Sudden death due to left coronary artery occlusion in infective endocarditis

A 44-year-old man died suddenly, shortly after admission to the hospital with complaints of abdominal pain. Medical history was significant for chronic alcoholism and homozygous hemoglobin C disease. Autopsy revealed vegetations on the aortic valve, especially on the left coronary cusp. There was anomalous origin of the coronary arteries from the left sinus of Valsalva. The large vegetation on the left coronary cusp had extended into the left main-stem coronary artery and obstructed it. There was evidence of prior embolization to the right coronary artery with mycotic aneurysm formation and myocardial infarction. Other lesions included a cerebral artery mycotic aneurysm and metastatic abscesses within the myocardium and spleen. Although the aortic valve was free of underlying chronic pathology, the causative organism was Streptococcus viridans. This case illustrates several unusual, and, in some instances, unique findings in infective endocarditis.     

Sudden coronary death in children

Coronary disease is an uncommon cause of severe morbidity and death in the young. The great majority of lesions are congenital, and anomalous origins and courses of the coronary arteries are the most common groups of diseases in the age group. Intimal proliferation and coronary arteritis are even less common causes of death in this age population. For this review, we retrospectively studied cases of coronary cardiac death in children and adolescents, from 2004 to 2007, from a statewide medical examiner's office. There were 197 natural deaths in children ages ≤16 years and 58 deaths from sudden infant death syndrome. Of these, the largest group of deaths showed no morphologic abnormalities and was attributed to arrhythmias. The next largest group of deaths comprised complex congenital heart disease (n=13; 20%), followed by myocarditis (n=11; 17%), coronary disease (n=9; 14%), cardiomyopathy (n=8; 12%), valve disease (n=4; 6%), and cardiac fibroma (n=1). Nine coronary deaths occurred in seven boys and two girls, ranging from 14 days to 16 years of age (mean, 8 years). An anomalous course of the coronaries was seen in seven of the nine coronary deaths, and the other two casualties were examples of intimal proliferations involving the coronaries. The rare examples of coronary lesions in the young are discussed in this article.     

Spontaneous coronary artery dissection mimicking aortic dissection

A 51-year-old woman suffered rapidly irreversible cardiogenic shock with left hemiparesis. Transesophageal echocardiography, which represents an essential imaging tool in the emergency room, ruled out aortic dissection involving branch vessels but did not allow an in vivo diagnosis of spontaneous coronary dissection. The in vivo diagnosis of spontaneous coronary dissection is rather difficult because of the dramatic clinical presentation and selective coronary angiography requirement.     

Sudden death outside the hospital from coronary heart disease: the state of the intercoronary artery collaterals

By means of postmortem selective coronary angiography coronary collaterals of 55 sudden out-of-hospital coronary death victims were investigated. The collaterals were found in most (58,5%) sudden coronary death cases. There was no correlation between the presence of coronary collaterals, on the one hand, and ischaemic heart disease symptoms during the life and focal myocardial lesions in deceased persons, on the other. The investigation showed the maximal formation of collaterals in cases with asymmetrical obstructive lesions of the right and left coronary arteries. This is in agreement with a hypothesis claiming the coronary collateral formation to be dependent on the pressure gradient between individual coronary arteries.     

Sudden cardiac death caused by coronary vasculitis

Coronary vasculitis is a rare and diagnostically challenging cause of sudden cardiac death (SCD). There are currently no large-scale series on this rare entity. A retrospective non-case–control observational study of SCD with coronary vasculitis referred to a tertiary cardiac pathology referral centre at the National Heart and Lung Institute at the Royal Brompton Hospital between 1996 and 2010 was completed. Ten cases of SCD with coronary artery vasculitis were retrieved from a database of 1,980 SCD cases (0.5%) with a 1:1 male/female ratio; median age was 39 years and range 15–71 years. Six deaths occurred in hospital following symptoms or cardiac arrest in the community; the remaining died at rest at home (n = 4). Appearances ranged from aneurysms of the coronary artery to occlusive lesions mimicking atheroma or masses imitating tumour. Types of vasculitis detected were: eosinophilic (n = 5), two associated with Churg–Strauss syndrome; lymphoplasmacytic vasculitis (n = 4); and idiopathic giant cell arteritis (n = 1). This study shows coronary vasculitis as a rare cause of SCD with a variable macroscopic and microscopic presentation that pathologists need to be aware of.     

