Pulmonary vascular occlusive disease presenting as sudden death

We report a case of pulmonary vascular occlusive disease (also termed pulmonary veno occlusive disease) which caused the sudden death of an eleven-year-old girl. The post-mortem findings are described and the current theories of aetiology and pathogenesis of this unusual condition are reviewed. The clinical picture is characterized by pulmonary hypertension with marked increase of pulmonary arterial pressure but often normal or only slightly elevated wedge pressure. The pulmonary vasculature, especially the veins, show progressive fibrotic occlusion. The aetiology is unknown but an infective cause is considered most likely. Definitive diagnosis usually depends on an open lung biopsy or, more commonly, autopsy examination.     

Exercise-related sudden death due to an unusual coronary artery anomaly

Coronary artery anomalies are, after hypertrophic cardiomyopathy, the second most common cause of exercise-related sudden cardiac death in young American athletes. These anomalies have been associated with myocardial ischemia, arrhythmia, and sudden death during exercise. A 14-yr-old male with no previous abnormal medical history collapsed during soccer practice and was successfully resuscitated without defibrillation. An extensive cardiac workup did not reveal any abnormalities. Two weeks later, he experienced a cardiac arrest while running and could not be resuscitated. Autopsy demonstrated an acute angle take-off of the left main coronary artery and a transverse slit-like opening with a fibrous cushion, which created a kink near its origin. This case report illustrates the difficulty in diagnosing coronary artery anomalies in general, and acute angle take-off and ostial ridges in particular.     

Pursuing enigmas on ischemic heart disease and sudden cardiac death

This article reviews what our colleagues have found as to how ischemic injury or cell death develop in myocardium through Ca²⁺-dependent protease calpain and how compensatory responses evolve through activation of intracellular signaling molecules including PKC isoforms, MAP kinase family enzymes and PI3 kinase. We also addressed how restraint or other psychological stress evokes hypertension and cardiovascular responses in signaling molecules or genes. Unexpectedly, carbon monoxide protects heart and cardiogenic cells against ischemia-resperfusion injury. When I think back, the unresolved cases of autopsies provided ideas for experimental study, which then taught us how the other cases died.     

心源性猝死

尽管近年来临床逐渐能够识别和改变猝死的危险因素,成功地进行心肺复苏(CPR),并且使用抗心律失常药物治疗防止再发,猝死率已有明显下降。但是人群中老年患者的增加,心源性猝死的总人数仍然保持不变[1]。从一般意义上讲,猝死是指在终末事件发生前,患者心脏功能稳定,死亡发生于症     

致猝死的冠状动脉起源异常及其临床意义

目的 了解起自对侧冠状动脉窦并可能引发青少年运动员猝死的冠状动脉畸形在国人中的发病率,结合文献资料比较其影像学特点和临床意义.方法 应用PACS系统顺序回顾14 343例不伴有其他心脏大血管畸形的国人CT冠状动脉成像所见,选取左、右冠状动脉起自对侧冠状动脉窦及左、右单冠状动脉的病例,对冠状动脉开口位置、走行路径、非粥样硬化狭窄截面形态、径线、狭窄程度及冠状动脉近段与主动脉壁夹角进行分析统计.结果 检出冠状动脉发自对侧冠状动脉窦(包括单冠状动脉)74例,其近段走行于主、肺动脉之间者59例.右冠状动脉起自左冠状动脉窦且走行于主、肺动脉之间55例,左冠状动脉起自右冠状动脉窦且走行于主、肺动脉间2例,左、右单冠状动脉且走行于大动脉间者各1例.国人此类冠状动脉近段走行于两大动脉之间的起源异常发病率为4.1‰(59/14 343).右冠状动脉起自左冠状动脉窦,起始段常伴非粥样硬化性狭窄,狭窄面积≥50%者29例(52.7%),≥70%者3例(5.4%).近段走行于主、肺动脉之间的右冠状动脉起自左窦是左冠状动脉起自右窦的18.7倍(56/3).结论 CT冠状动脉成像是诊断冠状动脉起源异常的最佳影像学手段.国人中该畸形的解剖类型分布有其特点,这一特点对引发我国青少年运动性猝死的影响值得探讨.     

