Implantable Defibrillator in the Systemic Right Ventricle: Are We Still Baffled?

The prevalence of congenital heart disease (CHD) has remained relatively stable in newborns over the past several decades. With improvements in cardiac management, there are now more adults with CHD (ACHD) than children in most of the developed world.1 A unique challenge that remains is the management of a patient with a systemic right ventricle (sRV). Two prevalent conditions are D-transposition of the great arteries (D-TGA) repaired with an atrial baffle procedure and congenitally corrected transposition of the great arteries (CCTGA).