Department of Clinical Genetics, University Medical Center Groningen / University of Groningen, Groningen, the Netherlands.
Department of Clinical Genetics, Academic Medical Center / University of Amsterdam, Amsterdam, the Netherlands.
Eur J Hum Genet. 2023 Dec;31(12):1381-1386. doi: 10.1038/s41431-023-01334-8. Epub 2023 Mar 27.
In inherited and familial cardiovascular diseases (CVDs), relatives without current symptoms can still be at risk for early and preventable cardiovascular events. One way to help people evaluate their potential risk of CVD is through a risk-assessment tool based on family health history. However, family criteria including inherited CVD risk to be used by laypersons are non-existent. In this project, we employed a qualitative study design to develop expert-based family criteria for use in individual risk assessment. In the first phase of the project, we identified potential family criteria through an online focus group with physicians with expertise in monogenic and/or multifactorial CVDs. The family criteria from phase one were then used as input for a three-round Delphi procedure carried out in a larger group of expert physicians to reach consensus on appropriate criteria. This led to consensus on five family criteria that focus on cardiovascular events at young age (i.e., sudden death, any CVD, implantable cardioverter-defibrillator, aortic aneurysm) and/or an inherited CVD in one or more close relatives. We then applied these family criteria to a high-risk cohort from a clinical genetics department and demonstrated that they have substantial diagnostic accuracy. After further evaluation in a general population cohort, we decided to only use the family criteria for first-degree relatives. We plan to incorporate these family criteria into a digital tool for easy risk assessment by the public and, based on expert advice, will develop supporting information for general practitioners to act upon potential risks identified by the tool. Results from an expert focus group, a Delphi method in a larger group of experts, and evaluation in two cohorts were used to develop family criteria for assessing cardiovascular disease risk based on family health history for a digital risk-prediction tool for use by the general population. CVD Cardiovascular disease, ICD Implantable cardioverter defibrillator, TAA Thoracic aortic aneurysm, AAA Abdominal aortic aneurysm.
在遗传性和家族性心血管疾病 (CVD) 中,没有当前症状的亲属仍然存在发生早期和可预防心血管事件的风险。帮助人们评估其 CVD 潜在风险的一种方法是使用基于家族健康史的风险评估工具。然而,用于评估个体风险的基于家族的标准(包括遗传性 CVD 风险)在非专业人士中并不存在。在这个项目中,我们采用了定性研究设计,为个体风险评估制定了基于专家的家族标准。在项目的第一阶段,我们通过一个在线焦点小组,与具有单基因和/或多因素 CVD 专业知识的医生一起确定了潜在的家族标准。然后,使用第一阶段的家族标准作为输入,进行了三回合德尔菲程序,让更多的专家医生参与,以就适当的标准达成共识。这就达成了共识,确定了五个家族标准,这些标准侧重于年轻时期的心血管事件(即猝死、任何 CVD、植入式心脏复律除颤器、主动脉瘤)和/或一个或多个近亲中遗传性 CVD。然后,我们将这些家族标准应用于一个临床遗传学部门的高危队列,并证明它们具有很高的诊断准确性。在一般人群队列中进一步评估后,我们决定仅使用一级亲属的家族标准。我们计划将这些家族标准纳入一个易于公众使用的数字工具,以便进行风险评估,并根据专家建议,为普通科医生制定相关支持信息,以便根据工具识别的潜在风险采取行动。使用来自专家焦点小组、更大专家群体中的德尔菲方法以及两个队列的评估结果,制定了基于家族健康史的 CVD 风险评估家族标准,用于为一般人群开发数字风险预测工具。CVD 心血管疾病,ICD 植入式心脏复律除颤器,TAA 胸主动脉瘤,AAA 腹主动脉瘤。