Nagamalesh U M, Shankar Shetty Ravi, Narayan Ravi, Kumar Anil, Naik Ravi, Uzma Sadath, Karthik Naidu K C
Department of Cardiology, MS Ramaiah Medical College and Memorial Hospital, Bengaluru, IndiaaIndia.
Department of Cardio-thoracic Surgery, MS Ramaiah Memorial Hospital, Bengaluru, IndiabIndia.
J Saudi Heart Assoc. 2017 Oct;29(4):300-304. doi: 10.1016/j.jsha.2017.03.002. Epub 2017 Mar 14.
Supravalvular aortic stenosis is an uncommon but well characterized congenital narrowing of the ascending aorta above the level of the coronary arteries. It can be a familial disorder, can occur sporadically, or can be associated with Williams syndrome. We are reporting a very rare presentation of supravalvular aortic stenosis with associated left ventricular diverticulum and cleft mitral valve. Repair consisted of resection of the ascending aorta, patch augmentation of the aortic root, and mitral valve repair. Follow-up echocardiography demonstrated normal mitral and aortic valve function and a postoperative three-dimensional computed tomographic scan showed a normal shape of the reconstructed ascending aorta.
瓣上主动脉狭窄是一种不常见但特征明确的先天性升主动脉狭窄，位于冠状动脉水平以上。它可以是一种家族性疾病，可散发发生，或与威廉姆斯综合征相关。我们报告了一例非常罕见的瓣上主动脉狭窄合并左心室憩室和二尖瓣裂的病例。修复包括切除升主动脉、主动脉根部补片扩大以及二尖瓣修复。随访超声心动图显示二尖瓣和主动脉瓣功能正常，术后三维计算机断层扫描显示重建的升主动脉形状正常。
