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.     

A comparison of respiratory symptoms and inflammation in sudden infant death syndrome and in accidental or inflicted infant death

Upper respiratory infection and pulmonary inflammation are common in sudden infant death syndrome, but their role in the cause of death remains controversial. Controlled studies comparing clinical upper respiratory infection and inflammation in sudden infant death syndrome with sudden infant deaths caused by accidents and inflicted injuries (controls) are unavailable. Our aim was to compare respiratory inflammation and upper respiratory infection within 48 hours of death and postmortem culture results in these two groups. A retrospective analysis of upper respiratory infection and pathologic variables in the trachea and lung of 155 infants dying of sudden infant death syndrome and 33 control infants was undertaken. Upper respiratory infection was present in 39% of sudden infant death syndrome cases and 40% of control cases. Upper respiratory infection was more likely to have occurred in association with more severe lymphocytic interstitial pneumonitis when sudden infant death syndrome cases and control cases were combined ( P=.04). Proximal and distal tracheal lymphocytic infiltration was more severe in control cases than in sudden infant death syndrome cases ( P=.01 and.01, respectively). Lymphocytic infiltrations of the bronchi, bronchioles, and pulmonary interstitium were similar between groups. Bronchial associated lymphoid tissue was more prominent in control cases ( P=.04). Cultures were positive in 80% of sudden infant death syndrome cases, 78% of which were polymicrobial. Among control cases, 89% were positive, with 94% being polymicrobial. This study confirms that microscopic inflammatory infiltrates in sudden infant death syndrome are not lethal.     

Inferior Vena Cava Tumor Thrombosis Secondary to Metastatic Uterine Cancer: A Rare Cause of Sudden Unexpected Death

Pulmonary tumor thromboembolus (PTTE) secondary to uterine cancer is a rare cause of sudden unexpected deaths in the elderly population. Additionally, tumor extension to the inferior vena cava (IVC) makes it rarer. No such cases have been previously reported in the forensic literature. We report this phenomenon in a previously healthy 70‐year‐old woman with no other known risk factors for the development of deep vein thrombosis. The deceased was found dead while watching television with her relatives. Autopsy examination revealed that her death was attributed to a saddle embolus lodged in the main pulmonary arteries. A uterine tumor was discovered, and microscopic examination revealed low‐grade endometrial stromal sarcoma with angiolymphatic invasion. The tumor cells were also found in the embolus and the remnant thrombosis attached to the inner wall of the IVC. This case demonstrates a sudden unexpected death caused by acute PTTE following seeding of uterine cancer into the IVC and raises awareness of such events.     

Sudden death from massive pulmonary tumor embolism due to hepatocellular carcinoma

Massive pulmonary embolism in cancer patients can be due to detached thrombi or tumor. Pulmonary tumor embolism is often undiagnosed antemortem. We report a 52-year-old Chinese man admitted for management of hepatocellular carcinoma (HCC). Computerized tomography showed tumor involvement of hepatic vein and inferior vena cava. He died suddenly on the day of admission. At autopsy the main pulmonary arteries of both lungs were blocked by large tumor emboli, the immediate cause of death. Although rapid death in patients with HCC is usually caused by intraperitoneal hemorrhage from spontaneous rupture of tumor, massive pulmonary tumor embolism should also be considered in these patients, especially when antemortem evidence of hepatic vein and/or inferior vena cava invasion is present.     

Traumatic venous aneurysm of the popliteal vein with outcome: a case report and review of the literature.

A case of sudden death due to recurrent pulmonary thromboembolism is described. The fatality took place three and a half weeks following blunt trauma to the left popliteal region. The patient died unexpectedly. Autopsy revealed the source of the emboli as a sacciform venous aneurysm of the popliteal vein, an entity seldom described, but important to consider in cases of soft tissue popliteal masses or unexplained pulmonary embolism, especially in otherwise healthy individuals.     

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.     

