Atrio-ventricular deformation and heart failure in Ebstein's Anomaly — A cardiovascular magnetic resonance study

PURPOSE: We aimed to quantify atrial and ventricular myocardial deformation in Ebstein's Anomaly (EA) in a case-control study with cardiovascular magnetic resonance (CMR) feature tracking and to correlate changes in cardiac performance with the severity of disease and clinical heart failure parameters. MATERIALS AND METHODS: Atrial and ventricular deformation was measured using CMR feature tracking in 30 EA and 20 healthy control subjects. Atrial performance was characterized using longitudinal strain and strain rate parameters for reservoir function, conduit function and booster pump function. Ventricular performance was characterized using RV and LV global longitudinal strain (εl) and LV circumferential and radial strain (εc and εr). Volumetric measurements for the ventricles including the Total Right/Left-Volume-Index (R/L-Volume-Index) and heart failure markers (BNP, NYHA class) were also quantified. RESULTS: EA patients showed significantly impaired right atrial performance, which correlated with heart failure markers (NYHA, BNP, R/L-Volume-Index). LA function in EA patients was also impaired with atrial contractile function correlating with NYHA class. EA patients exhibited impaired RV myocardial deformation, also with a significant correlation with heart failure markers. CONCLUSION: CMR feature tracking can be used to quantify ventricular and atrial function in a complex cardiac malformation such as EA. EA is characterized by impaired quantitative right heart atrio-ventricular deformation, which is associated with heart failure severity. While LV function remains preserved, there is also significant impairment of LA function. These quantitative performance parameters may represent early markers of cardiac deterioration of potential value in the clinical management of EA.