阴性解剖中的心脏性猝死

综述了近年来有关心脏性猝死的分子生物学和电生理学的研究进展,着重讨论了心震荡、先天性长QT间期综合征和Brugada综合征,可能诱发致死性心律失常,发生心脏性猝死。这些功能性病症死亡常可导致病理解剖时无明显客观器质性病理改变。提示法医和病理工作者在遇到阴性解剖猝死时,应注意了解猝死的诱因、继往病史和家族病史,注意排除可能存在的这些病症。     

A pathological study of sudden coronary death in China: report of 89 autopsy cases.

This paper reports the results of a forensic pathological study of 89 autopsy cases of Sudden Coronary Death (SCD). Of 89 cases, 63 (52 male, 11 female) were narrowed by 76-100% in cross-sectional area (XSA) of the coronary artery (CA) and 26 (22 male, 4 female), by 51-75%. Atherosclerotic plaques in the CA were serious and extensive, especially in the left anterior descending and often involved several branches of CA at the same time. Recent thrombosis was found in 18 cases, haemorrhage in plaques in 17 cases. Only 2 cases had visible acute myocardial infarction. Inflammatory cell infiltration was found in coronary plaques in 36 cases. Myocardial fibrosis or small scar formation was detected in 51 cases. It is suggested that although the incidence of coronary heart disease (CHD) is low in China, SCD is the commonest mode of Sudden Unexpected Death. The majority of SCD (52%) were middle aged males (30-49 years old). Most of the cases died suddenly during sleep without any clear precipitating factors. The characteristics of occurrence and pathological changes in the CA and myocardium and the pathological diagnosis of SCD are also analysed and discussed.     

Sellar fracture followed by sudden death: an autopsy case

The authors describe a case of sellar fracture followed by sudden death. The victim was involved in a wrangle. The autopsy revealed facial damage and sellar fracture and no evidence of cerebral damage, except for a mild subarachnoid hemorrhage in the left parietotemporal regions and undersurface of both frontal lobes. Sellar fracture is a rare and severe entity, associated with serious complications, which is frequently diagnosed postmortem. In any case, death is rarely a direct consequence of the sellar fracture itself and is usually considered to be the result of associated cerebral trauma. This case prompted us to screen the literature on sellar fracture to gain a better understanding of the mechanism of death.     

Pathology of sudden death during recreational sports activity: an autopsy study of 31 cases

A growing number of people are involved in recreational physical activity. It is therefore not uncommon for a medical examiner to encounter sports-related sudden deaths and to be faced with the legal implications. The authors examined the clinical and cardiac pathologic patterns in 31 persons who died suddenly during sports activities and underwent autopsy at the Institute of Forensic Medicine of Paris between 1991 and 2001. Twenty-nine male subjects, ranging in age from 7 to 57 years (mean 30 years) and two female subjects, 8 and 60 years old, died suddenly during sports activities. The sports involved were various, with running the most frequent: 13 cases. Cardiomyopathies (10 cases) and coronary artery disease (9 cases) were the most frequent causes of deaths. Despite the severity of lesions, only 4 subjects had a known cardiovascular disease. In conclusion, with regard to prevention, efforts should be continued to improve the sensitivity and specificity of diagnostic tools and screening strategies. In this regard, medicolegal autopsies should be systematically performed in cases of sudden death during sports activities, because they provide accurate and useful information for a better knowledge of sports-related mortality.     

心脏性猝死的机制研究进展

世界范围内心脏病性猝死发病率正呈逐年升高趋势并已成为第一大死亡原因,因此国内外学者在这方进行了广泛而深入的研究。本文综述了心脏病性猝死的病理学、神经体液、电生理特别是其分子生物学发病机制的研究成果。     

窦房结中央动脉狭窄与心源性猝死

近年来国内外学者相继报道由单纯窦房结中央动脉狭窄引起的猝死。本文就窦房结中央动脉的解剖、组织学特点、病变特点进行综述,探讨窦房结动脉狭窄引起猝死的机制,以期为相关法医病理学死因鉴定提供理论基础。     

Association of right coronary artery hypoplasia with sudden death in an eleven-year-old child

Congenital coronary artery abnormalities are a rare but well-documented cause of sudden and unexpected death in the pediatric age group. Most reported cases involve both an aberrant origin and course of the abnormal vessel. A case of unexpected death occurring in an otherwise healthy eleven-year-old boy, who had been previously investigated for sudden collapse, is described. The major finding at autopsy was marked disparity in diameter between the coronary arteries due to diffuse hypoplasia of the right coronary artery. This case demonstrates the importance of meticulous examination of the coronary artery system in cases of sudden death in childhood so that significant reduction in luminal cross section will be adequately documented. The possible role played by this finding in the etiology of sudden death is discussed.     

