Redefining Cirrhotic Cardiomyopathy for the Modern Era

Abstract

Cirrhotic cardiomyopathy (CCM) is cardiac dysfunction in patients with end-stage liver disease in the absence of prior heart disease. First defined in 2005 during the World Congress of Gastroenterology, CCM criteria consisted of echocardiographic parameters to identify subclinical cardiac dysfunction in the absence of overt structural abnormalities. Significant advancements in cardiovascular imaging over the past 14 years, including the integration of myocardial deformation imaging into routine clinical practice to identify subclinical cardiovascular dysfunction, have rendered the 2005 CCM criteria obsolete. Therefore, new criteria based on contemporary cardiovascular imaging parameters are needed. In this guidance document, assembled by a group of multidisciplinary experts in the field, new core criteria based on contemporary cardiovascular imaging parameters are proposed for the assessment of CCM. This document provides a critical assessment of the diagnosis of CCM and ongoing assessment aimed at improving clinical outcomes, particularly surrounding liver transplantation. Key points and practice-based recommendations for the diagnosis of CCM are provided to offer guidance for clinicians and identify gaps in knowledge for future investigations.

Conflict of interest statement

Potential conflict of interest: Nothing to report.

© 2019 by the American Association for the Study of Liver Diseases.

Figures

FIG. 1.

Illustration of strain imaging as a surrogate for cardiac systolic function. This “Bull’s eye” diagram of the myocardium shows (A) normal strain imaging (global longitudinal strain of −21%) and (B) abnormal strain imaging (global longitudinal strain of −14%). Diminished strain in patients with end stage liver disease in the absence of known heart disease is diagnostic of cirrhotic cardiomyopathy.

FIG. 2.

Changes in pressure, velocity, and volume during different grades of diastolic dysfunction diagram of left ventricle (LV)-left atrial (LA) pressure tracing (top), mitral inflow velocity (middle) and mitral annulus velocity (bottom) of normal and grade 1 to grade 3 diastolic dysfunction. Tricuspid regurgitation and LA volume index values of each category in general are shown at the bottom (with permission from The Echo Manual, 4th edition, Oh et al.)

FIG. 3.

Evaluation of diastolic function in patients with end-stage liver disease (A simplified algorithm, revised from the 2016 ASE guideline. [Adapted from Oh JK et al.(33) Submitted to JACC Imaging]). *In this algorithm, only medial annulus velocity is recommended. After applying the modified criteria, filling pressure is first assessed, then diastolic function is graded based on E/A ratio. **For values of PV, IVRT, and strain assessment in patients with indeterminate diastolic function, refer to Fig. 4. Advanced diastolic dysfunction (grade 2 or 3) in patients with ESLD in the absence of known heart disease is diagnostic of cirrhotic cardiomyopathy. Abbreviations: LA, left atrium; PV, pulmonary vein; IVRT, isovolumetric relaxation time.

FIG. 4.

Additional assessment to reclassify patients with indeterminate diastolic function based on Fig. 3 into normal versus different grades of diastolic dysfunction. Pictured are still frames of pulmonary vein pressures, values of isovolumetric relaxation (IVRT), Left atrial systolic strain (LAS), and LV global longitudinal strain (LVS) for normal and different stages of diastolic function.