Salve Gananjay G, Javali Satish R, Dalvi Bharat V, Krishnanaik Shivaprakash
Department of Pediatric Cardiovascular and Thoracic Surgery, Seven Hills Hospital, Marol-Maroshi Road, Andheri East, Mumbai, India.
Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):244-7. doi: 10.4103/0974-2069.189124.
Aneurysms of ascending aorta are rarely seen in pediatric age group. Only few cases with Marfans syndrome have been reported in the literature. Preferred treatment for these children has been the standard Bentall procedure (aortic root replacement with composite graft prosthesis). We report a 4-year-old male child with huge aneurysm of ascending aorta and aortic root dilation with severe aortic regurgitation, having phenotypic features of Loeys-Dietz syndrome type I. He underwent Bentall procedure with a novel modification (medial trap-door technique for coronary reimplantation). Short-term result of this procedure is encouraging and he is asymptomatic for the last 14 months of follow-up.
升主动脉瘤在儿童年龄组中很少见。文献中仅报道了少数患有马凡综合征的病例。对于这些儿童，首选的治疗方法一直是标准的Bentall手术（用复合移植假体置换主动脉根部）。我们报告了一名4岁男童，患有巨大的升主动脉瘤和主动脉根部扩张并伴有严重主动脉瓣反流，具有I型Loeys-Dietz综合征的表型特征。他接受了Bentall手术，并进行了一项新的改良（冠状动脉再植入的内侧活板门技术）。该手术的短期结果令人鼓舞，在最后14个月的随访中他没有症状。
