Cases reported "Death, Sudden, Cardiac"

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1/14. Supravalvular aortic stenosis, williams syndrome and sudden death. A case report.

    Supravalvular aortic stenosis (SVAS) is an uncommon but well characterized congenital narrowing of the ascending aorta above the level of the coronary arteries. It can be a familial disorder, can occur sporadically, or associated with williams syndrome (WS) which is a neurodevelopmental disorder affecting connective tissue and the central nervous system. Sudden death is a well-known complication of non-syndromic SVAS but few cases have been reported associated with WS. We present a case of sudden death in a woman with the diagnosis of SVAS and WS since the age of 3 years who refused surgical correction and died at the age of 27 years. At autopsy, the aorta and pulmonary trunk were narrowed and the walls showed peculiar microscopical characteristics. In the cardiac conduction system the His bundle was small and intramyocardial. The incidence, pathology, pathogenesis and prognosis of both conditions (SVAS and WS) are reviewed.
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2/14. Sudden death and variant angina.

    Variant angina is defined by chest pain occurring at rest associated with transitory ST segment elevation on ECG, and is caused by a spasm of a coronary artery. Frequently, variant angina is associated with atherosclerotic coronary obstruction and patients with normal coronary arteries are rare. patients with variant angina and normal coronary arteries have good prognosis, and the development of ventricular arrhythmias or sudden death is rare. The authors present two cases of sudden cardiac death in patients with variant angina and normal coronary arteries.
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3/14. The brugada syndrome.

    brugada syndrome describes the syndrome of sudden cardiac death in the setting of the following electrocardiographic findings: right bundle branch block pattern with ST-segment elevation in the right precordial leads. The right bundle branch block may be incomplete while the ST segment elevation is minimal. The electrocardiographic findings are not constant. patients suspected of having brugada syndrome should be promptly referred for electrophysiological testing and treatment. Rapid referral and placement of an implantable cardioverter defibrillator (ICD) is associated with an excellent prognosis, whereas failure to diagnose this condition is associated with a high risk for sudden death. Therefore, it is imperative that all emergency physicians be familiar with the typical ECG manifestations of brugada syndrome. Three illustrative cases are presented with a review of the syndrome.
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4/14. Two sudden death cases of idiopathic ventricular tachycardia.

    It is said that the prognosis of idiopathic ventricular tachycardia (IVT) without organic heart disease is good in general. In this paper, two sudden death cases of IVT are reported. The first case was a 44-year-old male, who was referred to our hospital for further examination and treatment of ventricular tachycardia. echocardiography did not show obvious organic heart disease. After the patient was diagnosed with IVT, he was given drug therapy at the outpatient clinic. However, about six months later, he died suddenly while playing mahjong late at night. The second case was a 17-year-old male. He was also referred to our hospital by another hospital after being diagnosed with ventricular tachycardia. A diagnosis of IVT was made, for which he was given drug therapy at outpatient clinic. However, the patient discontinued receiving the drug therapy. The patient died suddenly about 3 months after discontinuation of the therapy. Some idiopathic ventricular tachycardia cases have poor prognosis like the present ones. It was considered necessary to thoroughly control and guide patients with their daily life and monitor them carefully about the ingestion of anti-arrhythmic agents.
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5/14. Atrial paralysis in a patient with Emery-Dreifuss muscular dystrophy.

    Emery-Dreifuss disease is a benign X-linked muscular dystrophy characterized by a distinct pattern of muscle weakness, which is of insidious onset and slow progression. It is associated with atrial paralysis that results in sudden death in early adulthood if left untreated. The authors report the documentation of electrical and mechanical silence confined to the atria in a patient with this disease. electrocardiography and electrophysiological study document the absence of electrical atrial activity, and inability to pace the atria. Hemodynamic studies demonstrate the absence of A waves, and angiography revealed immobility of the atria. This patient has done well following the institution of permanent ventricular pacing. His brother, who also had muscular dystrophy, died a sudden cardiac death at the age of 29 after refusing medical intervention. Emery-Dreifuss muscular dystrophy is particularly worthy of recognition because of the preventable occurrence of sudden death in young patients with an otherwise excellent prognosis. Permanent ventricular pacing is indicated.
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6/14. Idiopathic Brugada-type electrocardiographic pattern in an octogenarian.

