Congenital Aortic Valvular Stenosis and Pseudocoarctation ("Kinking, Buckling") of the Arch of the Aorta

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Abstract
Pseudocoarctation is an uncommon congenital anomaly of the arch of the aorta that is embryologically and anatomically similar to coarctation but without aortic occlusion. Consequently, hemodynamic abnormalities do not occur and the arm and leg blood pressures are normal and equal; collateral circulation does not develop. In the conventional roentgenogram, there is a double rounded shadow along the left superior mediastinum. The upper mass may be mistaken for a mediastinal tumor or aneurysm; the lower may be interpreted as the aortic knob. Angiocardiography, however, readily delineates the unusually dilated and deformed arch of the aorta and demonstrates that the lower shadow is, in reality, the dilated ("poststenotic") descending aorta. Heretofore, except for 2 patients with aneurysmal dilatation of the aortic sinuses, associated anomalies of the aorta have not been reported in pseudocoarctation. In 4 patients with congenital aortic stenosis and pseudocoarctation, 2 had bicuspid aortic cusps. An adult with a bicuspid valve died of heart failure owing to parietal endocardial fibrosis and fibroelastosis. Accordingly, as in other types of congenital cardiovascular defects, patients with pseudocoarctation should have antibiotic or chemical prophylactic therapy whenever dental or surgical procedures are contemplated.