Congenitally corrected transposition of the great arteries (ccTGA) is a rare defect characterized by discordant atrioventricular and ventriculoarterial connections. Symptoms result from one or a combination of associated cardiovascular malformations, including ventricular septal defect, pulmonary stenosis or atresia, and tricuspid valve dysfunction. Survival of patients with ccTGA is often determined by the associated anomalies, but even in isolation ccTGA results in a diminished life expectancy. Regurgitation of the tricuspid valve and decreased morphologic right ventricular function tend to develop and worsen over time. Anatomic repair by double switch technique may improve survival of patients with ccTGA by establishing the morphologic left ventricle in the systemic circulation.
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