Cases reported "Death, Sudden, Cardiac"

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1/11. Can heart rate variability predict sudden death? A case of sudden death in a child with severe coronary sequelae of Kawasaki disease.

    We report the heart rate variability (HRV) of a child aged 11 years. Arrhythmia was the suspected cause of sudden death after 10 years of therapy for Kawasaki disease. The linear methods failed to show any features of the HRV that could have predicted the patient's sudden death, but the fractal scaling as a nonlinear method had suddenly decreased from 5.3 to 4.1 1 year before his death.
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2/11. Successful prevention of recurrent ventricular fibrillation by intravenous isoproterenol in a patient with brugada syndrome.

    Intravenous administration of isoproterenol restored the ST-segment configuration to nearly normal in the right precordial leads and completely prevented spontaneous VF attacks in a patient with brugada syndrome. The formation of a Brugada-type ECG has been attributed to the transmural dispersion of repolarization of the right ventricular epicardium and related to modulation of the autonomic nervous system. Our case may provide clues to the pathophysiological mechanism of this syndrome.
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3/11. Sudden death in supravalvular aortic stenosis: fusion of a coronary leaflet to the sinus ridge, dysplasia and stenosis of aortic and pulmonic valves.

    Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency.
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4/11. exercise-related sudden death due to an unusual coronary artery anomaly.

    Coronary artery anomalies are, after hypertrophic cardiomyopathy, the second most common cause of exercise-related sudden cardiac death in young American athletes. These anomalies have been associated with myocardial ischemia, arrhythmia, and sudden death during exercise. A 14-yr-old male with no previous abnormal medical history collapsed during soccer practice and was successfully resuscitated without defibrillation. An extensive cardiac workup did not reveal any abnormalities. Two weeks later, he experienced a cardiac arrest while running and could not be resuscitated. autopsy demonstrated an acute angle take-off of the left main coronary artery and a transverse slit-like opening with a fibrous cushion, which created a kink near its origin. This case report illustrates the difficulty in diagnosing coronary artery anomalies in general, and acute angle take-off and ostial ridges in particular.
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5/11. Successful resuscitation after sudden death in a one year old infant who sustained a blunt chest injury after a fall from 10 m.

    Sudden cardiac arrest due to blunt anterior chest wall impact (commotio cordis) usually occurs in young athletes who are struck by a baseball or other projectile in the precordium. survival is extremely rare if the induced ventricular fibrillation (VF) is not defibrillated immediately at the scene. We report here a rare case of a one-year-old infant survivor of cardiac arrest caused by blunt chest impact during an accidental fall from a fourth story window. eye witnesses reported to have seen him land on the front of his chest directly onto the plastic rain cover on the ground floor. He was transferred to a nearby hospital within minutes, where ventricular fibrillation was recorded. Immediate cardiopulmonary resuscitation and defibrillation and was successful. He recovered without any subsequent sequelae. To the best of our knowledge, this rare incident represents the first time that an infant has survived such cardiac arrest in these circumstances that has been recorded in the literature. This has implications for the management of paediatric fall injuries.
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6/11. swimming-triggered aborted sudden cardiac death in a patient with Andersen-Tawil syndrome.

    In this report we describe the case of a 42-year-old woman who experienced an episode of near drowning during recreational swimming. A diagnosis of Andersen-Tawil syndrome was made based on the patient's dysmorphic features, characteristic T-U-wave patterns and ventricular arrhythmias. To our knowledge, this is the first report of a swimming-triggered cardiac event in a patient with Andersen-Tawil syndrome.
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7/11. Homicidal blunt head trauma, diffuse axonal injury, alcoholic intoxication, and cardiorespiratory arrest: a case report of a forensic syndrome of acute brainstem dysfunction.

