Cirrhotic Cardiomyopathy

Cirrhotic cardiomyopathy is cardiac dysfunction in patients with cirrhosis that is not explained by other heart disease. It consists of a blunted contractile response to stress, diastolic dysfunction and electrophysiological abnormalities, often with a normal or high resting cardiac output.

It affects 40% to 50% of patients with cirrhosis, regardless of cause, and the prevalence rises with severity. It contributes to hepatorenal syndrome, refractory ascites, hypotension after paracentesis or shunting, and heart failure after transjugular intrahepatic portosystemic shunt (TIPS) or liver transplantation.

Pathophysiology.

Systemic vasodilatation, raised nitric oxide, carbon monoxide and endocannabinoids, beta adrenergic receptor down regulation, cardiomyocyte membrane changes and myocardial fibrosis impair contraction and relaxation. Delayed repolarisation prolongs the QT interval.

Usually silent at rest. Dyspnoea, fatigue, exercise intolerance, hypotension under stress, pulmonary oedema after volume load, and arrhythmia may appear with infection, bleeding, paracentesis, surgery or TIPS.

Red flags.

• Pulmonary oedema after TIPS or large volume paracentesis.
• Syncope or sustained QT prolongation.
• Rising creatinine with low blood pressure.

Echocardiography with tissue Doppler and strain: reduced global longitudinal strain, left ventricular ejection fraction under 55% at rest or failure to rise with stress, and diastolic dysfunction (E/A ratio under 1, deceleration time over 200 ms, E/e' over 10 or left atrial enlargement). ECG shows QTc over 440 ms. Troponin and natriuretic peptides are raised but are less specific in cirrhosis. Exclude coronary artery disease, valvular disease, alcoholic or iron overload cardiomyopathy and portopulmonary hypertension.

Differential diagnosis.

• Alcoholic cardiomyopathy.
• Haemochromatosis cardiomyopathy.
• Coronary artery disease.
• Portopulmonary hypertension.
• Hepatopulmonary syndrome.

There is no specific therapy. Optimise the underlying liver disease, avoid triggers for decompensation, and give supportive care.

• Albumin with paracentesis: 8 g of 20% albumin IV for each litre of ascitic fluid removed when more than 5 L is drained; maximum 100 g for each session; monitor blood pressure, creatinine and signs of volume overload; reduce in heart failure.
• Spironolactone: 100 mg orally once daily, increased every 3 to 5 days to 400 mg; maximum 400 mg daily; monitor potassium (4.0 to 5.0 mmol/L), sodium and creatinine; reduce or stop if potassium is over 5.5 mmol/L or creatinine clearance falls under 30 mL/min.
• Furosemide: 40 mg orally once daily, increased to 160 mg daily as needed (with spironolactone in a ratio of 40 mg to 100 mg); maximum 160 mg daily; monitor weight, sodium, potassium and creatinine; reduce with encephalopathy or hyponatraemia under 125 mmol/L.

Non-selective beta blockers are withheld when systolic pressure is under 90 mmHg or in refractory ascites. Avoid drugs that prolong the QT interval and keep potassium and magnesium (1.0 mmol/L or more) in range. Treat heart failure with sodium restriction and careful diuretic titration; avoid angiotensin converting enzyme inhibitors, which risk hypotension and renal failure. Echocardiographic assessment before TIPS and transplantation is mandatory. Liver transplantation reverses the dysfunction in most patients.

Monitoring. Echocardiogram and ECG at assessment for transplant or TIPS, and with new dyspnoea; renal function and electrolytes weekly during diuretic titration.

Referral and escalation. Hepatology and cardiology; transplant centre.