Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect that usually presents as dilated cardiomyopathy in infancy. From 1984 to 2005, 13 (five males and eight females, 0.13%) out of 9,950 patients with congenital heart disease were identified as ALCAPA at our institute. Corrective surgery was performed at a median age of 9 months (range: 2 months to 5 years). Eleven patients underwent direct reimplantation of the left coronary artery (LCA) to the aorta, while two received extrapulmonary baffling. The overall survival rate was 92%. Only one patient died 5 months after reimplantation of the LCA due to acute myocardial ischaemia. Left ventricular ejection fraction (LVEF) was significantly lower in the eight (62%) patients operated during infancy than in those operated beyond 5 months (median: 35% vs. 75%). Left ventricle function was normalised in 11 patients (85%). Among the eight patients without concomitant mitral annuloplasty, mitral regurgitation (MR) improved to a mild or trivial degree in six patients and remained at the pre-operative level in two patients. Pathologic Q wave was noted in 11 patients, which eventually regressed in all except two cases. The median interval of recovery was 16 days, 6 months and 24 months for MR, LVEF and electrocardiogram (ECG) changes, respectively. In conclusion, ALCAPA is also a rare disease in Asian countries, such as Taiwan. The subsequent recovery of MR, left ventricular (LV) function and even pathologic Q wave can be expected after dual coronary repair, regardless of the age at repair.
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