Sudden Death

猝死
  • 文章类型: Journal Article
    目的:探讨急诊护士如何照顾死者及其亲属,以及在紧急情况下阻碍或促进这种护理的因素。
    方法:使用解释性描述的定性研究。
    方法:从2023年2月至6月收集数据,对丹麦7个急诊科的13名护士进行个别访谈。
    结果:我们的分析揭示了总体主题“在有限的环境中导航提供整体护理的复杂性”,涵盖五个子主题:(1)一项重要但尚未得到认可的护理任务;(2)生者和死者的待定护理需求;(3)死者没有身心空间;(4)在没有正规教育和培训的情况下利用个人经历;(5)导航专业精神和同理心。
    结论:急诊科在为死者及其亲属提供护理方面提出了独特的挑战。
    照顾死者及其亲属的性质未被认可,这表明急诊科普遍低估了这种照顾。
    结论:对死者及其亲属的护理既不被认可,也不以证据为基础。这项研究引发了对为死者提供护理的情况的讨论,并对急诊科雇用的护士和护理负责人产生了影响。
    报告定性研究的综合标准(COREQ)。
    无。
    OBJECTIVE: To explore how emergency nurses experienced caring for brought-in-dead persons and their relatives, and what hindered or facilitated this care in an emergency setting.
    METHODS: A qualitative study using Interpretive Description.
    METHODS: Data were collected as individual interviews with 13 nurses at seven Danish emergency departments from February to June 2023.
    RESULTS: Our analysis revealed the overarching theme \'Navigating the complexities of providing holistic care in a constrained environment\', covering five sub-themes: (1) An important yet not recognized nursing task; (2) Pending care needs of the living and the dead; (3) No physical or mental room for the brought-in-dead persons; (4) Utilizing personal experiences in the absence of formal education and training and (5) Navigating professionalism and empathy.
    CONCLUSIONS: Emergency departments posed unique challenges in providing care to brought-in-dead persons and their relatives.
    UNASSIGNED: The unrecognized nature of caring for brought-in-dead persons and their relatives suggests a universal undervaluation of this care in emergency departments.
    CONCLUSIONS: Care for brought-in-dead persons and their relatives is neither recognized nor evidence-based. This study initiates a discussion of the circumstances for delivering care for persons brought-in-dead and has an impact on nurses and nursing leaders employed in emergency departments.
    UNASSIGNED: The Consolidated Criteria for Reporting Qualitative Research (COREQ).
    UNASSIGNED: None.
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  • 文章类型: Journal Article
    猝死约占工作年龄成年人死亡人数的10%,并且与空气质量差有关。目标:确定高风险人群和潜在的风险调节剂和介体,我们探讨了先前建立的细颗粒物(PM2.5)与按潜在危险因素分层的猝死之间的关联.
    韦克县的猝死受害者,NC,在2013年3月1日至2015年2月28日期间,通过急诊医疗系统筛查报告进行鉴定并作出裁决(n=399).空气质量数据集市上威克县的每日PM2.5浓度与事件和控制期有关。潜在的修饰符包括绿色空间指标,临床状况,左心室肥厚(LVH),和中性粒细胞与淋巴细胞比率(NLR)。使用案例交叉设计,条件逻辑回归估计猝死的OR(95CI)为PM2.5增加5μg/m3,滞后1天,根据温度和湿度进行调整,跨风险因素阶层。
    LVH或NLR高于2.5的个体的PM2.5相关性比没有[LVHOR:1.90(1.04,3.50);NLR>2.5:1.25(0.89,1.76)]的个体。PM2.5对居住在绿色空间较高地区的个人的影响通常较小。
    LVH和炎症可能是不良空气质量和传统危险因素引发心律失常或心肌缺血和猝死的因果途径的最后一步。统计证据与临床知识的结合可以告知医疗提供者其患者的潜在风险。虽然我们的发现可能有助于指导干预措施以减轻猝死的发生率。
    UNASSIGNED: Sudden death accounts for approximately 10% of deaths among working-age adults and is associated with poor air quality. Objectives: To identify high-risk groups and potential modifiers and mediators of risk, we explored previously established associations between fine particulate matter (PM2.5) and sudden death stratified by potential risk factors.
