Congenitally corrected transposition: not correct at all

Congenitally corrected transposition of the great arteries is a rare congenital defect with several management options. Disagreement continues on strategies, such as anatomic repair, physiologic repair or observation-only. This review discusses recent data that provide further guidance for clinical decision-making.

New data provide greater insights into practice patterns and outcomes. Recent data from high-volume centers show progressively high rates of systemic right ventricle dysfunction over time with lower rates of systemic left ventricle dysfunction following anatomic repair; there is a statistical trend towards better survival of anatomic repair patients. Data comparing anatomic repair to observation showed that anatomic repair patients had a lower hazard of reaching a composite adverse outcome. These complex operations are predominantly performed at a small subset of congenital heart surgery centers.

Anatomic repair compared with physiologic repair may have better outcomes, although there are relatively high rates of morbidity for both approaches. In the patient without associated lesions, nonsurgical management can have excellent outcomes but is complicated by right ventricular failure over time. Multicenter research will help determine risk factors for bad outcomes; management at high volume, experienced centers will probably be beneficial for this complex patient population.