Pulmonary embolism: a rare cause of sudden infant death

Pulmonary embolism can be observed at any age, but it occurs very rarely in the infant. The authors report the case of a 12-month-old boy who suddenly died during clinical improvement of gastroenteritis associated with otitis. Autopsy showed signs of dehydration and acute left pulmonary embolism, which was the cause of death. Thrombosis of the inferior vena cava extending into renal veins was seen. In this case, plasma hyperosmolality caused by dehydration might have favored the formation of venous thromboses. The various risk factors of pulmonary embolism in the child are discussed, including genetic factors.     

The significance of pulmonary interstitial emphysema in live birth determination

The utility of pulmonary interstitial emphysema (PIE) in live birth determination is not well established. Because the distinction between live birth and stillbirth may be important in criminal proceedings, we undertook this study to investigate the relationship between the histologic finding of PIE and live birth. Sixty-six cases of infant death were retrieved and compared with 21 stillborn infants. Histologic sections of the lungs were characterized as "florid PIE," "equivocal PIE," or "absent PIE." Sixteen cases of florid PIE were identified, all in live born infants. Forty-seven cases of equivocal PIE were found in 36 live born and 11 stillborn infants. In 24 cases (14 live born infants and 10 stillborns), no PIE was identified. We examined the relationship between florid PIE in infants with sudden infant death syndrome (SIDS) or "sudden unexpected death in infancy, manner undetermined" (SUDI), and also its relationship to other variables. No association was found. The presence of equivocal PIE may be an artifact of tissue processing. Florid PIE is found only in live born infants. No correlation between the presence of florid PIE and cause of death could be determined.     

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.     

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 and anomalous origin of the coronary arteries from the aorta. A case report and review

Based on a case report of sudden death in a young boy, this paper reviews the available information concerning the various combinations of anomalous origins of coronary arteries and associated sudden death. Left coronary arteries arising from the right sinus of Valsalva and passing between the aorta and pulmonary arteries are often associated with sudden death and myocardial ischemia in young people. Although right coronary arteries originating from the left sinus of Valsalva and passing between the aorta and pulmonary artery are less frequently associated with symptoms, this condition may be associated with sudden death. The incidence of symptoms associated with other anomalous origins is also discussed.     

Supine Hypotensive Syndrome as the Probable Cause of Both Maternal and Fetal Death

Abstract: Supine hypotensive syndrome is characterized by severe supine hypotension in late pregnancy, whose clinical presentation ranges from minimal cardiovascular alterations to severe shock, resulting from inferior vena cava compression by gravid uterus. We report a case of a 41‐year‐old 39‐week‐pregnant woman found dead supine. Autopsy revealed the following: cyanosis of the limbs; congestion of the jugular and subclavian veins; abundant abdominal subcutaneous fatty tissue; uterus displacing intestine and diaphragm; collapsed inferior vena cava; both femoral veins dilated and filled with blood; edematous and congested lungs; and placenta 790 g, fetus 3475 g, amniotic fluid 800 cm³. The diagnosis of supine hypotensive syndrome as the probable cause of death is supported by the position of the body and autopsy findings. This syndrome can be considered as the first stage of the physio‐pathological mechanism that led to death in the case presented herein and should be considered by pathologists as a cause of sudden death.     

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.     

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.     

A retrospective analysis of cardiovascular deaths: A 5-year autopsy study of 1045 cases in Antalya,Turkey

This study aims to identify the macroscopic and microscopic changes that occur in the heart in different causes of cardiovascular death and sudden cardiac death in autopsy cases and evaluate the difficulties that a forensic practitioner may encounter during autopsies. All forensic autopsy cases in the Morgue Department of the Council of Forensic Medicine, Antalya Group Administration between January 1, 2015, and December 31, 2019, were examined, retrospectively. The cases were chosen according to inclusion and exclusion criteria, and their autopsy reports were examined in detail. It was determined that 1045 cases met the study criteria, 735 of which were also met the sudden cardiac death criteria. The top three common causes of death were ischemic heart disease (n = 719, 68.8%), left ventricular hypertrophy (n = 105, 10%), and aortic dissection (n = 58, 5.5%). The frequency of myocardial interstitial fibrosis was significantly higher in deaths due to left ventricular hypertrophy than in deaths due to ischemic heart disease and other causes (χ²(2) = 33.365, p < 0.001). Despite detailed autopsy and histopathological examinations, some heart diseases that cause sudden death may still not be detected.     

