Reduced

Reversibility

Improves after transplant

Partial

LV dilation

Mild

Marked


🔟 Impact During TIPS

TIPS sudden preload unmask latent LV dysfunction acute pulmonary edema.

High-risk predictors before TIPS:

  • Diastolic dysfunction
  • Elevated BNP
  • QT prolongation
  • Older age


11️⃣ During Liver Transplant

Phases:

  1. Pre-anhepatic – hyperdynamic
  2. Anhepatic – decreased preload
  3. Reperfusion – massive hemodynamic stress

CCM patients:

  • Cannot augment cardiac output
  • Risk of post-reperfusion syndrome
  • Vasoplegia + myocardial depression


12️⃣ Management (Guideline-Oriented)

There is no specific therapy for CCM.

A. General Principles

  • Treat cirrhosis
  • Avoid volume overload
  • Monitor QT interval
  • Correct electrolytes


B. ICU Management

1️⃣ Septic Shock in Cirrhosis

  • Norepinephrine = first line
  • Vasopressin adjunct
  • Dobutamine if low cardiac output

2️⃣ Acute LV Failure

  • Loop diuretics
  • Non-invasive ventilation
  • Cautious vasodilators

3️⃣ Arrhythmia

  • Correct K+, Mg++
  • Avoid QT-prolonging drugs


13️⃣ Beta Blockers – Friend or Foe?

Non-selective beta blockers (NSBBs) like:

  • Propranolol
  • Carvedilol

Used for portal hypertension.

But:

  • May worsen chronotropic incompetence
  • Can precipitate hypotension in advanced cirrhosis

Use cautiously in refractory ascites or shock.


14️⃣ Reversibility

The only definitive treatment:

Liver Transplantation

After transplant:

  • QT normalizes
  • Diastolic function improves
  • Systolic response improves

However:

  • Severe pre-existing CCM perioperative mortality


15️⃣ Prognosis

Worse outcomes associated with:

  • Elevated BNP
  • QTc > 500 ms
  • Severe diastolic dysfunction
  • Child-Pugh C
  • MELD > 25