    UNASSIGNED: Sudden death victims in Wake County, NC, from 1 March 2013 to 28 February 2015 were identified by screening Emergency Medical Systems reports and adjudicated (n = 399). Daily PM2.5 concentrations for Wake County from the Air Quality Data Mart were linked to event and control periods. Potential modifiers included greenspace metrics, clinical conditions, left ventricular hypertrophy (LVH), and neutrophil-to-lymphocyte ratio (NLR). Using a case-crossover design, conditional logistic regression estimated the OR (95%CI) for sudden death for a 5 μg/m3 increase in PM2.5 with a 1-day lag, adjusted for temperature and humidity, across risk factor strata.
    UNASSIGNED: Individuals having LVH or an NLR above 2.5 had PM2.5 associations of greater magnitude than those without [with LVH OR: 1.90 (1.04, 3.50); NLR > 2.5: 1.25 (0.89, 1.76)]. PM2.5 was generally less impactful for individuals living in areas with higher levels of greenspace.
    UNASSIGNED: LVH and inflammation may be the final step in the causal pathway whereby poor air quality and traditional risk factors trigger arrhythmia or myocardial ischemia and sudden death. The combination of statistical evidence with clinical knowledge can inform medical providers of underlying risks for their patients generally, while our findings here may help guide interventions to mitigate the incidence of sudden death.
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  • 文章类型: Multicenter Study
    镰状细胞病(SCD)是全球范围内的主要公共卫生负担,发病率和死亡率不断增加。该研究评估了与SCD患者死亡率相关的危险因素,2006年至2020年在阿曼的三家医院。分析包括临床表现,血液学,生物化学,和放射学参数,使用抗生素,血液和交换输血。我们的队列包括123例患者(82例男性,41名女性),年龄中位数为27岁(四分位距21-35岁)。SCD相关并发症包括急性胸部综合征(ACS)占52.8%,脾隔离率为21.1%,右上腹综合征占19.5%,17.9%超过>6VOC/年,中风占13.8%。在终点站入场时,病人咳嗽,降低O2饱和度,起病和发烧占24.4%,49.6%,分别为53.6%和68.3%。胸部X线和胸部CT扫描异常占57.7%,和76.4%。实验室参数显示血红蛋白(Hb)和血小板计数从基线显著下降,随着白细胞的大幅上升,来自基线的LDH和CRP(p<0.05,Wilcoxon签名等级检验)。所有患者都接受了抗生素治疗,然而,95.9%和93.5%接受简单输血,和交换输血,66.6%需要无创通气。在死亡原因中,ACS见于32例(26%),脓毒症49例(40%),和杂项42(34%)。32例(26%)患者出现猝死。男性,HbF低,血红蛋白和血小板快速下降,白细胞增加,LDH,铁蛋白,CRP,该队列中与死亡率显著相关.
    Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. The study evaluates the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. The analysis includes clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (Interquartile Range 21-35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC/year in 17.9%, and stroke in 13.8%. At the terminal admission, patients had cough, reduced O2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest X-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline (p < 0.05, Wilcoxon Signed Ranks test). All patients received antibiotics, whereas, 95.9% and 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.
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  • 文章类型: Journal Article
    目的:Brugada综合征(BrS)的诊断和危险分层依赖于自发性1型(spT1)心电图(ECG)模式的存在;然而,其自发波动可能导致误诊和风险低估。这项研究旨在评估重复高心前导联(HPL)静息和动态心电图监测在识别spT1中的作用,并评估其预后作用。
    结果:对BrS受试者的HPL静息和动态监测心电图进行回顾性回顾,以及与室性心律失常和心源性猝死(SCD)相关的spT1的存在。三百五十八名受试者(77名在演示时具有spT1模式,第一组和281,没有,包括第2组)。总的来说,1651个静息HPL静息和621个动态监测心电图可供审查,或充分描述。在72个月的中位随访时间(四分位间距-IQR-75),第1组中的42/77(55%)受试者在至少一个ECG中显示spT1。在第2组中,有36/281名受试者(13%)新检测到spT1(每100人年1.9),在HPL门诊记录中有23名(8%)。七个以前无症状的受试者,其中5人患有spT1(4人在演示中,1人在随访中),经历过的心律失常事件;生存分析表明spT1,无论是在出现时还是在一生中,与事件有关。单变量模型显示,spT1与风险增加一致相关[spT1出现时:风险比(HR)6.3,95%置信区间(CI)1.4-28,P=0.016;spT1随访时:HR3.1,95%CI1.3-7.2,P=0.008]。
    结论:重复的ECG评估和HPL动态监测对于识别短暂性spT1Brugada模式及其相关风险至关重要。
    OBJECTIVE: Brugada syndrome (BrS) diagnosis and risk stratification rely on the presence of a spontaneous type 1 (spT1) electrocardiogram (ECG) pattern; however, its spontaneous fluctuations may lead to misdiagnosis and risk underestimation. This study aims to assess the role for repeat high precordial lead (HPL) resting and ambulatory ECG monitoring in identifying a spT1, and evaluate its prognostic role.