    The prognosis of idiopathic Brugada-type ECG pattern in asymptomatic people is unknown. We report a case of an 85-year-old man who had persistent Brugada-type ECG pattern without associated clinical symptoms. This illustrates that the persistent Brugada-type ECG can be present with normal longevity.
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7/14. microvascular angina, adverse outcome: a case report.

    We encountered a patient with microvascular angina (MVA) who was died suddenly, and observed ST-segment elevation during attack without epicardial coronary arterial vasoconstriction, suggesting the occurrence of microvascular spasm. Doppler guide wire (DGW) and N-13 ammonia positron emission tomography (PET) demonstrate severe impairment of the coronary microcirculation extending throughout the whole of the left ventricle. Conventional medical treatment was not effective in this case. We speculate that the prognosis of microvascular angina with severe coronary microcirculatory disturbance and accompanied by microvascular spasm might not always be good. Therefore, methods for treating such cases need to be established.
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8/14. Diffuse nonatherosclerotic coronary aneurysms: an unusual cause of sudden death in a young male and a literature review.

    Coronary artery aneurysms are uncommon, usually associated with atherosclerosis and rarely involve all 3 main coronary arteries. Sudden death from documented thrombosis within large coronary aneurysms has been rarely reported. The authors report a case of a previously healthy 36-year-old male who presented with myocardial infarction complicated by sudden cardiac death. The patient was successfully resuscitated, and coronary angiography revealed diffuse, severe aneurysmal disease without evidence of atherosclerosis. A thrombus was visualized in a large aneurysm of the proximal left anterior descending artery, and there was total occlusion of a second diagonal branch, presumably due to thrombus embolization. The patient had no history of Kawasaki disease, and evaluation revealed no inflammatory or autoimmune condition. Optimal treatment and prognosis for patients with nonatherosclerotic coronary aneurysms remains unclear. Our patient was treated medically with chronic warfarin and low-dose aspirin therapy and recovered without complication.
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9/14. Successful parental use of an automated external defibrillator for an infant with long-QT syndrome.

    Congenital long-QT syndrome with 2:1 atrioventricular block presenting in the perinatal period is rare, has a poor prognosis, and leads to high risk for lethal ventricular arrhythmic events. An implantable cardioverter-defibrillator seems to be the most effective treatment in the prevention of arrhythmic sudden cardiac death in patients with long-QT syndrome. Technical limitations and risks associated with implantable cardioverter-defibrillators in asymptomatic infants is considered too great to justify use for primary prophylaxis against sudden cardiac death. In this case report we describe the first successful parental use of an automated external defibrillator prescribed for primary prophylaxis against sudden cardiac death in an infant with long-QT syndrome.
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10/14. Apical segmental dysfunction in hypertrophic cardiomyopathy: progression into end-stage heart failure with sudden cardiac death.

    Apical segmental dysfunction is an unusual finding in hypertrophic cardiomyopathy (HCM). It is characterized by a poor clinical course and a high incidence of malignant ventricular tachyarrhythmias. Long-term prognosis of patients with HCM and apical segmental dysfunction is still unclear. We report 2 cases of apical segmental dysfunction in HCM in whom progressive apical dilatation and congestive heart failure developed. Both patients died suddenly, and intractable ventricular tachyarrhythmias were documented in 1 case during resuscitation. This report provides further evidence that apical segmental dysfunction might predict a subgroup of patients with HCM who are likely to develop end-stage heart failure and are at high risk of sudden cardiac death.
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