Non-atherosclerotic coronary pathology causing sudden death

Coronary atherosclerosis is responsible for >50% of all cases of sudden death and for 90% of sudden coronary death. Four cases encountered in routine autopsy evaluation at our institute in 2004 in which non-atherosclerotic coronary pathology was responsible for sudden cardiac death are reported. The cases of a 31-year-old man with epicardial coronary arteritis, a 57-year-old man with intramyocardial vasculitis, a 45-year-old woman with spontaneous coronary dissection and a 50-year-old man with vascular fibrosis are described. Searching for non-atherosclerotic coronary disease is relevant for both the clinician and the pathologist to prevent coronary causes of sudden death going unrecognised.     

Periarteritis of coronary arteries with severe eosinophilic infiltration. A new pathologic entity (eosinophilic periarteritis)?

A 40 year-old male presented symptoms of angina pectoris for about nine years and expired with symptoms of unstable angina, changing pattern at the terminal stage. At autopsy, both right and left coronary arteries of the subepicardial region were grayish white and elastic hard. Histologically, inflammatory infiltration was localized in adventitia of coronary arteries located in the subepicardial region. Inflammatory cells infiltrated into the adventitia were mostly eosinophiles. The medial smooth muscle cells were well preserved and the intima showed irregular thickening with fibrosis. Vascular obstruction or recanalization could not be observed. As a result of these findings, it was considered that these inflammatory changes of the coronary arteries could be termed eosinophilic periarteritis. These inflammatory changes could not be found in the intramural coronary arteries. Rather extensive fibrosis could be seen in the muscle layer centering about the posterior wall of the left ventricle. No findings of angiitis could be detected in the blood vessels except subepicardial coronary arteries.     

Kawasaki disease. Relationship with infantile periarteritis nodosa.

Until 1972, Kawasaki disease, or acute febrile infantile mucocutaneous lymph node syndrome (MCLS), was considered in Japan to be a nonfatal disease with a favorable prognosis. Based on the findings from two autopsy cases of MCLS, we believe that sudden and unexpected death during convalescence may be due to arterial lesions, especially those involving the coronary artery, that resemble those of periarteritis nodosa. Of 29 autopsy cases of MCLS, gathered from all over Japan, all exhibited arteritis lesions (eg, aneurysm due to coronary arteritis). Such vascular pathological findings may in fact represent the same entity as infantile periarteritis nodosa. Whether infantile periarteritis nodosa can be identified with the adult type is still debatable. The particular manifestations of infantile periarteritis nodosa might be related to severe vasculitis. Autopsy cases of infantile periarteritis nodosa without MCLS manifestations are being collected and studied.     

右冠状动脉起源于左主干5例临床分析

目的：分析右冠状动脉起源于左主干的临床特征，探讨其与心肌缺血的关系。方法：回顾分析5例右冠状动脉起源于左主干的临床资料及相关文献。结果：（1）本组5例患者中，女性1例，男性4例，年龄27～70岁。（2）临床表现主要为胸痛、胸闷等心绞痛症状，除1例老年患者外，其余4例病人都有典型的心绞痛症状和缺血心电图变化。（3）冠状动脉造影示3例合并严重冠状动脉粥样硬化，给予冠状动脉搭桥术，术后随访2月～6年无明显不适；另2例冠状动脉无明显病变，药物治疗后仍有劳累性心绞痛发作，其中1例半年后猝死。结论：右冠状动脉起源于左主干是一种罕见的冠脉畸形，对存在严重心肌缺血的患者应进行预防性冠状动脉搭桥术或介入治疗，预防不良事件的发生。     

Perivascular nerve lesion of the coronary artery involved in spasm in a patient with variant angina

An autopsy case of a 52-year-old man with typical variant angina is reported. He had recurrent attacks of chest pain at rest, particularly from midnight to early morning, associated with ST segment elevation in the electrocardiogram. At autopsy, degenerative changes and fibrosis were confirmed light microscopically in the perivascular nerves of the coronary artery involved in spasm. This finding indicates an intimate relationship between coronary spasm and the autonomic nervous system.     

Total anomalous origin of the coronary circulation from the right pulmonary artery

Origin of both coronary arteries from a branch of the pulmonary artery is rare and has not been reported as a cause of sudden unexpected death. We report autopsy findings of a 14-day male infant in previously good health who died suddenly. From the proximal right pulmonary artery arose a single coronary artery, which branched into the right and left main coronary arteries. The right proximal coronary artery coursed between the aorta and pulmonary trunk, and the left main traveled along the anterior proximal aorta. The distal course of the coronary circulation was normal. There was concentric subendocardial healing myocardial infarction. No other congenital anomalies were identified. Total anomalous origin of the coronary arteries from the pulmonary trunk or artery should be considered in cases of sudden unexpected death in infants.     

