High-Risk Congenital Coronary Abnormalities in Patients with Bicuspid Aortic Valve

Abstract

Objective: Abnormal coronary artery origin (ACAO) from the opposite sinus with inter-arterial course of the ectopic proximal vessel is associated with the greatest potential for clinical manifestations, specifically sudden death. Data remain limited regarding the association between bicuspid aortic valve (BAV) and this potentially dangerous coronary variant reported in up to 0.6% in the general population. We investigated the frequency of this high-risk ACAO with inter-arterial course in our surgical series of BAV patients. Methods and Results: We conducted a retrospective study to identify BAV patients with ACAO and inter-arterial course who underwent elective aortic valve/root surgery between 2010 and 2019 in our tertiary center. A total of 279 consecutive patients with BAV were identified. Among these, four patients (1.4%) had ACAO with inter-arterial course. Three patients had abnormal right coronary artery arising from the theoretical opposite coronary sinus with intramural course. The fourth patient presented an abnormal left coronary artery from a single coronary artery arising from the theoretical right coronary sinus with a long inter-arterial course. Conclusion: Our results from a single-center experience support high-risk ACAOS may be more frequently diagnosed in BAV patients, especially in surgical series, justifying a careful preoperative assessment.