Sudden death resulting from lesions of the cardiac conduction system

Sudden unexpected deaths in young persons with noncontributory histories, autopsy results, and drug screen results are a common problem in forensic pathology. As part of the evaluation of such cases, the cardiac conduction system (CCS) should be studied. To determine the type and incidence of lethal CCS lesions, the authors reviewed their files of sudden unexpected cardiac deaths with particular attention to cases with causes of death in the conduction system. Cases of sudden cardiac death in patients aged < or=40 years during a 10-year period (Michigan) and a 4 year-period (Spain) were selected from the files. From this group, cases were identified in which the cause of death was a lethal change in the CCS. The portions of the heart containing the CCS were excised, and at least one hematoxylin and eosin slide and at least one trichrome or elastic trichrome slide per block were studied. In the two centers, 381 cases of sudden cardiac death were identified. The most common causes of sudden cardiac death were arteriosclerotic narrowing of the coronary arteries, cardiomyopathy, and myocarditis. In 82 cases, there was no identifiable cause of death even after complete gross and microscopic autopsy was performed, a medical history was obtained, and a drug screen was performed. In 11 cases, the CCS contained lesions that were considered lethal: narrowing of the atrioventricular node artery by fibromuscular hyperplasia (7 cases) and atrioventricular node tumors (4 cases). The 11 cases accounted for 2.9% of the 381 cases of sudden cardiac death and 11.8% of the indeterminable cases. It was concluded that examination of the CCS in deaths in which the gross and microscopic autopsy, history, and drug screen fail to provide a cause of death can yield a cause of death in a significant percentage of cases. If heart block was not documented during life and no explanatory lesions were found during routine cardiac examination, examination of the CCS can yield valuable information.     

A retrospective analysis of cardiovascular deaths: A 5-year autopsy study of 1045 cases in Antalya,Turkey

This study aims to identify the macroscopic and microscopic changes that occur in the heart in different causes of cardiovascular death and sudden cardiac death in autopsy cases and evaluate the difficulties that a forensic practitioner may encounter during autopsies. All forensic autopsy cases in the Morgue Department of the Council of Forensic Medicine, Antalya Group Administration between January 1, 2015, and December 31, 2019, were examined, retrospectively. The cases were chosen according to inclusion and exclusion criteria, and their autopsy reports were examined in detail. It was determined that 1045 cases met the study criteria, 735 of which were also met the sudden cardiac death criteria. The top three common causes of death were ischemic heart disease (n = 719, 68.8%), left ventricular hypertrophy (n = 105, 10%), and aortic dissection (n = 58, 5.5%). The frequency of myocardial interstitial fibrosis was significantly higher in deaths due to left ventricular hypertrophy than in deaths due to ischemic heart disease and other causes (χ²(2) = 33.365, p < 0.001). Despite detailed autopsy and histopathological examinations, some heart diseases that cause sudden death may still not be detected.     

Total anomalous pulmonary venous drainage and sudden death in infancy.

The clinicopathologic features of four infants with undiagnosed total anomalous pulmonary venous return who died suddenly and unexpectedly are presented. Two infants were found dead in their beds and two collapsed following very non-specific episodes of mild tachypnoea and tachycardia, respectively. Pulmonary venous blood drained via a common channel to the left innominate vein, the superior vena cava, the inferior vena cava at the junction of the hepatic vein and the supradiaphragmatic portion of the inferior vena cava. All cases demonstrated right atrial and ventricular hypertrophy and in one case there was associated hypoplastic left heart syndrome. Although those mechanisms responsible for sudden infant death syndrome could not definitely be excluded in two cases, the presence of anomalous pulmonary drainage with significant right-sided cardiac cardiac hypertrophy in each patient strongly suggests a contributing role for the vascular malformation in the aetiology of sudden unexpected death. The importance of careful in situ dissection of the major vessels in all cases of paediatric sudden death is emphasised, particularly in the presence of other congenital cardiac anomalies.     