Temporal arteritis with giant cell aortitis, coronary arteritis, and myocardial infarction

Extracranial giant cell arteritis occurs in 10% to 15% of patients with temporal arteritis and polymyalgia rheumatica. Aorta and its major branches are most often involved and death may result from an unsuspected ruptured aortic aneurysm or aortic dissection. Involvement of coronary arteries by giant cell arteritis resulting in death from myocardial infarction is extremely rare. This article describes one such case: an 84-year-old man who died of acute myocardial infarction with the unexpected autopsy finding of giant cell aortitis and coronary arteritis, and who, three years earlier, had sudden onset of bilateral blindness and biopsy-proven temporal arteritis.     

Dissection of the aorta and coronary arteries associated with acute cocaine intoxication.

Acute myocardial ischemia, cardiac arrhythmias, and conduction disturbances are the most common cardiovascular complications of cocaine and its alkaloidal freebase "crack." Cocaine abuse-related acute aortic dissection has been reported only rarely: three cases in the last 5 years. Described herein, to our knowledge, is the first case of cocaine intoxication-associated acute aortic dissection that also involved the coronary arteries, causing sudden death.     

Multiple spontaneous coronary artery dissections in association with anomalous origin of right coronary and intramural coronary artery dysplasia.

We present a case of sudden death due to spontaneous acute coronary artery dissection. In addition, there was a healing spontaneous coronary dissection, intramural coronary artery dysplasia, and an anomalous origin of the right coronary artery from the pulmonary trunk. The coincidence of multiple spontaneous coronary dissections, coronary arterial dysplasia, and anomalous origin of the right coronary artery is unique.     

Anomalous coronary arteries arising from the aorta associated with sudden death in infancy and early childhood. An autopsy series

Anomalous coronary arteries arising from the aorta are a recognized cause of myocardial ischemia and sudden death. Death has been precipitated by exercise in most cases. We present the results of an autopsy study in which sudden and unexpected deaths associated with coronary anomalies were found in three children (less than 2 years of age). In two cases, death was not associated with exercise. In two case, the myocardium was morphologically normal, and in the third case, there was an extensive recent anterior myocardial infarct with a background of established fibrosis. Coronary artery anomalies may be easily overlooked in this age group because of small vessel size and difficulty in dissection; this is particularly so when there are normally placed ostia. One consequence of this is potential for confusion with sudden infant death syndrome.     

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a forgotten congenital cause of sudden death in the adult

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a very rare often fatal congenital coronary artery anomaly that occurs in approximately only 1 of 300,000 live births. Many cardiologists and pathologists may be unaware that cardiac ischemia and sudden death can also occur due to ALCAPA in adults in whom it is even rarer and thus may be missed at autopsy. The adult form may range clinically from asymptomatic to sudden cardiac death. We present four cases of adult ALCAPA that illustrate the variable presentations and survival. Two had died at rest and were previously asymptomatic. The other two died during exertion with history of recent chest pains in one and previous myocardial infarction in childhood in the other. This last case remained asymptomatic until death. These four cases highlight that surgery should be undertaken in all cases, even in those who are asymptomatic as newer imaging makes the diagnosis in life more frequent. Age is not important as cases vary widely in age from 22 to 68 in our small group. Furthermore, the most common site of left coronary artery origin is the left pulmonary sinus but our autopsy study shows that the origin can be variable extending above the pulmonary valve sinus to the main pulmonary artery.     

Accuracy of magnetic resonance imaging in determining cause of sudden death in adults: comparison with conventional autopsy

AIM: To determine the accuracy and define the limitations of post mortem magnetic resonance imaging (MRI) in determining the cause of sudden death in adults. METHODS AND RESULTS: Sudden unexpected adult deaths in the community, reported to the Coroner (n = 10), excluding suspicious, violent or potentially drug-related deaths, were submitted to whole body MRI, followed by full invasive autopsy. The MRI scans were reported independently by four radiologists, blinded to the autopsy findings; two had previous experience of post mortem MRI. An abnormality that related to the cause of death as identified at autopsy, was identified by at least one radiologist in eight cases. These were pulmonary consolidation (autopsy finding pneumonia) (n = 1), pneumoperitoneum (autopsy finding perforated peptic ulcer) (n = 2), left ventricular failure (autopsy finding ischaemic heart disease) (n = 4), and disseminated bronchial carcinoma (n = 1). However, in only one case were all radiologists able to provide a confident cause of death (disseminated bronchial carcinoma). In two cases, in which death occurred 2-6 days and 3-6 days before MRI, early decomposition prevented interpretation of the images. Severe coronary artery atheroma was detected at autopsy in 7/10, but these lesions were not detected by MRI. Previous experience in reporting post mortem MRI, without autopsy comparison, did not result in more accurate interpretation of the images. CONCLUSIONS: This pilot study suggests that post mortem MRI can identify some abnormalities relating to the common causes of sudden death in adults, but there is a need for greater experience in correlating MRI with autopsy findings before a reliable cause of death can be made by MRI alone. Inability to image coronary artery lesions, differentiating thrombus from clot and pulmonary oedema from pneumonic exudates, are specific problems that may be corrected with greater experience and higher resolution scans.     

