Dissecting intramural haematoma of the coronary artery with intimal tears.

A dissecting intramural haematoma of the coronary artery is an infrequent cause of sudden and unexpected death. Most cases are women and in a significant number of these women, the haematoma occurred at term to 80 days postpartum. Because the intimal rupture and communication with the lumen were not found in most of the cases, the site of rupture has been considered to be a vasa vasorum. The authors report on a case of one woman who was at a later postpartum period (106 days). The microscopic finding of the dissected coronary artery revealed two intimal tears in a section. The case showed that the dissecting intramural haematoma of the coronary artery is caused not only by the rupture of the vasa vasorum but also by the rupture of the intima.     

Sudden unexpected death due to papillary fibroma of the aortic valve. Report of a case and review of the literature

The case of the sudden unexpected death of a 21-year-old man due to embolization of segments of an aortic valve papillary fibroma to the left main and anterior descending coronary artery is presented. The literature regarding cardiac papillary fibroma is reviewed with particular reference to those cases associated with sudden death.     

Dissecting coronary artery aneurysm: a report of two cases

Two cases of sudden, unexpected death resulting from coronary artery dissection have been reported. Since sudden and unexpected death falls within medical examiners' jurisdiction, the systematic autopsy examination of such cases offers an opportunity to evaluate this entity as well as other rare causes of natural death. A review of the literature concerning the subject has been presented.     

"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.     

Sudden unexpected death due to atherosclerotic coronary artery aneurysm

The case of a 34-year-old white male who died suddenly and unexpectedly of a thrombosed coronary artery aneurysm is presented. Coronary artery aneurysms have a prevalence of approximately 2%; over half of these are caused by atherosclerosis with the remainder due to syphilis, infections, trauma, congenital malformations, vasculitis, neoplasms, and connective tissue disorders (Ehlers-Danlos and Marfan's syndromes). Replacement of the media by atherosclerotic debris is the cause of atherosclerotic aneurysms. Complications include rupture and thrombosis. Sudden death has been reported in eight other cases of atherosclerotic aneurysms; sudden unexpected death as the presenting symptom of atherosclerotic coronary artery aneurysm, as in our case, is rare.     

Circumflex artery from the right coronary sinus: a not-so-benign anomaly

The origin of the circumflex coronary artery from the right sinus of Valsalva is generally considered a benign anomaly. Herein we report the sudden unexpected death of a 6-year-old boy who died after jumping into a swimming pool and whose only pathologic finding was this usually inconsequential coronary artery anomaly. A discussion of the pathophysiology of the disorder and a literature review are included. This is the youngest patient to die with this entity.     

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.     

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.     

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 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.     

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.     

Aortic Dissection and Sudden Unexpected Deaths: A Retrospective Study of 31 Forensic Autopsy Cases

Acute aortic dissection (AAD) is the most common cause of sudden unexpected death related to aortic diseases. A retrospective study of 31 sudden unexpected deaths caused by AAD was conducted at Xi'an Jiaotong University Forensic Center from 2001 to 2012. We summarized the forensic characteristics of AAD and assessed the clinically diagnostic accuracy of AAD. The characteristics of sudden unexpected death due to AAD were male predominant (male: female = 6.7:1), relatively young with the mean age of 44, and predominance of type A dissection (77.4%). Cardiac tamponade was the most frequent cause of sudden death (87.1%). Of the 31 cases, 26 (83.9%) patients were not recognized clinically and were misdiagnosed with acute myocardial infarction, coronary artery disease, cholecystitis, acute gastroenteritis, renal/urinary lithiasis, or acute pancreatitis. In summary, AAD can be difficult to recognize, diagnosis is therefore sometimes delayed or missed. The medicolegal death investigation can help physicians have a better understanding of AAD.     

Sudden death due to unsuspected coronary vasculitis.

