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.     

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.     

Anomalous origin of the right coronary artery from the left sinus of Valsalva: report of two cases.

Anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva (LSV) is rare and has only recently been recognized as a cause of sudden cardiac death in otherwise healthy individuals. In this report, we describe two cases of anomalous origin of the RCA from the LSV. In the first case, a previously healthy 11-year-old girl died suddenly and unexpectedly. An autopsy revealed no abnormalities, except for anomalous origin of the RCA from the LSV. In the second case, a 21-year-old woman died by drowning in a bath, probably after an episode of syncope brought about by anomalous origin of the RCA from the LSV.     

Sudden death due to anomalous right coronary artery in a 26-year-old marathon runner

Sudden death due to an anomalous right coronary artery is reported. Once considered a normal variant, there is now evidence that origin of the right coronary artery from the left sinus of Valsalva may cause morbidity and mortality. Salient features of the condition are discussed.     

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.     

Two consecutive fatal cases of acute myocardial infarction caused by free floating thrombus in the ascending aorta and review of literature

Free floating thrombus in the ascending aorta is an uncommon source of acute myocardial infarction. We report on two cases of young women who died of acute myocardial infarction caused by a free floating thrombus in the sinus of Valsalva obstructing the coronary arteries' ostia. The first case reports on a 30-year-old pregnant woman who anamnestically had episodes with short loss of consciousness and weakness. The second case presents a 37-year-old woman suffering from multiple sclerosis with no previous history of thrombotic events. The review of literature revealed a predominance of women (eight females and three males). Interestingly, the coronary arteries bear no preference concerning the right (RCA) or left coronary artery (LCA) being more often occluded by a free floating thrombus. Especially, younger women (mean age 45.5 years, range 30-59 years) with no history of cardiac symptoms and without atherosclerotic changes seem to be predispositioned. The hypothesis that thrombus formation in cases without plaque disruption may depend on an endothelial erosion which seems to be more common in younger women and promoted by a hyperthrombogenic state is supported by our two cases. A comprehensive literature search revealed, that these are the first two reports on a free floating thrombus being the cause of fatal acute myocardial infarction in a pregnant woman, respectively, a woman suffering from multiple sclerosis.     

Fatal paradoxical air embolism diagnosed by postmortem imaging and autopsy

The recognition and visualization of an arterial gas embolism are difficult. We report a case of sudden death caused by paradoxical air embolism of coronary and cerebral arteries, diagnosed by the pre autopsy computed tomography (CT) scanning. A 54-year-old woman suddenly died after the self-removal of the jugular vein catheter. Postmortem imaging examination using CT scanning showed multiple gas embolisms in the cerebral arteries, pulmonary artery, right atrium and ventricle, left ventricle, aorta, and coronary arteries. These findings suggested that the occurrence of acute ischemia of the brain and heart caused by massive air inflow to the artery. Conventional autopsy revealed a patent foramen ovale of the heart. These results indicated that the patient died of paradoxical air embolization of the coronary and cerebral arteries through a patent foramen ovale because of right-to-left shunting. The use of postmortem imaging as an aid for conventional autopsy has proved to be of advantage in the case of gas embolism.     

Supravalvular aortic stenosis, Williams syndrome and sudden death. A case report

Supravalvular aortic stenosis (SVAS) is an uncommon but well characterized congenital narrowing of the ascending aorta above the level of the coronary arteries. It can be a familial disorder, can occur sporadically, or associated with Williams syndrome (WS) which is a neurodevelopmental disorder affecting connective tissue and the central nervous system. Sudden death is a well-known complication of non-syndromic SVAS but few cases have been reported associated with WS. We present a case of sudden death in a woman with the diagnosis of SVAS and WS since the age of 3 years who refused surgical correction and died at the age of 27 years. At autopsy, the aorta and pulmonary trunk were narrowed and the walls showed peculiar microscopical characteristics. In the cardiac conduction system the His bundle was small and intramyocardial. The incidence, pathology, pathogenesis and prognosis of both conditions (SVAS and WS) are reviewed.     

Sudden unexpected death following exercise and congenital anomalies of coronary arteries. A report of two cases

Two cases of anomalous origin of the left coronary artery from the pulmonary trunk with unusually prolonged post-childhood survival are presented. In both cases, death was associated with physical exertion. The pathological findings included enlarged right coronary ostium, myocardial hypertrophy, endocardial fibrosis, and focal myocardial scarring.     

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.     

