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.
Kosaka
Yoshimichi
Y
Department of Cardiovascular Surgery, Toyama Prefectural Central Hospital, Toyama, Japan.
Hoshino
Shuichi
S
Azuma
Takashi
T
Nishiya
Yasushi
Y
D001013:Aorta, Thoracic / Q000473:pathology / Q000601:surgery; D001015:Aortic Arch Syndromes / Q000601:surgery; D021921:Aortic Stenosis, Supravalvular / Q000209:etiology* / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D002315:Cardiopulmonary Bypass; D002648:Child; D018450:Disease Progression; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008297:Male; D011666:Pulmonary Valve Stenosis / Q000601:surgery; D012086:Reoperation