    RESULTS: HPL resting and ambulatory monitoring ECGs of BrS subjects were reviewed retrospectively, and the presence of a spT1 associated with ventricular dysrhythmias and sudden cardiac death (SCD). Three-hundred and fifty-eight subjects (77 with spT1 pattern at presentation, Group 1, and 281 without, Group 2) were included. In total, 1651 resting HPL resting and 621 ambulatory monitoring ECGs were available for review, or adequately described. Over a median follow-up of 72 months (interquartile range - IQR - 75), 42/77 (55%) subjects in Group 1 showed a spT1 in at least one ECG. In Group 2, 36/281 subjects (13%) had a newly detected spT1 (1.9 per 100 person-year) and 23 on an HPL ambulatory recording (8%). Seven previously asymptomatic subjects, five of whom had a spT1 (four at presentation and one at follow-up), experienced arrhythmic events; survival analysis indicated that a spT1, either at presentation or during lifetime, was associated with events. Univariate models showed that a spT1 was consistently associated with increased risk [spT1 at presentation: hazard ratio (HR) 6.3, 95% confidence interval (CI) 1.4-28, P = 0.016; spT1 at follow-up: HR 3.1, 95% CI 1.3-7.2, P = 0.008].
    CONCLUSIONS: Repeated ECG evaluation and HPL ambulatory monitoring are vital in identifying transient spT1 Brugada pattern and its associated risk.
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  • 文章类型: Observational Study
    儿童心脏猝死是一种罕见的事件,但具有重大的社会意义。一般来说,它与心脏病有关,有心脏猝死(SCD)的风险,可能伴随心血管症状和/或心电图标志物而发生;因此,初级保健儿科医生(PCP)可以发现它们.因此,我们提出了一项研究,评估如何在六岁和十二岁的常规健康儿童检查中付诸实践并进行心血管评估;反映心血管体征和症状,以及选定人群中存在SCD风险的儿童的心电图改变;并评估PCP的心电图(ECG)解释能力。与PCP合作,初级护理护士,和儿科心脏病专家,一个观察,描述性,描述性多中心,横断面研究在巴利阿里群岛(西班牙)进行,从2021年4月到2022年1月,包括在内。PCP通过表格收集患者数据(病历,心电图,和体检),并将其发送给调查员,连同知情同意书和心电图。调查人员将心电图交给儿科心脏病专家阅读,与儿科医生填写的表格相同。收集了变量,并进行了描述性分析。三位儿科心脏病专家,十二个PCP,来自七个公共卫生中心的九名护士参加了会议。他们收集了641名患者的数据,但233例患者未参与(81.11%因PCP的工作量).因此,研究覆盖率约为64%,代表参与的患者总数的商,除以符合研究条件的患者总数.我们检测到30例心电图改变与SCD风险相符的患者。其中9个曾由儿科心脏病专家检查过(8/9功能性杂音),五个人报告说运动时呼吸急促,四个人报告有猝死的家族史。所有心电图与SCD风险相符的患者的体格检查均正常。在分析PCP和儿科心脏病专家的心电图结果在多大程度上一致后,最终解释(正常/改变)的一致性百分比为91.9%,科恩的卡帕系数为31.2%(CI95%:13.8-48.6%)。PCP进行ECG解释以检测与SCD风险相容的ECG的敏感性为29%,阳性预测值为45%。结论:本研究为未来儿童SCD风险筛查奠定了基础。由PCP执行。然而,以前,重要的是优化他们在阅读和解释儿科心电图方面的培训。什么是已知的:•目前在西班牙,有一个项目可以检测有猝死风险的心脏病[1],但它只针对作为一项联邦运动开始或正在进行体育活动的儿童。在几个国家[2],实施此类筛查计划已被证明是有效的。然而,一些研究表明,参加体育活动的儿童心源性猝死的发生率并不比不参加任何运动的儿童高[3]。这构成了道德冲突,因为目前,不参加任何联合会运动的儿童将被排除在筛查之外。根据修订后的文献,到目前为止,只有在两项研究中,他们对学校的儿童群体进行了筛查,在两者中,他们成功地检测到心脏病患者与猝死风险相关[4,5]。我们没有发现任何研究将这些特征的筛查纳入初级保健儿科医生的常规健康儿童检查中。新增内容:•我们不知道是否-在我们的设置中,目前,初级保健儿科医生可以在常规的健康儿童检查中进行筛查方法,为了检测可能有心脏猝死风险的健康儿童,因为它们存在SCD风险之一。在这方面,我们提出了我们的项目:评估初级保健儿科医生如何在健康儿童人群中实施和通过SCD风险筛查进行心血管评估,并评估初级保健儿科医生识别与SCD风险相关的心电图改变的技能.这项试点研究的最终目的是使之成为可能,在未来,设计和证明一项旨在普及心血管筛查和实现儿童心源性猝死事件长期减少的研究。
    Sudden cardiac death in children is a rare event, but of great social significance. Generally, it is related to heart disease with a risk of sudden cardiac death (SCD), which may occur with cardiovascular symptoms and/or electrocardiographic markers; thus, a primary care paediatrician (PCP) could detect them. Therefore, we proposed a study that assesses how to put into practice and conduct a cardiovascular assessment within the routine healthy-child check-ups at six and twelve years of age; that reflects cardiovascular signs and symptoms, as well as the electrocardiographic alterations that children with a risk of SCD in the selected population present; and that assesses the PCP\'s skill at electrocardiogram (ECG) interpretation. In collaboration with PCPs, primary care nurses, and paediatric cardiologists, an observational, descriptive, multicentre, cross-sectional study was carried out in the Balearic Islands (Spain), from April 2021 to January 2022, inclusive. The