Ad N, Vidne B A
Department of Cardiothoracic Surgery, Rabin Medical Center, Petach Tikva, Israel.
Ann Thorac Surg. 1999 Apr;67(4):1125-9. doi: 10.1016/s0003-4975(99)00047-8.
The combination of right aortic arch and coarctation of the aorta has seldom been reported. This rare abnormality occurs as an isolated lesion or in association with other congenital defects, such as mixed gonadal dysgenesis and Turner's syndrome.
The medical records of 2 patients who underwent operation for right aortic arch and aortic coarctation in our institution were reviewed together with case reports in the literature. Various surgical options have been reported: synthetic grafts, subclavian flap, and end-to-end anastomosis. End-to-end anastomosis is our method of choice for coarctation of the aorta and for right aortic arch with coarctation, even though an excessive amount of dissection is needed.
Both patients from our institution are doing well, with no sign of recoarctation in either patient. Our experience and recent advances in the understanding of the anatomy of this lesion led us to develop an alternative simplified classification for right aortic arch, which is presented here.
Right aortic arch and coarctation of the aorta is a rare morphologic combination. On the basis of our experience with repair of coarctation of the aorta and our review of the literature, we think that end-to-end anastomosis is the operative technique of choice with the best long-term results. Our simplified classification is easy to understand when dealing with right aortic arch.
右位主动脉弓合并主动脉缩窄的情况鲜有报道。这种罕见的异常可作为孤立病变出现，或与其他先天性缺陷相关，如混合性性腺发育不全和特纳综合征。
回顾了我院2例接受右位主动脉弓和主动脉缩窄手术患者的病历，并结合文献中的病例报告。已报道了多种手术方式：人工血管移植、锁骨下皮瓣和端端吻合术。尽管需要进行大量的解剖分离，但端端吻合术是我们治疗主动脉缩窄以及右位主动脉弓合并缩窄的首选方法。
我院的这2例患者情况良好，均未出现再缩窄迹象。我们的经验以及对该病变解剖结构认识的最新进展促使我们开发了一种针对右位主动脉弓的简化分类方法，现予以介绍。
右位主动脉弓合并主动脉缩窄是一种罕见的形态学组合。基于我们对主动脉缩窄修复的经验以及对文献的回顾，我们认为端端吻合术是首选的手术技术，长期效果最佳。我们的简化分类在处理右位主动脉弓时易于理解。