Characteristics and prognosis of IgG4-related periaortitis/periarteritis: A systematic literature review

ObjectiveImmunoglobulin G4 (IgG4)-related disease is a systemic chronic fibroinflammatory disease that can affect almost every organ of the body. IgG4-related periaortitis/periarteritis is a newly recognized subset of IgG4-related disease, and its characteristics and prognosis remain unclear. We investigated the clinical characteristics and prognosis of IgG4-related periaortitis/periarteritis.MethodsWe performed a systematic literature review of IgG4-related periaortitis/periarteritis. Additionally, we have summarized the characteristics and prognosis of IgG4-related coronary arteritis.ResultsWe investigated 248 patients with IgG4-related periaortitis/periarteritis. All studies reported the condition in elderly patients, and male predominance was observed. The infra-renal abdominal aorta and iliac arteries were the most commonly affected sites. Most reports showed the serum C-reactive protein elevation in this disease entity, in contrast to non-vascular IgG4-related disease. Based on radiological findings observed in 27 patients with IgG4-related coronary arteritis, vasculitic lesions were classified into 3 types: stenotic (67% of patients), aneurysmal (42%), and diffuse wall thickening type (92%). Serum IgG4 level, but not C-reactive protein level, was associated with the number of affected organs in IgG4-related coronary arteritis. Corticosteroid treatment with or without cardiac surgery or percutaneous coronary intervention was effective in most patients with IgG4-related coronary arteritis; however, 33% of patients showed an unfavorable clinical course including disease progression, relapse, or death. Pre-treatment stenosis and/or aneurysms were associated with progression of stenosis or aneurysm after corticosteroid treatment.ConclusionMost clinical characteristics were similar between the IgG4-related periaortitis/periarteritis and the non-vascular IgG4-related disease groups; however, serum C-reactive protein level elevation was observed only in the former. Although corticosteroid treatment was effective, this disease can be life-threatening secondary to myocardial infarction, aortic dissection, and aneurysmal rupture. Pre-treatment evaluation of stenosis or aneurysms is important for predicting progression of stenosis or aneurysm after corticosteroid treatment.     

Spontaneous coronary artery dissection presenting as acute myocardial infarction

Spontaneous coronary artery dissection is a rare entity being increasingly diagnosed as a cause of acute myocardial infarction, especially in cases of low cardiac risk female patients. This is one such case report of a black female patient, who suffered an acute anterior wall myocardial infarction due to an idiopathic spontaneous coronary artery dissection of the left anterior descending artery. She was treated with a thrombolytic agent in the acute phase, uneventfully. An urgent coronary angiogram demonstrated an intimal tear with a dissection of the left anterior descending artery. She survived the acute event and her subsequent hospital course was uncomplicated. Hence she was treated medically for her ischemic event and left ventricular systolic dysfunction with a favorable outcome. This case is yet another report of a survivor treated with a thrombolytic agent for the acute myocardial infarction due to spontaneous coronary artery dissection.     

Hypoplastic coronary artery disease causing sudden death. Report of two cases and review of the literature

Congenital coronary anomalies represent a condition often unrecognized in the living and in the dead. Investigating this condition is relevant for both clinicians and pathologists in order to identify potentially unrecognized coronary causes of sudden death.Hypoplastic coronary artery disease (HCAD) is a rare congenital abnormality reported to be associated with sudden death.We report two additional cases of previously apparently healthy people who died suddenly and were found to have HCAD at postmortem evaluation. The clinicopathologic findings are discussed along with a review of the literature.     

