Ascending Aortic Rupture through a Penetrating Atherosclerotic Ulcer: A Rare Cause of Sudden Unexpected Death

Spontaneous rupture of the aorta through an atherosclerotic lesion without preexisting aortic aneurysm, dissection, or history of trauma is very rare. Without prompt aortic repair, all cases result in sudden death with a definitive diagnosis made only intraoperatively or during autopsy. The phenomenon has been uniformly found in individuals with hypertension. The author reports a sudden unexpected death caused by spontaneous rupture of the ascending aorta in a 57‐year‐old man with a history of hypertension. The ascending aortic wall showed a longitudinal intimal tear measuring approximately 1 cm in length and rupture of the ascending aorta through an atherosclerotic ulcer, leading to massive hemopericardium and eventual death. Chronic hypertension and a penetrating atherosclerotic ulcer of the ascending aorta were the apparent underlying etiologies of the aortic rupture in the present case. This case illustrates not only the association between a rupture and a penetrating atherosclerotic ulcer with a silent death, but also raises awareness of possible such deaths.     

Descending thoracic aortic aneurysm rupture during postpartum period

Aortic aneurysm refers to the pathological dilatation of the normal aortic lumen involving one or several segments. Thoracic aortic aneurysms are much less common than aneurysms of the abdominal aorta. Descending thoracic aortic aneurysm leading to dissection and spontaneous rupture is a potentially catastrophic illness. Although rare, dissection and rupture of a preexisting aortic aneurysm have been reported during pregnancy and early puerperium. To the best of our knowledge, such cases among young pregnant women are rarely reported in literature. Herein, an autopsy case of spontaneous rupture of a clinically undiagnosed descending thoracic aortic aneurysm during early puerperium in a young woman is presented along with the review of relevant literature. The victim was found dead on her hospital bed on the seventh day of puerperium. Autopsy with ancillary investigations revealed that the young woman died because of hemothorax from a ruptured dissecting descending thoracic aortic aneurysm secondary to chronic aortitis.     

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.     

Fourteen years' survival with an untreated traumatic rupture of the thoracal aorta

Untreated traumatic rupture of the aorta generally results in sudden death. A case with 14 years' survival after an unrecognized traumatic rupture of the aorta is presented. Chronic traumatic aneurysm of the aorta should be kept in mind in a patient who develops sudden symptoms of arteriosclerotic heart disease months to years after a relevant thoracic trauma.     

Fatal Hemorrhage from an Arteriovenous Fistula

In this study, we present two cases of sudden deaths of people with end‐stage kidney with arteriovenous fistulas for long‐term hemodialysis treatment. This procedure is associated with a number of known complications. While stenosis, thrombosis, and infection are well known, lethal hemorrhage from arteriovenous fistula is much less commonly encountered. Inspection of the bodies at the scene of the death by a medical examiner suggested that the deaths were due to exsanguination. Autopsies revealed visible defects on the front wall of the arteriovenous fistulas. Microscopic examination showed wall necrosis with infiltration of various inflammatory cells. Deaths were due to exsanguination from the ulcerated arteriovenous fistulas in patients with chronic renal failure. Further investigation revealed that complications in the area around the arteriovenous fistulas were known and were being treated until a sudden rupture of the vessels and hemorrhage from the arteriovenous fistulas resulted in the deaths.     

Sudden and Unexpected Death in Three Cases of Ehlers‐Danlos Syndrome Type IV*

Abstract: Ehlers–Danlos syndrome (EDS) type IV is a connective tissue disorder characterized by the inability to produce sufficient amounts of collagen or a defect in the structure of collagen. The most serious complications include a rupture of a viscus or vascular rupture with or without mural dissection. Death may result from internal hemorrhage. This report describes three cases of sudden and unexpected death caused by EDS type IV. Two cases involved hemothorax as a result of dissection of the subclavian artery and aorta, respectively. The third case represented spontaneous pulmonary rupture and hemorrhage. A detailed family history should be sought, and additional specimens collected to confirm the diagnosis, including skin fibroblasts for collagen testing and blood for DNA testing. The forensic pathologist should consider the possibility of EDS type IV upon discovery of spontaneous visceral or arterial rupture and should alert the family members of this hereditary and potentially fatal condition.     

