Sudden Death Due to Undiagnosed Rheumatic Heart Disease in a Child

We report the case of a 5‐year‐old boy who died from complications of rheumatic heart disease with atypical presentation. He was hospitalized for recent inflammatory and neurological symptoms. He was diagnosed with viral encephalitis. He died the day after he was discharged. The macroscopic autopsy findings were unremarkable. Histology revealed typical rheumatic heart disease. Neuropathology showed cerebral infarction due to an embolic event linked with the rheumatic valvulitis. The cause of death was determined as heart failure due to rheumatic heart disease secondary to an undiagnosed acute rheumatic fever. It is related to an autoimmune response to infection with group A streptococcus. It mainly affects children in developing countries. In our case, viral encephalitis was consistent with the medical history and the proper diagnosis was made on histological analysis. Forensic pathologists should consider this diagnosis facing a sudden unexpected death in childhood, even in industrialized countries.     

Metastatic calcification of the cardiac conduction system with heart block: an under-reported entity in chronic renal failure patients

Systemic metastatic calcification is a common complication of chronic renal failure. Cardiac involvement is particularly ominous, especially when the cardiac conduction system is affected. Conduction defects, arrhythmias, and sudden death have all been reported with conduction system calcification; however, these are relatively under-reported or unrecognized causes of cardiac morbidity and mortality. We describe a 40-year-old man with Von Hippel-Lindau disease who had been maintained on hemodialysis for two years following bilateral nephrectomies for renal cell carcinoma. The patient presented with symptomatic complete heart block that had progressed from Mobitz type I atrioventricular block. Two months later, while being internally paced, the patient died unexpectedly after a complicated hospital admission. Postmortem revealed extensive vascular, myocardial, and conduction system calcification. Conduction system calcification may cause sudden death in chronic renal failure patients during hospital admission, or unexpectedly while the patient is in the community. Knowledge of this condition is necessary to detect it, as the conduction system is not routinely examined. A routine abbreviated conduction system examination is warranted for patients with systemic metastatic calcification, especially if they have sudden death or a known history of heart block.     

Fatal amphetamine-associated cardiotoxicity and its medicolegal implications

Amphetamines are popular drugs of abuse, particularly among youngsters and at dance scenes. Cardiotoxicity (manifested as cardiomyopathy, acute myocardial infarction/necrosis, heart failure, or arrhythmia) after the recreational (mis)use of amphetamine and its synthetic derivatives has been documented but is rather rare. Amphetamine-related cardiac fatalities are even more rare. We present 6 cases of young persons who died unexpected after the chronic abuse of amphetamines. Death was not attributed to a lethal intoxication but to an acute myocardial necrosis, a right ventricle rupture, a cardiomyopathy, or an arrhythmia. Two of the deceased persons presented prior to their death to the emergency department, but their complaints were not considered (probably due to their young age) to be of cardiac origin. One case was a sport-related fatality where medical screening failed to identify the underlying cardiac pathology or the amphetamine abuse, and 1 case was a so-called idiopathic dilated cardiomyopathy where substance abuse was not considered by the treating physician. We think that amphetamine-associated cardiotoxicity is a rare but probably genuine entity that should be considered both in forensic and clinical/emergency medicine because of its potential medicolegal implications.     

Hemopericardium caused by endocarditis ulcerosa: an unusual cause of sudden death in adolescence

The authors report on the case of a 18-year-old student who died suddenly and unexpectedly of a pericardial tamponade after perforated ulcer-polypous endocarditis of the aortic valve. The detection of aerococcus urinae--which usually causes urinary tract infection--on the inflamed aortic valve demonstrates an unusual pathogenetic chain linking complexes of findings in the urogenital system and the heart.     