118例心源性猝死分析

心源性猝死(sudden cardiac death,SCD)在成人猝死的死因中占首位,本文对1998-2005年陕西地区发生的118例SCD案例进行统计分析,结果显示男性是女性的5.9倍,其中冠心病占55.1%,心肌炎占17.8%,心肌病占9.3%,先天性心脏病、瓣膜病、主动脉夹层瘤破裂各占2.5%,其他疾病占4.5%。说明SCD多发于男性,冠心病仍是SCD的主要基础疾病,心肌炎是青少年SCD的主要原因,心肌病是青壮年SCD的危险因素,无结构异常的心脏病也同样严重威胁生命健康。     

缝隙连接、连接蛋白与冠心病猝死

缝隙连接(gapjunction,GJ)构成细胞之间的跨膜水相通道,协调细胞间化学及电信息的交流。心脏上缝隙连接的主要功能是为心肌细胞间的电冲动提供低压传导通路,并维持心肌的协调运动。近年来的研究显示,心肌细胞(特别是心室肌工作细胞)上构成缝隙连接的连接蛋白含量、分布及功能的异常与各种心律失常的产生和持续关系密切。这很可能为临床及法医实践中,诊断冠心病猝死心肌早期病变提供一个新的思路。     

Sickle cell lung disease and sudden death: a retrospective/prospective study of 21 autopsy cases and literature review

Sudden death in the setting of sickle cell lung disease (SCLD), is periodically seen in the practice of medical examiners. The goal of the present study was to identify the most common pathologic findings of SCLD associated with sudden or unexpected death. A retrospective/prospective review of 21 autopsy cases from sickle cell patients between 1990 and 2004 was performed. Review of medical records, autopsy reports, and H&E-stained slides of lung tissue was performed. Oil-Red-O and elastic staining of lung tissue were evaluated. All cases were screened for both acute and chronic forms of SCLD. Patients admitted for sickle cell pain crisis ranged in age from 8 months to 65 years. Fifteen out of 21 cases (71.4%) showed significant pulmonary pathology. The most frequent lung findings included pulmonary edema (47.6%), pulmonary thromboembolism (38.1%), fat emboli (33.3%), pulmonary hypertension, grades I-IV (33.3%), and microvascular occlusive thrombi (28.5%). Our study demonstrates higher-than-expected percentages of acute and chronic sickle cell-related lung injury such as fat embolism (33.3%) and pulmonary hypertension (33.3%), with right ventricular hypertrophy (33.3%). Therefore, we propose a simple and high-yield autopsy algorithm of ancillary procedures that should be applied on all known and suspected autopsy cases of sickle cell disease.     

A case of sudden death due to spontaneous coronary artery dissection

Spontaneous dissection of the coronary arteries is an extremely rare event that occurs usually in middle-aged women and is mostly recognized at postmortem examination in victims of sudden death. It is a rare coronary pathologic finding whose precise incidence, etiology, pathogenesis, treatment, and evolution have not been clearly established. We present a sudden death case of a 53-year-old woman with spontaneous dissection of the left anterior descending coronary artery with eosinophilic coronary arteritis.     

