Cases reported "Death, Sudden"

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1/69. Clinical and pathologic study of two siblings with arrhythmogenic right ventricular cardiomyopathy.

    ARVC is a cardiomyopathy in which the right ventricular myocardium is replaced by fibroadipose tissue. Males are affected slightly more often than females and, in those cases which are familial, the pattern of inheritance is usually autosomal dominant with incomplete penetrance. We examined the hearts of two sisters, ages 17 and 14, with no family history of heart disease. The older sibling, who was previously considered healthy, died suddenly, while the younger sibling developed congestive heart failure and received a cardiac transplant. An autopsy of the older sibling and examination of the younger sibling's excised heart revealed severe examples of ARVC with minor differences. A thick cap of fibroadipose tissue covered most, if not all, of each right ventricle and was transmural in some areas. Microscopically, lelt ventricles contained extensive myocyte disarray and multifocal fibrosis. The coronary arteries displayed intimal hyperplasia with disruption of the internal elastic lamina, similar to fibromuscular dysplasia. These two cases comprise a unique familial grouping in a polymorphic disease. Despite the male predominance and autosomal dominant inheritance in ARVC, the only members affected in this family were female, and an autosomal dominant pattern of inheritance, even with incomplete penetrance, would be unusual. In addition, we identified changes in the coronary arteries similar to fibromuscular dysplasia and corroborated recently reported changes in the left ventricle of patients with ARVC, providing evidence that this disease, in its most severe form, involves the entire heart.
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ranking = 1
keywords = arrhythmogenic, arrhythmogenic right, right ventricular cardiomyopathy, ventricular cardiomyopathy, cardiomyopathy, dysplasia
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2/69. A case of sudden death caused by fibromuscular dysplasia.

    After sudden unexpected death in a previously healthy 42 year old woman, necropsy examination showed myocardial infarction caused by occlusion of a vital coronary artery by fibromuscular dysplasia. This is a rare arterial disease with a clinical onset usually in the third or fourth decades of life. The aetiology is not fully understood but since it affects vital (cerebral and coronary) blood vessels it often has fatal consequences.
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ranking = 0.0016540929291291
keywords = dysplasia
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3/69. Postictal central apnea as a cause of SUDEP: evidence from near-SUDEP incident.

    While undergoing video-EEG monitoring, a 20-year-old woman had a 56-second convulsive seizure, after which she developed persistent apnea. The rhythm of the electrocardiogram complexes was unimpaired for approximately 10 seconds, after which it gradually and progressively slowed until it stopped 57 seconds later. Evaluation after successful cardio-respiratory resuscitation showed no evidence of airway obstruction or pulmonary edema. The patient had a previous cardio-respiratory arrest after a complex partial seizure without secondary generalization. Although epileptic seizures are known to be potentially arrhythmogenic to the heart, our observations strongly suggest that one probable mechanism of sudden unexplained death in epilepsy is the marked central suppression of respiratory activity after seizures.
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ranking = 0.11913894640946
keywords = arrhythmogenic
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4/69. Arrhythmogenic left ventricular dysplasia' and sudden death.

    A 32-year-old man who died suddenly and unexpectedly was found at autopsy to have prominent fatty infiltration of his left ventricle with fibrous scarring, in the presence of normal coronary arteries. The right ventricle was minimally involved. A diagnosis of ventricular dysplasia largely limited to the left ventricle was made. Subsequent family screening identified a brother with clinical manifestations of ventricular dysplasia. This case provides further evidence for the association of left ventricular dysplasia with sudden death, and demonstrates that left ventricular involvement may also be inheritable. Whether predominantly left ventricular dysplasia is a manifestation of right ventricular dysplasia, or is a separate entity, is yet to be determined.
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ranking = 4.096304425214
keywords = ventricular dysplasia, right ventricular dysplasia, dysplasia
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5/69. Hypertrophic cardiomyopathy complicated by severe bradycardias: a pedigree report.

    We describe four patients with nonobstructive hypertrophic cardiomyopathy (HCM) from an extended Chinese family. The patients had remarkably similar physical and echocardiographic findings including a harsh localized systolic murmur and apical left ventricular hypertrophy. All four had severe sinus bradycardia and atrial-ventricular conduction block (AVB) manifest by recurrent syncope. Two died suddenly due to bradycardia. Holter monitoring showed no tachycardias. Late potentials were not present. We conclude that this unusual form of HCM may be caused by a new and as yet unknown gene mutation.
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ranking = 0.0039061452188289
keywords = cardiomyopathy
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6/69. The spectrum of intramyocardial small vessel disease associated with sudden death.