Previously Unreported Complication of Coronary Artery Bypass Grafting: Suture Rupture Resulting in Patient's Death

Traditionally, the manner of death in most hospital autopsy cases is natural, in which death is due to the natural course of disease or reasonably anticipated outcomes of medical interventions. Some cases fall into a potential gray zone between natural and accident, including rare or unanticipated outcomes of medical interventions. We present a case of a patient postcoronary artery bypass graft. Autopsy revealed the proximal anastomosis of the aorta‐to‐first‐diagonal‐coronary‐artery‐to‐second‐obtuse‐marginal‐artery graft was detached from the aorta. A broken suture was present at the disconnected anastomosis, with intact knots but was broken along its length. In‐hospital mortality rates of CABG range from 1% to 3%, with several autopsy studies identifying surgical complications as the cause of death in one‐third of perioperative deaths. No publications were found that described suture rupture as directly relating to the cause of death. This case report describes a previously unreported complication of coronary artery bypass grafting.     

Complete Cardiac Rupture Associated with Closed Chest Cardiac Massage: Case Report and Review of the Literature

Chest skeletal injuries are the most frequent complications of external chest massage (ECM) during cardiopulmonary resuscitation, but heart and great vessels lacerations that are indeed very rare. We report the case of a 35‐year‐old workman who collapsed and underwent ECM by his co‐workers for almost 30 min. At autopsy, no external injuries, fractures or bruises of the ribs or sternum, were observed. A hemopericardium with a rupture of the heart was found, with no signs of pre‐existent cardiac disease. Bruises of thoracic aortic wall, lung petechiae, a contusion of the liver, and bruises of lumbar muscles were found. The cause of death was due to sudden cardiac death with an extensive cardiac rupture. This is an unusual report of massive heart damage without any skeletal or muscle chest injuries, secondary to cardiopulmonary resuscitation. This kind of cardiac lesions may be considered when thoracic–abdominal trauma, or medical history, is unclear.     

An Autopsy Case of Sudden Unexplained Death Caused by Malaria*

Abstract: Sudden unexplained deaths, especially those unwitnessed can lead to forensic issues and would necessitate the need for a meticulous and complete postmortem examination including ancillary investigations to discover the cause of death. We herein report a case of sudden unexplained death caused by malaria in an apparently healthy individual. This fatal case is presented to remind the forensic pathologist of the possibility of malaria as a cause of sudden unexplained death in malaria‐endemic regions. In the present case, histopathological examination demonstrated the presence of parasitized red blood cells with malarial pigment in the blood capillaries in the brain, myocardium, pericardium, lungs, kidneys, liver, and the spleen. Cerebral malaria with acute renal insufficiency or pulmonary edema with an acute respiratory distress syndrome might have been the cause of death.     

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.     

Acute Aortic Dissection Diagnosed after Embalming: Macroscopic and Microscopic Findings

A 58‐year‐old man died suddenly in Madagascar and poisoning was suspected. The body was embalmed after death and the general state of preservation was good. We found a major aortic dissection with a large false lumen from the aortic root to the common iliac arteries and a hemopericardium with formalinized blood clot. The intimal tear was on the ascending aorta, and an intramural hemorrhage was noted at the right coronary artery, attesting to a retrograde dissection. Microscopic studies confirmed aortic dissection with extensive intramural hemorrhage and also confirmed the retrograde dissection to the right coronary artery with a reduction of 90% of the true lumen. Classically, aortic dissection occurs in individuals with hypertension and individuals with genetic disorders of collagen formation. The diagnosis is often first established at the postmortem examination. Aortic dissection is therefore dealt with largely in necropsy studies. The usual cause of death is rupture into the pericardial sac. One case of bloodless dissection has been reported but the sudden death was explained by acute myocardial ischemia secondary to dissection of the left coronary artery. In our case, we found major hemopericardium and also intramural hemorrhage at the right coronary artery. We were able to make the diagnosis of aortic dissection and exclude the suspicion of homicide 15 days after death and after embalming.     

