Cirrhotic Cardiomyopathy

Maxwell Eyram Afari, M.D.
Joe Aoun, M.D.

Basic Information

Definition

Cirrhotic cardiomyopathy (CCM) is a clinical syndrome that describes cardiac dysfunction in patients with liver cirrhosis who have no other evidence of an etiology for heart disease. Patients are characterized by presentation with normal or increased cardiac output but with the absence of an appropriate contractile responsiveness to stress, altered diastolic dysfunction, and/or electrophysiological abnormalities

Synonym

ICD-10CM CODES
I43	Cardiomyopathy in diseases classified elsewhere

Epidemiology & Demographics

Incidence

There is not enough data available at this point to identify specific incidence rates for this disorder.

Prevalence

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Prevalence between 40% to 50% in cirrhotic patients independent of liver disease etiology and 3% to 23.4% of patients undergoing orthotopic liver transplantation
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Cardiac dysfunction seen in 50% of patients with advanced cirrhosis
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Heart failure is seen in 7% to 21% of patients who undergo orthotopic liver transplantation

Risk Factors

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Advanced liver disease is the principal risk factor. Moderate to severe cirrhosis (Child Pugh B or C) have at least one feature of CCM.
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Posttransjugular intrahepatic portosystemic shunt (TIPS): shift of splanchnic blood into the heart via the artificial shunts suddenly overloads a noncompliant left ventricle, leading to overt heart failure.

Physical Findings & Clinical Presentation

CCM should be suspected in any patient with advanced liver cirrhosis. Patients usually remain asymptomatic for a long time because it is initially tolerated.

Clinical and physical features include:

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Symptoms and signs of heart failure: reduced exercise capacity, dyspnea on exertion, tachycardia, edema, ascites, paroxysmal nocturnal dyspnea, pulmonary congestion, increased jugular venous pressure, and an S3 gallop.
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Electrophysiological disturbances: QTc interval prolongation (>440 msec), Torsades de pointes, chronotropic incompetence, and late ventricular potentials.

Etiology and Pathophysiology

Etiology is multifactorial in patients with liver disease. Fig. 1 illustrates proposed pathophysiology of cirrhotic cardiomyopathy.

FIG.1

Proposed pathophysiology of cirrhotic cardiomyopathy. *QTc,* rate-corrected QT interval.

From Feldman M, et al.: *Sleisenger and Fortran’s gastrointestinal and liver disease*, ed 10, Philadelphia, 2016, Elsevier.

Mechanisms of cardiac dysfunction include the following:

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Downregulation of β-adrenergic receptor leads to systolic dysfunction.
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Nitric oxide overproduction causes splanchnic arterial vasodilation, which masks the presence of blunted cardiac function. The decreased effective volume activates the renin angiotensin aldosterone (RASS), leading to fibrosis and myocardial dysfunction (diastolic dysfunction due to left ventricle wall stiffness).
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Ion channel defect (decreased K+ current density) leads to electrophysiological imbalances such as QT prolongation, chronotropic incompetence, and mechanical dyssynchrony.
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Hyperdynamic circulation: in cirrhotic patients there is an increased volume expansion and poor volume distribution (to the splanchnic circulation). The relative hypovolemia activates the RASS and sympathetic nervous system and leads to tachycardia and increased cardiac output.

Diagnosis

The 2005 World Congress of Gastroenterology proposed the following criteria for CCM:

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Systolic dysfunction (LV EF <55%, blunted increase in cardiac output with exercise)
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Diastolic dysfunction on echocardiogram (E/A <1.0, deceleration time >200 msec, isovolumetric relaxation time >80 msec)
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Supportive criteria: electrophysiological abnormalities, abnormal chronotropic response, electromechanical uncoupling/dyssynchrony, prolonged QTc interval, enlarged left atrium, increased myocardial mass, increased brain natriuretic peptide (BNP) and pro-BNP, or increased troponin

Differential Diagnosis

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Alcoholic cardiomyopathy (ACM)
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Cardiac cirrhosis
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Iron-induced cardiac disease (hemochromatosis)
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Dilated cardiomyopathy
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Hypertrophic cardiomyopathy
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Restrictive cardiomyopathy
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Coronary atherosclerosis with left ventricular dysfunction

Workup

Laboratory Tests

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Brain natriuretic peptide (BNP) and pro-BNP: markers of fluid overload, and correlate with the severity of cirrhosis
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Troponin I: marker of cardiac dysfunction, increased LV mass

Imaging Studies

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Electrocardiography: QTc >440 msec, ST depressions, bundle branch block
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Chest x-ray: pulmonary congestion, left atrial and ventricular enlargement
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Echocardiogram: resting systolic dysfunction; diastolic dysfunction (E/A <1, prolonged deceleration time [>200 msec] and prolonged isovolumetric relaxation time [>80 msec]); strain rate can be used to identify cardiac dysfunction, even when ejection fraction is normal
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MRI: morphological evaluation of the liver and the heart (shunts, chamber quantification, tissue characterization)

Diagnostic Tests

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Exercise testing (upright bicycle ergometry): suppressed increase in cardiac output with exercise
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Echocardiogram with dobutamine stress test: lack of contractile reserve with dobutamine (limited utility due to downregulation of the β-receptors)

Treatment

There are no well-established guidelines for the management of CCM. Management should follow the same guidelines as in noncirrhotics.

Nonpharmacologic Therapy

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Risk-factor modification: weight loss, smoking cessation, exercise, blood pressure control
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Oxygen supplementation if signs of hypoxia
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Dietary sodium restriction (<2 g/day)
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Fluid restriction (1.5 L/day)
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Pneumococcal and influenza vaccination

Acute General Rx

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Acute pulmonary edema: head elevation ≥30 degrees, early diuretic use (furosemide, bumetanide, torsemide); nitrates are useful if concomitant hypertension or chest pain
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Cardiogenic shock: inotropes (dobutamine, milrinone), vasopressors (norepinephrine, dopamine), and mechanical support (intraaortic balloon pump, impella, ECMO, tandem heart) if needed

Chronic Rx

Chronic management is consistent with the treatment of patients with heart failure.

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Diuretics: Furosemide and spironolactone are the mainstay for treating fluid overload in cirrhosis.
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β-Blockers: They promote the reduction of the QT interval. Nonselective β-blockers are known to prevent variceal bleeding. One should exercise caution because β-blockers can reduce cardiac output.
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Liver transplantation is the cure for cirrhosis and its associated cardiomyopathy. It has been shown to restore exercise capacity within 6 to 12 months.

Disposition

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Mortality rate is unclear because most patients die from repercussions of liver failure.
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Liver transplant can reverse CCM features, including systolic and diastolic dysfunction and QT prolongation.

Referral

Follow-up with a cardiologist is recommended.

Pearls & Considerations

Comments

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CCM should be considered in cirrhotic patients who present with symptoms and signs of heart failure symptoms or arrhythmia.
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Diagnostic criteria are based on echocardiogram (systolic or diastolic dysfunction), electrophysiological disturbances and laboratory abnormalities.
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Liver transplantation is the definitive treatment for this rare clinical entity.

Prevention

Treatment of liver disease prior to progression to cirrhosis.

Patient/Family Education

A multidisciplinary team involving a hepatologist and cardiologist may be useful in long-term management and patient education.

Ferri – Cirrhotic Cardiomyopathy