“1/3 Yacoub” for correction of supravalvular aortic stenosis: valve-sparing root enlargement and ascending aorta replacement in an adult patient

Congenital supravalvular aortic stenosis is a rare form of left ventricular outflow-tract obstruction, which is frequently associated with genetic disorders such as Williams-Beuren syndrome, despite spontaneous occurrence still being possible. Over the years, different surgical approaches to relieve the stenosis have been proposed; among them the McGoon, the Doty and the Brom repair are the best-known. Nevertheless, all these techniques especially address a paediatric population, and treatment options in adults are rarely described. Older patients, indeed, do not present the concern of preserving the growth potential of the aorta, therefore root replacement with or without aortic valve-sparing is an attractive alternative. We report our surgical approach in a 40-year-old female affected by severe supravalvular aortic stenosis, consisting of a modification of the Yacoub technique, in order to perform an enlargement of both the aortic root and sinotubular junction, sparing the aortic valve. The aim is to provide practical insights into the evolving field of valve-sparing repair for supravalvular aortic obstruction in young adults.