TRANSPOSITION OF THE GREAT-ARTERIES (S,D,L) - PATHOLOGICAL ANATOMY, DIAGNOSIS, AND SURGICAL-MANAGEMENT OF A NEWLY RECOGNIZED COMPLEX

The transposition of the great arteries (S,D,L) complex is delineated for the first time from the anatomic, diagnostic, and surgical standpo ints in this study of 26 cases: 16 surgical and 10 postmortem, Transpo sition of the great arteries with situs solitus of the viscera and atr ia (S), D-loop ventricles (D), and L-transposition (L) was characteriz ed by six additional interrelated anomalies that largely determined su rgical management: (1) ventricular septal defect, usually conoventricu lar, in 96%; (2) malalignment of the conal septum, typically leftward and posteriorly, in 80%; (3) right ventricular hypoplasia in 50%; (4) pulmonary outflow tract stenosis in 27%; (5) ventricular malposition, such as superoinferior ventricles, in 23%; and (6) absent left coronar y ostium resulting in ''single'' right coronary artery in 23%, Complet e surgical repair was done in 81% of the surgical patients with a 12.5 % hospital mortality rate and no late deaths, When there was no pulmon ary outflow tract stenosis and intracardiac anatomy was uncomplicated, we undertook anatomic repair before 1 month of age, However, when pul monary outflow tract stenosis coexisted, complete repair was deferred until after age 1 year, our currently preferred operation being the RE V procedure (reparation a l'etage ventriculaire), When complex intraca rdiac anatomy precluded biventricular repair, a palliative procedure w as performed in 19% without mortality, Hence, this experience indicate s that surgical management of patients with the transposition of the g reat arteries (S,D,L) complex is feasible.