PCPs gathered patient data through forms (medical record, electrocardiogram, and physical examination) and sent them to the investigator, together with the informed consent document and electrocardiogram. The investigator passed the electrocardiogram on to the paediatric cardiologists for reading, in an identical form to those the paediatricians had filled in. The variables were collected, and a descriptive analysis performed. Three paediatric cardiologists, twelve PCPs, and nine nurses from seven public health centres took part. They collected the data from 641 patients, but 233 patients did not participate (in 81.11% due to the PCP\'s workload). Therefore, the study coverage was around 64%, representing the quotient of the total number of patients who participated, divided by the total number of patients who were eligible for the study. We detected 30 patients with electrocardiographic alterations compatible with SCD risk. Nine of these had been examined by a paediatric cardiologist at some time (functional murmur in 8/9), five had reported shortness of breath with exercise, and four had reported a family history of sudden death. The physical examination of all the patients whose ECG was compatible with a risk of SCD was normal. Upon analysing to what extent the ECG results of the PCP and the paediatric cardiologist agreed, the percentage of agreement in the final interpretation (normal/altered) was 91.9%, while Cohen\'s kappa coefficient was 31.2% (CI 95%: 13.8-48.6%). The sensitivity of the ECG interpretation by the PCP to detect an ECG compatible with a risk of SCD was 29% and the positive predictive value 45%.     Conclusions: This study lays the foundations for future SCD risk screening in children, performed by PCPs. However, previously, it would be important to optimise their training in reading and interpreting paediatric ECGs. What is Known: • In Spain at present, there is a programme in place to detect heart disease with a risk of sudden death [1], but it targets only children who are starting on or are doing a physical activity as a federated sport. Implementing such screening programmes has proven effective in several countries [2]. However, several studies showed that the incidence of sudden cardiac death is no higher in children competing in sport activities than in those who do not do any sport [3]. This poses an ethical conflict, because at present, children who do not do any federated sport are excluded from screening. According to the revised literature, so far, only in two studies did they screen the child population at schools, and in both, they successfully detected patients with heart disease associated to the risk of sudden death [4, 5]. We have found no studies where the screening of these features was included within the routine healthy-child check-ups by primary care paediatricians. What is New: • We did not know whether-in our setting, at present-the primary care paediatrician could perform a screening method within the routine healthy-child check-ups, in order to detect presumably healthy children at risk of sudden cardiac death, as they present one of the SCD risks. In this regard, we proposed our project: to assess how to put into practice and conduct a cardiovascular assessment via SCD risk screening in the healthy child population by primary care paediatricians and appraise primary care paediatricians\' skills in identifying the electrocardiographic alterations associated with SCD risk. The ultimate intention of this pilot study was to make it possible, in the future, to design and justify a study aimed at universalising cardiovascular screening and achieving a long-term decrease in sudden cardiac death events in children.
