Kosaka Yoshimichi, Hoshino Shuichi, Azuma Takashi, Nishiya Yasushi
Department of Cardiovascular Surgery, Toyama Prefectural Central Hospital, Toyama, Japan.
Jpn J Thorac Cardiovasc Surg. 2003 Dec;51(12):675-7. doi: 10.1007/s11748-003-0009-4.
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.
一名6岁男孩出现主动脉弓弥漫性狭窄。他在2岁时因局限性瓣上主动脉狭窄接受了补片扩大手术。初次手术前，发现主动脉弓和降主动脉发育不全，直径为主动脉瓣环直径的60%至73%，无压力阶差。4年后，发现主动脉弓出现进行性弥漫性狭窄，直径为主动脉瓣环直径的42%。遂再次手术，包括对主动脉弓进行补片扩大。修复部位远端再次新出现了40 mmHg的压力阶差。这是一例罕见的瓣上主动脉狭窄病例，因远端主动脉弥漫性狭窄进展而需要再次手术。
