Diagnosis of Congenitally Corrected Transposition of the Great Arteries

概要

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Congenitally corrected transposition of the great arteries (CCTGA) is characterized by atrioventricular and ventriculoarterial discordance. Because of double discordance, the circulation is physiologically corrected, but the morphologic right ventricle supports systemic circulation, and 90% of patients have associated cardiac anomalies. The clinical presentation is influenced by the presence of associated anomalies. Since progressive systemic right ventricular failure and tricuspid regurgitation occur by the fourth decade in many patients with CCTGA, early intervention is recommended. The unique anatomy of CCTGA is malalignment between the atrial septum and the inlet part of the ventricular septum. This produces a gap into which is deeply wedged the pulmonary out flow tract and the abnormal course of atrioventricular conduction tissue. The aorta is usually anterior and to the left. Laboratory investigations including chest radiograph, electrocardiogram, echocardiography, MRI, and MDCT, reflect these morphological characteristics. Echocardiography is the imaging modality that provides the most accurate diagnostic assessment of CCTGA. It permits the identification of the basic anatomy by sequential segmental analysis as well as of associated anomalies. The role of echocardiography to evaluate the systemic right ventricular function is to be elucidated.