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  • 文章类型: Journal Article
    背景:Brugada综合征(BrS)的风险分层仍存在争议。在这方面,电生理学研究(EPS)的作用一直是争论的主题。在一些中心,通常的做法是在EPS阴性后使用植入式环路记录仪(ILR)来帮助进行风险分层.然而,这种方法的诊断价值从未得到具体解决.
    目的:描述在BrS中EPS阴性后使用ILR进行诊断整理策略的基线特征和主要发现。
    方法:我们进行了一项回顾性国际注册,其中包括BrS和EPS阴性的患者(即,非诱导型VT/VF)在ILR监测之前。
    结果:来自荷兰8家转诊医院的65名患者,包括西班牙和英国(平均年龄39±16岁,72%男性)。ILR监测的主要指征是原因不明的晕厥/晕厥前(66.1%)。在39.0个月的中位随访期间(Q125.0-Q347.6),18例患者(27.7%)经历了21次心律失常事件(AE)。随访期间无一例患者死亡。缓慢性心律失常是最常见的发现(47.6%),其次是房性快速性心律失常(38.1%)。只有3例患者出现室性心律失常。12例患者(66.7%)被认为是偶然的不良事件。在11名患者(61.1%)中,AE导致治疗的特定变化。
    结论:在BrS中EPS阴性后使用ILR是一种安全的策略,反映了EPS对该综合征室性心律失常的高阴性预测值。此外,它允许在相当比例的患者中检测到AE,其中大多数都有治疗意义。
    BACKGROUND: Risk stratification in Brugada syndrome (BrS) remains controversial. In this respect, the role of the electrophysiology study (EPS) has been a subject of debate. In some centers, it is common practice to use an implantable loop recorder (ILR) after a negative EPS to help in risk stratification. However, the diagnostic value of this approach has never been specifically addressed.
    OBJECTIVE: The aim of this study was to describe the baseline characteristics and the main findings of a diagnostic workup strategy with an ILR after a negative EPS in BrS.
    METHODS: We conducted a retrospective international registry including patients with BrS and negative EPS (ie, noninducible ventricular tachycardia or ventricular fibrillation) before ILR monitoring.
    RESULTS: The study included 65 patients from 8 referral hospitals in The Netherlands, Spain, and the United Kingdom (mean age, 39 ± 16 years; 72% male). The main indication for ILR monitoring was unexplained syncope/presyncope (66.2%). During a median follow-up of 39.0 months (Q1 25.0-Q3 47.6 months), 18 patients (27.7%) experienced 21 arrhythmic events (AEs). None of the patients died during follow-up. Bradyarrhythmias were the most common finding (47.6%), followed by atrial tachyarrhythmias (38.1%). Only 3 patients presented with ventricular arrhythmias. AEs were considered incidental in 12 patients (66.7%). In 11 patients (61.1%), AEs led to specific changes in treatment.
    CONCLUSIONS: The use of ILR after a negative EPS in BrS is a safe strategy that reflected the high negative predictive value of EPS for ventricular arrhythmia in this syndrome. In addition, it allowed the detection of AEs in a significant proportion of patients, with therapeutic implications in most of them.