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 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.     

Postmortem absorption of drugs and ethanol from aspirated vomitus--an experimental model.

Using human cadavers an experimental model was developed to simulate the agonal aspiration of drug- and alcohol-laden vomitus. By needle puncture, an acidified (N/20 HCl) 60-ml slurry of drugs (paracetamol 3.25 g, dextropropoxyphene 325 mg) and ethanol 3% w/v was introduced into the trachea. After 48 h undisturbed at room temperature, blood samples were obtained from ten sites. Ethanol and drug concentrations were highest in the pulmonary vessels in all five cases studied. Pulmonary vein mean ethanol was 58 mg% (range 13-130), paracetamol 969 mg/l (range 284-1934), propoxyphene 70 mg/l (range 11-168). Pulmonary artery mean ethanol was 53 mg% (range 10-98), paracetamol 476 mg/l (range 141-882), propoxyphene 29 mg/l (range 7.6-80). Ethanol and drug concentrations in aortic blood were higher than in the left heart and concentrations in the superior vena cava were higher than in the right heart, suggesting direct diffusion into these vessels rather than diffusion via the pulmonary and cardiac blood. Potential interpretive problems arising from this phenomenon can be avoided by using femoral vein blood for quantitative toxicological analysis.     

Sarcomeric gene mutations in sudden infant death syndrome (SIDS)

In developed countries, sudden infant death syndrome (SIDS) represents the most prevalent cause of death in children between 1 month and 1 year of age. SIDS is a diagnosis of exclusion, a negative autopsy which requires the absence of structural organ disease. Although investigators have confirmed that a significant percentage of SIDS cases are actually channelopathies, no data have been made available as to whether other sudden cardiac death-associated diseases, such as hypertrophic cardiomyopathy (HCM), could be responsible for some cases of SIDS. The presence of a genetic mutation in the sarcomeric protein usually affects the force of contraction of the myocyte, whose weakness is compensated with progressive hypertrophy and disarray. However, it is unclear whether in the most incipient forms, that is, first years of life, the lack of these phenotypes still confers a risk of arrhythmogenesis. The main goal of the present study is to wonder whether genetic defects in the sarcomeric proteins, previously associated with HCM, could be responsible for SIDS. We have analysed 286 SIDS cases for the most common genes implicated in HCM in adults. A total of 680 mutations localised in 16 genes were analysed by semi-automated matrix-assisted laser desorption/ionisation time-of-flight mass spectrometry (MALDITOF-MS) using the Sequenom MassARRAY(?) System. Ten subjects with completely normal hearts showed mutated alleles at nine of the genetic variants analysed, and one additional novel mutation was detected by conventional sequencing. Therefore, a genetic mutation associated with HCM may cause sudden cardiac death in the absence of an identifiable phenotype.     

Occult Adrenocortical Carcinoma and Unexpected Early Childhood Death

A four‐year‐old previously well boy collapsed unexpectedly and was taken immediately to hospital, where he developed seizures and cardiogenic shock with lethal, rapidly progressing multi‐organ failure. At autopsy, the height was >90th percentile and there were indications of early virilization. Internally, a friable tumor of the left adrenal gland was identified that had invaded the left renal vein and inferior vena cava. Histology revealed typical features of an adrenocortical carcinoma with aggregated trabeculae of cells containing abundant eosinophilic cytoplasm and large pleomorphic nuclei. There was strong positive cytoplasmic staining for inhibin; mitochondria were shown on electron microscopy to contain prominent electron‐dense granules. Death was due to massive pulmonary tumor embolism. Although adrenocortical carcinomas are very rare and are more commonly found in adults, the current case demonstrates that they may also occur in childhood and be responsible for unexpected death by the very unusual mechanism of tumor embolism.