    Sudden death can occur in drunk individuals who are severely beaten about the face. The structural basis for this forensic syndrome is unknown. We herein describe the case of an intoxicated 23-year-old man (blood alcohol 234 mg%, 51 mmol/l) who was involved in an altercation and received blows and kicks to his head. A cardiorespiratory arrest occurred during the assault. He was resuscitated in hospital 23 min later but died 90 h after admission of severe ischemic encephalopathy and bronchopneumonia. Postmortem examination revealed diffuse scalp bruising, no evidence of a skull fracture, multiple small hemispheric contusions, severe cerebral edema secondary to ischemic encephalopathy, and axonal swellings in the corpus callosum, subcortical white matter, midbrain, right rostral inferior cerebellar peduncle, and medulla. This case of near sudden death confirms that blunt head trauma sustained during an assault can cause mild diffuse axonal injury. In addition, it is possible that sudden, alcohol intoxication-associated, craniofacial traumatic death is caused by acute dysfunction of the brainstem cardiorespiratory centers, whose capacity to correct potentially fatal dysrhythmias or apnea, induced by injury to their afferent axons, can be compromised by alcohol ingestion.
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8/11. The automated implantable cardiac defibrillator. Prophylaxis in cardiac sarcoidosis.

    A patient with cardiac sarcoidosis proved by biopsy specimen and no history of sudden death or clinical sustained ventricular tachycardia prophylactically received an implantable cardioverter defibrillator (ICD) that later reversed an episode of near syncope. The patient was supported with the ICD until heart transplantation. The physiology and treatment of arrhythmias associated with cardiac sarcoidosis is described. Consideration for use of the ICD in asymptomatic patients and as bridge therapy until heart transplantation is discussed.
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9/11. apoptosis as a possible cause of gradual development of complete heart block and fatal arrhythmias associated with absence of the AV node, sinus node, and internodal pathways.

    BACKGROUND: Gradually progressive development of complete heart block in young people often is associated with cardiac arrhythmia and sudden death, but the pathogenesis remains unexplained. methods AND RESULTS: A young woman with complete heart block died suddenly. Her mother had serological but no clinical evidence of antiphospholipid syndrome. Five brothers of another family had arrhythmia and heart block. Three died suddenly; the other two have automatic defibrillators and are alive. The hearts from the young woman and two of the three brothers who died were available for our histological examination of their cardiac conduction systems. In two of the three hearts, the AV node was absent; in the third heart, only fragments of the AV node remained. In all three hearts, the sinus node was nearly destroyed by a noninflammatory degeneration with no abnormal fibrosis or infiltrate. In each heart, the interatrial and internodal pathways were similarly involved, and in the young woman, there were no myocardial cells in which these pathways normally exist. CONCLUSIONS: In these three subjects with progressive development of complete heart block and various arrhythmias, all of whom died suddenly, the histological abnormalities of their cardiac conduction systems are best interpreted as resulting from apoptosis. Programmed cell death is a logical explanation for the pathogenesis of this puzzling clinical picture.
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10/11. Sudden cardiac death in familial hypertrophic cardiomyopathy: are "benign" mutations really benign?

    Familial hypertrophic cardiomyopathy (FHC) is an autosomal dominant disorder characterised predominantly by left ventricular hypertrophy and sudden cardiac death. Mutations in the cardiac beta-myosin heavy chain gene have been identified in several families and designated as "benign" or "malignant". We describe a family (family L) with a "benign" mutation in which early sudden cardiac death has occurred. The family was studied by clinical, electrocardiographic and echocardiographic assessment. dna analysis involved screening for the six most common cardiac beta-myosin heavy chain gene mutations using allele specific oligonucleotide probes and restriction enzyme analysis. The Val606Met missense mutation was found. This mutation has been described in four families as being "benign" since it was associated with low penetrance and a near normal life span. Sudden cardiac death was an infrequent finding. In contrast, family L has a more malignant clinical picture with one sudden death in three affected individuals. The proband died suddenly at age 14 years during exercise. Designating gene mutations in FHC as benign or malignant has major clinical implications. As these mutations have only been described in a limited number of families, caution needs to be taken when interpreting genotype-phenotype correlations in this disorder.
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