Progressive diffuse aortic stenosis after localized supravalvular aortic stenosis repair.

A 6-year-old boy presented with diffuse stenosis of the aortic arch. He had undergone patch augmentation for localized supravalvular aortic stenosis at 2 years of age. Before the initial operation, the aortic arch and descending aorta were noticed to be hypoplastic with a diameter of 60 to 73% of that of the aortic annulus, without a pressure gradient. 4 years later, progressive diffuse stenosis of the aortic arch with a diameter of 42% of that of the aortic annulus was found. Reoperation, consisting of patch augmentation of the aortic arch, was carried out. A pressure gradient of 40 mmHg distal to the repair was newly recognized, again. This is a rare case of supravalvular aortic stenosis, which required reoperation because of the progression of diffuse stenosis of the distal aorta.