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1/31. Rapid progression of cardiomyopathy in mitochondrial diabetes.

    Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes.
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2/31. Effects of extracorporeal shock wave lithotripsy on tiered therapy implantable cardioverter defibrillators.

    The effects of extracorporeal shock wave lithotripsy (ESWL) were tested on four advanced generation implantable cardioverter-defibrillators (ICDs) in vitro and in vivo in two patients. During in vitro testing, advancement of nonsustained episode counters occurred in one device, and a set screw and power source cell loosened in another, which was connected to an external power source. No arrhythmias occurred during in vivo procedures, but programmed parameters were reset and elective replacement indicated after one procedure. ESWL can be performed safely in selected patients with ICDs, but testing should be performed afterwards to confirm satisfactory function and component continuity.
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3/31. Giant negative T waves in guillain-barre syndrome.

    A guillain-barre syndrome patient showed giant negative T waves on electrocardiography at the height of the disease, with large left ventricular hypokinesis on echocardiography and extensive defects on 123I-meta-iodobenzylguanidine myocardial scintigraphy. Gamma-globulin improved the neurological symptoms, and the above abnormalities resolved. We speculate that cardiac sympathetic nerve endings were transiently damaged, with consequent myocardial injury, due to norepinephrine toxicity.
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4/31. Twenty-seven-year follow-up of arrhythmogenic right ventricular dysplasia.

    This case report describes clinical features, especially of surface ECG changes, observed for 27 years in a patient with arrhythmogenic right ventricular dysplasia (ARVD). The course of this patient was characterized by progressive deterioration of right ventricular function and progression of delayed potentials (so-called epsilon waves) following QRS complexes. However, the relation between ventricular arrhythmias and ECG changes or the degree of right ventricular abnormality was difficult to discern.
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5/31. Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction. angina pectoris-myocardial infarction Investigations in japan.

    OBJECTIVES: To determine the clinical features of a novel heart syndrome with transient left ventricular (LV) apical ballooning, but without coronary artery stenosis, that mimics acute myocardial infarction, we performed a multicenter retrospective enrollment study. BACKGROUND: Only several case presentations have been reported with regard to this syndrome. methods: We analyzed 88 patients (12 men and 76 women), aged 67 /- 13 years, who fulfilled the following criteria: 1) transient LV apical ballooning, 2) no significant angiographic stenosis, and 3) no known cardiomyopathies. RESULTS: Thirt-eight (43%) patients had preceding aggravation of underlying disorders (cerebrovascular accident [n = 3], epilepsy [n = 3], exacerbated bronchial asthma [n = 3], acute abdomen [n = 7]) and noncardiac surgery or medical procedure (n = 11) at the onset. Twenty-four (27%) patients had emotional and physical problems (sudden accident [n = 2], death/funeral of a family member [n = 7], inexperience with exercise [n = 6], quarreling or excessive alcohol consumption [n = 5] and vigorous excitation [n = 4]). Chest symptoms (67%), electrocardiographic changes (ST elevation [90%], Q-wave formation [27%] and T-wave inversion [97%]) and elevated creatine kinase (56%) were found. After treatment of pulmonary edema (22%), cardiogenic shock (15%) and ventricular tachycardia/fibrillation (9%), 85 patients had class I New York heart association function on discharge. The LV ejection fraction improved from 41 /- 11% to 64 /- 10%. Transient intraventricular pressure gradient and provocative vasospasm were documented in 13/72 (18%) and 10/48 (21%) of the patients, respectively. During follow-up for 13 /- 14 months, two patients showed recurrence, and one died suddenly. CONCLUSIONS: A novel cardiomyopathy with transient apical ballooning was reported. Emotional or physical stress might play a key role in this cardiomyopathy, but the precise etiologic basis still remains unclear.
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6/31. hypotension and functional left ventricular obstruction during dobutamine stress echocardiography--two case reports.

