Corrected transposition of the great vessels without associated defects

Abstract

Two patients with complete atrioventricular block and corrected transposition of the great vessels without intracardiac shunts have been described. One patient had mild associated mitral insufficiency. The diagnosis is difficult in uncomplicated cases, which probably accounts for the paucity of case reports of this interesting syndrome. The prognosis is good. The diagnosis should be suspected in children or young adults when heart block or congenital mitral insufficiency is present. In the absence of these findings, an x-ray silhouette demonstrating a globular heart with a narrow pedicle, an electrocardiogram with abnormal P waves and absent septal Q waves, together with the presence of a loud, single second sound at the pulmonic area, are important additional diagnostic clues.