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  • 文章类型: Case Reports
    胶体囊肿是由上皮组织膜覆盖的凝胶状材料制成的非恶性肿瘤生长。它通常位于门罗孔的后面,在大脑第三脑室的前部。由于它的位置,会导致梗阻性脑积水,颅内压升高,和心源性猝死,儿茶酚胺介导的,通过下丘脑压迫.所有的机制仍然存在争议,但是儿茶酚胺的作用已被强调心肌损伤的组织学发现证实(凝血性肌细胞溶解和收缩带坏死,CBN)。这项研究介绍了一名先前健康的22岁男性因第三脑室的胶体囊肿而突然死亡的情况。进行了完整的尸检,在大脑中突出显示大量的脑脊液(CSF)和2厘米的浅灰绿色圆形囊肿形成,部分填充并扩张了第三脑室。通过免疫组织化学研究证实了诊断:高碘酸希夫(PAS)染色阳性和CK7表达。在这里报道的这种情况下,联合的尸检方法,组织学,和免疫组织化学是强制性的,以确定新形成的位置和形态结构特征,以进行正确的鉴别诊断,以及确定死因.
    The colloid cyst is a non-malignant tumor growth made of a gelatinous material covered by a membrane of epithelial tissue. It is usually located posterior to the foramen of Monro, in the anterior aspect of the third ventricle of the brain. Due to its location, it can cause obstructive hydrocephalus, increased intracranial pressure, and sudden cardiac death, catecholamine-mediated, through hypothalamus compression. All the mechanisms are still controversial, but the role of catecholamine has been confirmed with histological findings that highlighted myocardial injury (coagulative myocytolysis and contraction band necrosis, CBN). This study presents a case of sudden death in a previously healthy 22-year-old male due to a colloid cyst of the third ventricle. A complete autopsy was performed, highlighting in the brain an abundant quantity of cerebrospinal fluid (CSF) and a 2 cm pale grayish-green rounded cyst formation partially filling and distending the third ventricle. The diagnosis was confirmed through immunohistochemical investigation: positivity for Periodic acid-Schiff (PAS) staining and CK7 expression. In cases such as the one reported here, a combined approach of autopsy, histology, and immunohistochemistry is mandatory in order to identify the neoformation\'s location and morpho-structural characteristics for a correct differential diagnosis, as well as to identify the cause of death.
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  • 文章类型: Journal Article
    法布里病(FD)是α-半乳糖苷酶-A的X连锁缺乏症,导致鞘脂在多个器官中的溶酶体储存。心肌积聚会导致心律失常和猝死,FD死亡的最常见原因。因此,有必要对目标设备治疗进行风险分层和预测。植入式环路记录器(ILR)允许连续节律监测长达3年。这里,我们进行了一项回顾性研究,以评估FD中ILR的当前使用情况,并量化检测到的心律失常的负担,这导致了治疗的改变。这是2000年1月1日至2022年9月1日期间英格兰三个专科中心915例FD患者的快照评估。总的来说,22例(2.4%)患者接受了临床指示的ILR植入。平均植入年龄为50岁,13例(59%)患者为女性。植入后,9名(41%)患者接受了心律失常检测,需要干预(六个ILR和三个ILR后电池耗尽)。三名患者经历了持续的心房高速率发作,并开始接受抗凝治疗。三人患有非持续性快速性心律失常,并开始使用β受体阻滞剂。ILR电池耗尽后,一个患有完全的心脏传导阻滞,两个患有持续性室性心动过速,都需要设备治疗。心律失常患者的心电图PR间期较短。这项研究表明,在FD中植入ILR可发现心律失常的高负担。ILR可能在这个致心律失常队列中没有得到充分利用,可能仅限于患有晚期FD心肌病的患者。在上述三名患者的电池耗尽后,对于ILR监测后出现主要心律失常事件的患者,建议提高警惕性和心律失常监测.需要进一步的工作来确定谁将从植入中受益最大。
    Fabry disease (FD) is an X-linked deficiency of alpha-galactosidase-A, leading to lysosomal storage of sphingolipids in multiple organs. Myocardial accumulation contributes to arrhythmia and sudden death, the most common cause of FD mortality. Therefore, there is a need for risk stratification and prediction to target device therapy. Implantable loop recorders (ILRs) allow for continual rhythm monitoring for up to 3 years. Here, we performed a retrospective study to evaluate current ILR utilisation in FD and quantify the burden of arrhythmia that was detected, which resulted in a modification of therapy. This was a snapshot assessment of 915 patients with FD across three specialist