We report a case of a 23-day-old girl who presented with repeated life-threatening myocardial ischemia from intermittent aortic insufficiency because of fixation of the left coronary cusp against the aortic wall. The patient underwent aortic valve repair with partial commissuroplasty of the left-right and left-noncommissures. After the surgical intervention the patient has been well, with no ischemic event for over 1 year. This diagnosis should be considered as a differential diagnosis in pediatric cases with myocardial ischemia.
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