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.     

Sudden death due to a paraganglioma of the organs of Zuckerkandl

A 20-year-old woman died suddenly in a hospital emergency room after presenting with nausea, vomiting, back pain, and hypertension. At autopsy, an extra-adrenal pheochromocytoma (paraganglioma) of the organs of Zuckerkandl was found, with microscopic focal myocardial necrosis similar to that described in death from adrenal pheochromocytomas. Tumors of the organs of Zuckerkandl are extremely rare; less than 100 such cases have been reported in the world's literature, and only six, including the present case, have presented as a sudden, unexpected death. The symptoms of catecholamine storm may mimic those of acute drug intoxications, leading to misdiagnosis by both clinical physicians and pathologists.     

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.     

Sudden death due to primary diffuse leptomeningeal gliomatosis

Tumors of the central nervous system are an unusual cause of sudden death. This report describes the sudden death of a presumed healthy 28-year-old woman from primary diffuse leptomeningeal gliomatosis. She presented to an emergency room with headache and vomiting, subsequently became unresponsive and was pronounced dead 14 h later. Autopsy revealed a diffuse extensive infiltrate of well-differentiated astrocytoma in the leptomeninges of the brain and spinal cord without an underlying parenchymal tumor. Primary diffuse leptomeningeal gliomatosis is a rare tumor that arises within the leptomeninges from small neuroglial heterotopic rests that undergo neoplastic transformation. Grossly. this tumor can mimic leptomeningeal carcinomatosis, pachymeningitis, tuberculosis, sarcoidosis, and fungal infections. However, the histologic features of primary diffuse leptomeningeal gliomatosis should allow it to be readily distinguished from grossly similar conditions. The mechanism of death in this case is most likely tumor obstruction of cerebrospinal fluid outflow resulting in the usual complications seen with increased intracranial pressure. Although this tumor is aggressive and is associated with a rapidly progressive fatal course, it has not been previously associated with sudden death.     

Sudden death in an adolescent due to undiagnosed classic Hodgkin lymphoma

Few cases of natural sudden death presenting as an undiagnosed lymphoma have been reported in the literature, especially in adolescents. Herein we provide a report of sudden death caused by undiagnosed classic Hodgkin lymphoma (cHL). We describe an 18-year-old female who collapsed after several weeks of weight loss, decreased appetite, and dyspnea. At autopsy, a bulky mass arising in the mediastinum and neck compressed the esophagus and trachea, surrounded the great vessels, obliterated the pericardial sac, and infiltrated the myocardium. The lungs were collapsed and large pleural effusions were present. The tumor burden, which weighed at least 2710 g in aggregate, was entirely above the diaphragm. Microscopic examination of the masses showed features typical for nodular sclerosis cHL including large bands of sclerosis, numerous Hodgkin/Reed-Sternberg (HRS) cells, and an eosinophil-rich mixed inflammatory cell infiltrate. Immunohistochemical stains showed the HRS cells to be uniformly positive for CD30 and CD15 and negative for CD3, CD20, CD45, and PAX5. This case exemplifies a rare sudden natural death due to previously undiagnosed cHL in a young patient.     

Sudden, unexpected death due to fourth ventricular subependymoma

A case of sudden, unexpected death due to fourth ventricular subependymoma (subependymal glomerate astrocytoma) in a 60-year-old man is presented. Sudden, unexpected death due to uncomplicated subependymoma has not been previously described.     

Sudden death associated with solitary intracavitary right atrial metastatic tumour deposit.

We report a case of cardiac metastasis of primary adrenal cortical carcinoma to the endocardium of the right atrium resulting in an intracavitary mass which we believe caused sudden death by blocking the tricuspid valve orifice. We have been unable to find a similar case previously reported although primary atrial myxoma is well known to be a rare cause of sudden cardiac death by the same mechanism. Modern non-invasive imaging techniques have facilitated recognition of intracavitary cardiac metastases, and with surgical intervention, when feasible, the risk of sudden cardiac death can be reduced.     

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 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.     

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.     

