Combined atrial and arterial switch operations for congenitally corrected transposition

Conventional repair of congenitally corrected transposition of the great ar teries (CCTGA) is directed at eliminating the associated defects and leaves the right ventricle ill a systemic position. Tile long-term outcome of thi s procedure may involve deterioration of right ventricular function with tr icuspid regurgitation and failure of the conduction system. Mie describe tw o consecutive patients with CCTGA, one of whom had apicocaval juxtaposition . The patients were aged 19 and 16 months, respectively, and both underwent a combination of atrial and arterial switch. These are the first two repor ted cases of successful completion of this type of operation in Taiwan. Our review of previously reported cases suggested that no significant differen ce exists in the outcome of patients with this condition who undergo either arterial switch or Rastelli-type repair plus atrial redirection. However, reported patients who underwent anatomic repair had lower early mortality, late mortality, and incidence of complete heart block than those who underw ent conventional repair. The present two cases and our review of the litera ture suggest that, among patients with apicocaval juxtaposition, 1) Mustard operation is optimal for patients with small atrial volume; 2) one-and-one -half ventricular repair may be helpful to the outcome, especially when tre atment is combined with Rastelli-type repair. and 3) excellent access to th e ventricular septal defect through the tricuspid valve is afforded via a l eft atriotomy. From the present two cases and our review of the literature, we conclude that anatomic repair is superior to conventional repair of CCT GA in terms of protection against dysfunction and failure of the anatomic r ight ventricle, tricuspid valve, and conduction system. Long-term follow-up is mandatory.