centres in England during the period between 1 January 2000 and 1 September 2022. In total, 22 (2.4%) patients underwent clinically indicated ILR implantation. The mean implantation age was 50 years and 13 (59%) patients were female. Following implantation, nine (41%) patients underwent arrhythmia detection, requiring intervention (six on ILR and three post-ILR battery depletion). Three patients experienced sustained atrial high-rate episodes and were started on anticoagulation. Three had non-sustained tachyarrhythmia and were started on beta blockers. Post-ILR battery depletion, one suffered complete heart block and two had sustained ventricular tachycardia, all requiring device therapy. Those with arrhythmia had a shorter PR interval on electrocardiography. This study demonstrates that ILR implantation in FD uncovers a high burden of arrhythmia. ILRs are likely to be underutilised in this pro-arrhythmic cohort, perhaps restricted to those with advanced FD cardiomyopathy. Following battery depletion in three patients as mentioned above, greater vigilance and arrhythmia surveillance are advised for those experiencing major arrhythmic events post-ILR monitoring. Further work is required to establish who would benefit most from implantation.
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  • 文章类型: Multicenter Study
    目的:了解本病的流行病学特征,临床病理发现以及最近在性活动相关猝死(SArSD)中滥用物质和处方药的使用。
    方法:在西班牙27个省进行了12年(2010-2021年)的基于多中心人群的法医尸检研究。
    结果:在18046例尸检自然死亡病例中,调查了64例(0.35%)SArSD(男性87%)。女性比男性年轻(50.5±13.4岁vs37.2±14.2;p=0.017)。心脏猝死(SCD)占病例的87%。缺血性心脏病是主要病理(58%),主要影响年龄≥36岁的男性。脑出血(8%)和哮喘(5%)是主要的非心脏原因。在年轻人中,SADS(36%)和哮喘(27%)是主要原因。在7名受试者的生活中诊断出了导致SCD的疾病。64%的患者有心血管危险因素,主要是肥胖。毒理学分析发现非法药物(23%),主要是可卡因,治疗勃起功能障碍的药物(9%),乙醇≥0.5g/L(8%)。死亡通常发生在异性恋性交期间或之后。最常见的位置是在家里(63%)。在12名男性中,性伴侣是性工作者。
    结论:SArSD在影响中年男性与异性性伴侣性交时的普通人群中发病率较低。它起源于心血管,主要是由于生活中经常保持沉默的缺血性心脏病。经常与肥胖有关,使用可卡因(和,在较小程度上,勃起功能障碍的药物)和进行非常规的性行为。法医调查有助于制定预防策略。
    OBJECTIVE: To investigate the epidemiological characteristics, clinic-pathological findings and recent use of substances of abuse and prescribed drugs in sexual activity-related sudden death (SArSD).
    METHODS: Multicenter population-based study on forensic autopsies conducted in 27 provinces of Spain over 12 years (2010-2021).
    RESULTS: Out of 18046 autopsied natural deaths, 64 cases (0.35 %) of SArSD were investigated (87 % males). Women were younger than males (50.5 ± 13.4 years vs 37.2 ± 14.2; p = 0.017). Sudden cardiac deaths (SCD) accounted for 87 % of cases. Ischemic heart disease was the predominant pathology (58 %), mainly affecting men ≥ 36 years of age. Cerebral haemorrhage (8 %) and asthma (5 %) were the leading non-cardiac causes. In young adults, SADS (36 %) and asthma (27 %) were the main causes The disease responsible of SCD was diagnosed in life in 7 subjects. In 64 % there were cardiovascular risk factors, mainly obesity. Toxicological analysis detected illicit drugs (23 %), mainly cocaine, medications for erectile dysfunction (9 %), and ethanol ≥ 0.5 g/L (8 %). Deaths occurred usually in the context of heterosexual intercourse and during or immediately after sexual activity. The most common location was at home (63 %). In 12 men the sexual partner was a sex worker.
