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A 14 year old girl returned for follow up. She initially presented with coarctation and right aortic arch at two months of age. Cardiac catheterisation determined the coarctation to be between the right common carotid (RCC) and right subclavian arteries (RSA), with a retro-oesophageal left subclavian artery. The intracardiac anatomy was normal. The patient underwent patch augmentation of the coarctation site at 2 months of age and again at 8 months of age for recurrent coarctation with a gradient of 80 mm Hg. An ascending aorta to the upper abdominal aorta conduit was placed at 15 months of age when she presented with recurrent coarctation. At 14 years of age cardiac catheterisation demonstrated a widely patent conduit but a residual coarctation between the right carotid and subclavian arteries with a 16 mm Hg gradient. A magnetic resonance angiogram including three dimensional reconstruction provided excellent definition of the isolated coarctation between the RCC and RSA, in addition to identifying additional areas of stenosis in the RSA and upper descending thoracic aorta (below left and right).
Right aortic arch in association with coarctation is extremely rare. This is in keeping with the principle of flow related development of the central great vessels. Coarctation is more likely to occur in situations of right to left shunting through the ductus arteriosus with decreased blood flow across the aortic isthmus. Additionally right aortic arch is strongly associated with right side obstructive lesions, in which there is reversed ductal flow and increased antegrade flow across the isthmus, which makes this entity even rarer.
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