Left Ventricular Aneurysm: Sudden Unexpected Deaths in a 29‐Year‐Old Man

Left ventricular aneurysm (LVA) is an abnormal dilated heart structure, either congenital or acquired. LVA is a rare cardiac condition with no symptoms in most cases, thus occasionally diagnosed during investigations of other diseases. Its association with certain cardiac complications and sudden cardiac deaths has been reported. However, its role as a cause of sudden unexpected death is rare. The author reported a sudden cardiac death in a 29‐year‐old man with LVA. Without a significant coronary artery disease and known etiologies of LVA, such an abnormal heart structure in the present case was considered congenital LVA. As no other possible mechanisms of death could be identified other than LVA with its associated pathologic lesions, mural thrombi, and dilated cardiomegaly, his death was attributable to fatal cardiac arrhythmia (most commonly ventricular tachycardia) secondary to LVA.     

Sudden Cardiac Deaths in Adults with Congenital Heart Disease with Structural Abnormalities: A Retrospective Review of Cases in the Cook County Medical Examiner's Office

Sudden cardiac death is a significant cause of mortality in adults with congenital heart disease (CHD). The Cook County Medical Examiner's Office database was queried for cases of CHD as a cause of death in the period between July 2008 and April 2019. Twenty-two cases were identified, including 11 decedents with simple defects and 10 decedents with complex defects. All of the subjects were in apparent good health at the time of death. In the absence of other obvious causes of death, simple defects were considered cases of sudden cardiac death. Significant cardiac morphological changes were common in complex defects. While 16 cases had known, diagnosed/treated CHD, 5 cases had no diagnosis prior to autopsy. In these cases, the ability to recognize CHD (sometimes subtle) helped in determining the causes of death. Therefore, forensic pathologists must be able to properly recognize various forms of CHD and request consultations, when needed.     

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.     

Cardiovascular Conditions and the Evaluation of the Heart in Pregnancy‐Associated Autopsies

Abstract: Pregnancy‐associated death is defined as the death of a woman from any cause during pregnancy or in the year after delivery. This review concentrates on cardiac conditions that may result in pregnancy‐associated death including, but not limited to, acute myocardial infarction, endocarditis, peripartum cardiomyopathy, and prolonged QT syndrome. Lethal vascular conditions may also occur involving arterial dissection and thromboembolism, on occasion exacerbated by hypercoagulability, and altered hormonal and physiologic states. The autopsy evaluation of these patients includes a careful assessment of the medical history particularly for prior pregnancy‐related conditions, fetal loss, and episodes of unexplained collapse. A family history of sudden death at an early age may be significant. At autopsy, evaluation for underlying syndromes such as Marfan, or evidence of intravenous narcotism should be undertaken. Autopsy examination involves careful dissection of the heart and vessels with consideration of conduction tract studies and possible genetic evaluation for prolonged QT syndrome.     

6例甲状腺功能亢进性心脏病猝死法医学分析

目的分析甲状腺功能亢进性心脏病(以下简称甲亢性心脏病)猝死案例,探讨其死亡的一般情况及法医病理学特点,为此类案件的法医病理学鉴定提供参考。方法收集中国医科大学法医学院2001—2016年6例甲亢性心脏病猝死案例,回顾性分析基本信息(性别与年龄)、临床表现、病史、解剖所见和组织病理学所见、生物化学检测指标、死亡原因。结果 6例案例多具有明确的甲状腺功能亢进病史,并表现出不同程度的心血管病症状;均具有明显的死亡诱因;甲状腺病理学检验符合弥漫性毒性甲状腺肿的表现;心脏质量均增加,心腔扩张,心肌肥大,灶状坏死;死后心包液的生物化学检测可作为甲亢性心脏病猝死的辅助手段。结论对甲亢性心脏病猝死案例进行诊断时应参考临床病史、尸体检验、组织病理学检验、死后毒(药)物检验等结果综合判定,必要时进行死后甲状腺和心功能的生物化学检测。     

Fatal ischemic stroke due to dissecting aneurysm of the intracranial arteries presenting as sudden unexpected death in childhood

