Congenitally corrected transposition of the great arteries (CCTGA)





Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac defect characterized by discordance at the atrio-ventricular and ventriculo-arterial level. This is due to an abnormal, leftward looping of the primitive heart tube in utero, resulting in the morphological right ventricle being on the left side of the morphological left ventricle. Because of the presence of double discordance, this defect is physiologically corrected while maintaining blood flow from the left atrium to the aorta and from the right atrium to the pulmonary artery.


CCTGA is frequently associated with the presence of dextrocardia, mesocardia, ventricular septal defects, pulmonary stenosis or atresia, and Ebsteinoid malformation of the tricuspid valve. Progressive tricuspid valve disease and systemic right ventricular failure are worrisome complications that contribute significantly to the morbidity and mortality of these patients. Conduction disorders on ECG represent another characteristic feature of this condition.




Figure 1


Apical four-chamber view in a patient with CCTGA demonstrating atrio-ventricular discordance. The arrows indicate attachment of the septal leaflet of the tricuspid valve to the interventricular septum. Dashed lines represent the mild apical displacement of the tricuspid valve in relation to the mitral valve. These features, among others, characterize the morphological right ventricle, which is on the left side of the heart in this child. LA , left atrium; mLV , morphological left ventricle; mRV , morphological right ventricle; RA , right atrium.



Figure 2


(A) Subcostal long-axis view showing ventriculo-arterial discordance in CCTGA. The aorta arises anteriorly from the morphological right ventricle, which is coarsely trabeculated. The pulmonary artery is posterior to the aorta and originates from the morphological left ventricle, which has a smooth endocardial surface. Note the parallel orientation of the great arteries. (B) Corresponding color flow mapping in the same child. Ao , aorta; mLV , morphological left ventricle; mRV , morphological right ventricle; MV , mitral valve; PA , pulmonary artery; RA , right atrium.



Figure 3


(A) Apical five-chamber view demonstrating ventriculo-arterial discordance in a patient with CCTGA. Color flow mapping illustrating the origin of the pulmonary artery from the (right-sided) morphological left ventricle. (B) More anterior plane showing the origin of the aorta from the (left-sided) morphological right ventricle. The aorta is to the left of the pulmonary artery and both vessels have a parallel course. Ao , aorta; LPA , left pulmonary artery; mLV , morphological left ventricle; mRV , morphological right ventricle; PA , pulmonary artery; RA , right atrium; RPA , right pulmonary artery.



Figure 4


(A) Subcostal short-axis view demonstrating ventriculo-arterial discordance in a patient with CCTGA. The pulmonary artery arises from the (right-sided) morphological left ventricle. (B) The aorta is connected to the (left-sided) morphological right ventricle. The interventricular septum is intact. Ao , aorta; mLV , morphological left ventricle; mRV , morphological right ventricle; PA , pulmonary artery.

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Feb 2, 2021 | Posted by in CARDIOLOGY | Comments Off on Congenitally corrected transposition of the great arteries (CCTGA)

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