Congenitally Corrected Transposition of the Great Arteries (ccTGA)

Rare conditionCongenitally corrected transposition characterized by atrio-ventricular and ventriculo-arterial discordance—this creates a physiologically ‘corrected’ circulation but leaves the morphologic right ventricleVentricles (mRV) in the systemic position. Very heterogenous condition—majority of cases have associated lesions, most commonly a VSDVentricular septal defect (VSD) and the presence or absence of pulmonary or sub-pulmonary obstruction. Management depends on the associated lesions but has to take into account the unpredictable function of both the mRV and the tricuspid valveTricuspid valve (TV) in the systemic position. This makes for complex management strategies that can either focus on ‘physiological correction’ of the associated lesions (leaving the mRV as the systemic ventricleVentricles) or ‘anatomical correction’ that also restores the morphologic left ventricleVentricles (mLV) to the systemic position. The latter are referred to as the ‘double switch’ procedures that involve both an atrial switch and correction of the outflow tracts—either arterial switch or RastelliRastelli-type procedure. Anatomical repair is generally reserved for the younger age-groups and may require re-training of the mLV with pulmonary artery bandingPulmonary artery banding (PAB) if it has become deconditioned. Management is controversial and has to be individualized since occasionalLeft ventricle re-training cases never require any intervention yet the majority require surgery and anatomical repair if possible isLevo-transposition preferredL-tga double-switch when suitable.