Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart defect, occurring in only 0.5% of patients exhibiting congenital heart defects. Because it is not seen in a routine transthoracic echocardiography (TTE) examination, CCTGA may be missed. The echocardiography diagnosis of CCTGA is difficult and not well explained in most textbooks. The present report provides key sonographic images that contribute to the diagnosis. In the present case, the patient had no prior history of heart disease. TTE revealed that the patient had CCTGA and early tricuspid regurgitation with right ventricle enlargement and hypertrophy. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:375-379, 2017.

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