DISSECTING ANEURYSMS OF THE CORONARY ARTERIES

This paper reports two cases of coronary dissecting aneurysms of the heart. These were found among 45 consecutive autopsy cases with cardiac diseases from January, 1978 to April, 1981. The first case, a 60-year-old woman, revealed dissecting aneurysm involving the aorta and three main coronary arteries in association with cystic medial necrosis. The second case, a 55-year-old man, revealed dissecting hematoma limited to the right coronary artery without any presumptive causal lesion to the dissection. The dissections could not be identified by either clinically or even macroscopic examinations of the autopsy materials.
It is likely that the dissecting coronary aneurysm is not so uncommon nor fatal lesion as previously thought. Considerable number of the cases would be concealed in those of sudden death not autopsied or carelessly autopsied, or in those survived myocardial infarction not angiographied.     

Fatal coronary artery intimal hyperplasia due to amphetamine use

BackgroundAmphetamines have been reported to cause myocardial infarct, cerebral hemorrhage, aortic dissection, hypertension, vasculitis, aneurysms, and, occasionally, death from direct toxicity. To date, there have been no reports of coronary intimal hyperplasia in an amphetamine user.MethodsA 29-year-old male was found collapsed after jogging. He had been taking amphetamines for 11 years for attention deficit/hyperactivity disorder. Past medical history was positive for mild hypertension and migraine headaches. He underwent a complete autopsy and drug screen.ResultsAt gross autopsy his left anterior descending (LAD) coronary artery was occluded and he had an old myocardial infarct in the anterior wall of the left ventricle. Microscopic findings included occlusive intimal hyperplasia (positive for smooth muscle actin) in the LAD and an old left ventricular anterior wall infarct surrounded by acute infarct.ConclusionIn addition to other reported adverse effects, amphetamines can also cause occlusive intimal hyperplasia of the coronary arteries.     

C-reactive protein in vulnerable coronary plaques

BACKGROUND: An increased level of serum C-reactive protein (CRP) is a known prognostic factor for acute coronary events and sudden cardiac death, and it is associated with coronary calcification. CRP is expressed in coronary arteries, but its role in the development of coronary plaques is unclear. AIM: To investigate CRP immunoreactivity in relation to the severity of coronary artery disease and plaque morphology in human left anterior descending coronary arteries (LAD). METHODS: A prospective, consecutive autopsy series of 66 patients (mean age 63.4 years) in Tampere University Hospital, Tampere, Finland. RESULTS: CRP immunoreactivity was seen in 59% of the cases. In logistic regression analysis with age, sex and body mass index as confounders, CRP immunoreactivity in LAD was associated with >50% stenosis and plaque calcification. All three cases with acute coronary thrombosis due to rupture or erosion of the plaque showed a clear immunopositive reaction. CRP-positive cells were never detected in normal arteries, but were often found in early fibrous plaques (75%) and almost invariably present in the shoulder area of plaques with necrotic core (96%). CRP immunoreactivity adjacent to calcified areas in more stable plaques (71%) was less consistent with one-third of these plaques showing no immunoreactivity. CONCLUSIONS: CRP immunoreactivity is associated with the progression of atherosclerosis, and especially with unstable coronary plaques. The immunoreactivity could cease at the stable calcified stages of atherosclerosis.     

Risk factors for sudden and non-sudden coronary death

The impact of risk factors for sudden and non-sudden coronary death was investigated in 3,589 Finnish men aged 40-59 years at entry from a prospective population survey. During a mean follow-up time of 11 years, 234 coronary deaths occurred, 150 of which were sudden, i.e. ensuing within 1 hour of the onset of symptoms. The severity of the manifestations of CHD at baseline investigation appeared to be a powerful predictor of sudden coronary death. Smoking and high serum cholesterol were significant predictors of sudden coronary death. High serum cholesterol was an equally significant predictor of sudden and non-sudden coronary death. High blood pressure did not appear to significantly increase sudden coronary death but increased the incidence of non-sudden death significantly. Obesity and diabetes did not appear to be independent risk factors for sudden coronary death. Smoking and high serum cholesterol were significant risk factors for sudden coronary death in men with manifestations of coronary heart disease. The results suggest that reduction of primary risk factors, especially smoking and high serum cholesterol, is important even after coronary heart disease has become manifest.     

Maximizing the effectiveness of the autopsy in cases of sudden death

Autopsies are essential in the investigation of sudden unexpected deaths. To maximize the effectiveness of the autopsy in these cases, the pathologist must assess the circumstances leading to the person's death, should be knowledgeable of the diseases most often responsible for sudden death, and should use dissection techniques most appropriate for their disclosure. In adults, the great majority of sudden deaths are cardiogenic and related to diseases of the coronary arteries, cardiac valves, or myocardium. In infants and young children, however, extracardiac diseases are prevalent in the causation of sudden deaths. Approximately one third of these are inexplicable (sudden infant death syndrome).