Department of Pediatric Cardiology, Cleveland Clinic Children's and The Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
Heart Institute, Johns Hopkins All Children's Hospital, St Petersburg, Florida; Congenital Heart Center, UF Health Shands Hospital, Division of Cardiovascular Surgery, Departments of Surgery and Pediatrics, University of Florida, Gainesville, Florida.
Ann Thorac Surg. 2023 Jul;116(1):6-16. doi: 10.1016/j.athoracsur.2023.03.023. Epub 2023 Jun 8.
Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization.
在过去的 20 年中，已经提出了几种主动脉根部异常的分类方法。这些方案大多没有先天性心脏病专家的参与。本综述的目的是从这些专家的角度提供一种分类方法，基于对正常和异常形态发生和解剖结构的理解，重点介绍具有临床和手术相关性的特征。我们认为，当以认识到正常根部由 3 个瓣叶组成的方式来描述先天性畸形的主动脉根部时，描述变得更加简单，瓣叶由各自的窦支持，窦之间由瓣间三角分隔。畸形的根部通常在 3 个窦的情况下发现，也可以在 2 个窦的情况下发现，很少在 4 个窦的情况下发现。这允许分别描述三叶、双叶和四叶变体。这一特征为目前存在的瓣叶解剖和功能数量的分类提供了基础。通过提供标准化的术语和定义，我们认为我们的分类适用于所有心脏专业的人员，无论他们是儿科还是成人。在获得性或先天性心脏病的情况下同样具有价值。我们的建议将有助于修订和/或补充现有的国际儿科和先天性心脏病代码，以及世界卫生组织提供的第十一次国际疾病分类迭代。