Abnormal heart rate response during newborn sucking behavior study: Subsequent sudden infant death syndrome with cardiac conduction abnormality

This report concerns a four-month-old white female infant who exhibited abnormal feeding behavior and EKG irregularities during a newborn sucking behavior study. The immediate post-birth history showed an irregular heart rate on two occasions, but a cardiac consultation elicited no unusual findings. During sucrose sucking conditions, the heart rate increased with a beat-to-beat variation of 50 beats per minute, noted to be due to premature atrial beats. At 39 days, an EKG showed a marked sinus tachycardia of 156, a PR interval of 0.08, QRS of 0.05 and a QT of 0.26. The infant was diagnosed as a sudden infant death syndrome (SIDS) following an unexpected death at home. Subsequent cardiac pathology revealed an anomalous tract between the right atrium and the atrioventricular (AV) bundle which formed an extensive bypass of the AV node (atrio-His tract), and two accessory AV connections between the left atrium and posterior left ventricle. These findings are consistent with the Wolff-Parkinson-White syndrome type A. Only further studies can determine whether such abnormal feeding behavior with EKG irregularities can be used to identify infants who are at high risk for sudden death.     

Subendocardial small infarct in the superior ventricular septum as a cause of sudden death

In autopsy files from April 1999 to April 2001, five cases showed macroscopic subendocardial small infarct above 1 cm diameter in the superior ventricular septum (SVS) near the atrioventricular (AV) junction, and all five were finally considered to be sudden cardiac death after full investigation. All these small infarcts in these Japanese patients were located at the posterior site of the SVS, an area mainly nourished by branches which ramified from the AV node artery and which branched from the right coronary artery (RCA). Four of the five showed acute (A) or subacute (SA) foci in or around the healed (H) lesion and surviving myocytes were visible in infarcts, in all cases, which suggested a recurrent or chronic prolonged ischemia in the territory. Four of the five had a significant stenosis of the RCA and in the other one, there was an anomalous origin of the RCA. As all five had also small artery disease in the SVS, small infarct of the posterior SVS may have formed by hemodynamic impairment in the territory of the AV node artery caused by RCA disorders. We consider the evidence of macroscopic small infarct of the posterior SVS greatly aids in determining the cause of sudden death in forensic autopsy and may be notable lesion for discussing the pathogenesis of sudden cardiac death with RCA disorder.     

Pericardial Cyst: Cause of Sudden Cardiac Death?

Cardiovascular disease is the leading cause of sudden death in the world. The etiology of sudden cardiac death involves a wide range of diseases, but seldom pericardial cysts. A pericardial cyst is an uncommon cyst usually located in the middle mediastinum and rarely in the posterior part. They are usually harmless and asymptomatic. Here, we present a case of a 63‐year‐old woman who presented with dyspnea and hoarseness, but died suddenly after a CT scan was attempted. The detailed forensic pathologic and histologic examination revealed a pericardial cyst located in the posterior mediastinum. Toxicology and biochemistry tests, including tryptase, found no competing cause of death.     

Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy

Abstract:  Sickle cell disease (SCD) and sickle cell trait (SCT) can be associated with sudden unexpected death in the pediatric population, usually due to pulmonary complications occurring within the acute chest syndrome (ACS). Musculoskeletal complications can occur and are classically limited to bone infarcts. The occurrence of bone pathology centered upon the epiphyseal growth plate in SCD/SCT is extremely rare, and multiple such injuries in a single patient have not been previously reported. Herein, we describe a case of sudden unexpected death in a 5-year-old child with undiagnosed SCT due to the ACS, with widespread epiphyseal and periosteal bone lesions mimicking multiple inflicted injuries at autopsy. This case highlights the importance of clinicopathological correlation and is the first to describe SCT pathology as a mimic of nonaccidental injury.     

Sudden Death Due to Recent Hemorrhage From an Intrathoracic Chronic Expanding Hematoma

Chronic expanding hematoma (CEH) is a rare disease that can develop in any region of the body, but it most frequently develops in the thorax. When intrathoracic CEH is left untreated, gradually expanding hematoma can be life‐threatening, leading to respiratory failure or hemoptysis. We encountered an 89‐year‐old man with cardiopulmonary arrest on arrival. He had been healthy, and it was unclear whether CEH had previously been detected. A very large mass was observed on chest computed tomography (CT), but the cause of death could not be determined. In the autopsy, this mass was identified as CEH and no malignant findings were noted. A fresh hemorrhage had occurred in the hematoma and perforated the bronchial lumen, which caused airway obstruction/asphyxia and resulted in sudden death. CEH should be suspected when a very large tumorous lesion occupying the entire hemithorax is observed on chest imaging, and it is important to recognize that sudden death can occur in the natural course of CEH.     

Sudden death of an infant with 'an early epileptic encephalopathy'.