    CONCLUSIONS: SArSD has a low incidence in the general population affecting middle-aged males during intercourse with a heterosexual partner. It is of cardiovascular origin, mainly due to ischemic heart disease that frequently remained silent during life. There is a frequent association with obesity, use of cocaine (and, to a lesser extent, medications for erectile dysfunction) and performing unconventional sexual practices. Forensic investigation is useful for developing prevention strategies.
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  • 文章类型: Journal Article
    背景:猝死是药物难治性癫痫死亡的主要原因。正在调查患有癫痫(PWE)的中年人的死亡风险和心血管疾病(CVD)负担。
    方法:使用英国生物库,我们确定了7,786名(1.6%)被诊断为癫痫的参与者和6,171,803人年的随访(平均12.30年,SD1.74);排除了566例有中风史的个体。7,220PWE包括研究队列,其余494,676名无癫痫作为比较组。使用经过验证的诊断代码确定CVD的患病率。Cox比例风险回归用于评估全因死亡率和猝死风险。
    结果:高血压,冠状动脉疾病,心力衰竭,心脏瓣膜病,先天性心脏病在PWE中更为普遍。包括心房颤动/扑动在内的心律失常(12.2%vs6.9%;p<0.01),缓慢性心律失常(7.7%vs3.5%;p<0.01),传导缺陷(6.1%vs2.6%;p<0.01),室性心律失常(2.3%vs1.0%;p<0.01),以及心脏可植入电子设备(4.6%vs2.0%;p<0.01)在PWE中更为普遍。PWE有较高的调整后全因死亡率(HR3.9[95%CI,3.01-3.39]),和猝死特异性死亡率(HR6.65[95%CI,4.53-9.77]);并且在死亡时几乎年轻2岁[68.1vs69.8;p<0.001]。
    结论:中年PWE增加了全因死亡率和猝死死亡率,和更高的心血管疾病负担,包括心律失常和心力衰竭。需要进一步的工作来阐明中年PWE中全因死亡率和猝死风险的潜在机制,在PWE中确定预后生物标志物并开发预防性治疗。
    BACKGROUND: Sudden death is the leading cause of mortality in medically refractory epilepsy. Middle-aged persons with epilepsy (PWE) are under investigated regarding their mortality risk and burden of cardiovascular disease (CVD).
    METHODS: Using UK Biobank, we identified 7786 (1.6%) participants with diagnoses of epilepsy and 6,171,803 person-years of follow-up (mean 12.30 years, standard deviation 1.74); 566 patients with previous histories of stroke were excluded. The 7220 PWE comprised the study cohort with the remaining 494,676 without epilepsy as the comparator group. Prevalence of CVD was determined using validated diagnostic codes. Cox proportional hazards regression was used to assess all-cause mortality and sudden death risk.
    RESULTS: Hypertension, coronary artery disease, heart failure, valvular heart disease, and congenital heart disease were more prevalent in PWE. Arrhythmias including atrial fibrillation/flutter (12.2% vs 6.9%; P < 0.01), bradyarrhythmias (7.7% vs 3.5%; P < 0.01), conduction defects (6.1% vs 2.6%; P < 0.01), and ventricular arrhythmias (2.3% vs 1.0%; P < 0.01), as well as cardiac implantable electric devices (4.6% vs 2.0%; P < 0.01) were more prevalent in PWE. PWE had higher adjusted all-cause mortality (hazard ratio [HR], 3.9; 95% confidence interval [CI], 3.01-3.39), and sudden death-specific mortality (HR, 6.65; 95% CI, 4.53-9.77); and were almost 2 years younger at death (68.1 vs 69.8; P < 0.001).
    CONCLUSIONS: Middle-aged PWE have increased all-cause and sudden death-specific mortality and higher burden of CVD including arrhythmias and heart failure. Further work is required to elucidate mechanisms underlying all-cause mortality and sudden death risk in PWE of middle age, to identify prognostic biomarkers and develop preventative therapies in PWE.
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