Sudden death due to atlantoaxial subluxation in marfan syndrome

Marfan syndrome is 1 of the commonest inherited connective tissue disorders. Sudden death may occur and is usually attributed to cardiovascular manifestations of the syndrome. Atlantoaxial hypermobility, increased odontoid height, and rotatory subluxation are well described in this syndrome, but this paper details what seems to be the first reported case of sudden and unexpected death due to spontaneous atlantoaxial subluxation in Marfan syndrome.     

Ruptured Syphilitic Aneurysm: A Cause of Sudden Death in a Man with Human Immunodeficiency Coinfection

Syphilis, a sexually transmitted infection caused by the bacterium Treponema palladium, is experiencing a worldwide resurgence. The risk of syphilis infection is particularly high in men who have sex with men (MSM), especially those who are human immunodeficiency virus (HIV)‐positive. Untreated syphilis can lead to rare but severe late‐stage complications, including syphilitic aortitis. Herein, we present an autopsy case of a ruptured thoracic aneurysm that resulted from an undetected case of syphilitic aortitis in an HIV‐positive Japanese MSM with undiagnosed syphilis. Although no syphilitic skin lesions were observed on the body, anatomical changes consistent with a syphilitic etiology were present at the site of the rupture, including medial aortic scarring with “tree‐bark”‐like atherosclerotic plaque. In addition, heart blood was positive for T. palladium in a latex agglutination test. This case highlights for forensic pathologists the importance of recognizing syphilis as a possible underlying cause of sudden death among HIV‐positive MSM.     

Influenza A/H1N1 (2009) Infection as a Cause of Unexpected Out‐of‐Hospital Death in the Young*

Abstract: In March 2009, a new strain of influenza A/H1N1 virus was identified in Mexico, responsible for a pandemic. Worldwide, more than 13,500 patients died, most often from acute respiratory distress syndrome. Because sudden death cases were rare, involving mostly young apparently healthy persons, influenza A/H1N1 (2009)‐related deaths may be misdiagnosed, which can raise medico‐legal issues. Case history: we report on an unexpected out‐of‐hospital death involving a young male with no past medical history and no vaccination. Fever was his only symptom. Laboratory tests: histology showed patchy necrotic foci with mononuclear inflammation in the lungs. The heart was histologically normal, but virological analyses using molecular biology on frozen myocardial samples showed high virus load. In conclusion, this case report shows that influenza A/H1N1 (2009) virus can be a cause of sudden cardiac death in the young and demonstrates the importance of quantitative virological analyses for the diagnosis of myocarditis.     

Sudden Infant and Early Childhood Death and Sinus of Valsalva Pseudoaneurysms

Two cases of occult pseudoaneurysms of the sinus of Valsalva (SoV) are reported to demonstrate different etiologies and lethal mechanisms. A previously well 9‐month‐old boy who suffered an unexpected fatal cardiorespiratory arrest was found at autopsy to have a ruptured pseudoaneurysm of the left SoV resulting in lethal hemopericardium. A 13.5‐month‐old boy died soon after presenting with a 24‐h history of poor feeding and reduced urine output. At autopsy, destruction of the noncoronary cusp of the aortic valve by bacterial endocarditis was found with extension of the inflammation into a pseudoaneurysm of the SoV with surrounding abscess formation involving the atrioventricular node. Death was most likely due to an arrhythmia. Pseudoaneurysms of the SoV are exceedingly rare but may result in sudden/unexpected death in infancy and early childhood from rupture or involvement of the conduction system or myocardium. Detailed cardiac examination is essential in all unexpected deaths in early life.     

