COARCTATION OF THE AORTA WITH RIGHT AORTIC-ARCH - A RARE CASE AND ATYPICAL CLINICAL PICTURE

We report the case of a 13-year-old girl with a diffusely hypoplastic right aortic arch, anomalous origin of the left subclavian artery, and a small, insignificant ventricular septal defect. The patient's pulse was forceful at the carotid arteries, but it was markedly weaker at a il extremities. Catheterization revealed that both common carotid arte ries arose from the dilated ascending aorta; the right subclavian and vertebral arteries arose from the hypoplastic posterior segment of the aortic arch, and the left subclavian artery arose from the normally d eveloped descending aorta. The pressure gradient between the ascending and descending aorta was 80 mmHg. A bypass grafting procedure was per formed to connect the ascending and the upper abdominal aorta. No pres sure gradient remained after the operation, nor was a gradient detecte d during 2 years of follow-up. The origin of both subclavian arteries distal to the area of coarctation resulted in an atypical clinical pic ture and delayed diagnosis. Ten previously reported cases of coarctati on of the aorta with right aortic arch are reviewed.