Thorac Cardiovasc Surg 2016; 64 - OP14
DOI: 10.1055/s-0036-1571855

Atrioventricular Mechanics and Heart Failure in Ebstein's Anomaly—A Cardiovascular Magnetic Resonance Imaging Study

M. Steinmetz 1, M. Broder 1, J. T. Kowallick 2, P. Llamata 3, S. Kutty 4, M. Seehase 1, W. Staab 2, C. Unterberg-Buchwald 2, J. Lotz 2, T. Paul 1, G. Hasenfuß 5, A. Schuster 5
  • 1Georg-August-Universität Göttingen, Herzzentrum, Pädiatrische Kardiologie und Intensivmedizin, Göttingen, Germany
  • 2Georg-August-Universität Göttingen, Herzzentrum, Diagnostische und Interventionelle Radiologie, Göttingen, Germany
  • 3King's College London, Division of Imaging Sciences and Biomedical Engineering, London, United Kingdom
  • 4University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha, United States
  • 5Georg-August-Universität Göttingen, Herzzentrum, Kardiologie und Pneumologie, Göttingen, Germany

Objectives: To assess quantitative atrial and ventricular function in EA with CMR feature tracking and to correlate changes in biatrial and biventricular performance with the severity of disease and clinical parameters of heart failure

Background: Ebstein's anomaly (EA) is a rare but clinically important congenital heart disease with potential affection of right ventricular, right atrial, left ventricular and left atrial function that may play a role in heart failure development.

Methods: Atrial and ventricular deformation parameters were calculated from 30 EA and 20 healthy control subjects at 1.5 Tesla. Right and left atrial (RA/LA) performance was characterized using longitudinal strain and strain rate parameters quantifying reservoir function (total strain [Ells], peak positive SR [SRs]), conduit function (passive strain [Elle], peak early negative SR [SRe]) and booster pump function (active strain [Ella], late peak negative SR [SRa]). Ventricular performance was characterized using right ventricular (RV) and left ventricular (LV) global longitudinal strain (Ell) and LV circumferential and radial short axis strain (Ecc and Err). Additionally, volumetric measurements for all cardiac chambers including the Total right/left volume-index and heart failure markers (BNP, NYHA class) were quantified.

Results: EA patients showed a significantly impaired right atrial performance which correlated with markers of heart failure (NYHA, BNP and Total R/L-Volume Index). The left atrial function in EA patients was also significantly impaired compared with healthy controls with atrial contractile function correlating with NYHA class. Furthermore, EA patients exhibited an impaired right ventricular function, also with a significant correlation with heart failure markers, whereas left ventricular parameters only showed a non-significant trend toward reduced performance.

Conclusion: EA is characterized by impaired quantitative right heart atrioventricular deformation, which is associated with heart failure severity even in the presence of normal RV EF. While left ventricular function remains preserved there is significant impairment of LA function potentially representing early stages of diastolic dysfunction. These novel quantitative performance parameters may represent early markers of cardiac deterioration of potential value to improve the clinical management of EA patients.