Association of right coronary artery hypoplasia with sudden death in an eleven-year-old child

Congenital coronary artery abnormalities are a rare but well-documented cause of sudden and unexpected death in the pediatric age group. Most reported cases involve both an aberrant origin and course of the abnormal vessel. A case of unexpected death occurring in an otherwise healthy eleven-year-old boy, who had been previously investigated for sudden collapse, is described. The major finding at autopsy was marked disparity in diameter between the coronary arteries due to diffuse hypoplasia of the right coronary artery. This case demonstrates the importance of meticulous examination of the coronary artery system in cases of sudden death in childhood so that significant reduction in luminal cross section will be adequately documented. The possible role played by this finding in the etiology of sudden death is discussed.     

Total anomalous pulmonary venous drainage and sudden death in infancy.

The clinicopathologic features of four infants with undiagnosed total anomalous pulmonary venous return who died suddenly and unexpectedly are presented. Two infants were found dead in their beds and two collapsed following very non-specific episodes of mild tachypnoea and tachycardia, respectively. Pulmonary venous blood drained via a common channel to the left innominate vein, the superior vena cava, the inferior vena cava at the junction of the hepatic vein and the supradiaphragmatic portion of the inferior vena cava. All cases demonstrated right atrial and ventricular hypertrophy and in one case there was associated hypoplastic left heart syndrome. Although those mechanisms responsible for sudden infant death syndrome could not definitely be excluded in two cases, the presence of anomalous pulmonary drainage with significant right-sided cardiac cardiac hypertrophy in each patient strongly suggests a contributing role for the vascular malformation in the aetiology of sudden unexpected death. The importance of careful in situ dissection of the major vessels in all cases of paediatric sudden death is emphasised, particularly in the presence of other congenital cardiac anomalies.     

Occlusion of left and right coronary arteries and coronary sinus following blunt chest trauma

Blunt chest trauma from rapid automobile airbag deployment causing coronary artery occlusion and myocardial infarction is a rare but potentially fatal condition. We present the case of a 37-year-old man who developed extensive anterior and inferior myocardial infarction because of occlusion of both left anterior and right coronary arteries following blunt injury to the chest in a car accident. The patient was scheduled for emergency coronary angiography but left and right coronary ostia were not cannulated because of thrombus formation probably. The patient died, and the autopsy revealed external compression by epicardial hematomas involving separately left and right coronary arteries and the coronary sinus without signs of coronary and/or aortic dissection. To our knowledge, this is the first case presenting occlusion of both coronary arteries secondary to blunt chest trauma causing acute myocardial infarction in a young man without signs of prior coronary artery disease.     

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.     

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.     

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.     

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.     

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.     

Sudden unexplained death among persons 1-35 years old

Sudden unexplained death (SUD) on children and young people is unusual, although the real magnitude is unknown. The clinical and physiopathological characteristics are poorly defined. The aim of this work is to analyse the epidemiological, clinical and pathological characteristics of SUD on children and young people. In this population observational study, all sudden non-violent deaths between 1 and 35 years occurred in Bizkaia (north Spain) from 1991 to 1998 were investigated, analysing those diagnosed as SUD. Pathological records, circumstances of death and autopsy findings were obtained. Out of 107 cases of sudden death (SD), 19 were SUD. The mortality rate of SUD was 0.43/100,000 persons per year. Five had pathological antecedents: syncopal episodes in three cases and tachycardia and ventricular extrasystoles one each. The initial symptom was sudden collapse (N=13). In four cases, a precipitating factor was identified (two physical exercise and two emotional stress). Six cases died during sleep. Minimal pathological findings in coronary arteries, myocardium or conduction system was found in nine cases. Heart weight increased (under interval of confidence of 95%) was present in 12 cases. In conclusion, the SUD is very infrequent in children and young people. However, it has great clinical significance because it affects people in good health and occurs without warning symptoms. Some of these cases can be due to cardiac arrhythmias. In deaths in bed there is a high frequency of SUD. In the future, it may be possible that abnormalities at a molecular level will be detected in some of the cases identified today as SUD.     

Fatal traumatic rupture of an aortic aneurysm of the sinus of Valsalva: an autopsy case

This report describes an autopsy case of a rare type of aortic sinus of Valsalva aneurysm, which caused fatal rupture from a blunt chest impact. A 51-year-old male was hit in the chest with a fist, lost consciousness after about 15min and died after approximately 7h. The postmortem examination revealed a large saccular aneurysm of the right coronary sinus bulging on the right atrium, which had a full laceration causing pericardial hematoma (cardiac tamponade). A related chest wall injury was observed in the right outer mammary region. A rare type of bulging onto the right atrium and subsequent sclerosis of the right coronary artery appeared to have greatly contributed to the fatal rupture.