Congenitally corrected transposition of great arteries with severe rhythm disturbances

Within recent years, much scientific attention has been devoted to adults with congenital heart disease (CHD) and probable complications. Congenitally corrected transposition of the great arteries (CCTGA) is a rare, complex form of congenital heart defects. CCTGA is characterized by atrioventricular (AV) and ventriculoarterial (VA) discordance and, hence, by a physiologically normal direction of blood flow. The development of complete AV block and global ventricular dysfunction has been identified as the cause of cardiac death. Although the development of arrhythmias represents a major cause of morbidity and mortality in patients with CHD, the account of all implantations of pacemakers and implantable cardioverter defibrillators (ICD) is less than one percent. This paper presents a case of CCTGA with severe rhythm disorders, discusses probable treatment options, and offers indications of ICD implantation in patients with CHD.