Congenitally corrected transposition of the great arteries

Case contributed by Dr Vincent Tatco

Presentation

Diagnosed case of congenital heart disease on echocardiography

Patient Data

Age: 5
Gender: Female

There is atrioventricular and ventriculoarterial discordance. The morphologic right ventricle which is on the left side is hypertrabeculated with top normal wall thickness. The left atrium is located on the left side. The morphologic left ventricle which is on the right side is normal in size and wall thickness. The right atrium is located on the right side. The aorta, which comes from the morphologic right ventricle, is anterior and to the left of the main pulmonary artery, which comes from the morphologic left ventricle. The single arch and descending thoracic segment of the aorta are left-sided.  The aortic arch gives off three main branches without aberrant vessels. No evidence of coarctation or abnormal dilatation is identified. There are two superior and two inferior pulmonary veins draining into the left atrium. The inferior vena cava and a single right-sided superior vena cava drain into the right atrium.

Case Discussion

This is a diagnosed case of congenitally corrected transposition of the great arteries, which is a rare anomaly that comprises less than 1% of all congenital heart diseases 1-2. In this anomaly, there is atrioventricular and ventriculoarterial discordance (double discordance) wherein the right atrium communicates with the morphologic left ventricle, which gives rise to the pulmonary artery, while the left atrium communicates with the morphologic right ventricle, which gives rise to the aorta. The blood flows in the normal direction but passes through the wrong ventricular chambers.

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