To evaluate the agreement between dual-source computed tomography (DSCT) and two-dimensional transthoracic echocardiography (2D-TTE) with respect to the assessment of global left ventricular (LV) function in patients with severe arrhythmia. With 2D-TTE serving as the reference method, we performed both DSCT and 2D-TTE, at an interval of less than 2 days, in 54 patients with severe arrhythmia (average heart rate difference >30 beats per min) before open heart surgery for evaluation of valvular heart disease (VHD) and coronary artery disease. DSCT was performed using retrospective electrocardiography (ECG) without dose modulation. Ten phases of the cardiac cycle were analyzed for identification of end-diastolic and end-systolic phases with ECG-editing. Pearson’s correlation coefficient (r) and Bland–Altman analysis were used to determine agreement for parameters of LV global function. Correlation between DSCT and 2D-TTE measurements was good or excellent in terms of the values of the LV ejection fraction (51.0 ± 11.4% vs. 55.8 ± 11.6%; r = 0.8), LV end-diastolic volume (179.5 ± 98.6 ml vs. 152.1 ± 73.8 ml; r = 0.95), LV end-systolic volume (90.7 ± 60.7 ml vs. 69.1 ± 46.8 ml; r = 0.90), and LV stroke volume (89.0 ± 48.1 ml vs. 82.9 ± 37.3 ml; r = 0.89). Left ventricular ejection fraction measured using DSCT was less than that measured using 2D-TTE by an average of −4.8 ± 7.3%. Dual-source CT with ECG editing can provide results comparable to those of 2D-TTE for assessment of LV global function in patients with severe arrhythmia.