This article reports an autopsy case of sudden death of an infant with an infrequent encephalopathy involving epileptic episodes. The infant was a 1-year and 10-month-old boy, who had a history of the first convulsive seizures in the third month after birth. The clinical diagnosis was described as 'an early infantile epileptic encephalopathy with suppression-bursts' (Ohtahara syndrome). On a winter day, he was collapsed following a high fever and was already dead on the arrival at a hospital. The body was small for the age and poorly nourished. The autopsy and postmortem magnetic resonance imaging scan (MRI) of formalin-fixed brain revealed advanced unsymmetric brain atrophy with cortical dysplasia, which were prominent in the left temporal and right occipital lobes, and sclerotic atrophy of the parahippocampal gyri, additionally showing a feature of the olivo-ponto-cerebellar atrophy. However, the cause of death was pathologically and microbiologically determined as bacterial bronchopneumonia following pulmonary infection of the influenza A virus. In sudden death cases of physically handicapped infants, the investigation of viral infection in consideration of an epidemiological survey is important even when the death can be pathomorphologically explained.     

Pulmonary macrophage counts in deceased infants: baseline data for further study of infant mortality

Infant lung samples were obtained prospectively at autopsy by medical examiner pathologists in five areas of the United States and regardless of the cause of death. Four sections were examined for each case and were taken from the anterior and posterior aspects of the right and left upper lung lobes. Lung sections were stained with HAM-56 immunostain, which is specific for macrophages. Sixty-one cases were evaluated for the study. Three pathologists independently counted the number of macrophages per 40x field (10x ocular) in 10 contiguous fields near the center of each lung section examined. There was good agreement between pathologists on the average number of macrophages observed in each case. The mean macrophage count for all fields counted was 16.5 per 40x field (range 0-136), and the mean for individual cases was 16 (range 6.6-39.4). There was no observed difference between right, left, anterior, and posterior lung sections. There was a tendency for cases certified as sudden infant death syndrome to show lower macrophage counts than those with other causes of death, but the difference was of only marginal statistical significance. Seven of 10 cases in which infants died after a survival period in the hospital had a mean macrophage count greater than the overall mean of 16 per 40x field. These data suggest that mean pulmonary macrophage counts > 16 per 40x field may be a marker for causes of death other than sudden infant death syndrome or that there was a survival interval. These data may be useful as baseline data for further studies of infant mortality possibly involving pathologic changes in the lungs.     

Sudden death owing to right atrial hemangioma

Primary cardiac tumors are rare, with an autopsy incidence of 0.03%. Seventy-nine percent to 85% of primary cardiac tumors are benign, and of the benign tumors, cardiac hemangiomas account for 5-10% of cases. Most cardiac hemangiomas are asymptomatic and are discovered incidentally at autopsy, or by echocardiography, computerized tomography, or magnetic resonance imaging. We report a case of sudden death owing to cardiac hemangioma in a 22-year-old woman who collapsed while shopping and became unresponsive. The autopsy revealed a hemorrhagic mass on the surface of the right atrium which was infiltrating and replacing the wall of the right atrium; histopathological examination confirmed the tumor was a cavernous hemangioma. Fewer than 20 cases of right atrial cavernous hemangiomas have been reported in English literature, emphasizing the rarity of our case.     

青壮年猝死综合征心肌心钠素免疫组化研究

研究青壮年猝死综合征病理形态学诊断依据。应用免疫组化LSAB,观察10例SMDS及10例非心性死亡对照组死者ANP变化情况。结果发现,SMDS中7例ANP呈阴性,3例呈弱阳性;10例非心性死亡ANP呈强阳性。研究表明,SMDS死者心房内ANP数量减少是导致死亡的重要因素,可通过ANP免疫组化观察找到SMDS死亡的形态学依据。     

Vertebral artery dissection complicating occipital injection of heparin for treatment of thoracic outlet syndrome

A 38-year-old woman with a 2-year history of chronic neck pain radiating down her right arm underwent radiological and neurological evaluations, which revealed no anatomical cause for her pain. She sought alternative therapies including intramuscular heparin injections. Following a right occipital injection of heparin, cyanocobalamin, and lidocaine, she had a sudden cardiorespiratory arrest and was successfully resuscitated, but did not regain consciousness.Computed tomography of the head and neck and subsequent autopsy revealed a right vertebral artery dissection, but at autopsy, no significant subarachnoid hemorrhage was noted at the base of the brain. This is the first case report where heparin (a potent anticoagulant) used in an occipital injection was documented to cause a vertebral artery dissection. It is also the first reported case where radiographically and histologically documented vertebral artery dissection did not present with overwhelming subarachnoid hemorrhage at the base of the brain. The subtle gross anatomical findings in this case highlight the importance of evaluating the cervical spinal cord in any case of sudden cardiorespiratory arrest following even apparently minor neck injury.