主动脉夹层动脉瘤合并肺动脉夹层动脉瘤猝死病例报道及文献回顾

目的：对罕见的主动脉及肺动脉同时发生的夹层动脉瘤进行报道并讨论其死亡原因。方法：分析1例因主动脉夹层动脉瘤合并肺动脉夹层动脉瘤猝死病例的解剖所见、组织学特征,结合文献分析讨论。结果：死者男性,76岁,因外力打击后猝死于家中。解剖所见主动脉、肺动脉夹层动脉瘤形成,肺动脉各级分支夹层动脉瘤形成伴动脉管腔狭窄。组织学证实大动脉动脉粥样硬化。结论：主动脉合并肺动脉夹层动脉瘤非常罕见,病情凶险,易引起猝死。     

Sudden cardiac death.

The rate of cardiac deaths that are sudden is approximately 50%, and decreases with age. The causes of sudden cardiac death are diverse, and are a function of age. In children and adolescents, coronary anomalies, hypertrophic cardiomyopathy and myocarditis are frequent substrates for lethal arrhythmias; in adults, coronary atherosclerosis and acquired forms of cardiomyopathy are the most common findings at autopsies of sudden cardiac death. This review focuses on coronary causes of sudden cardiac death, especially congenital coronary artery anomalies, which result in sudden death almost exclusively in adults younger than age 35, and coronary thrombosis. The most lethal coronary artery anomaly is the left coronary artery arising from the right sinus of Valsalva; this anomaly often results in fatal arrhythmias, often with exercise. The right coronary artery arising from the left sinus of Valsalva may also be lethal in adolescents and young adults, but, unlike the anomalous left, is more often an incidental finding at autopsy. Approximately 60% of sudden coronary death is caused by coronary thrombosis, the rest die with severe coronary disease in the absence of thrombosis. The two major substrates of coronary thrombosis are plaque rupture and plaque erosion, and are not only different pathologically, but are seen in patients with divergent risk factor profiles. Plaque rupture is the most common cause of fatal coronary thrombus, and is characterized by necrotic core with a thin fibrous cap, infiltrated by macrophages. The factors that result in plaque instability and rupture are largely unknown, and are under intense scrutiny; morphologic studies have identified serum lipid abnormalities as a key risk factor in the development of plaque rupture. Plaque erosion, in contrast to plaque rupture, is seen in younger men and women, is not associated with lipid abnormalities, and does not result from exposure of the lipid core to the lumen. The heterogeneity of the atherosclerotic plaque and the diverse mechanics of plaque progression and thrombosis have only been relatively recently explored, and are largely elucidated by autopsy studies of victims of sudden coronary death.     

Sudden cardiac death not related to coronary atherosclerosis

Sudden cardiac death (SCD) accounts for approximately 300,000 cardiac events in the United States each year, representing an overall incidence of 0.1-0.2% per year. Although the vast majority of these may be attributed to coronary atherosclerosis, a wide variety of nonatherosclerotic-related cardiac diseases have been associated with SCD. This review highlights three general categories of cardiac disease not related to atherosclerosis: the cardiomyopathies, inflammatory myocardial diseases, and ion channel disorders. The important role played by genetics in some of these cardiovascular diseases is presented as well as toxic and drug-related etiologies.     

Sudden coronary death due to IgG4-related disease

A 54-year-old male entered the emergency room in cardiorespiratory arrest after syncope at home. Resuscitation was attempted, but the patient died a few hours later. At necropsy, aneurysms were found at the right and left anterior descending coronary arteries. At microscopic examination, there was no significant coronary atherosclerosis, and a dense inflammatory infiltrate was detected, with a high number of igG4-positive cells (94.0 positive cells/hpf). The case illustrates that IgG4-related disease can cause coronary disease and sudden cardiac death.