What is cirrhotic cardiomyopathy?

Cirrhotic Cardiomyopathy: Definition and Clinical Significance

Cirrhotic cardiomyopathy (CCM) is a chronic cardiac dysfunction in patients with established cirrhosis, characterized by a blunted contractile response to stress, altered diastolic relaxation, and electrophysiological abnormalities such as QTc prolongation, occurring in the absence of any other cardiac disease. 1

Pathophysiology

Cirrhotic cardiomyopathy develops through multiple mechanisms:

• Systemic inflammation plays a key role in inducing myocardial dysfunction, leading to impaired diastolic relaxation and decreased left ventricular ejection fraction 1
• Shear stress generated by portal hypertension exerts mechanical forces on myocardial fibers, contributing to cardiac dysfunction 1
• Portal hypertension leads to hyperdynamic circulation and altered neurohumoral regulation 2
• Bacterial translocation and endotoxemia trigger inflammatory cascades affecting cardiac function 2
• Hepatocellular insufficiency results in altered synthesis or metabolism of substances that affect cardiac function 2

Clinical Features and Diagnosis

Systolic Dysfunction

• Defined as impaired left ventricular contractile responses to stress, with resting LVEF <55% 1
• Often masked at rest due to hyperdynamic circulation and reduced afterload in cirrhosis 1
• Requires stress induction (pharmacological or exercise) to manifest as an inadequate contractile response 1
• As cirrhosis advances, progressive reduction in peripheral vascular resistance unmasks systolic dysfunction 1

Diastolic Dysfunction

• Often the first manifestation of CCM 1
• Should be diagnosed using ASE guidelines: Average E/e'>14, Tricuspid velocity >2.8 m/s, and LAVI >34 ml/m2 1
• Associated with higher degree of ascites, elevated plasma renin levels, and increased risk of hepatorenal syndrome 1
• E/e' ratio is an independent predictor of survival 1

Electrophysiological Abnormalities

• QTc interval prolongation is common in cirrhosis and may indicate poor outcome 1
• Found in approximately 50% of cirrhotic patients, though rarely leads to fatal arrhythmias 3
• Medications that prolong QT interval should be used cautiously in these patients 1

Clinical Significance and Prognostic Impact

• CCM is largely subclinical but significantly influences prognosis in advanced liver disease 1
• Reduced cardiac output (a manifestation of CCM) is associated with development of acute kidney injury after infections like spontaneous bacterial peritonitis 1
• Diastolic dysfunction correlates with mortality: survival rates of 95% without diastolic dysfunction, 79% with grade I dysfunction, and only 39% with grade II diastolic dysfunction 1
• CCM impacts outcomes of interventions such as transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation 1, 4
• Several studies show association between diastolic dysfunction at time of TIPS placement and poor survival 1

Diagnostic Approaches

• Echocardiography remains the primary diagnostic tool 4
• Advanced techniques include:
  • Myocardial strain imaging to evaluate shortening of myocardial muscle fibers 1
  • Global longitudinal systolic strain (GLS) as a sensitive marker of left ventricular function 1
  • Cardiac MRI to detect diffuse myocardial fibrosis 1
• Biomarkers such as BNP levels can help grade severity of cardiac dysfunction 1

Impact on Interventions

TIPS Placement

• Cardiac reserve is a major clinical consideration for elective TIPS placement 1
• 2D echocardiography to assess LVEF is standard practice before TIPS 1
• Despite screening, some patients develop cardiac decompensation post-TIPS insertion 1
• Studies consistently show increased left ventricular and atrial volume over time after TIPS, suggesting increased risk of future heart failure 1

Liver Transplantation

• Detailed cardiac assessment is essential before liver transplantation 1
• Patients with higher BNP levels (>391) on day one post-transplant tend to have higher mortality and longer dialysis requirements 1
• Some patients with BNP levels >567 and ejection fractions <50% have died of cardiogenic shock within 72 hours post-transplant 1
• Following transplantation, there can be significant improvement in systolic strain and reduced left ventricular mass, suggesting some CCM changes are reversible 1

Management Considerations

• Currently, no specific treatment exists for CCM 2, 5
• Careful cardiac evaluation is necessary before major interventions 4
• Standardized criteria and protocols for assessment of systolic and diastolic function in cirrhosis are needed 1
• Agents that prolong QT interval should be used with caution 1
• Emerging treatment strategies targeting the pathophysiological mechanisms are under investigation 2

Common Pitfalls and Caveats

• CCM is often unrecognized due to its subclinical nature at rest 1
• Hyperdynamic circulation in cirrhosis may mask systolic dysfunction 1
• Different echocardiographic criteria and thresholds lead to heterogeneous data on cardiac dysfunction impact 1
• Variability in diagnostic techniques can lead to inconsistent assessment of cardiac function 1
• The 2005 World Congress of Gastroenterology criteria for CCM are now considered obsolete due to advances in cardiovascular imaging 6