    Intramyocardial small vessel abnormalities are not commonly recognized. The best known abnormality is fibromuscular dysplasia involving the sinoatrial or atrioventricular nodal arteries. Small vessel disease has been reported as an isolated cardiac anomaly in individuals with sudden death, and may also be associated with other cardiac conditions including hypertrophic cardiomyopathy and mitral valve prolapse. The nature of the association is unknown, and the mechanism causing sudden death is sometimes obscure. We describe pathological changes of the intramyocardial small vessels of three individuals with sudden death. Abnormalities involved small vessels at different levels. In all the cases, the abnormalities were thought to have caused or contributed to the individual's death. The possible mechanisms of this are discussed.
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ranking = 0.0011120476295916
keywords = cardiomyopathy, dysplasia
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7/69. Bilateral fronto-occipital polymicrogyria and epilepsy.

    We present a clinico-pathological case report in which both cortical dysplasia and epilepsy coexisted: a 30 year old male who was dead on arrival at hospital. One and a half hours earlier he had developed complex partial status with peri-oral cyanosis. At post mortem examination his brain showed bilateral occipital frontal polymicrogyria with unlayered neuronal migration disorder; in other parts there were fourth layer migration disorders. The white matter exhibited multicystic encephalopathy. In the heart there was chronic interstitial and perivascular fibrosis, although he died of a cardiac arrest. Bilateral frontal-occipital polymicrogyria is highly epileptogenic. This was a sporadic case and we cannot define a clear aetiology. There was a pathological cardiac condition without previous vascular risk factors which might be related to repetition of seizures and possibly to his sudden death during status epilepticus.
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ranking = 0.00033081858582583
keywords = dysplasia
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8/69. Childhood sporting deaths.

    exercise-induced collapse and sudden death are unusual in childhood. For this reason, a study was undertaken of a series of 12 cases of sudden death in childhood occurring during physical exertion associated with sporting activities. The age range was 7 to 16 years (mean 12.3 years, M:F ratio 5:1). Deaths resulted from trauma associated with the sporting activity, from an idiosyncratic response to exertion, or from exacerbation of a known underlying disease. Trauma was directly fatal (n = 4: vascular trauma in 1, head injury in 2, drowning in 1), exacerbated an underlying medical condition (n = 1: hypertrophic obstructive cardiomyopathy), or followed collapse from underlying organic disease (n = 1: drowning in epilepsy). Deaths after exertion occurred when there was an unexpected response to underlying occult disease (n = 4: aortic stenosis in 1, cerebral arteriovenous malformation in 1, hypertrophic obstructive cardiomyopathy in 1, coronary atherosclerosis in 1) or to preexisting known disease (n = 2: surgically corrected transposition of the great vessels in 1, asthma in 1). The fatal episodes often resulted from a complex interplay of a variety of factors, including physical exertion, possible trauma, and underlying organic disease. Testing of other family members may be indicated in cases where a rare, possibly familial, disease is found. Evaluation of cases required descriptions of activities before death, information from the medical history of the deceased, and detailed findings from the autopsy.
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ranking = 0.0015624580875316
keywords = cardiomyopathy
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9/69. De subitaneis mortibus. XVII. Multifocal stenoses due to fibromuscular dysplasia of the sinus node artery.

    The sinus node artery was focally narrowed by fibromuscular dysplasia in two examples of sudden unexpected death reported here. Although both cases had additional histological abnormalities in the conduction system of the heart, the more striking feature was the focal fibromuscular dysplasia. These findings are discussed in relationship to a large number of similar examples of focal fibromuscular dysplastic narrowing of the sinus node artery observed in other victims of sudden unexpected death, considering some possible mechanisms for lethal electrical instability of the heart and also the possible pathogenesis of such fibromuscular dysplasia.
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ranking = 0.0023157301007808
keywords = dysplasia
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10/69. Kallmann's syndrome and dilated cardiomyopathy.

    A 52-year-old male previously known to have Kallmann's syndrome was admitted with congestive cardiac failure. He was found to have a dilated cardiomyopathy and died despite medical therapy. This report is the first known case of Kallmann's syndrome and dilated cardiomyopathy.
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ranking = 0.0046873742625947
keywords = cardiomyopathy
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