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Whereas anomalous origin of the left coronary artery from the pulmonary artery is rather well known (Bland-White-Garland syndrome, ALCAPA), only a few cases have been described with anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA).
A 12 year old patient was referred with this provisional diagnosis. He had been followed since the age of 5 years because of a muscular ventricular septal defect which had closed spontaneously. There were no pathological signs at physical examination; exercise tolerance was normal. The ECG showed incomplete right bundle branch block without signs of ischaemia. Echo revealed trivial tricuspid and pulmonary valve regurgitation with normal right ventricular pressure. The left coronary artery (LCA) appeared with a large central part of the left anterior descending artery (LAD) but without a visible circumflex artery. The right coronary (diameter 3 mm) was visible anterior to the aortic root (AO) arising from the pulmonary artery (PA) (top panel). There was a “retrograde” laminar blood flow into the PA (middle panel). Angiography confirmed the normal course of the left coronary artery with delayed retrograde visualisation of the right coronary artery via collaterals and run-off of the contrast medium into the pulmonary artery (bottom panel).
Although there are no signs of myocardial ischaemia orthotopic direct reimplantation of the anomalous right coronary artery is planned. The variable and not precisely predictable course of Bland-White-Garland syndrome were taken into consideration for this decision.
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