Unexpected sudden death of a 12-year-old male with congenital single coronary artery

A rare autopsy case of a 12-year-old male with a single coronary artery is reported in which he suddenly and unexpectedly died after a brief period of physical and mental excitation. The single coronary artery originated from the left aortic sinus, then branched out the left circumflex artery and afterwards descended as the left anterior descending artery, while the right coronary artery originated almost at a right angle from the left anterior descending artery. The entire heart as well as the cardiac conduction system depended exclusively on the single coronary artery for oxygenated blood supply, and the unbalanced blood distribution on his exertion probably led to sudden cardiac death. Moreover, in this case, both the deceased's mother (at the age of 20 years) and grandmother on the mother's side died suddenly, thus suggesting a possibility of maternal transmission of this congenital anomaly in his family.     

Sudden death due to undiagnosed intracranial hemangiopericytoma

A previously healthy 9-year-old Japanese boy with a 4-day history of vomiting and headache died suddenly and unexpectedly. An external examination revealed no abnormalities other than foam around the mouth and nose. An internal examination revealed severe pulmonary edema and hemorrhagic hemangiopericytoma arising from the choroid plexus of the right lateral ventricle. The cause of death was thought to be neurogenic pulmonary edema caused by the rapid growth of a hemangiopericytoma, with intratumoral hemorrhage.     

Severe Cardiac Rupture by Only One Blow to the Chest in a Young Boy: An Autopsy Case

Cardiac rupture by blunt chest trauma is commonly seen after motor vehicle accidents and falls; however, it is rarely caused by a blow to the chest. We herein report an autopsy case of a high school boy who sustained severe right ventricular rupture by only one knee kick to the chest during a quarrel. He was hospitalized and developed cardiopulmonary arrest. Emergency surgery was performed, but the patient died. The autopsy revealed no external severe trauma or deformation, but the side wall of the right ventricle contained a large V‐shaped laceration. The other thoracic organs had no injuries. This case illustrates that severe cardiac rupture can occur by only one blow to the chest. Blunt cardiac injuries can occur even if no severe injuries are present on the body surface. We should consider the possibility of severe cardiac injuries regardless of the presence of external injuries.     

Rupture of a cardiac diverticulum resulting in sudden death

Cardiac diverticula have been documented in approximately 80 patients. In over 70% of cases, this lesion is associated with midline thoracoabdominal defects or other congenital malformations of the heart. The diverticulum is thought to occur early in embryogenesis from an outpouching of the endomyocardium through a weak region in the left ventricular wall, but its exact etiology is uncertain. The case of a 6-year-old white boy who died suddenly while playing is presented. Autopsy revealed a ruptured diverticulum of the left ventricular apex with massive hemopericardium and cardiac tamponade. No other abnormalities were found. The pertinent literature is reviewed.     

Marfan syndrome. A contribution to forensic medicine cases

A 19-year-old school boy suffered from fluctuating uncharacteristic chest pain in the last 20 h before his death. He died unexpectedly within a few minutes of a hemopericardium, which resulted from an aneurysmal rupture of the ascending aorta. The patient's past history as well as the autopsy and ultrastructural findings led to the diagnosis of Marfan's syndrome with alterations of the cardiovascular skeletal system but no ophthalmological involvement ("oligosymptomatic" form of Marfan's syndrome). Appraisal of the cause of death is made more difficult by the fact that medical treatment was undertaken on the same day, whereby subtotal liver crushing was established that had resulted from attempts at resuscitation.     

A Case of Sudden Death Due to Persistent Severe Pulmonary Arterial Hypertension After Late Atrial Septal Defect Closure

Atrial septal defects (ASDs) are one of the most prevalent congenital cardiac anomalies in adults. These interatrial communications can produce changes in the right heart (remodeling and failure) and the lungs (pulmonary hypertension). Most adults with ASDs are surgically treated with excellent results. However, a small fraction of patients is at risk for postoperative complications, particularly the persistence of pulmonary hypertension. A case of a 47‐year‐old woman who was found unresponsive in the bathroom of her house and died despite resuscitative efforts is described. According to medical records, the woman underwent a surgical repair of an atrial septal defect at the age of 37. At the autopsy, macroscopic and microscopic signs of advanced pulmonary hypertension were detected, highlighting the importance for the forensic pathologists to recognize pulmonary hypertension as a cause of sudden death in adults with a history of late surgical closure of an atrial septal defect.     

