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1/64. 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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2/64. Regional left ventricular dysfunction in a patient with severe prolonged anemia.

    A 47-year-old woman with severe prolonged anemia developed heart failure. After treatment of the heart failure and anemia, she showed regional dysfunction of the left ventricular wall and myocardial fatty acid metabolism was disturbed in these sites. Coronary arteriography showed normal images. It took about 4 months to recover both left ventricular wall motion and fatty acid metabolism. Prolonged decrease of oxygen supply to the myocardium, which is caused by severe prolonged anemia, seemed to affect the myocardial function in this case, which could be another model of anemia-related myocardial dysfunction.
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3/64. Dynamic outflow obstruction due to the transient extensive left ventricular wall motion abnormalities caused by acute myocarditis in a patient with hypertrophic cardiomyopathy: reduction in ventricular afterload by disopyramide.

    A 65-year-old woman was admitted to the coronary care unit because of acute pulmonary edema. Immediate 2-dimensional and Doppler echocardiograms revealed extensive left ventricular wall motion abnormalities and left ventricular hypertrophy with extreme outflow obstruction. Although an ECG showed ST-segment elevation in the anterolateral leads, a coronary arteriogram revealed normal epicardial arteries. heart failure was relieved after diminishing the dynamic outflow obstruction with disopyramide administration. An endomyocardial biopsy from the right ventricle on the 8th hospital day showed borderline myocarditis. Wall motion abnormalities gradually normalized within 2 weeks. It is speculated that her pulmonary edema would not have been relieved so readily without the immediate reduction in ventricular afterload by disopyramide. These clinical changes over time were observed with serial echo-Doppler examinations.
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4/64. Transformation of mitral valve prolapse to dynamic left ventricular outflow tract obstruction and back again in a patient with acute transient myocardial depression.

    We describe an unusual case of transient resolution of preexisting mitral valve (MV) prolapse during acute cardiac dysfunction and the development of dynamic left ventricular (LV) outflow tract obstruction. The patient presented with lightheadedness, chest pain, and compromised hemodynamic status. echocardiography revealed akinesis and deformation of the LV anterior wall and apex, hyperdynamic activity in the bases, anterior MV leaflet systolic anterior motion without prolapse, and a dynamic outflow tract gradient. Myocardial function fully recovered over 1 month. Repeat ultrasonography showed posterior MV leaflet prolapse and no anterior MV leaflet systolic anterior motion. Elongated MV leaflets may have contributed to dynamic outflow tract obstruction and life-threatening hemodynamic compromise during LV conformational change.
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5/64. myocardial stunning in hyperthyroidism.

    The cases of two patients with hyperthyroidism and acute left ventricular (LV) dysfunction with segmental wall motion abnormalities resulting in heart failure are reported. Both had electrocardiographic changes mimicking ischemic coronary artery disease. Treatment with antithyroid medications, beta blockers, and angiotensin-converting enzyme inhibitors rapidly restored LV function. The rapid reversibility suggests a role for myocardial stunning, an important entity to recognize in hyperthyroidism since this form of LV dysfunction can be reversed with appropriate treatment.
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6/64. Segmental degradation of left ventricular wall motion after persistent coronary fistula in a posttransplantation patient: a case report and short review of literature.

    A 50-year-old man received an orthotopic heart transplant because of severe coronary heart disease and congestive heart failure. Two years after the transplantation, a continuous murmur occurred at the left sternal edge after repeated endomyocardial biopsies. echocardiography and coronary angiography revealed a dilated left anterior descending artery with a fistula to the right ventricle. The circumflex was large with an equally postero-lateral branch, and the right coronary artery was rather small with collaterals to the distal part of the left anterior descending branch. The patient had refused any intervention to close the fistula. The left ventricular levogram was normal. Two years later, in a follow-up angiogram, the left ventricular ejection fraction had decreased as a result of hypo- and akinesis of the apex and posterior wall. We suggest that this local wall motion disturbance derives from a steal phenomenon rather than being a sequela of rejection. The decrease in left ventricular ejection fraction was associated with shortness of breath upon moderate exercise. Standard heart failure medication relieved the patient's symptoms. The observation of local wall motion disturbances in this case, as well as conflicting views in the literature, raises the question whether postbiopsy coronary fistulas in transplant patients should be closed.
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7/64. Coronary microvascular abnormality in the reversible systolic dysfunction observed after noncardiac disease.

    Acute reversible left ventricular wall motion abnormalities mimicking myocardial stunning have been reported with noncardiac disease and their coronary angiograms did not demonstrate organic stenosis or vasospasm in the epicardial coronary arteries. Thus, this mechanism has not yet been fully clarified. Two patients are reported as demonstrating acute reversible wall motion abnormalities after noncardiac disease. The electrocardiographic and echocardiographic findings mimicked myocardial stunning and confirmed the previous reports. The coronary angiograms did not show any corresponding coronary stenosis or vasospasm, but did show a reduced coronary flow reserve. Cardiac metaiodobenzylguanidine scintigraphy demonstrated regional defects involving the apex, a decreased heart/mediastinum ratio and an enhanced washout rate, which partially returned to normal after 3 months. Microvascular dysfunction and sympathetic nervous abnormalities might be responsible for the reversible contractile impairment.
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8/64. 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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9/64. 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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10/64. 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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