Bilateral Coronary Ostial Atresia: A Malignant Anatomic Variant.

BACKGROUND

Bilateral coronary ostial atresia (BCAO) is one of the rarest congenital cardiac abnormalities. Consequently, outcomes in patients with bilateral coronary ostial atresia are poorly characterized.

CASE SUMMARY: We report the clinical course of a term girl with prenatally diagnosed ventricular septal defect and normal ventricular function, who rapidly deteriorated at 6 weeks. Catheterization confirmed BCOA with the right coronary artery connecting to the right atrium via a fistula and the left coronary filling from the right ventricular outflow tract.

DISCUSSION: This case highlights the malignant clinical course of BCOA. Early detection is limited by difficulty detecting coronary anatomy on echocardiography. Outcome was poor despite extracorporeal membrane oxygenation support. To our knowledge, this variant of BCOA has not been described.

TAKE-HOME MESSAGES: BCOA is rare and lethal; early stability may mask rapid deterioration. Suspicious coronary imaging warrants urgent angiography. Limited management options include extracorporeal membrane oxygenation or transplantation.