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Although only about 10% of infants born with tetralogy of Fallot survive without surgical treatment beyond 20 years, it still remains the most common cyanotic congenital heart disease in adults. A 25 year old man presented with complaints of dyspnoea on exertion and orthopnoea. Physical examination was abnormal for cyanosis, clubbing, and a loud pansystolic murmur. A two dimensional echocardiogram confirmed the presence of tetralogy of Fallot. The patient underwent right and left cardiac catheterisation. A 6 French Judkins left coronary catheter (size 4) was used for cannulation of the coronaries. Coronary angiography (right) revealed all three coronaries arising from a single coronary artery originating from the left coronary sinus.
A single coronary artery arising from the aorta is associated with 5–20% of major coronary artery anomalies. The single artery may arise from the right or left coronary sinus. It has several variations and different classifications have been proposed. Usually it is asymptomatic but premature deaths have been associated with it. Atherosclerosis in the single coronary artery can have dire consequences since it is supplying the whole heart. Other congenital malformations associated with this coronary anomaly include transposition of great vessels, truncus arteriosus, coronary-cameral fistula, and bicuspid aortic valves. These anomalies have important surgical implications for patients undergoing corrective surgery for these malformations.