Anatomic repair of complex transposition with en bloc rotation of the truncus arteriosus: 10-year experience†

Transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction are commonly called complex transposition. The traditional method of repair is the Rastelli procedure. Aortic translocation (Nikaidoh 1984) provides a more anatomic repair of this malformation. En bloc rotation of the truncus arteriosus (double root translocation, half turn truncal switch procedure) was introduced in 2003 (Yamagishi), and offers a complete anatomic repair with growth potential in all tubular structures. The aim of this study was to analyse our general experience with this method and the mid-term results concerning growth of the tubular structures as well as the function of the semilunar valves, if preserved.

Nineteen patients with transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction or similar cases of double outlet right ventricle (DORV) have been treated by an en bloc rotation of the truncus arteriosus in our centre since 2003. Patient age ranged between 4 days and 6.46 years. The median age was 0.39 [0.1; 2.25] years. Weight ranged between 3.1 and 18.8 kg. Median weight was 5.6 [3.6; 9] kg. Five patients had received between 1 and 4 palliative procedures prior to the definitive repair. The pulmonary valve could be preserved in 15 cases, whereas a transannular patch was necessary in 4 cases.

One patient died of chronic left ventricular failure during the hospital stay. One patient acquired a severe cerebral haemorrhage 3 weeks after the operation. She was discharged and died 6 months later. One patient is not in a follow-up programme. Sixteen patients are now followed over a period of 153 days to 9.96 years. Aortic and pulmonary valves showed proportional growth during the follow-up period. The preserved pulmonary valves were small for age, but kept their competence satisfactorily. Three patients required a reoperation: one aortic valve repair, 1 permanent pacemaker, VSD closure.

Up to now, reoperations had been caused by technical issues. The reoperation rate can be kept low, by understanding some important features of this procedure and avoiding these problems. Complete anatomic repair with growth potential and satisfactory preserved pulmonary valve function is possible.