Quadricuspid aortic valve is a rare anomaly, and most patients require surgery for aortic regurgitation in the 5th or 6th decades of life; only a few cases of aortic valve repair in childhood have been reported. A 3-year-old boy was scheduled for ventricular septal defect closure and aortic valve repair. Quadricuspid aortic valve was an incidental finding at operation; it was repaired by joining the left anterior and right anterior cusps. At the 9-month follow-up, the patient had no more than mild aortic regurgitation. We emphasize the importance of detecting this anomaly, especially in children with aortic valve regurgitation.
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