Heart Rate Induced Dynamic Coronary Ischemia Secondary to Aortic Valve Insufficiency from a Diminutive Aortic Valve Leaflet: Diagnosis and Repair

Hemodynamically significant congenital obstruction of the coronary arteries is a rare phenomenon. The potential etiology of these obstructions is variable including coronary os stenosis, occlusive fibrous webs, or a dysplastic aortic valve leaflet resulting in obstruction. In infants, diagnosis of these coronary lesions is often difficult due to the small anatomic size and limitations of imaging modalities in the setting of the underlying rapid heart rates. We present a case of an infant presenting in heart failure secondary to dynamic coronary artery insufficiency from a diminutive aortic valve leaflet and aortic sinus and variable levels of aortic valve insufficiency based on heart rate.