Methods: Nine patients with ALCAPA who had dual coronary system repair were retrospectively analyzed. The age of the patients ranged from 1.5 months to 23 years and seven of them were younger than 3.5 months old. Echocardiography showed that left ventricles of the patients were severely dilated with a left ventricular shortening fraction of 8-17%. The diagnosis was confirmed by multidedector computed tomography and conventional angiocardiography in three patients. Four patients were diagnosed with dilated cardiomyopathy previously. All patients underwent restoration of dual coronary artery system repair: Takeuchi procedure in three, autologous pericardial tube interposition in two, extrapulmonary tunnel modification in three and direct re-implantation in one patient.

Results: No mortality was seen in the early postoperative period, except a nine-month-old infant with preoperative severe mitral regurgitation. Left ventricular mechanical support was needed for an 1.5 monthold infant who developed severe left ventricular dysfunction while weaning off cardiopulmonary bypass and discontinued at 36 hours postoperatively In all eight patients with preoperative dilated left ventricle, the ventricular function returned to normal level within three months following surgery (shortening fraction ranging from 32% to 44%). Mild and moderate preoperative mitral regurgitation resolved with improved left ventricular function. The patients were followed-up with a mean of 7.5 years (range, 9 months to 14 years) in the late term. No mortality was seen in the late term.

Conclusion: Dual coronary artery system restoration improves myocardial function early after the operation in ALCAPA patients. Mild to moderate mitral regurgitation may resolve without any additional surgical intervention after coronary artery system restoration with improved left ventricular function. In children, particularly in neonates and infants, ALCAPA should be carefully investigated before diagnosis of dilated cardiomyopathy.