Single coronary artery associated with severe bicuspid aortic valve stenosis

Abstract

A 75-year-old male with history of hypertension and hypercholesterolemia, presented with recurrent chest pain. Transthoracic echocardiography revealed severe bicuspid aortic valve stenosis. Pressure gradient over the aortic valve was 152 mm Hg and 94 mm Hg, peak and mean respectively; and calculated aortic valve area (AVA) was 0.77 cm2, with concentric left ventricular hypertrophy and preserved global left ventricular systolic function (LVEF) at 65%. Coronary angiography demonstrated a single coronary artery arising from the right sinus of Valsalva (Fig. 1). The right coronary artery (RCA) was hyperdominant and it branched into the left descending coronary artery (LAD) giving rise from the distal branch of acute marginal artery, and the circumflex coronary artery (CX) giving rise from the distal branch of posterolateral artery. All branches of a single coronary artery were free of significant atherosclerotic disease. Cardiac computed tomography was subsequently performed and it confirmed normal course of all branches (Fig. 2) as well as severe calcifications of the aortic valve (Fig. 3). The patient eventually underwent surgical aortic valve replacement with uneventful hospital outcome. Figure 1 Left anterior-oblique view of the coronary angiography showing single coronary artery arising from the right sinus of Valsalva, with the hyperdominant right coronary artery branching into the left ascending coronary artery and the circumflex coronary ... Figure 2 Cardiac computed tomography in the left anterior-oblique view demonstrating single coronary artery with normal course arising from the right sinus of Valsalva and the origin of left anterior descending artery from acute marginal branch and the origin ... Figure 3 Cardiac computed tomography in the right anterior-oblique view showing severe calcifications of the aortic valve (arrow). Single coronary artery is an extremely rare congenital coronary artery anomaly where only one coronary artery arises from the aorta from a single coronary ostium to supply the entire heart, occurring in 0.02–0.06% of coronary angiograms [1]. Although some patterns of a single coronary artery may cause clinical manifestations of myocardial ischemia and sudden death, most cases are asymptomatic [2]. The clinical significance of a single coronary artery originating from the right sinus of Valsalva depends on its relationship with the aorta and the main pulmonary trunk [3]. The diagnosis and understanding of anomalies in coronary circulation are particularly important for further therapeutic decisions and management in cases with additional coronary and/or valvular heart surgery. Our patient presented two particularities: this is the oldest age at which such a diagnosis has been made. The second particularity is the combination of a single coronary artery arising from the right sinus of Valsalva associated with severe bicuspid aortic valve stenosis undergoing surgical aortic valve replacement.