Kommerell diverticulum (KD) with aberrant left subclavian artery (ALSA) is a rare congenital variation of vascular structure. We reported a case of 3-year-old boy with KD and ALSA, which was preliminarily diagnosed by transthoracic echocardiography and verified by computed tomography angiography (CTA). The patient was treated successfully with KD resection and anastomosed the left subclavian artery directly to the left carotid artery under cardiopulmonary bypass. Our case illustrates that echocardiography and CTA should be used to comprehensively assess all the internal and external cardiac structures in order to determine the appropriate surgical plan.
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