遗传性心律失常猝死的分子解剖研究现状及展望

遗传性心律失常所致猝死的死因鉴定是法医病理学领域亟待解决的难题之一。近年来心律失常易感基因/突变的发现和高通量组学技术的推广,使得利用分子遗传学方法筛查猝死的遗传学病因（即＂分子解剖＂）成为可能。本文通过汇总心律失常分子遗传研究的进展,综述传统遗传分析和近期全基因组关联性研究（GWAS）筛查的结果,为心源性猝死的＂分子解剖＂研究提供候选基因列表;并进一步比较针对不明原因猝死所开展的回顾性＂分子解剖＂筛查的结果,探讨新技术在该领域的应用前景。这一综述有助于更好的认识心律失常所致猝死的分子机制,并为借助新一代遗传分析技术进行分子解剖提供有益参考。     

Digoxin, magnesium, and potassium levels in a forensic autopsy material of sudden death from ischemic heart disease

In 91 cases where the cause of death was heart disease, digoxin, Mg and K concentrations in serum and ventricular myocardium were measured post mortem. Forty per cent were positive for digoxin in both serum and myocardium. The mean serum level was 5.1 +/- 2.4 nmol/l and the mean myocardial level was 42.6 +/- 27.5 ng/g. Correlation could be established between serum and myocardial concentrations of digoxin. There were statistically significant differences in serum as well as in myocardial digoxin levels in persons on 0.13 mg and 0.25 mg per day, respectively. Myocardial levels of Mg and K were low as generally found in persons with ischemic heart disease. There was no correlation between these levels and myocardial digoxin concentrations. Caution must be exercised in the assessment of digoxin results from cadaver samples because of the postmortem rise of digoxin serum concentrations. Considering this fact, the results still indicate that the prevalence of toxic digoxin concentrations might be more common than previously thought.     

急性出血坏死怀肠炎猝死7例尸检分析

对牡丹江等城市 1986～ 1998年因急性出血坏死性肠炎猝死 7例尸检资料进行分析 ,探讨其年龄、性别、临床症状和生前临床诊断与病理解剖结果。结果表明因其病变不典型、不特异、患者的临床症状有所不同 ,所以在临床上容易造成漏诊、误诊、引起医疗纠纷 ,其病理变化因患者临床表现不同可有程度不同的差别。     

Stressful events as a trigger of sudden death: a study of 43 medico-legal autopsy cases

The reports relating emotional stress to sudden death are largely anecdotal. In addition to experimental and electrophysiological studies, an opportunity for a better understanding of possible stress-related sudden death (SSD) may be provided by medico-legal autopsies. The goal of our autopsy study was to analyze cardiovascular pathologic findings in cases of SSD and if possible identify mechanisms by which the stressful event (SE) could be the cause. Forty three cases were studied (29 males and 14 females). In all cases, the SE and the death were witnessed. The age range was 22 to 90 years in males (mean, 52) and 30 to 92 years in females (mean, 64). Death occurred in all cases without premonitory symptoms. In 20 cases, death occurred during the SE and in the other 23 cases occurred within 2 h of the event. SE included fear, 15 cases; altercation, 21 cases; sexual activity, 3 cases; police questioning or arrest, 4 cases. According to police reports, in 40 cases (90%), the victims had no previous clinical history of cardiovascular disease. At autopsy, the heart weight in males ranged from 255 to 1000 g with a mean of 517 g and in females the range was 250–700 g with a mean of 417 g. In only 3 cases, gross and microscopic examination of the heart was normal. In 2 of the remaining 40 cases the subjects died of subarachnoid hemorrhage. In 38 cases, a cardiac cause of death was found as follows: coronary heart disease, 27 cases; cardiomyopathy, 6 cases; aortic valvular stenosis, 2 cases and right ventricular dysplasia, 3 cases. A coronary artery thrombosis was found in 8 cases of sudden coronary death. Post myocardial infarction fibrosis was present in 25 cases (92%) of sudden coronary death. In conclusion, it appears from our autopsy study that SSD occurs primarily in those individuals with severe heart disease, especially coronary heart disease.     

An autopsy case of sudden unexpected death due to atherosclerotic coronary heart disease associated with simple right ventricular outflow tract stenosis

