Sudden unexpected infant death due to fibroma of the heart

A 7-month-old previously healthy female infant was found dead in her crib by her mother shortly after having been laid down to sleep following the noontime feeding. Because the child did not suffer from an acute illness and no other evidence pointed to a cause of death, it was initially assumed by the police that she had died of sudden infant death syndrome. At autopsy, however, the cause of death was determined to be cardiac arrhythmia secondary to fibroma of the heart.     

Papillary fibroelastoma of the aortic valve: a sudden death case of coronary embolism with myocardial infarction

Papillary fibroelastoma is a rare benign tumor, occasionally causing angina or sudden death. We report an autopsy case of an aortic valve papillary fibroelastoma with coronary artery embolism. The patient was a 68-year-old Japanese man who had collapsed suddenly in his house. He was a heavy drinker and had a history of liver disease but no notable cardiac event. The autopsy revealed extensive transmural infarction of the inferior wall of the left and right cardiac ventricles. The distal portion of the right coronary artery (segment 4, NYHA) was completely occluded by tumor emboli of the fibroelastoma. At the site of closure of the aortic non-coronary cusp, there was a typical papillary fibroelastoma, which was considered to have originated the coronary embolization.     

Sudden Death Due to Infiltration of Left Bundle Branches by Interventricular Septal Cardiac Fibroma

Cardiac fibromas are benign conditions; however, their location and size may cause ventricular arrhythmias and sudden cardiac death. We report a case of a 68‐year‐old female who died suddenly. Postmortem investigation detected a huge cardiac fibroma in the pars muscularis of the interventricular septum, occupying almost the entire muscular septum, and restricting the volume of left ventricular chamber. Histological examination revealed numerous foci of calcification in the alternating complex interlacing or strictly parallel collagenous fiber mass. Tumor mass was mainly demarcated, but in some places, fibrous infiltration of surrounding working cardiac muscle was found. We present a case when direct tumor involvement in the descending left bundle branches was evidenced. Mainly, the branches of septal fascicle were disrupted, entrapped, and degenerated by the tumor mass. This case report emphasizes that postmortem histological examination of conduction system in all sudden cardiac death cases may substantially improve the accuracy of postmortem diagnosis.     

Anomalous Anterior Papillary Muscle as an Autopsy Finding in Two Cases

Anomaly in the anterior papillary muscle (APM) is known to cause left ventricle outflow tract (LVOT) obstruction, and this rare congenital condition could be a cause of sudden cardiac death. This anomaly and its hemodynamic effects is similar to valvular heart disease. In our two described cases, in which cause of death was, respectively, hypertrophic cardiomyopathy and suicide by hanging, the cephalad portion of the left APM was inserted directly into the ventricular surface of the anterior mitral leaflet and chordae tendineae were absent in the area of the direct anomalous muscle insertion; the aberrant papillary muscle was very large and showed an exaggerated anterior displacement within the left ventricular cavity. The described anomaly is a cause of LVOT obstruction. This condition is considered to be rare, although incidence estimates do not exist. In the absence of other possible causes, this finding may indicate arrhythmia as being the immediate cause of death.     

冠心病猝死的法医病理学免疫组织化学研究进展

冠心病猝死(sudden coronary death,SCD)是各种猝死中最常见的原因,免疫组织化学技术(immuno-histochemistry,IHC)是近年来研究冠心病猝死的有效方法。本文根据国内外文献对冠心病猝死的法医病理学免疫组织化学研究进展作如下综述。     

A Rare Presentation of Sudden Death due to Pulmonary Thromboembolism Possibly as a Result of Giant Hepatic Hemangioma

Hepatic hemangiomas are usually detected incidentally when investigating for other nonrelated pathologies. When symptomatic, they are usually large and cause symptoms such as abdominal discomfort or other digestive issues. Occasionally, uncommon presentations such as bleeding with coagulopathy (Kasabach–Merritt syndrome) or even fever of unknown origin may occur. As hepatic hemangiomas are benign, they do not result in tumor emboli and pulmonary thromboembolism due to hepatic hemangiomas is extremely rare. This is a case report of a rare case of a female with no known past medical history who presented with sudden death due to pulmonary thromboembolism, possibly as a result of an underlying giant hepatic hemangioma. Incidentally, she also had three other tumors—pancreatic cystic lymphangioma, cerebral capillary telangiectasia, and papillary thyroid carcinoma, and their potential contribution to the cause of death will be discussed.     

