Congenitally Corrected Transposition of the Great Arteries

Classically, identifying a chamber with a right ventricular (RV) morphology on two-dimensional echocardiography relies on: (1) a coarse and irregular endocardial surface, (2) chordae tendineae attaching directly to the ventricular septum, (3) the presence of an infundibulum, (4) a triangular-shaped ventricular cavity, (5) the presence of a prominent moderator band, and (6) recognition of the atrioventricular (A-V) valve as tricuspid. 

The features associated with left ventricular (LV) morphology include: (1) a smooth and uniform endocardial surface, (2) two distinct groups of papillary muscles, (3) an ellipsoid-shaped ventricular cavity, and (4) identification of the A-V valve as mitral.

In Van Praagh's classification, congenitally corrected transposition of the great arteries (CCTGA) is referred to as ventricular inversion or L-transposition and is described as follows:

Key Features in Van Praagh's Classification:

Echocardiography example

Case of cc-TGA (L-TGA) with pulmonary atresia. Here there is no heart block during the echocardiography. 

Case of atrial situs solitus; laevocardia (heart predominantly in the left hemithorax) and congenitally corrected transposition of great arteries (discordant atrioventricular & ventriculo-arterial connections, ventricular septal defect and pulmonary atresia, Ebstein's malformation of tricuspid valve. Tortuous patent arterial duct (pulmonary atresia type - aortic arch to pulmonary artery) shunting left to right. Large nonrestrictive perimembranous central ventricular septal defect (VSD) and Left aortic arch.

In this apical view, one may appreciate the coarse trabeculation of the morphological right ventricles on the left side of the patient, with a inferiorly displaced atrio-ventricular valve with attachments to the septum. This tricuspid valve is Ebstanoid. 

The morphological left ventricle which has a smooth surface is on the right side of the patient. There is a large inlet ventricular septal defect. The right sided atrio-ventricular valve is superiorly placed compared to the left sided atrio-ventricular valve.

Focus on the morphological left ventricle on the right side of the chest. It is connected to the atrium receiving the SVC and IVC (unseen in this image). 

Focus on the left-sided morphological right ventricle which receives the blood flow from the atrium that received the pulmonary venous return (not show here). 

Sweep outlining that the left-sided morphological right ventricle leads to an anteriorly placed vessel that does not bifurcate, which is the aorta. 

Colour flow from the morphological RV to the aorta. 

Subcostal sweep with the morphological RV and its outflow tract leading to the ascending aorta. 

In this particular patient, there is a IVC and SVC (no interruption) that are connected to the atrium that feeds the morphological left ventricle. 

The branch pulmonary arteries are being fed by the patent ductus arteriosus (pulmonary atresia type configuration). The morphological left ventricle on the right side of the patient does not have a patent connection towards the pulmonary artery ("pulmonary atresia" setup). 

Aortic arch and ductus arteriosus seen. 

It is important to outline the coronary branching in cases of ccTGA. 

Presentation by Dr Sariya Sahussarungsi and Dr Abdullah Alghamdi - January 2025

Great presentation prepared by Dr Sariya Sahussarungsi and Dr Abdullah Alghamdi for the NICU Cardio Rounds at McGill University of January 2025. Reviewed in collaboration with Dr Tiscar Cavallé Garrido (pediatric and fetal cardiologist). 

EDITEDccTGA AA_SS 01-08.pdf

Important Articles

van-praagh-1977-terminology-of-congenital-heart-disease-glossary-and-commentary.pdf

© NeoCardioLab - Gabriel Altit - 2020-2025
Contact us / Contactez-nous