    Although hypotension during dobutamine stress echocardiography has been reported, the mechanism of this response is still controversial. In two patients, a 72-year-old woman and 64-year-old man, with exercise-induced ST-T change, continuous-wave Doppler examination of the left ventricular cavity was performed at baseline and peak dobutamine infusion. No echocardiographic abnormalities at rest or angiographic coronary lesions were observed in either patient. The intracavitary pressure gradient at peak dosage of dobutamine for both patients was 121 mm Hg and 100 mm Hg, and was reproducibly confirmed by cardiac catheterization. During dobutamine infusion, echocardiography or left ventriculography revealed that papillary muscle motion was dramatically augmented by dobutamine and mid-left ventricular obstruction was produced at the systolic phase. Although blood pressure response improved following beta-blocker treatment, intracavitary pressure gradient during dobutamine infusion remained the same. A hypotensive response during dobutamine stress echocardiography may be produced by the development of dynamic intraventricular obstruction and a vasodepression reflex. The exercise-induced electrocardiographic changes may have been related to the systolic pressure augmentation in the mid-to-apical left ventricular cavity.
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7/31. Reversible left ventricular systolic dysfunction--reversibility of coronary microvascular abnormality.

    Reversible left ventricular wall motion abnormalities mimicking myocardial infarction have been reported in patients with a noncardiac illness. Their coronary angiograms do not demonstrate organic stenosis or epicardial coronary vasospasm. In this article, two cases of reversible left ventricular contraction abnormality are presented. electrocardiography showed deep inverted T waves in precordial leads, and the echocardiography revealed diffuse akinesis of the apical region in the acute phase. coronary angiography showed no significant stenosis or occlusion in either patient. thallium scintigraphy showed no defect, while the metaiodobenzylguanidine scintigraphy demonstrated significant defects in the apex. The relative coronary flow reserve ratio, measured with an intracoronary Doppler flow wire, was significantly reduced in both patients. Myocardial contrast echocardiography revealed a reversible perfusion defect in the apex in the acute phase in case 2. Transiently impaired coronary microcirculation was thought to be involved in the pathogenesis of the reversible left ventricular dysfunction observed in these patients.
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8/31. Extreme pulsus alternans with P wave alternans in a child.

    Pulsus alternans was observed in a child with left ventricular dysfunction secondary to systemic hypertension. The Echo-Doppler of supravalvular aorta and a simultaneous electrocardiographic trace (ECG) clearly demonstrated the mechanism of an apparent abrupt halving of pulse frequency compared with the ECG, similar to an electromechanical dissociation 2:1. Besides the extreme pulsus alternans, a P wave and a PP cycle length change were also observed. Both mechanical and electrical alternans disappeared with afterload reduction by nitroprusside treatment.
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9/31. Left ventricle dynamics during pulsus alternans: insights from tissue velocity imaging.

    A 34-year-old female patient with idiopathic dilated cardiomyopathy presented with hemodynamic pulsus alternans. Mitral annular tissue Doppler velocities showed reciprocal beat-to-beat alterations during systolic ejection and diastolic filling periods. Tissue velocity waves were unaltered during the isovolumic relaxation and contraction periods.
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10/31. Reversible left ventricular dysfunction "takotsubo" cardiomyopathy related to catecholamine cardiotoxicity.

    An 80-year-old female was admitted for a persistent consciousness disturbance. electrocardiography revealed ST-segment elevation in leads II, III, aVf, and V3-V6. coronary angiography demonstrated normal arteries, while left ventriculography revealed asynergy of apical akinesis and basal hyperkinesis. The creatinine kinase and creatinine kinase MB levels were not elevated after the initial measurement on admission. The diffuse ST-segment elevation reached a maximum level 24 hours after admission. In leads V3-V6, ST-segment elevation continued for 48 hours, and was followed-up by deep inverted T waves. Within 24 days, the asynergy improved without any specific treatment, but the giant negative T waves were present on the electrocardiogram. The plasma norepinephrine and brain natriuretic peptide levels on the first hospital day were 2.9ng/mL and 906pg/mL, respectively. The left ventricular dysfunction appeared to be induced by the exposure to high-level plasma catecholamines. (123)I-metaiodobenzyl guanidine scintigraphy also revealed transient dysfunction of the cardiac catecholamine dynamics.
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