Severity Scores for Ebstein Anomaly: Credibility and Usefulness of Echocardiographic versus Magnetic Resonance Assessments of the Celermajer Index

Abstract Background Severity score of Ebstein anomaly (EA) that correspond to clinical status is still under research, with the Celermajer index (Cel-ind) being one of those. The agreement between echocardiographic and CMR assessment of Cel-ind is not known. We determined the agreement between echo- and CMR-derived Cel-ind and its relationship with heart failure markers. Methods 37 unoperated EA adults (mean age 43.0±14.4y) underwent echocardiography, CMR, cardiopulmonary test. For Cel-ind end-diastolic areas in echocardiography, or end-diastolic volumes in CMR were used according to the following formula: Cel-ind=(RA+aRV)/(fRV+LA+LV). Based on this assumption patients were classified as follows: grade1= Cel-ind 1.5. The agreement between echocardiographic and CMR was determined with the Intraclass Correlation Coefficient or Cohen’s Kappa ( Results The median echoCel-ind was 0.9 (range 0.4-2.3), the median cmrCel-ind was 0.7 (range:0.3-5.3). Grade 1 or 2 was found in 19pts (51.3%) in echo and in 27pts (72.9%) in CMR. The agreement between imaging methods was only fair (kappa=0.39, p=0.002) for the four-grade classification; and moderate (ICC=0.43, 95%CI 0.13-0.66) for Cel-ind calculation. Significant correlations between Cel-ind in CMR and cardiopulmonary parameters were found (for peakVO2: R=-0.35, p=0.034; for VE/VCO2slope: R=0.46, p=0.005). Neither of them correlated with echocardiographic severity score. Conclusions The agreement between echocardiographic and CMR assessment of the Celermajer index is at most moderate; echocardiography usually overestimates, but rarely underestimates, EA severity. Celermajer index by CMR seems to be more valuable, as it is associated with heart failure markers.