Sudden death and anomalous origin of the coronary arteries from the aorta. A case report and review

Based on a case report of sudden death in a young boy, this paper reviews the available information concerning the various combinations of anomalous origins of coronary arteries and associated sudden death. Left coronary arteries arising from the right sinus of Valsalva and passing between the aorta and pulmonary arteries are often associated with sudden death and myocardial ischemia in young people. Although right coronary arteries originating from the left sinus of Valsalva and passing between the aorta and pulmonary artery are less frequently associated with symptoms, this condition may be associated with sudden death. The incidence of symptoms associated with other anomalous origins is also discussed.     

过敏性休克死亡法医学诊断的研究现状

过敏性休克死亡在临床医学及法医学鉴定中较为常见,但过敏性猝死的死后诊断一直是法医病理学鉴定的一大难点。近年来,国内外学者研究了过敏性死亡血清IgE、组胺、肥大细胞类胰蛋白酶和P物质的含量以及其在肺脏和胃肠等组织中的免疫表达,试图为过敏性猝死的法医学鉴定提供客观、准确的形态学依据和诊断指标。本文就过敏性休克死亡法医学诊断的研究进展和存在的问题加以综述。     

类胰蛋白酶和IgE测定在过敏性猝死诊断中的应用研究

目的探讨过敏性猝死法医学鉴定的诊断方法和指标。方法采取10例正常人、9例过敏性猝死和19例其他死因（排除过敏反应、冠心病）尸体的静脉血，采用荧光酶联免疫法（Pharmacia UniCAP100过敏原定量分析仪）和酶联免疫吸附试验ELISA法分别测定血清肥大细胞类胰蛋白酶和]gE含量，采用免疫组化方法观察过敏性猝死和其他死因的肺组织中的肥大细胞类胰蛋白酶免疫组化染色。结果过敏性猝死者的血清类胰蛋白酶和IgE含量升高，与其他死因之间的差异具有显著性意义（P〈0．01），其他死因和正常人之间的差异无统计学意义（P〉0．05）；与其它死因相比，过敏性猝死肺组织中的肥大细胞类胰蛋白酶免疫组化阳性染色增强（P〈0．01）。结论过敏性猝死者血清IgE和肥大细胞类胰蛋白酶含量显著升高；过敏性猝死者肺组织中肥大细胞类胰蛋白酶染色增强。     

Is there progress in the autopsy diagnosis of sudden unexpected death in adults?