Sudden unexpected death is any nontraumatic death in healthy individuals with normal activities for age until acute demise. Whereas not uncommon in adults and infants, it is considered a rarity in children, with most cases due to infection or occult cardiovascular abnormalities. Pathologic intracranial findings are rarely found in sudden unexpected death in childhood, with most cases due to occult intracranial neoplasms. Cerebral arterial dissection is an uncommon cause of arterial ischemic stroke in childhood. Outcome is poor, with more than 20% of patients dying. We report 4 cases of sudden unexpected death due to arterial ischemic stroke after cerebral arterial dissection in childhood and present a review of the literature.     

97例心性猝死的法医病理学分析

目的探讨心性猝死（SCD）的特点、病理基础及致死因素和诱因等。方法对本系2002年12月至2006年12月期间，所作450例法医病理检案的97例心性猝死案例进行分析研究。结果97例SCD患者中，冠心痛猝死38例，心肌炎23例，心肌痛16例，高血压性心脏病12例，主动脉瘤破裂4例，肺栓塞4例。结论SCD病程短骤、凶险，以老年男性多见，冠心病占首位。由于猝死的因素繁多，因此对猝死事件的法医学鉴定要根据其发生特征和变化规律，作出客观、全面、准确的签定结论。     

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.     

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。     

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.     

Comparison of heart mass in seizure patients dying of sudden unexplained death in epilepsy to sudden death due to some other cause

Proposed mechanisms by which sudden unexplained death syndrome in epilepsy (SUDEP) occurs include cardiac dysrhythmias. We hypothesized that individuals dying of SUDEP would have enlarged hearts compared with normal, increasing the risk of sudden cardiac death should the autonomic nervous system initiate a dysrhythmia. We performed a retrospective case-control study in a medical examiner population, comparing the mean heart mass in a group of individuals who died of SUDEP to a group of individuals with epilepsy who died suddenly due to some unrelated cause (non-SUDEP). We found no significant difference in the mean heart mass between the 2 groups when analyzing the unadjusted data. Upon stratifying the cases by age, however, we found a significant reduction in the frequency of SUDEP in individuals 40 or more years of age with an increased heart mass compared with those younger. This reduced frequency disappeared when cases where the cause of death was indeterminate between SUDEP and heart disease were reclassified from non-SUDEP to SUDEP. With increasing age, the likelihood of finding a cause of death that competes with the possibility of SUDEP increases, making SUDEP appear to be a phenomenon of the young. The inclusion of seizure deaths evaluated in a medical examiner office in studies of SUDEP would provide the benefit of a more certain diagnosis in each given case. Moreover, the inclusion of cases from the medical examiner population would stem attrition in a clinical study due to loss to follow-up.     

"Spontaneous" coronary artery dissection. The challenge of detection, the enigma of cause

Sudden death secondary to acute dissection of a coronary artery is a rare, but increasingly recognized, cause of sudden, unexpected death in apparently healthy persons. It has been reported more frequently in women and has been associated with sudden death during the puerperium. It has also been reported that these involved coronary vessels contain increased numbers of eosinophils and often show areas of cystic medial necrosis. In this article, we report a case of sudden death in a 47-year-old white woman due to dissection of the distal segment of her left anterior descending coronary artery. There was marked involvement of the coronary arterial walls with cystic degeneration of the media with accumulation of glycosaminoglycans as demonstrated by Alcian blue staining. There was no eosinophilic infiltrate within the arterial walls. This case is unusual in that this woman's mother and brother both have had aneurysms, which stresses the importance of not only searching carefully for these lesions, but also of obtaining family history in such cases.     

