Recommendations for surgical repair of a congenital heart defect in children with trisomy 13 or trisomy 18 remain controversial, are subject to biases, and are largely unsupported with limited empirical data. This has created significant distrust and uncertainty among parents and could potentially lead to suboptimal care for patients. A working group, representing several clinical specialties involved with the care of these children, developed recommendations to assist in the decision-making process for congenital heart defect care in this population. The goal of these recommendations is to provide families and their health care teams with a framework for clinical decision making based on the literature and expert opinions. This project was performed under the auspices of the AATS Congenital Heart Surgery Evidence-Based Medicine Taskforce. A Patient/Population, Intervention, Comparison/Control, Outcome process was used to generate preliminary statements and recommendations to address various aspects related to cardiac surgery in children with trisomy 13 or trisomy 18. Delphi methodology was then used iteratively to generate consensus among the group using a structured communication process. Nine recommendations were developed from a set of initial statements that arose from the Patient/Population, Intervention, Comparison/Control, Outcome process methodology following the groups' review of more than 500 articles. These recommendations were adjudicated by this group of experts using a modified Delphi process in a reproducible fashion and make up the current publication. The Class (strength) of recommendations was usually Class IIa (moderate benefit), and the overall level (quality) of evidence was level C-limited data. This is the first set of recommendations collated by an expert multidisciplinary group to address specific issues around indications for surgical intervention in children with trisomy 13 or trisomy 18 with congenital heart defect. Based on our analysis of recent data, we recommend that decisions should not be based solely on the presence of trisomy but, instead, should be made on a case-by-case basis, considering both the severity of the baby's heart disease as well as the presence of other anomalies. These recommendations offer a framework to assist parents and clinicians in surgical decision making for children who have trisomy 13 or trisomy 18 with congenital heart defect.

中文翻译：

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美国胸外科协会 (AATS) 2023 年专家共识文件：13 三体或 18 三体和先天性心脏缺陷儿童的护理建议

对 13 三体或 18 三体儿童先天性心脏缺陷进行手术修复的建议仍然存在争议，存在偏见，并且在很大程度上得不到有限的经验数据的支持。这在父母之间造成了极大的不信任和不确定性，并可能导致对患者的护理效果不佳。一个代表涉及这些儿童护理的多个临床专业的工作组制定了建议，以协助该人群先天性心脏病护理的决策过程。这些建议的目标是为家庭及其医疗团队提供基于文献和专家意见的临床决策框架。该项目是在 AATS 先天性心脏手术循证医学工作组的赞助下进行的。使用患者/人群、干预、比较/对照、结果流程生成初步声明和建议，以解决与 13 三体或 18 三体儿童心脏手术相关的各个方面。然后迭代使用德尔菲法在小组中达成共识使用结构化的沟通流程。小组审查了 500 多篇文章后，根据患者/人群、干预、比较/控制、结果过程方法论得出的一组初步陈述制定了九项建议。这些建议由该专家组使用修改后的德尔菲流程以可重复的方式进行裁决，并构成了当前的出版物。建议的类别（强度）通常为 IIa 类（中等益处），证据的总体水平（质量）为 C 级限制数据。这是多学科专家组整理的第一套建议，旨在解决患有先天性心脏缺陷的 13 三体或 18 三体儿童的手术干预指征的具体问题。根据我们对最近数据的分析，我们建议不应仅根据是否存在三体性做出决定，而应根据具体情况做出决定，同时考虑婴儿心脏病的严重程度以及是否存在其他异常情况。这些建议提供了一个框架，帮助父母和临床医生为患有先天性心脏病的 13 三体或 18 三体儿童做出手术决策。