ABSTRACT
Congenitally corrected transposition of the great arteries is very much rarer than dextro (d-) transposition representing only 0.7% of all congenital anomalies.1 It is unique in that the circulation is physiologically normal with no shunts, no pressure load, and no cyanosis. Early in life, in the absence of associated anomalies, there are usually no symptoms. However, the right ventricle and tricuspid valve must function at systemic pressure and ultimately can fail. Frequently, however, associated anomalies are present, including abnormalities of atrioventricular conduction, pulmonary valvar and subvalvar stenosis, a large conoventricular or inlet VSD, and often an Ebstein-like malformation of the tricuspid valve. Dextrocardia is also present in approximately 25%. Corrected transposition is also a common form of single ventricle when the left-sided RV is hypoplastic and often little more than an infundibular outflow chamber. This form of corrected transposition is discussed in Chapter 25, ThreeStage Management of Single Ventricle.
