The results of a two-stage double switch operation for congenital corrected transposition of the great arteries with a deconditioned morphologically left ventricle.

OBJECTIVES: The purpose of this retrospective study was to evaluate a two-stage double switch operation, morphological left ventricular (mLV) retraining followed by an atrial-arterial switch operation, in the management of patients with congenitally corrected transposition of the great arteries (CCTGA) and a deconditioned mLV.

METHODS: Between May 2005 and May 2011, 14 patients with CCTGA and a deconditioned mLV anomaly underwent the two-stage double switch operation. There were eight males and six females aged between 2.5 and 72 months (mean: 34.4 ± 24.0 months) old and weighing from 5 to 23 kg (mean: 12.7 ± 4.9 kg). The major associated malformations included: tricuspid regurgitation (TR, n = 13); restrictive ventricular septal defect (n = 10); atrial septal defect or patent foramen ovale (n = 7); mild pulmonary stenosis (n = 5) and patent ductus arteriosus (n = 4). These patients underwent morphological left ventricular retraining by means of pulmonary artery banding under general anaesthesia, which was then followed by a double switch operation under general anaesthesia and cardiopulmonary bypass.

RESULTS: There were no deaths or complications during the hospital stay or follow-up for the mLV retraining. In comparison with preoperative conditions, the mLV end-diastolic diameter (mLVEDd), the posterior wall thickness of the mLV and the mLV/mRV pressure ratio were all increased; the interventricular septum had moved partially to the midline position and TR had decreased. After the atrial-arterial switch procedure, 2 patients died during the perioperative period. The causes of death included serious cardiac arrhythmia with circulatory collapse and sudden death. The others were followed up for 2-8 years: 1 patient died from serious cardiac arrhythmias with circulatory collapse in the follow-up period. With regard to the others, 8 were evaluated as New York Heart Association Functional Class I, and the other 3 as Class II. Moderate aortic valve regurgitation was noted in 3 patients and moderate mitral regurgitation in 1 patient.

CONCLUSIONS: For CCTGA children with degraded mLV, the two-stage double switch procedure can be performed with low mortality and morbidity and may be an appealing alternative to conventional repair. mLV retraining should be performed as early as possible. The second-stage atrial-arterial switch procedure showed satisfactory early and mid-term results. More attention should be paid to the long-term function of the mLV and the aortic valve.