Multimodal Imaging Directs Decision Making in Intraseptal L-AAOCA

Congenital coronary artery abnormalities represent an uncommon class of congenital heart disease with an associated risk of sudden cardiac death. There is a paucity of data for the evaluation and management of affected patients with an intraseptal course.

A 27-year-old woman was found to have a single coronary trunk arising from the right sinus with a 2.7 cm intraseptal course of the left main coronary artery. She underwent stress cardiac magnetic resonance and catheterization, to assess for inducible ischemia before surgical repair with transconal unroofing. Her recovery was uneventful, and she remains free of symptoms.

Management of congenital coronary artery anomalies requires an exhaustive evaluation. Further studies are warranted to define the ideal work-up, indication, and timing of intervention and the efficacy of medical management in improving symptoms.

Congenital coronary anomalies represent a rare but consequential cause of exertional chest pain. This diagnosis requires multimodal imaging to inform decision making.