ObjectiveTo evaluate clinical results of left ventricular retraining followed by double switch operation (DSO) for patients with congenitally corrected transposition of the great arteries (CCTGA) and a deconditioned morphologically left ventricle (mLV). MethodsClinical data of 14 patients with CCTGA and a deconditioned mLV who underwent surgical therapy in Fu Wai Hospital from May 2005 to May 2011 were retrospectively analyzed. There were 8 male and 6 female patients with their age of 2.5-72.0 (34.4±24.0) months and body weight of 5.1-23.0 (12.7±4.9) kg. Preoperative diagnosis was confirmed by echocardiography, angiography or cardiac catheterization. Major concomitant anomalies included tricuspid regurgitation (TR) in 13 patients, restrictive ventricular septal defect in 10 patients, atrial septal defect or patent foramen ovale in 7 patients, mild pulmonary valve stenosis in 4 patients, patent ductus arteriosus in 4 patients, and third-degree atrioventricular block in 1 patient. All the patients underwent first-stage morphologic left ventricular retraining under general anesthesia followed by second-stage atrial switch and arterial switch operations (DSO) under cardiopulmonary bypass with the interval of 0.67-34.0(10.23±9.47)months. ResultsAfter the first-stage morphologic left ventricular retraining, there was no postoperative complication or death. During follow-up, mLV end-diastolic diameter (mLVEDd) and posterior wall thickness of mLV were significantly larger than preoperative parameters (P < 0.05). The interventricular septum moved partially towards morphologically right ventricle (mRV). TR degree was significantly decreased, the pressure gradient across the pulmonary artery band was significantly increased (P < 0.05), and left ventricular ejection fraction (LVEF) was not statistically different from preoperative LVEF. And mLV/mRV pressure ratio was significantly increased (P < 0.05). After the second-stage DSO, 2 patients died with the in-hospital mortality of 14.3% (2/14). The causes of death included serious arrhythmia, circulatory collapse and sudden death. Early postoperative complications included pulmonary infection in 6 patients, atrial arrhythmias in 2 patients, pleural effusion in 2 patients, pneumothorax in 1 patient, diaphragmatic paralysis cured by diaphragm placation in 1 patient, respiratory tract hemorrhage in 1 patient, mild aortic insufficiency in 1 patient, peritoneal dialysis for 1 patient, extracorporeal membrane oxygenation for 1 patient, and tracheal intubation for a second time for 1 patient. All the 12 patients who were discharged alive were followed up for 2 to 8 years. One patient died during follow-up with the late mortality of 8.33% (1/12), and the cause of death was serious arrhythmia and circulatory collapse. Eight patients were in New York Heart Association (NYHA) classⅠ, and 3 patients were in NYHA class Ⅱ. Major late complications included left ventricular dysfunction in 3 patients, moderate aortic valve regurgitation in 3 patients, and moderate mitral valve regurgitation in 1 patient. ConclusionShort-term clinical results of left ventricular retraining followed by DSO for patients with CCTGA and a deconditioned mLV are satisfactory, and its middleand long-term results need further follow-up. But postoperative left ventricular dysfunction and new-onset aortic valve regurgitation deserve more attention.