Congenitally Corrected Transposition of the Great Arteries
Congenitally corrected transposition of the great arteries (ccTGA) is a complex congenital heart defect that is defined by atrioventricular and ventriculoarterial discordance. Figure 10.1 shows a coronal view of a heart with ccTGA and ventricular septal defect (VSD). The morphologic right atrium (systemic collecting chamber) is connected to the morphologic left ventricle through a mitral valve, which is connected to the pulmonary artery. The morphologic left atrium (pulmonary venous collecting chamber) is connected to the morphologic right ventricle through a tricuspid valve, which is connected to the aorta. This arrangement results in physiologically corrected but not anatomically corrected circulation. Circulatory aberrations are generally owing to associated lesions that include VSDs, pulmonary stenosis (PS) or atresia, and tricuspid valve abnormalities. Congenitally corrected transposition of the great arteries may also occur in association with situs solitus (normal atrial situs) or situs inversus. In addition, the conduction system in patients with ccTGA is notably atypical, and the incidence of spontaneous heart block is high even without surgical correction.
KeywordsAdult congenital heart surgery Atrioventricular discordance ccTGA Double switch operation Mustard-Rastelli operation Surgical treatment Ventriculoarterial discordance
Congenitally corrected transposition of the great arteries
Inferior vena cava, also known as inferior caval vein
Left ventricular outflow tract
Superior vena cava, also known as superior caval vein
Ventricular septal defect