Investigation of sudden infant deaths in the State of Maryland (1990-2000)

The Office of the Chief Medical Examiner (OCME) has recorded a significant decline in the deaths of sudden infant death syndrome (SIDS) in the state of Maryland since 1994. However, infants who died of accidental or non-accidental injuries remained consistent during the same time period. This report focuses on the epidemiological characteristics and scene investigation findings of infant victims who died suddenly and unexpectedly in Maryland between 1990 and 2000. A retrospective study of OCME cases between 1990 and 2000 yielded a total of 1619 infant fatalities. 802 infant deaths were determined to be SIDS, which represented 50% of the total infant deaths in our study population. Five hundred and twenty-three (31.8%) deaths were due to natural diseases, 128 (7.9%) deaths were accidents, and 74 (4.6%) were homicides. The manner of death could not be determined after a thorough scene investigation, review of history and a complete postmortem examination in 92 (5.7%) infants. SIDS deaths most often involved infants who were male and black. The peak incidence of SIDS was between 2 and 4 months of age. The majority of SIDS infants (60%) were found unresponsive on their stomach. Among SIDS infants, 269 (33.4%) were found in bed with another person or persons (bed sharing). Of the bed-sharing SIDS cases, 182 (68%) were African-American. In the past 11 years, 52 infants died of asphyxia due to unsafe sleeping environment, such as defective cribs, ill-fitting mattresses, inappropriate bedding materials. Of the 74 homicide victims, 53 (70%) involved infants less than 6 months of age. Twenty (27%) exhibited the classical abuse syndrome characterized by repeated acts of trauma to the infants.     

Endocardial fibroelastosis as a cause of sudden unexpected death

We present a case of primary endocardial fibroelastosis (EFE) which had been diagnosed in a 16-year-old girl who died suddenly and unexpectedly. This exceptional cause of death in adolescence led to a short literature review comparing our findings with previous medicolegal reports.     

Myocardial dysplasia of the right ventricle (Uhl's anomaly) as a possible cause of sudden, unexpected death

Partial absence and fatty replacement of the myocardial compacta of the right ventricular wall was observed in five young male adults, four of whom died suddenly and unexpectedly and one in a car accident. In one of these cases there was only one coronary orifice. Four cases of the so-called Uhl anomaly had histological findings indicating dysmorphic myocardial cells associated with minimal lymphohistiocytic infiltrates within the fatty tissue. In one case, chronic destructive inflammation was a prominent feature. Marked dysmorphic alterations in the myocardial fibers suggested the occurrence of arrhythmic episodes as the main cause of sudden heart failure and death.     

Heart weights of white men 20 to 39 years of age. An analysis of 218 autopsy cases

Review of autopsy data for 218 white men between 20 and 39 years of age who died of injuries or suddenly and unexpectedly indicated a mean heart weight of 371 g. The mean for those dying of external causes was 364 g whereas the mean for those dying of natural causes was 446 g. A coefficient for heart weight expressed as a percentage of body weight was lower in heavy individuals than lightweight individuals; it ranged between 0.38% and 0.55%, with a mean of 0.48%. Heart weight, including epicardial fat, increased with age and body weight. These data may be useful to those who are called upon to investigate sudden and unexpected deaths.     

Sudden death related to small coronary artery disease

Two cases of sudden death of young people in apparently good health are reported. The only pathologic change found was a fibromuscular dysplasia of the artery supplying the conduction system of the heart with an important narrowing of the lumen and strong thickening of the arterial wall. The first case was of a 12-year-old girl who died suddenly while skiing; the second was of a 32-year-old man who died while talking to his wife. No other pathologic changes were found at autopsy, and the results of toxicologic analysis were negative. There was no individual or family history of cardiac diseases. These cases illustrate the importance of an analysis of the conduction system, including examination of the intramural coronary arteries supplying the conduction system.     

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.     

