Congenitally corrected transposition of the great arteries (L-TGA) and dextrocardia

Case contributed by Dr Alexandra Stanislavsky


Known congenital cardiac anomaly, not surgically corrected. Sinus tachycardia.

Patient Data

Age: 51 years
Gender: Male

The cardiac apex is directed to the right (dextrocardia). Left sided aortic arch. 

The small hypertrophic morphological left ventricle is on the right. This is the subpulmonic ventricle. The hypertrophic morphological right ventricle with its moderator band is present on the left. This is the subaortic ventricle. 
Superior vena cava an inferior vena cava inflow to the right atrium passes through the AV valve to the morphologic left ventricle. 
Pulmonary venous return to a dilated left atrium passes through an AV valve to the morphologic right ventricle. Appearances are that of atrioventricular discordance and ventricular arterial discordance ie L -TGA ( congenitally corrected TGA.) 
A large ASD and small muscular VSD are present.


Case Discussion

Congenitally corrected transposition of the great arteries (L-TGA).

In this complex cardiac anomaly, systemic venous inflow to the right atrium travels (anomalously) to the morphologic left ventricle, but then (again anomalously) to the pulmonary arteries. 

Concurrently, pulmonary venous inflow to the left atrium travels anomalously to the morphologic right ventricle which is indicated by the moderator band, but then (again anomalously) to the aorta.

Thus, is there is both atrioventricular and ventriculo-arterial discordance (double discordance) and as a result, blood flow occurs in the correct direction. Cyanosis does not ensue at birth, and this patient has reached adulthood without surgical correction. He did however require a pacemaker soon after this scan.

Associated cardiac anomalies are common - in this case there is an ASD, VSD and dextrocardia.

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