Cases reported "Cardiomyopathies"

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1/23. A case of hereditary amyloidosis transthyretin variant Met 30 with amyloid cardiomyopathy, less polyneuropathy, and the presence of giant cells.

    Transthyretin-Met 30 (TTR-Met 30) is a variant of transthyretin and is usually associated with familial amyloid polyneuropathy. It is rare that patients with TTR-Met 30 will primarily develop amyloid cardiomyopathy. This report presents a patient with late-onset TTR-Met 30 who primarily developed amyloid cardiomyopathy, with less amyloid polyneuropathy in the peripheral nervous system than is usually seen. An autopsy was performed, and histological examination revealed many foreign-body giant cells and macrophages in the area of amyloid deposition that was found in nearly all of the organs.
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2/23. Sudden death caused by myocardial tuberculosis: case report and review of the literature.

    A 25-year-old fit man died suddenly while playing social soccer. autopsy revealed an infiltrative lesion involving the left ventricle with overlying pericarditis. No other significant pathologic changes were observed. Histologic examination showed necrotizing granulomatous inflammation. No acid-fast bacilli were demonstrated in the pericardial fluid or on histologic examination. The presence of mycobacterium tuberculosis dna complex was confirmed by use of the ligase chain reaction technique. The differential diagnosis of myocardial tuberculosis includes sarcoidosis, rheumatic fever, rheumatoid arthritis, giant-cell-containing tumors, idiopathic (giant-cell) myocarditis, and bacterial infections such as tularemia and brucellosis. This case illustrates the protean manifestations of tuberculosis and highlights the use of molecular biologic techniques in arriving at a definitive diagnosis in cases of suspected tuberculosis.
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3/23. Left ventricular apical aneurysm in cardiac sarcoidosis.

    A 53-year-old woman was hospitalized for general fatigue and palpitations. An electrocardiogram showed ST elevation and T wave inversion in leads II, III, aVF, and V4-6. cardiac catheterization was performed since the echocardiogram demonstrated the existence of a left ventricular apical aneurysm. Left ventriculography showed an aneurysm of the apex. An endomyocardial biopsy specimen from the left ventricular apical wall demonstrated typical noncaseating granulomas with giant cells. The patient was diagnosed as having cardiac sarcoidosis. There was no evidence suggesting involvement of other systemic organs. Cardiac sarcoidosis should be considered within a spectrum of diseases that cause left ventricular aneurysm.
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4/23. A case of anomalous pulmonary venous drainage from the entire left lung associated with complete heart block.

    A 22-year-old female patient presenting complete atrioventricular block and giant P waves in electrocardiogram had anomalous pulmonary venous drainage from the entire left lung. There was normal drainage from the right lung and no associated atrial septal defect or other intracardiac abnormalities. After a permanent pacemaker was implanted, she manifested signs and symptoms of heart failure. Although the anomalous pulmonary vein was anastomosed to the left atrium, intractable heart failure continued. She died six months later after surgical intervention. Postmortem examination revealed diffuse interstitial fibrosis throughout the myocardium.
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5/23. 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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6/23. Giant hydatid cyst in the interventricular septum of a pregnant woman.

    A 25-year-old woman, pregnant for 38 weeks, was admitted to our clinic with dyspnea. Transthoracic echocardiography revealed a large cyst that originated from the left side of the interventricular septum, decreasing left ventricular volume and almost entirely obstructing the left ventricular outflow tract. Cardiac magnetic resonance imaging confirmed a grade 1 hydatid cyst, which measured 61 x 59 x 66 mm. The cyst was excised after cesarean section. Extirpation required the creation of a small septal defect, which we closed without a patch, by suturing the septum directly to the left ventricular wall. The patient was discharged without symptoms. The case reported here is of particular interest not only because a hydatid cyst is rarely seen in the interventricular septum, but because a giant hydatid cyst obstructing the left ventricular outflow tract is very rare in pregnancy.
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7/23. An autopsy case of sudden death in a patient with giant cell myocarditis.

    We present a case of a 28-year-old male weight lifter who died suddenly while driving a bicycle. Forensic autopsy and histopathological examinations revealed granulomatous lesions in the myocardium and hilar lymph nodes. The latter had massive caseation necrosis, epithelioid granulomas and Langhans' giant cells suggestive of tuberculosis. However, the myocardium showed predominantly multinucleated foreign body and myogenic giant cells, lymphocytes, plasma cells, macrophages, eosinophils. Staining for acid-fast bacilli was negative. The present case highlights the difficulty in establishing whether the granulomatous lesions in the hilar lymph nodes and the myocardium are incidental findings or whether they are related by the same pathogenetic mechanisms.
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8/23. Electroanatomic mapping of the right ventricle in a patient with a giant epsilon wave, ventricular tachycardia, and cardiac sarcoidosis.

    A 47-year-old man presented with sustained monomorphic ventricular tachycardia of right ventricular origin. Surface ECG recorded during sinus rhythm showed a bizarre "double QRS" pattern. Biventricular cardiomyopathy was found with predominant right ventricular involvement, due to cardiac sarcoidosis. Electroanatomic mapping was used to characterize the right ventricular substrate abnormalities and to decipher the specific activation abnormalities responsible for the ECG findings.
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9/23. Cardiomyopathy with prominent autophagic degeneration, accompanied by an elevated plasma brain natriuretic peptide level despite the lack of overt heart failure.

    A 75-year-old man without overt heart failure showed an abnormally high level of brain natriuretic peptide (BNP) in plasma: 600 pg/ml. The left ventricular endomyocardial biopsy revealed prominent vacuolar degeneration in the myocytes, most of which were positive for PAS stain and BNP immunoreaction. Ultrastructurally, degenerative changes of myocytes were marked, such as deposits of glycogen and lipofuscin granules in the cytoplasm, but the most prominent finding was giant vacuoles containing degraded mitochondria, glycogen granules, myofibrils, and myelin-like structures (autophagosomes). This case may belong to one of the unclassified cardiomyopathies characterized by prominent autophagic vacuoles.
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10/23. Congenital giant diverticulum of the left ventricle in an adult.

    Congenital diverticulum of the left ventricle is a rare anomaly. echocardiography, cardiac catheterization, and magnetic resonance imaging of the heart diagnosed a giant left ventricular diverticulum in a 20-year-old male with a history of dyspnea and palpitations. He underwent successful surgical correction of the left ventricular diverticulum.
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