An asymptomatic 2-month-old boy was referred because of a cardiac murmur. Chest radiography revealed borderline cardiomegaly. An electrocardiogram showed left ventricular hypertrophy and myocardial ischaemic changes, including ST depression in V1-3 and QRS-T discordance. His creatine kinase-MB was 17.6 ng/mL, and cardiac troponin-I 0.23 ng/mL. Transthoracic echocardiography in the parasternal short axis view (Fig. 1a) demonstrated anomalous origin of the right coronary artery from the left sinus of Valsalva, near the origin of the left coronary artery. The proximal part of the right coronary artery passed between the aortic root and the subpulmonary infundibulum, and was narrow proximally, but markedly dilated beyond its middle portion (Fig. 1b). A coronary arterial fistula permitting continuous turbulent flow was present communicating with the apex of the right ventricle (Fig. 1c). Aortography in the true posterior-anterior (Fig. 2a) and lateral views (Fig. 2b) confirmed the findings.

Figure 1

Figure 2

Congenital anomalies of the coronary artery are commonly associated with other congenital cardiac malformations, but a coronary arterial anomaly combined with a fistula is rare. Several reports indicate a relatively high risk of sudden death during exercise when a coronary artery passes between the aortic root and the subpulmonary infundibulum.Reference Maresi, Argo, Spano, Novo, Cabibi and Procaccianti¹ This will be taken into account when planning further follow-up for our patient.