Coronary artery vasculitis is a well-recognized complication of polyarteritis nodosa and is occasionally seen in other forms of systemic vasculitis. However, involvement of the major epicardial coronary arteries leading to myocardial infarction and death is uncommon. Isolated coronary arteritis is even more rare. We report three cases of sudden death due to myocardial ischemia associated with arteritis of the major coronary arteries. All three decedents were previously healthy young to middle-aged men who had died suddenly after complaints of chest pain and shortness of breath. The autopsy findings and differential diagnoses are presented. Such cases are of particular interest to the medical examiner because of the sudden, unexpected nature of the deaths. An approach to the correct diagnosis is discussed.     

Is right coronary artery hypoplasia and sudden death an underdiagnosed association?

Determining whether hypoplasia of a coronary artery has caused or contributed to death is often complicated by an absence of histologic evidence of myocardial ischemia in the area of the heart supplied by the affected artery and also by the lack of data for assessing coronary artery size at autopsy. A 45-year-old woman is reported who collapsed and died and who was found at autopsy to have a dominant, small-caliber, right coronary artery, with acute and chronic ischemic changes in the posterior interventricular septum supplied by the diminutive vessel. This case provides evidence that small-caliber coronary arteries may be associated with a lethal outcome. Given the difficulties that may occur in determining whether there is a causal link between small coronary artery caliber and death, it is possible that this may be an underdiagnosed cause of sudden cardiac death, rather than a coincidental finding of minimal significance.     

Littre hernia--a rare cause of unexpected death in the elderly

A rare case of sudden and unexpected death is reported in an 87-year-old woman who was found dead at her home. At autopsy, the most striking finding was of a right-sided direct inguinal hernia containing a 20 mm infarcted Meckel diverticulum, with proximal small intestinal obstruction. Significant stenosing atherosclerosis was present in all three major epicardial coronary arteries, but with no histological evidence of acute or chronic ischemic myocardial damage. Death was attributed to small intestinal obstruction due to direct inguinal herniation (and infarction) of a Meckel diverticulum (a Littre hernia), complicating ischemic heart disease. Although Littre hernias are not a reported cause of sudden death in the elderly, congenital gastrointestinal anomalies may rarely play a significant role in terminal episodes well beyond childhood years. The autopsy assessment of sudden death at any age involves detailed examination of the entire length of the intestinal tract.     

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.     

Sudden Cardiac Death in a Young Man with Migraine-associated Arrhythmia

A 31-year-old man with migraine-induced syncope and bradycardia with subsequent pacemaker implantation died unexpectedly. Clinically unsuspected cardiac anomalies were found at autopsy including myocardial bridging of the left anterior descending artery and shelf-like coronary artery ostia. Nortriptyline was identified by toxicologic analysis. A review of the autopsy findings, the historical information, and the effects of the possible arrhythmogenic circumstances is undertaken and the potential contributions to the death are discussed. Cardiac arrhythmias have been documented during migraines. Coronary artery bridging has been known to lead to ischemia and infarction, ventricular tachycardia, and sudden death; however, these are very rare sequelae. Congenital coronary artery anomalies have been linked to sudden cardiac death, but only rarely cause death in people younger than 31 years. Migraines and the autopsy findings described have been associated with cardiac arrhythmia and sudden death. Altogether, they led to the unexpected death of this young man.     

Poor results in attempting to demonstrate the cause of death by examination of the conduction system of the heart in cases of sudden death

A microscopic examination of the proximal part of the conduction system in the heart was undertaken in seven cases of sudden death due to coronary arteriosclerosis; the same technique was applied to 31 control cases. A few infiltrations of mononuclear cells were demonstrated in relation to the conduction system in cases of sudden death as well as in controls. In one case severe narrowing of the sinus node artery was found without particular arteriosclerosis of the coronary arteries.     

Spontaneous dissection of the distal obtuse marginal coronary artery: a rare cause of sudden death

Spontaneous coronary artery dissection is a rare cause of acute myocardial infarction and sudden death. It typically, but not always, occurs in healthy postpartum women without traditional risk factors for atherosclerosis. Moreover, the site of dissection usually involves the proximal, major coronary arteries: left main coronary artery and/or the left anterior descending artery, and in men, more often the right coronary artery. We report a case of sudden death caused by dissection of the obtuse marginal branch of the left circumflex artery, in a 49-year-old man, a very rare site of fatal coronary dissection.