Bicuspid Aortic Valve: A Neglected Feature of Shone’s Complex?

Excerpt

The traditional description of complete Shone complex includes a supravalvular mitral membrane, parachute mitral valve, subaortic stenosis, and coarctation of the aorta. These anomalies present with varying degrees of severity. However, of the eight cases reported by Shone et al. [10] in 1963, only two exhibited all four features; in the remaining six cases, only two or three of these features coexisted. Only supravalvular mitral ring and subaortic stenosis were found in all eight patients, whereas parachute mitral valve and coarctation of the aorta were found in 50 %. Likewise, a bicuspid aortic valve was present in 50 % of patients [10]. Indeed, bicuspid aortic valve has been reported in several recent Shone complex patient series [4‐6, 8]. Grimaldi et al. [5] reported six Shone complex patients of whom five had bicuspid aortic valves and all six had a history of aortic coarctation. The bicuspid valve—Shone complex association was reported before by Bolling et al. [1] in their experience of surgical outcomes in 30 patients with Shone’s complex. Only 63 % of patients had all four lesions. Supravalvar mitral ring was present in 73 %, parachute mitral valve and subaortic stenosis in 86 %, coarctation of the aorta in 96 %, and bicuspid aortic valve in 63 % of patients. …