Abstract
A 6 year old girl was referred to our service after an incidental murmur was heard during a GP review for prominent neck veins when she was upset. She had a grade 1-2/6 soft systolic murmur at the left sternal edge. She was clinically asymptomatic and had good exercise tolerance. Chest X-ray was unremarkable and a 12 lead ECG showed deep Q waves in the inferolateral leads. An echocardiogram showed a significantly dilated left coronary artery (LCA) with multiple collaterals in the interventricular septum. The right coronary artery (RCA) was seen to arise from the pulmonary artery as shown in Figure 1. There was normal biventricular systolic function with no regional wall motion abnormalities. A cardiac CT confirmed that the RCA arose from the anterior and right-ward aspect of the pulmonary artery as shown in Figure 2. The LAD was dilated and tortuous, supplying the RCA via the posterior descending artery.Figure 2View Large Image Figure ViewerDownload Hi-res image Download (PPT) ARCAPA is an extremely rare anomaly, estimated to occur in 0.002% of the general population. It often presents asymptomatically with massive collateralisation between the normal and anomalous coronary artery required to maintain adequate myocardial perfusion. ARCAPA is associated with structural heart defects in a third of cases, where it tends to be diagnosed earlier. Reimplantation is the treatment of choice but surgical ligation of the RCA with saphenous grafts have also been described.
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