Cases reported "Cardiomyopathies"

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1/71. Hereditary cardiac amyloidosis associated with the transthyretin Ile122 mutation in a white man.

    An 83 year old white man with atrial fibrillation was admitted to hospital after a cerebral infarct. echocardiography was characteristic of cardiac amyloid deposition and subsequent tests confirmed amyloidosis of transthyretin (TTR) type, in association with the Ile122 mutation of the TTR gene; this has only been reported previously in african americans in whom it occurs with an allele frequency of 2%. Haplotype analysis did not suggest a different founder than for the African Ile122 mutation. Cardiac amyloidosis should be considered among elderly patients presenting with cardiac failure and/or arrhythmia, particularly if they are resistant to conventional treatment; if confirmed, it should be followed by precise characterisation of amyloid fibril type. The prevalence of autosomal dominant cardiac TTR amyloidosis in elderly white people is unknown but early diagnosis and supportive treatment may prevent complications among affected family members.
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2/71. Sleeve lobectomy of the middle lobe for hilar lung cancer with accompanying cardiomyopathy and actinomycosis.

    The indication for sleeve lobectomy of the middle lobe is limited, because the frequency of cancer occupying both the middle lobe and its bifurcation is low and the more commonly used middle and lower lobectomy is an alternate procedure. However, if a patient has accompanying cardiopulmonary restriction, lung preservation is paramount. We present a patient with hilar lung cancer (TisN0M0) associated with progressive cardiomyopathy, who underwent sleeve lobectomy of the middle lobe; the cancer took a favorable course.
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3/71. Electrocardiographic manifestations of CNS events.

    Certain intracranial events produce electrocardiographic abnormalities, most often involving the T wave with diffuse, deep inversions. The amplitude of the T wave inversion is impressive, approaching 15 mm in some cases. Morphologically, the T wave is asymmetric with a characteristic outward bulge in the ascending portion. In the setting of a CNS event, relatively minor degrees of ST segment elevation are also seen in leads with obviously abnormal T waves; the ST segment elevation frequently is less noticeable than the T wave changes and is usually less than 3 mm. The T wave inversions with associated ST segment elevation are most pronounced in the mid-precordial and lateral precordial leads; such findings are also noted to a less extent in the limb leads. Other electrocardiographic features associated with acute CNS injury include prominent U waves of either polarity and QT interval prolongation, often exceeding 60% of its normal value, as well as malignant forms of bradycardia and tachycardia.
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4/71. Cardiac findings in an individual with neurofibromatosis 1 and sudden death.

    Vascular lesions in neurofibromatosis 1 (NF1) are infrequently recognised as manifestations of the disease, yet they can produce serious complications. Most individuals with NF1 vasculopathy are asymptomatic, which may contribute to underestimation of its frequency. A recent study indicates that vascular changes in individuals with NF1 contribute to mortality at younger ages. We report the sudden death of a young man with NF1. On autopsy examination there was evidence of an intramyocardial vasculopathy characteristic of the vascular pathology previously described in NF1. Other cardiac findings included non-specific cardiomyopathic changes, myocardial fibrosis, and a "floppy" mitral valve.
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5/71. 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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ranking = 61.822314895252
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6/71. 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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ranking = 123.6446297905
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7/71. Isolated noncompaction of ventricular myocardium associated with fatal ventricular fibrillation.

    A female infant with isolated noncompaction of ventricular myocardium who developed ventricular tachyarrhythmia is described. wolff-parkinson-white syndrome was shown by electrocardiography. At 9 months of age, the patient suddenly developed cardiac arrest. electrocardiography following resuscitation with DC cardioversion demonstrated sinus rhythm without delta wave. The QT interval was normal. Frequent premature ventricular captures caused ventricular fibrillation. DC cardioversion was necessary to terminate frequent attacks of ventricular fibrillation until the introduction of beta blockers and lidocaine. Two-dimensional echocardiogram confirmed the diagnosis of isolated non-compaction of ventricular myocardium. Three months later, the patient died of ventricular fibrillation during respiratory syncytial viral infection.
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ranking = 61.822314895252
keywords = wave
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8/71. 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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ranking = 61.822314895252
keywords = wave
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9/71. Spontaneous intramyocardial hematoma presenting as a rapidly expanding mass followed by echocardiography.

    A50-year-old man with an intramyocardial mass was referred to us. He had undergone resection of both a coronary arterial aneurysm of unknown origin and a postinfarction left ventricular (LV) aneurysm 5 years before. Peak creatine kinase was slightly over the upper normal range, and an electrocardiogram revealed neither a new Q-wave nor an additional ST-segment change. Repeated two-dimensional echocardiography indicated rapid expansion of a heterogeneous mass in the posterior wall of the LV. The mass was intraoperatively diagnosed as intramyocardial hematoma without connection to the LV cavity. Via posterolateral thoracotomy with partial cardiopulmonary bypass, the outer layer of the hematoma was completely resected, and xenopericardium was applied with a continuous suture to cover the space which the hematoma had occupied. The patient made an uneventful recovery.
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10/71. Primary systemic amyloidosis presenting as angina pectoris due to intramyocardial coronary artery involvement: a case report.

    We describe a 76-year-old Japanese woman with primary systemic amyloidosis who presented with angina pectoris associated with ST-segment and T-wave abnormalities resulting from intramyocardial coronary artery amyloidosis. The patient was admitted to our hospital because of dyspnea and pretibial edema 7 years after the diagnosis of variant angina. A diagnosis of primary systemic amyloidosis (AL amyloid protein) was made after examination of gastric and endomyocardial biopsy specimens. The patient died of progressive, uncontrolled heart failure 3 months later. An autopsy study demonstrated only mild-to-moderate atherosclerosis in the epicardial coronary arteries. However, histological examination of the heart revealed diffuse stenoses and obstructions in the intramural coronary arteries by amyloid deposits. This patient had small-vessel coronary disease with ST-segment changes and angina caused by cardiac amyloidosis. A correct diagnosis of ischemic heart disease due to primary amyloidosis is important for estimation of the prognosis and for appropriate management.
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ranking = 61.822314895252
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