Methods: We retrospectively reviewed the charts of seven patients (2 females, 5 males, mean age 53.3±52.1 months; range 6 to 151 months) with the diagnosis of ALCAPA who underwent Takeuchi operation combined with pulmonary artery reconstruction between January 2007 and June 2010 in our clinic. Detailed cardiovascular examination including electrocardiography, chest X-ray, echocardiography, computed tomography coronary angiography and cardiac catheterization were performed preoperatively on all subjects. Demographic characteristics as well as pre- peri- and postoperative data were documented and evaluated.

Results: The most common symptom was periodic dyspnea (57.1%), however 28.6% of the patients were asymptomatic. The mean cardiopulmonary bypass duration and mean aortic cross-clamp time were 105.8±33.0 and 69.4±35.1 min, respectively. Postoperative durations of mechanical ventilation, Intensive Care Unit stay, and normal ward stay were 15.0±14.9 hours, 1.8±0.5 days, and 6.0±2.7 days, respectively. Malignant arrhythmia was the only complication of surgery which was identified in one patient who died at the 36 hour of post cardiac surgery. Six of seven patients who underwent Takeuchi operation with pulmonary artery reconstruction (85.7%) survived and among all of the surviving patients were symptom-free within a mean follow-up of 11.7±10.3 months.

Conclusion: ALCAPA is a kind of pathology which can be corrected by surgery and an early diagnosis and intervention are of utmost importance for the long-term prognosis.