pediatrics
Congenitally Corrected Transposition of the Great Arteries
Key Points
In congenitally corrected transposition of the great arteries, the right atrium enters a right-sided morphologic left ventricle, which gives rise to the pulmonary artery, and the left atrium connects to a left-sided morphologic right ventricle, which then leads to the aorta. There is a wide range of clinical manifestations due to the multiple associated anomalies. As patients reach adulthood, a concern is the development of dysfunction of the morphologic RV, which serves as the systemic ventricle. The diagnosis is usually made via echocardiogram, which demonstrates the morphology of the ventricles to be reversed with the right-sided morphologic left ventricle giving rise to a posterior pulmonary root and left-sided morphologic right ventricle giving rise to an anterior and leftward aortic root. Treatment is with a combination of medical, surgical, and rhythm device modalities. 