Differentiating cause-of-death terminology for deaths coded as sudden infant death syndrome, accidental suffocation, and unknown cause: an investigation using US death certificates, 2003-2004

We compared written text on infant death certificates for deaths coded as sudden infant death syndrome (R95), unknown cause (R99), and accidental suffocation (W75). Using US mortality files supplemented with the death certifiers' written text for all infant deaths with International Classification of Diseases (ICD)-10 assigned codes R95, R99, and W75, we formed cause-of-death subcategories from common themes identified from the written text. Among all infant deaths in 2003-2004, the underlying cause of death was listed as R99 for 2128 deaths, R95 for 4408 deaths, and W75 for 931 deaths. Among the postneonatal deaths, the differences in subcategories varied between assigned ICD-10 codes: for R99-coded deaths, 45.8% were categorized as "Unknown" and 48.6% as "Pending"; for R95-coded deaths, 67.7% were categorized as "sudden infant death syndrome (SIDS)"; and for W75-coded deaths, 76.4% were categorized as "Suffocation." Examination of the written text on the death certificates demonstrates variability in the assigned ICD-10 codes which could have an important effect on the estimates of SIDS cases in the United States.     

Manifestations and Medicolegal Significance of Loeys–Dietz Syndrome

Loeys–Dietz syndrome is a recently described autosomal dominant disorder with underlying vasculopathy characterized by aortic and other vascular aneurysmal dissection/rupture. A 61‐year‐old man is reported who died suddenly and unexpectedly and at autopsy was found to have a ruptured abdominal aortic aneurysm. Additional findings included dolichostenomelia, high‐arched palate, and pectus excavatum. There was a strong family history of Loeys–Dietz syndrome, although the decedent had never been tested. Death was, therefore, due to a ruptured abdominal aortic aneurysm in a case of probable Loeys–Dietz syndrome. Although Loeys–Dietz syndrome shares common characteristics with Marfan and other connective tissue syndromes, it is a distinct entity with a much more aggressive clinical course. Lethal events may not occur until later adult life; however, the identification of the syndrome and differentiation from other connective tissue disorders at autopsy is important so that genetic investigation of close relatives can be undertaken with prophylactic surgical treatment if necessary.     

KCNE、KCNQ1与心性猝死的相关性研究进展

部分心性猝死由于缺乏明确的病理学改变,其鉴定工作一直是法医工作者的一大难题。近年来,与长QT综合征、心房颤动等致死性心律失常疾病相关基因（KCNE基因家族与KCNQl）等研究逐渐增多。国内外研究发现KCNE和KCNQ1基因编码心肌钾离子通道,其基因异常可引起严重的心律失常,甚至导致心性猝死。因此,死后KCNE和KCNQl的基因检测对于心性猝死鉴定具有重要意义。本文对KCNE、KCNQl与心性猝死的相关性研究进展进行综述,希望能为法医学研究和实践提供参考。     

The Interplay Between Diabetes and Pancreatitis: Two Case Reports of Sudden,Natural Deaths and a Review of the Literature

Diabetes mellitus (DM) is a common disease involving insulin resistance or deficit that, when left unchecked, may cause severe hyperglycemia and subsequent end‐organ damage. Acute pancreatitis (AP) is inflammation of the pancreas that can lead to significant morbidity and mortality. AP and DM both account for a significant amount of sudden deaths, and rarely both disease processes may be present in the same decedent, causing some difficulty in wording the cause of death statement. Although much research has been directed at studying the causes and risk factors for AP and DM, there is a complex interplay between these diseases that is not fully understood. This study presents two autopsy cases of sudden, natural deaths that illustrate this interplay, along with a review of the literature. An algorithm for differentiating AP and DM is then discussed in the context of the presented cases as a proposed aid for forensic pathologists in the certification of such deaths.     

Natural,Unexpected Deaths: Reliability of a Presumptive Diagnosis*

Abstract: We retrospectively analyzed 100 deaths because of suspicions and concerns expressed by the family. We compared the preautopsy cause of death, as determined by a thorough review of the clinical data and circumstances, to the autopsy‐derived cause of death. In the majority (91/100), the preautopsy and postautopsy proximate causes of death were in agreement. In 9%, the autopsy provided information that resulted in a proximate cause of death different than anticipated. In four instances, the manner of death also was incorrect and was determined to be an accident rather than the originally presumed natural. No homicide or suicide would have been misclassified. In another nine instances, where the premortem and postmortem proximate causes of death were in agreement, the autopsy provided a specific mechanism of death. With a quality initial medicolegal death investigation, a subset of sudden deaths in adults may be reliably certified without an autopsy.