Cardiomyopathy in cirrhosis: From pathophysiology to clinical care

Abstract

Cirrhotic cardiomyopathy (CCM) is defined as systolic or diastolic dysfunction in the absence of prior heart disease or other identifiable cause in patients with cirrhosis and is an important determinant of outcome. Its underlying pathogenic/pathophysiological mechanisms are rooted in two distinct pathways: 1) factors associated with portal hypertension, hyperdynamic circulation and gut bacterial/endotoxin translocation with inflammatory phenotype. 2) Hepatocellular insufficiency with altered synthesis or metabolism of substances such as proteins, lipids, carbohydrates, bile acids and hormones. Different criteria have been proposed to diagnose CCM; the first in 2005 by the World Congress of Gastroenterology (WCG), and more recently in 2019 by the Cirrhotic Cardiomyopathy Consortium (CCC). These criteria mainly utilized echocardiographic evaluation, with the latter refining diastolic function evaluation and introducing global longitudinal strain to evaluate systolic function, an important addition since the hemodynamic changes that occur in advanced cirrhosis may lead to overestimation of systolic function by left ventricular ejection fraction. Advances in cardiac imaging such as cardiac magnetic resonance imaging and incorporating exercise challenge may help further refine the diagnosis of CCM. Clinical implications of cirrhotic cardiomyopathy have been elucidated over the past two decades and include contributing to increased mortality and morbidity after major interventions such as liver transplantation and TIPS stent insertion, and a pathophysiologic role in the genesis of hepatorenal syndrome. In this review, we discuss the pathogenesis/pathophysiology, role of cardiac imaging modalities including MRI, comparison of the WCG and CCC criteria, electrocardiographic QT prolongation and clinical implications. At present, no definitive medical therapy exists, but some promising potential treatment strategies for CCM are reviewed.