Pseudocoarctation of the aortic arch in a patient with Turner's syndrome

Pseudocoarctation of the aortic arch is a rare congenital anomaly which resembles true coarctation by the presence of a narrowing in the descending thoracic aorta immediately distal to the origin of the left subclavian artery. However, it is differentiated from that entity by the absence of a significant hemodynamic obstruction and by the presence of aneurysmal dilatation proximal and distal to the area of narrowing. As a result, the commonly observed clinical findings of coarctation such as a prominent collateral circulation and decreased blood pressure in the lower extremities are not observed. "Kinking" or "buckling" are common adjectives used to describe the roentgenographic appearance of the aortic arch in this condition. The association of various congenital cardiac abnormalities with pseudocoarctation has been reported, and are similar to those commonly seen with true coarctation. We now present the first reported case of pseudocoarctation associated with a probable bicuspid aortic valve in a patient with Turner's syndrome.