Khoshhal Saad Q
Taibah University, Medical College - Paediatric Department, Almadinah Almunawwarah, KSA.
J Taibah Univ Med Sci. 2018 Dec 1;14(1):1-7. doi: 10.1016/j.jtumed.2018.10.006. eCollection 2019 Feb.
No standard protocol is available for the management of children with Down's syndrome (DS) and a functional single ventricle. This review attempts to determine the outcomes of the single ventricular surgical palliation pathway in high-risk children with DS.
Several databases were searched using the following MeSH terms: 'Congenital heart disease', 'Atrioventricular septal defect', 'Balanced AVSD', 'Unbalanced AVSD', 'Down's syndrome', 'Univentricular repair', 'bidirectional Glenn procedure', and 'Fontan procedure'. A structured algorithm was used for the selection of studies for an in-depth analysis.
There was no universal agreement on the best surgical approach for unbalanced atrioventricular septal defect in DS. The majority of paediatric cardiac surgeons did not recommend the complete Fontan procedure; conversely, the use of a Glenn shunt (superior cavopulmonary connection) was preferred.
Careful assessment of the suitability for Fontan surgery, including the absence of elevated pulmonary vascular resistance, pulmonary arterial anatomy, and function of the dominant ventricle, is mandatory. A staged surgical procedure ending with complete Fontan repair provides acceptable medium-term results.
目前尚无用于管理唐氏综合征（DS）合并功能性单心室患儿的标准方案。本综述旨在确定高危DS患儿单心室手术姑息治疗路径的结果。
使用以下医学主题词检索了多个数据库：“先天性心脏病”、“房室间隔缺损”、“平衡型房室间隔缺损”、“非平衡型房室间隔缺损”、“唐氏综合征”、“单心室修复”、“双向格林手术”和“Fontan手术”。采用结构化算法选择研究进行深入分析。
对于DS中非平衡型房室间隔缺损的最佳手术方法，尚无普遍共识。大多数小儿心脏外科医生不推荐完全Fontan手术；相反，更倾向于使用格林分流术（上腔静脉肺动脉连接）。
必须仔细评估Fontan手术的适用性，包括不存在肺血管阻力升高、肺动脉解剖结构以及优势心室的功能。以完全Fontan修复结束的分期手术可提供可接受的中期结果。
