Ebstein valve rotation angle: a novel quantitative index of anatomical severity and higher clinical risk in Ebstein's anomaly

Abstract Funding Acknowledgements Type of funding sources: None. Introduction The study aimed to identify cardiovascular magnetic resonance (CMR) imaging parameters representative Ebstein's Anomaly (EA) anatomical and clinical severity. Materials Methods Consecutive CMR patients with EA referred our Center, were evaluated retrospectively. Displacement index (DI) was defined as the distance between septal leaflet hinge point atrio-ventricular groove over body surface area. Ebstein’s valve rotation angle (EVRA) quantified by connecting inferior systolic closure plane. Celermajer calculated using area (Cel-a) volumes (Cel-v) ratio atrialized RV (aRV) plus right atrium sum functional (fRV) left chambers. occurrence unfavorable outcome presence sustained brady-tachyarrhythmia or New York Heart Association (NYHA) class ≥ II for unoperated an at least moderate tricuspid regurgitation a during post-operative hospital stay undergone surgical correction. Results included 45 (age 28 +/- 15 years, 64% males). DI, Cel-a, Cel-v EVRA tend increase worsening Carpentier classification (p = 0.002, p =0.02, 0.01, < 0.0001 respectively). only parameter that showed discriminate both in group operated (68 ± 13.9° vs 43.5 26.8° 0.03) (58 27° 30 27°, 0.02). Among other analyzed parameters, aRV indexed end-diastolic volume ml/m2 31 22 , (192 81 128 32 ml/m2, significantly higher follow-up, while fRV/ARV axis (0.52 0.14 0.66 0.2, 0.04) (0.67 0.11 0.87 0.29, lower. fRV indexed-end diastolic lower (110 145 stay. value best who had follow-up event 44.5 °, sensitivity 0.57 (0.37–0.76, 95% CI) specificity 0.88 (0.69–0.96, CI). Conclusions is emerging may help provide quantitative assessment severity be integrated risk stratification planning.