Sudden death is now currently described as natural unexpected death occurring within 1h of new symptoms. Most studies on the subject focused on cardiac causes of death because most of the cases are related to cardiovascular disease, especially coronary artery disease. The incidence of sudden death varies largely as a function of coronary heart disease prevalence and is underestimated. Although cardiac causes are the leading cause of sudden death, the exact incidence of the other causes is not well established because in some countries, many sudden deaths are not autopsied. Many risk factors of sudden cardiac death are identified: age, gender, heredity factors such as malignant mutations, left ventricular hypertrophy and left ventricle function impairment. The role of the police surgeon in the investigation of sudden death is very important. This investigation requires the interrogation of witnesses and of the family members of the deceased. The interrogation of physicians of the rescue team who attempted resuscitation is also useful. Recent symptoms before death and past medical history must be searched. Other sudden deaths in the family must be noted. The distinction between sudden death at rest and during effort is very important because some lethal arrhythmia are triggered by catecholamines during stressful activity. The type of drugs taken by the deceased may indicate a particular disease linked with sudden death. Sudden death in the young always requires systematic forensic autopsy performed by at least one forensic pathologist. According to recent autopsy studies, coronary artery disease is still the major cause of death in people aged more than 35 years. Cardiomyopathies are more frequently encountered in people aged less than 35 years. The most frequent cardiomyopathy revealed by sudden death is now arrhythmogenic right ventricular cardiomyopathy also known simply as right ventricular cardiomyopathy (RVC). The postmortem diagnosis of cardiomyopathies is very important because the family of the deceased will need counseling and the first-degree relatives may undergo a possible screening to prevent other sudden deaths. In each case of sudden death, one important duty of the forensic pathologist is to inform the family of all autopsy results within 1 month after the autopsy. Most of the recent progress in autopsy diagnosis of sudden unexpected death in the adults comes from molecular biology, especially in case of sudden death without significant morphological anomalies. Searching mutations linked with functional cardiac pathology such as long-QT syndrome, Brugada syndrome or idiopathic ventricular fibrillation is now the best way in order to explain such sudden death. Moreover, new syndromes have been described by cardiologists, such as short-QT syndrome and revealed in some cases by a sudden death. Molecular biology is now needed when limits of morphological diagnosis have been reached.     

Cardiac pathological study of sudden death due to myocarditis: analysis of 17 cases

17 cases of sudden death due to myocarditis were presented. The problem of the classification and mechanism of sudden death is discussed. It is concluded that heart function failure is the main reason for the sudden death of myocarditis.     

原发性心肌病猝死心肌mtDNA~(4977)缺失变化

目的探讨原发性心肌病(PCM)猝死者心肌线粒体DNA(m tDNA4977)缺失情况及其与猝死的关系。方法对18例PCM猝死和28例对照组病例心肌组织蜡块,用常规方法提取心肌m tDNA,以PCR、琼脂糖紫外凝胶成像技术确定扩增产物激光密度,初步定量检测m tDNA4977缺失率。结果PCM猝死18例中,检见13例m tDNA4977缺失,占72.44%。对照组28例中,检见3例m tDNA4977缺失,占10.71%;两组病例m tDNA4977缺失率均值分别为0.5795和0.0744,差异有非常显著性意义。结论多数PCM,特别是扩张型心肌病猝死者心肌可检见m tDNA4977缺失;提示其心肌m tDNA4977缺失变化与PCM猝死的发生可能有一定关系。     

Cardiac concussion: definition, differential diagnosis, and cases presentation and the legal ramification of a misdiagnosis

A cardiac concussion is caused by a sudden, nonpenetrating, localized impact to the chest that is theorized to result in almost simultaneous sudden death from a disruption to the conductive system. The detailed external/internal forensic examination of the body reveals no evidence of structural, pathologic, or histologic signs of trauma to the heart. A cardiac concussion is a rare and often overlooked cause of sudden death. This type of sudden death is typically seen among younger individuals participating in sports involving projectiles and, to a lesser degree, where collisions occur. Cardiac concussions are clinically, pathologically, and chemically different from a cardiac contusion. The objective of this paper will be to define cardiac concussion, differentiate between cardiac concussion and cardiac contusion, and describe the clinical and pathologic features of a 32-year-old white male who died of a cardiac concussion following a collision with a catcher during a softball game. The civil ramification of incorrectly diagnosing the manner of death in cases of death involving a cardiac concussion will also be addressed.     

连接蛋白43与心性猝死的相关性研究进展

部分心性猝死者死后尸检时心脏没有发现明显病理学异常征象,过去均将其归属原因不明的猝死范畴。近年来研究显示,心肌连接蛋白43（connexin43,Cx43）表达对于心性猝死的诊断具有十分重要的意义。本文就Cx43结构、功能以及在心血管疾病发生发展中的作用与法医学意义作一综述。     

The psychodynamics of the presidential assassin and an examination of the theme/graphic variables of his threatening correspondence

A case of fatal Reye's syndrome presenting as the second sudden infant death in a family is described. The increasing interest in fatty change in the liver in cases of sudden infant death syndrome is noted and it is suggested that standardised interpretation of findings is essential if its significance is to be correctly evaluated.     