Impact of Myocardial Infarction and Abnormalities of Cardiac Conduction System on Sudden Cardiac DeathCSCD

Sudden cardiac death (SCD), most commonly seen in coronary heart disease, is a kind of sudden death caused by series of cardiac parameters, which usually combines with myocardial infarction. However, some SCDs (including early myocardial infarction) happen suddenly and cause death in a very short time. In these circumstances, typical morphological changes are lack in macroscopic or microscopic fields, which make such SCDs become the emphasis and difficulty in the present research. SCD caused by myocardial infarction and abnormalities of cardiac conduction system (CCS) is related to atherosclerosis of coronary artery closely. This paper reviews cardiac dysfunction caused by myocardial infarction and diseases of CCS from morphology and molecular biology, and explores potential relationship between them. This paper aims to provide clues to the mechanism of myocardial infarction related sudden death and possible assistance for forensic diagnosis of SCD. © 2017 by the Editorial Department of Journal of Forensic Medicine.     

Sudden death after a cold drink: case report

We report a case of sudden cardiac death in a 12-year-old boy after rapid ingestion of a frozen slurry drink. The cause of death was determined to be a cardiac arrhythmia secondary to a previously undiagnosed cardiac rhabdomyoma with associated myocardial scarring. Ingestion of cold liquids has been associated with syncope, but not sudden cardiac death. In this case, bradycardia induced by cold-induced vasovagal reflex may have precipitated the terminal arrhythmia. Ingestion of cold liquids should be considered a potential trigger for fatal cardiac arrhythmias in patients with underlying heart disease.     

Subvalvular aortic stenosis as a cause of sudden death: two case reports

Sudden death is defined as a death that occurs suddenly, develops during an unpredictable course, and is due to natural or unnatural causes. Although there is no universally standardized definition on how "sudden" a sudden death is, WHO defines sudden death as a death that occurs within 24 hours after the onset of symptoms. The aim of this study is to present 2 rarely reported autopsy cases and to emphasize the importance of systemic autopsy at sudden death. On macroscopic examination, crescent-shaped, thick, fibrous membranes, located 5 mm and 3 mm away from the aortic valves, were detected. Fibrous membranes extended from the ventricular septum to the left ventricular outflow tract, thus apparently narrowing this region. Left ventricular wall and septum were slightly thickened, and there were scattered grayish-white areas of a small diameter. These became more intense in the septum and myocardium of the left ventricle on the anterior plane of the myocardial sections. In both cases, the aortic valves of were thickened and also markedly narrowed on one of them. In this case, the fibrous membrane adhered to the aortic valve and extended to the anterior leaflet of the mitral valve at one side. Both aortic valves comprised 3 leaflets. Other valves and coronary arteries showed no macroscopic pathologic findings. Microscopic examination of both cases demonstrated that the fibrous membrane comprising abundant collagen fibers was situated on the ventricular septum. Hypertrophy, moderate to severe interstitial fibrosis, and focal areas of scarring were observed in the specimens taken from the septal and ventricular myocardium. No abnormality was found on the conduction system examinations. Toxicologic analysis results in blood were negative. Based on the findings, membranous-type (discrete type) subvalvular aortic stenosis, diagnosed during the autopsy, was considered as the cause of sudden death in both cases.     

Metastatic calcification of the cardiac conduction system with heart block: an under-reported entity in chronic renal failure patients

Systemic metastatic calcification is a common complication of chronic renal failure. Cardiac involvement is particularly ominous, especially when the cardiac conduction system is affected. Conduction defects, arrhythmias, and sudden death have all been reported with conduction system calcification; however, these are relatively under-reported or unrecognized causes of cardiac morbidity and mortality. We describe a 40-year-old man with Von Hippel-Lindau disease who had been maintained on hemodialysis for two years following bilateral nephrectomies for renal cell carcinoma. The patient presented with symptomatic complete heart block that had progressed from Mobitz type I atrioventricular block. Two months later, while being internally paced, the patient died unexpectedly after a complicated hospital admission. Postmortem revealed extensive vascular, myocardial, and conduction system calcification. Conduction system calcification may cause sudden death in chronic renal failure patients during hospital admission, or unexpectedly while the patient is in the community. Knowledge of this condition is necessary to detect it, as the conduction system is not routinely examined. A routine abbreviated conduction system examination is warranted for patients with systemic metastatic calcification, especially if they have sudden death or a known history of heart block.