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Aim: This study aims to elucidate the morphological and functional differences of patients' with Ebstein's anomaly (EA) and healthycontrols (C).Methods: Right ventricular (RV) 2D longitudinal global strain (2DGS) and isovolumic acceleration of basal segment of free RV wall (IVA) were measured by transthoracic echocardiography. RV and left ventricular (LV) ejection fraction (RVEF, LVEF), indexed RV and LV enddiastolic volumes (RVEDVI, LVEDVI) and tricuspid regurgitation fraction (TI) were determined by magnetic resonance imaging. Cardiopulmonary exercise testing was performed to evaluate maximum oxygen uptake (VO2max, EA patients only).Results: 69 (30 men) individuals in both groups each were included. Age did not differ between both groups (p=0.829). In EA RVEDVI was increased but LVEDVI, RV- and LVEF were diminished: RVEDVI ml/m2: 146±80 vs. 81±14, p=0.0013, RVEF %: 44±6 vs. 61±12, p<0.001, LVEDVI ml/m2: 65±3 vs. 73±3, p=0.015. LVEF %: 60±5 vs. 64±10, p=0.017. 2DGS and IVA were reduced in EA: 2DGS %: -16.4±6.7 vs. -22.9±5.3, p<0.001. IVA m/sec2: 1.2±1.1 vs. 1.9±0.9, p<0.001. TI was related directly to RVEDVI and inversely to LVEDVI: TI=51.3+2.3 RVEDVI, r=0.737, p<0.001, TI=79.3–0.556 LVEDVI, r=0.340, p=0.048. VO2max was inversely correlated to TI: VO2max=29.42-0.131 TI, r=0.453, p=0.007. LVEDVI was directly correlated to VO2max (ml/min/kg): LVEDVI=16.3+0.099 VO2max, r=0.255, p=0.036. No relationship was found of any RV or LV systolic function parameter to VO2max.Conclusions: Patients with EA present with RV dilatation and diminished LV volume. The severity of TI is directly related to VO2max. This might be an argument to correct TI by surgery even early in the disease course.