Shih Beatrice Chia-Hui, Lim Jae Hong, Min Jooncheol, Kim Eung Re, Kwak Jae Gun, Kim Woong-Han
Department of Pediatric Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Korean J Thorac Cardiovasc Surg. 2019 Apr;52(2):100-104. doi: 10.5090/kjtcs.2019.52.2.100. Epub 2019 Apr 5.
Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.
肖恩综合征是一种罕见的先天性疾病,涉及左心的4处梗阻性病变,具体如下:降落伞样二尖瓣、二尖瓣瓣上环、主动脉瓣下狭窄和主动脉缩窄。文献中很少报道成年患者存在其中2种或3种病变的不完全形式,这意味着关于手术策略和临床随访的总体数据不足。在此,我们报告一例31岁女性患者,诊断为伴有降落伞样二尖瓣和主动脉缩窄的不完全形式的肖恩综合征,该患者接受了成功的一期手术修复。