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A 25 year old man with a history of congenitally corrected transposition of the great vessels was referred for cardiac catheterisation before possible surgical management of systemic atrioventricular valve regurgitation. The catheterisation was notable for the finding of a single coronary artery supplying the entire myocardium (panels A and B).
The incidence of an isolated single coronary artery is noted to be 0.07% of adults referred for coronary arteriography. However, the incidence is much higher in patients with congenital heart disease and in particular those with conotruncal malformations. Approximately a third of the cases of isolated coronary arteries have been reported in the setting of transposition of the great vessels and tetrology of Fallot. Several authors have noted that 19% of patients undergoing coronary angiography in the setting of truncus arteriosus, 17% of patients in the setting of pulmonary atresia, and 2% in the setting of tetrology of Fallot have the finding of an isolated single coronary artery. It is important to know the presence of anomalies of the coronary arteries before cardiac surgery, especially in the setting of congenital heart disease, in order to minimise the risk of accidental ligation or transection.
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