A case of a 35-year-old male who died suddenly after a blow on the chest by his opponent during a quarrel. From both the clinical and autoptical examinations there was no evidence of trauma found anywhere, even in the chest. The heart was the only one of all the internal organs whereon significant pathologic changes were observed during autopsy. It's lesions were as follows: (I) The right ventricle was greatly enlarged simply due to the simple right ventricular outflow tract stenosis (SRVOTS); the heart weight was 587 g. (II) All the main trunks of coronary arteries were involved by atherosclerosis. In addition, the appearance of the lumen at the proximal end of the left anterior descending artery narrowed by an atheromatous plaque (grade II) was completely occluded by a newly formed thrombus. The authors believed that both the coronary atherosclerotic changes and the enlarged heart due to the SRVOTS played a role in the pathogenesis of death (Kelberlus, H.E. and Wellens, H.J.J., Sudden Death, Developments in Cardiovascular Medicine, Vol. 4, Martinys Nijhoff Publishers, London, 1980, pp. 34-48; Jinming, Qin, A study of cardiac sudden death (51 cases) on the clinical pathologic view, Tianjin Med., 8 (1980) 458-461). The quarrel and blow were the disposing factor.     

Myxoid heart disease: A review with special emphasis on sudden cardiac death

Myxoid heart disease (MHD), more commonly known as mitral valve prolapse, is a very common caridac abnormality affecting 5–10% of the general population. This article reviews the history, symptoms, physical findings, pathology, associated conditions and complications of this entity. Special emphasis is given to sudden cardiac death, which occurs as a result of acute heart failure or due to a fatal arrhythmia. Theories regarding the origin of the arrhythmias are discussed. Once the origin is known, those at risk for this devastating complication can be identified and perhaps a preventative therapeutic regimen developed.     

Myxoid heart disease: a review with special emphasis on sudden cardiac death

Myxoid heart disease (MHD), more commonly known as mitral valve prolapse, is a very common cardiac abnormality affecting 5-10% of the general population. This article reviews the history, symptoms, physical findings, pathology, associated conditions and complications of this entity. Special emphasis is given to sudden cardiac death, which occurs as a result of acute heart failure or due to a fatal arrhythmia. Theories regarding the origin of the arrhythmias are discussed. Once the origin is known, those at risk for this devastating complication can be identified and perhaps a preventative therapeutic regimen developed.     

冠心病猝死冠状动脉粥样硬化斑块病理形态学观察

目的观察冠心病猝死(SCD)和对照组心冠状动脉(CA)粥样硬化斑块的差异,探讨其在SCD中的意义。方法从本教研室2001年~2003年尸检档案中挑选64例有严重CA粥样硬化病变的病例及心脏标本,分SCD组(36例)和对照组(28例冠心病非猝死者)。除常规检查心脏外,重点检查CA左主干(LM)、左前降支(LAD)、左旋支(LC)和右主支(RM)的开口、类型、走向及粥样硬化斑块情况。每支CA分近、中、远3段,检测每个斑块距开口的距离、狭窄程度、长度、数目和形状(分偏心、环形、偏心+环形三种)。用SPSS11.5统计软件进行统计分析。结果SCD组中CA粥样硬化病变4级21例,3级15例;对照组4级12例,3级16例。两组斑块病变的严重程度无显著性差异(P>0.05)。SCD组粥样斑块长(x-2.0365 cm)、以CA近段较多、与CA开口或分支处的距离近(x-0.7457 cm);对照组斑块短(x-1.4283 cm)、以中段多见,与CA开口或分叉处的距离远(x-2.1942 cm);两组斑块的长度和距开口的距离均有非常显著性差异(P<0.01)。两组间斑块的形状在LAD和RM有显著性差异(P<0.05),而LM和LC则无(P>0.05)。结论SCD与冠心病非猝死者的CA粥样硬化斑块在部位、长度、形状和距CA开口的距离存在差异,而狭窄程度无差异。这对SCD的病理诊断和法医学鉴定具有十分重要的意义。     

Unexpected sudden death of a 12-year-old male with congenital single coronary artery

A rare autopsy case of a 12-year-old male with a single coronary artery is reported in which he suddenly and unexpectedly died after a brief period of physical and mental excitation. The single coronary artery originated from the left aortic sinus, then branched out the left circumflex artery and afterwards descended as the left anterior descending artery, while the right coronary artery originated almost at a right angle from the left anterior descending artery. The entire heart as well as the cardiac conduction system depended exclusively on the single coronary artery for oxygenated blood supply, and the unbalanced blood distribution on his exertion probably led to sudden cardiac death. Moreover, in this case, both the deceased's mother (at the age of 20 years) and grandmother on the mother's side died suddenly, thus suggesting a possibility of maternal transmission of this congenital anomaly in his family.