Sheppard Mary N, Westaby Joseph, Zullo Emelia, Fernandez Belmira V E, Cox Steve, Cox Alison
Cardiac Risk in the Young (CRY, UK) Cardiovascular Pathology Unit, St George's University of London, London, UK.
Histopathology. 2023 Jun;82(7):1056-1066. doi: 10.1111/his.14889. Epub 2023 Mar 20.
Sudden cardiac death (SCD) is defined as natural unexpected death in witnessed cases occurring < 1 h and in unwitnessed cases as last seen alive < 24 h. SCD due to ischaemic heart disease (IHD) is frequent in older age groups; in younger people genetic cardiac causes, including channelopathies and cardiomyopathies, are more frequent. This study aimed to present the causes of SCD from a large specialist pathology registry.
Cases were examined macroscopically and microscopically by two expert cardiac pathologists. The hearts from 7214 SCD cases were examined between 1994 and 2021. Sudden arrhythmic death syndrome (SADS), a morphologically normal heart, which can be underlaid by cardiac channelopathies, is most common (3821, 53%) followed by the cardiomyopathies (1558, 22%), then IHD (670, 9%), valve disease (225, 3%), congenital heart disease (213, 3%) and myocarditis/sarcoidosis (206, 3%). Hypertensive heart disease (185, 3%), aortic disease (129, 2%), vascular disease (97, 1%) and conduction disease (40, 1%) occur in smaller proportions.
To our knowledge, this is the largest SCD cohort with autopsy findings ever reported from one country. SADS and cardiomyopathies predominate. This study highlights the importance of the autopsy in SCD, which is a significant public health concern in all age groups. Knowing the true incidence in our population will improve risk stratification and develop preventative strategies for family members. There is now a national pilot study integrating molecular autopsy and family screening into the assessment of SCD victims.
心源性猝死（SCD）定义为在有目击者的情况下发生在＜1小时内的自然意外死亡，在无目击者的情况下为最后一次被看到存活＜24小时。缺血性心脏病（IHD）导致的SCD在老年人群中很常见；在年轻人中，遗传性心脏病因，包括离子通道病和心肌病，更为常见。本研究旨在从一个大型专业病理登记处呈现SCD的病因。
由两位心脏病理学专家对病例进行大体和显微镜检查。在1994年至2021年期间检查了7214例SCD病例的心脏。突然心律失常死亡综合征（SADS），一种形态学上正常的心脏，可能由心脏离子通道病引起，最为常见（3821例，53%），其次是心肌病（1558例，22%），然后是IHD（670例，9%）、瓣膜病（225例，3%）、先天性心脏病（213例，3%）和心肌炎/结节病（206例，3%）。高血压性心脏病（185例，3%）、主动脉疾病（129例，2%）、血管疾病（97例，1%）和传导疾病（40例，1%）的比例较小。
据我们所知，这是一个国家报告的有尸检结果的最大SCD队列。SADS和心肌病占主导地位。本研究强调了尸检在SCD中的重要性，SCD是所有年龄组中一个重要的公共卫生问题。了解我们人群中的真实发病率将改善风险分层并为家庭成员制定预防策略。现在有一项全国性试点研究将分子尸检和家族筛查纳入SCD受害者的评估中。