Progressive muscular dystrophy of the Duchenne type

A case of sudden death is reported that occurred as a result of Duchenne type progressive muscular dystrophy with extreme myogenic changes. These changes consisted of extensive muscular lipomatous transformation, typical of the advanced stage of the disease. The heart showed myocardial fibrosis, the respiratory system acute tracheobronchitis with early signs of bronchopneumonia. The pattern of distribution of the changes in the musculature is discussed with regard to the sudden death as well as to the possibility that medical malpractice may have occurred.     

Sudden infant death with anomalous origin of the left coronary artery.

Autopsy of a 3-month-old girl, an apparent case of sudden infant death syndrome, revealed anomalous origin of the left coronary artery from the right aortic sinus. Acute angulation of the left coronary artery along the aortic root, as well as a focal intramyocardial course within the ventricular septum, may have contributed to episodic luminal narrowing. Anomalous coronary origins of similar type have been associated with sudden death in children, teenagers, and young adults, but have not necessarily been associated with sudden death in older adults. Somewhat similar malformations have been reported in sudden infant death; two cases involved the left coronary artery and six involved the right.     

过敏性和冠心病猝死者心肌组织中肥大细胞类胰蛋白酶、脑利钠肽的表达

目的探讨肥大细胞类胰蛋白酶、脑利钠肽(brain natriuretic peptide,BNP)在过敏性猝死和冠心病猝死鉴别诊断中的意义。方法选取山西医科大学法医病理学教研室2010—2015年尸检案例心肌标本共30例,分为颅脑损伤致死组、过敏性猝死组、冠心病猝死组,每组各10例。采用免疫荧光染色和Western印迹法分析各组心肌组织肥大细胞类胰蛋白酶和BNP的表达。结果过敏性猝死组、冠心病猝死组心肌组织内肥大细胞类胰蛋白酶免疫荧光染色均出现阳性染色;三组间两两比较,表达差异均具有统计学意义(P0.05)。冠心病猝死组心肌组织内BNP的表达量高于过敏性猝死组、颅脑损伤致死组(P0.05),过敏性猝死组与颅脑损伤致死组之间差异无统计学意义(P0.05)。结论联合检测心肌组织内肥大细胞类胰蛋白酶、BNP有望为过敏性猝死和冠心病猝死的法医学鉴别诊断提供帮助。     

Sudden Death Caused by Anomalous Origin of the Coronary Artery During Exercise

Anomalous origin of the coronary artery (AOCA) is a rare, but important cause of sudden cardiac death among young athletes. Nine autopsy cases (8 male, 1 female; mean age, 17.9 years; age range, 11–31 years) of sudden death during or just after exercise caused by AOCA were reviewed. The exercises performed at the time of death were running (4 cases), soccer (2 cases), and baseball, swimming and kendo (Japanese swordsmanship) (1 case each). In 6 cases, the left coronary artery arose from the right sinus of Valsalva, and in 3, the right coronary artery from the left sinus. The coronary arteries passed between the pulmonary artery and the aorta with an acute angle takeoff from the orifice. Three cases had cardiovascular manifestations prior to death. In cases with cardiovascular manifestations, novel imaging methods should be considered to prevent sudden death.     

过敏性猝死者咽喉和胃肠组织内P物质的表达

目的观察P物质在过敏性猝死人体咽喉和胃肠组织的表达,并探讨其在法医学鉴定中的作用和意义。方法应用免疫组化方法对15例过敏性猝死者咽喉和胃肠组织的P物质表达进行研究,同时以冠心病猝死者作为对照;应用计算机图像分析系统对免疫组化染色结果进行分析,计算阳性指数(PI)。结果与对照组相比,实验组咽喉和胃肠组织P物质表达明显增加(P<0.001)。结论咽喉和胃肠组织的P物质表达可作为过敏性猝死的法医学鉴定的形态学依据和参考指标。