Pulmonary thromboembolism after air travel: Two case reports, the review of literature and forensic implications

Anabolic androgenic steroids (AAS) are the main class of doping agents and their consumption produces adverse effects involving several organs and systems. Three cases of sudden cardiac death (SCD) and one of death due to congestive heart failure of previously healthy athletes who were AAS users are herein reported. Concentric cardiac hypertrophy with focal fibrosis (one case), dilated cardiomyopathy with patchy myocyte death (two cases) and eosinophilic myocarditis (one case) were observed and most probably relate to the final event. Molecular investigation for viral genomes was positive in one case (Ebstein virus). Our data confirm previous findings, showing that the most typical cardiac abnormality in AAS abusers is left ventricular hypertrophy, associated with fibrosis and myocytolysis. An exceptional cardiovascular substrate was represented by the case with drug induced eosinophilic myocarditis. These features are at risk of ventricular arrhythmias as well as congestive heart failure. The cause-effect relationship between AAS abuse and cardiac death can be established only by a rigorous methodology with the use of standardized protocols, including precise morphological studies of all target organs to search for chronic toxic effects. Laboratory investigations should focus on AAS searching on a wide range of biological matrices to demonstrate type, magnitude and time of exposure.     

Comparison of heart mass in seizure patients dying of sudden unexplained death in epilepsy to sudden death due to some other cause

Proposed mechanisms by which sudden unexplained death syndrome in epilepsy (SUDEP) occurs include cardiac dysrhythmias. We hypothesized that individuals dying of SUDEP would have enlarged hearts compared with normal, increasing the risk of sudden cardiac death should the autonomic nervous system initiate a dysrhythmia. We performed a retrospective case-control study in a medical examiner population, comparing the mean heart mass in a group of individuals who died of SUDEP to a group of individuals with epilepsy who died suddenly due to some unrelated cause (non-SUDEP). We found no significant difference in the mean heart mass between the 2 groups when analyzing the unadjusted data. Upon stratifying the cases by age, however, we found a significant reduction in the frequency of SUDEP in individuals 40 or more years of age with an increased heart mass compared with those younger. This reduced frequency disappeared when cases where the cause of death was indeterminate between SUDEP and heart disease were reclassified from non-SUDEP to SUDEP. With increasing age, the likelihood of finding a cause of death that competes with the possibility of SUDEP increases, making SUDEP appear to be a phenomenon of the young. The inclusion of seizure deaths evaluated in a medical examiner office in studies of SUDEP would provide the benefit of a more certain diagnosis in each given case. Moreover, the inclusion of cases from the medical examiner population would stem attrition in a clinical study due to loss to follow-up.     

A fatal chronic ketamine poisoning

A few papers in the literature reported incident deaths by acute ketamine poisoning. In this paper, we report an unusual homicide caused by chronic ketamine poisoning. The victim was a 34-year old married woman with no previous medical history (except as reported herein) who died in her own home. The court investigation revealed that she was chronically poisoned by her husband over a period of about one year in an act of homicide. Determination of ketamine concentrations in autopsy specimens was carried out with gas-chromatography/mass spectrometry (GC-MS). The results showed that ketamine concentration was 21 microg/mL in gastric contents, 3.8 microg/mL in blood and 1.2 microg/mL in urine. The most striking forensic findings were cardiac muscle fibrosis and hyaline degeneration of small arteries in victim's heart, the pathological features of ketamine poisoning previous reported only in animal studies.     

Sudden,unexpected death following typhoid-cholera vaccination

A previously healthy 33-year-old Australian male died suddenly and unexpectedly 8 h after a typhoid-cholera vaccination. Such facalities are extreme rarities and the present case is the first in which postmortem measurement of serum immunoglobulins has been undertaken. The clinical course and necropsy findings suggest that death was the result of a slowly evolving systemic anaphylactic reaction which terminated in hypotension and righs heart failure. The deceased was probably atopic. The current recommendations for the vaccination of international travellers against typhoid and cholera are discussed.