Verdonschot Job A J, Paulussen Aimee D C, Lakdawala Neal K, de Die-Smulders Christine E M, Ware James S, Ingles Jodie
Department of Clinical Genetics, Maastricht University Medical Centre+, Maastricht, The Netherlands.
Department of Cardiology, Cardiovascular Research Institute Maastricht, Maastricht, The Netherlands.
Nat Rev Cardiol. 2025 Mar;22(3):199-211. doi: 10.1038/s41569-024-01073-3. Epub 2024 Sep 17.
In the past decade, genetic testing for cardiac disease has become part of routine clinical care. A genetic diagnosis provides the possibility to clarify risk for relatives. For family planning, a genetic diagnosis provides reproductive options, including prenatal diagnosis and preimplantation genetic testing, that can prevent an affected parent from having a child with the genetic predisposition. Owing to the complex genetic architecture of cardiac diseases, characterized by incomplete disease penetrance and the interplay between monogenic and polygenic variants, the risk reduction that can be achieved using reproductive genetic testing varies among individuals. Globally, disparities, including regulatory and financial barriers, in access to reproductive genetic tests exist. Although reproductive options are gaining a prominent position in the management of patients with inherited cardiac diseases, specific policies and guidance are lacking. Guidelines recommend that prenatal diagnosis and preimplantation genetic testing are options that should be discussed with families. Health-care professionals should, therefore, be aware of the possibilities and feel confident to discuss the benefits and challenges. In this Review, we provide an overview of the reproductive options in the context of inherited cardiac diseases, covering the genetic, technical, psychosocial and equity considerations, to prepare health-care professionals for discussions with their patients.
在过去十年中,心脏病的基因检测已成为常规临床护理的一部分。基因诊断为明确亲属的患病风险提供了可能。对于计划生育而言,基因诊断提供了生殖选择,包括产前诊断和植入前基因检测,这可以防止患病父母生育具有遗传易感性的孩子。由于心脏病复杂的遗传结构,其特征为疾病外显不全以及单基因和多基因变异之间的相互作用,利用生殖基因检测所能实现的风险降低程度因人而异。在全球范围内,获取生殖基因检测存在差异,包括监管和经济障碍。尽管生殖选择在遗传性心脏病患者的管理中占据突出地位,但缺乏具体的政策和指导。指南建议,产前诊断和植入前基因检测是应与家庭讨论的选择。因此,医疗保健专业人员应了解这些可能性,并自信地讨论其益处和挑战。在本综述中,我们概述了遗传性心脏病背景下的生殖选择,涵盖遗传、技术、心理社会和公平性等方面的考虑因素,以便让医疗保健专业人员为与患者的讨论做好准备。