Journal of Pediatric Cardiology and Cardiac Surgery

A 25-year-old woman with congenital aortic stenosis and severe intellectual disability associated with ring chromosome 18 syndrome was referred for surgical evaluation. She had previously undergone aortic valvuloplasty twice. Her aortic stenosis and regurgitation were severe, therefore another surgical intervention was planned. Preoperative contrast-enhanced computed tomography showed a narrow aortic annulus (19.0 mm diameter). Because anticoagulation after surgery was not considered feasible, bioprosthetic aortic valve replacement was performed in conjunction with the Konno procedure to enlarge the annulus. Her postoperative course was uneventful. Contrast-enhanced computed tomography after surgery showed the sinus of Valsalva properly enlarged, and distances between the coronary ostia and the leaflets of the bioprosthetic valve were sufficiently large, which may lower the risk of coronary flow obstruction when the aortic valve would be replaced further in a transcatheter valve-in-valve way. Bioprosthetic aortic valve replacement in anticipation of future use of such a transcatheter technique is a surgical option that should be considered for young adult patients with severe AS and difficulty in anticoagulation.