Abstract：
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.
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.
摘要：
目的：关于13三体或18三体患儿先天性心脏缺陷的手术修复建议仍存在争议，受到偏见，并且基本上没有有限的经验数据支持。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欠佳。一个工作组，代表几个与照顾这些儿童有关的临床专业，提出了一些建议，以协助该人群的先天性心脏病治疗决策过程。这些建议的目标是为家庭及其医疗保健团队提供基于文献和专家意见的临床决策框架。
方法：该项目是在AATS先天性心脏手术循证医学工作组的主持下进行的。患者/人口，干预,比较/控制,结果过程用于生成初步陈述和建议，以解决与13三体或18三体患儿心脏手术有关的各个方面。然后迭代地使用Delphi方法，使用结构化的沟通过程在小组之间产生共识。
结果：从患者/人群的一组初始陈述中得出了九项建议，干预,比较/控制,在对500多篇文章进行分组审查之后，成果流程方法。这些建议由专家组以可重复的方式使用改良的Delphi过程进行裁决，并构成了当前的出版物。建议的类别（强度）通常是IIa类（中等收益），证据的总体水平（质量）为C级限制数据。
结论：这是一个多学科专家小组整理的第一套建议，旨在解决13三体或18三体先天性心脏病患儿手术干预适应症的具体问题。根据我们对最近数据的分析，我们建议决策不应该仅仅基于三体的存在，相反，应该根据具体情况而定，考虑到婴儿心脏病的严重程度以及其他异常的存在。这些建议提供了一个框架，可以帮助父母和临床医生为患有先天性心脏病的13三体或18三体的儿童进行手术决策。
方法：该项目是在AATS先天性心脏手术循证医学工作组的主持下进行的。患者/人口，干预,比较/控制,结果过程用于生成初步陈述和建议，以解决与13三体或18三体患儿心脏手术有关的各个方面。然后迭代地使用Delphi方法，使用结构化的沟通过程在小组之间产生共识。
结果：从患者/人群的一组初始陈述中得出了九项建议，干预,比较/控制,在对500多篇文章进行分组审查之后，成果流程方法。这些建议由专家组以可重复的方式使用改良的Delphi过程进行裁决，并构成了当前的出版物。建议的类别（强度）通常是IIa类（中等收益），证据的总体水平（质量）为C级限制数据。
结论：这是一个多学科专家小组整理的第一套建议，旨在解决13三体或18三体先天性心脏病患儿手术干预适应症的具体问题。根据我们对最近数据的分析，我们建议决策不应该仅仅基于三体的存在，相反，应该根据具体情况而定，考虑到婴儿心脏病的严重程度以及其他异常的存在。这些建议提供了一个框架，可以帮助父母和临床医生为患有先天性心脏病的13三体或18三体的儿童进行手术决策。