A case of sudden cardiac death due to mitochondrial disease

A 25-year-old, emaciated man without medical treatment was found to have died suddenly at home by his mother. At autopsy, there were no injuries to his body, but significant circulatory insufficiency was observed. Electron microscopy revealed abnormal mitochondria in cells of the cardiac conduction system. The conduction system was filled with mitochondrial size abnormalities and mitochondrial cristae abnormalities. No notable abnormal findings were observed in other organs. Genetic examination of the blood revealed the mitochondrial pathogenetic variant m.3243A>G. Epileptic seizures, diabetic ketoacidosis, and hyperosmolar hyperglycemic state were unlikely to be the cause of sudden death. The cause of death was diagnosed as arrhythmia possibly induced by the failure of the cardiac conduction system due to mitochondrial disease. This is a rare case of sudden death caused by an accumulation of abnormal mitochondria in the cardiac conduction system.     

Parapharyngeal abscess and sudden death

This case-report illustrates a sudden asphyctic death of a 68-year-old female caused by an inflammatory obstruction in the upper airway. The autopsy revealed a peritonsillar-retropharyngeal abscess. Possible explanations for this rapid and lethal progress of a tonsillar infection are discussed.     

Tonsillitis and sudden childhood death

Critical reduction in upper airway diameter may result from tonsillar enlargement due to infection or from associated abscess formation. Other potentially lethal complications include hemorrhage and disseminated sepsis. Two cases are reported to illustrate features of specific cases: Case 1: a 12-year-old girl who exsanguinated from a pharyngocarotid fistula caused by a retropharyngeal abscess due to acute tonsillitis, and Case 2: a 17-year-old girl who asphyxiated from an aspirated blood clot following tonsillectomy. While most cases of acute tonsillitis resolve without sequelae, occasional cases may be associated with a lethal outcome. Massive hemorrhage may occur due to erosion of tonsillar vessels or subjacent larger vessels, or it may follow surgical extirpation of the tonsils. The autopsy assessment of cases where there has been possible lethal tonsillar pathology requires review of the presenting history and possible operative procedures, with careful dissection of Waldeyer's ring, adjacent soft tissues and major vessels. Presentations may not be straightforward and there may be misleading histories of epistaxis, hemoptysis, hematemesis and even melena.     

Adrenocortical carcinoma and sudden death

A 26-year-old man who presented with a 2-year history of intermittent gynecomastia with recent onset of fever, night sweats, and abdominal distension was found to have a left-sided adrenocortical carcinoma with metastases to the liver and spine. Sudden death occurred 1 month after his presentation. At autopsy a saddle pulmonary thromboembolus was found occluding the pulmonary outflow tract, with smaller more peripheral pulmonary thromboemboli. No tumor deposits were identified in the thromboemboli. The thromboemboli had arisen from a tongue of tumor that had grown through the left adrenal vein into the inferior vena cava. Despite a high rate of angio-invasion there are very few reports of sudden death resulting from this phenomenon in patients with adrenocortical carcinoma.     

Cytokines and sudden infant death

Background

It has been hypothesised that inflammatory reactions could play an important role in the pathway(s) leading to sudden and unexpected death in infancy. On a molecular level, these reactions are regulated by various cytokines.

Methods

To characterise the role of IL-1?, IL-6 and TNF?? more precisely, the concentrations of these cytokines were determined quantitatively using specific ELISA techniques in serum and cerebrospinal fluid (CSF) in 119 cases of sudden infant death. The infants were grouped into four categories (SIDS, SIDS with infection, natural death due to infection and unnatural death).

Results

A good correlation was found between CSF and serum for IL-6 (Spearman correlation coefficients (SCC), 0.73) and also for TNF?? (SCC, 0.57), although the CSF concentrations were lower than that from the serum. There were no significant differences between the categories of death for any of the serum or CSF cytokines. Compared with normal values, increased serum concentrations of IL-1?, IL-6 and TNF?? were found in 70%, 69% and 38% of the cases respectively, indicating possible agonal or post-mortem changes of cytokine concentrations. In three cases very high cytokine concentrations were found (mainly for IL-6). This may have contributed to the mechanism of death (cytokine storm) in two of the cases.

Conclusions

In a small group of patients, very high cytokine concentrations are a possible explanation for the cause of death (??cytokine storm??).     

Sudden death in an alcoholic with intramyocardial small vessel disease

A case of sudden death in an alcoholic with intramyocardial small vessel disease is reported. The histological findings and their medicolegal significance are discussed. In particular, emphasis is placed on the importance of histological examination of the intramyocardial small vessels in alcoholic subjects dying suddenly and in whom no obvious satisfactory cause of death is found at necropsy.