1/20. biopsy-proven cardiomyopathy in heterozygous Fabry's disease.A 23-year-old woman with heterozygous Fabry's disease who had acroparesthesia was admitted to hospital for precise examination of the disease before childbearing. She had no cardiac-related symptoms and no abnormality on physical examination. The alpha-galactosidase A activity in her leukocytes was present, but lower than normal. However, the endomyocardial biopsy showed specific changes for Fabry's disease. As Fabry's disease is a rare X-linked recessive inborn error of glycosphingolipid metabolism, heterozygous females are usually asymptomatic, but rarely can be affected as severely as hemizygous males. This is an isolated case of heterozygous Fabry's disease in a female in whom cardiac involvement was detected by endomyocardial biopsy, although she had no cardiac abnormality on physiological examinations. In conclusion, endomyocardial biopsy is useful for evaluation of the cardiac involvement of Fabry's disease even in an asymptomatic case.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
2/20. Left ventricular assist system as a bridge to myocardial recovery.BACKGROUND: Despite recent advances in medical therapy, heart transplantation, and mechanical circulatory support, the mortality of patients with congestive heart failure remains high. methods: Retrospective data on 5 patients were obtained from our hospital's medical records. Each patient was supported by a left ventricular assist system (LVAS) because of severe congestive heart failure. The duration of LVAS support averaged 229 days (range, 46 to 447 days). In 3 patients, the LVAS was removed electively after the patient showed recovery of myocardial function. In the other 2, it was removed because of a malfunction. RESULTS: In response to LVAS support, hemodynamic variables were significantly improved. The mean cardiac index increased from 1.45 to 2.69 L x min(-1) x m(-2) (p < 0.001) and the mean left ventricular ejection fraction increased from 0.144 to 0.288 (p < 0.025). All patients were in new york Heart association functional class IV at LVAS implantation and class I at its explantation. One patient died of noncardiac-related causes 10 days after LVAS removal. The remaining 4 patients are alive and well 35, 33, 14, and 2 months after LVAS removal. CONCLUSIONS: In select patients with severe congestive heart failure, mechanical unloading with an LVAS can result in recovery of myocardial function. These patients can return to a normal physical status, thereby avoiding heart transplantation. More research is required to determine optimal modes of LVAS support, to predict which patients are likely to recover, and to assess long-term outcomes.- - - - - - - - - - ranking = 0.19951741846193keywords = physical (Clic here for more details about this article) |
3/20. 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.- - - - - - - - - - ranking = 0.39903483692386keywords = physical (Clic here for more details about this article) |
4/20. Amyloid myopathy: evidence for mechanical injury to the sarcolemma.Myopathy is a rare clinical manifestation in primary systemic amyloidosis. The clinical phenotype and muscle histology are well described but the pathophysiological mechanisms remain poorly understood. We report a 40-year-old man who presented with hypertrophic cardiomyopathy and a limb girdle syndrome associated with deposition of amyloid and free lambda light chains in skeletal muscle. Electron microscopy showed amyloid fibrils, physically disrupting the plasma membrane and basal lamina, while laminin immunocytochemistry revealed a reduction of laminin beta1 and upregulation of laminin alpha1. We believe that one of the possible pathophysiological mechanisms in amyloid myopathy is mechanical disruption of the sarcolemma by the abutting amyloid fibrils.- - - - - - - - - - ranking = 0.19951741846193keywords = physical (Clic here for more details about this article) |
5/20. selenium deficiency associated with cardiomyopathy: a complication of the ketogenic diet.PURPOSE: The ketogenic diet (KD) is an efficacious treatment for intractable epilepsy, associated with infrequent side effects. The KD is known to be deficient in most vitamins and minerals and may be deficient in trace minerals. We report biochemical selenium deficiency in nine patients on the KD, including one who developed cardiomyopathy. methods: A whole-blood selenium level was obtained on the symptomatic patient after noting the patient's poor appearance on physical examination. Children already treated and children beginning the KD were then evaluated prospectively for selenium status by measuring whole-blood or serum selenium as part of routine laboratory evaluation every 3 months. RESULTS: The index case had no detectable whole-blood selenium. Cardiac physical examination and ECG were normal, but the echocardiogram revealed cardiomyopathy. Thirty-nine additional children had the selenium status evaluated. Eight had selenium levels below the normal range (six initially, and two developed low selenium levels on serial testing). They were referred for cardiology evaluations, which were normal. selenium supplementation improved levels in all children. Low levels were seen in some children after only a few months of treatment. CONCLUSIONS: The nutrient adequacy of the currently used KD has not been fully evaluated. The nutrient content of KD with usual supplements may not meet recommended dietary allowances (RDA) for selenium and may not provide other trace minerals in adequate amounts. At our center, selenium deficiency was found in 20% of the patients evaluated. Screening for selenium deficiency is suggested if the patient KD regimen does not meet > or =75% of the RDA or if the child is symptomatic. Nutrient supplementation should provide adequate trace elements for children treated with the KD. The KD requires close monitoring of the overall nutritional status.- - - - - - - - - - ranking = 2keywords = physical examination, physical (Clic here for more details about this article) |
6/20. A 28 year old woman with ventricular tachycardia and an abnormal chest radiograph.A 28 year old white woman with no medical history presented to the emergency room with symptomatic non-sustained ventricular tachycardia. She was asymptomatic up to a few days before presentation. Her physical examination was essentially normal and hence did not help with the differential diagnosis of the problem. Bronchoscopic transbronchial biopsy led to the final diagnosis of cardiac sarcoidosis.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
7/20. Cardiomyopathy in coffin-lowry syndrome.coffin-lowry syndrome (CLS) is a rare but well-documented X-linked disorder characterized by small size, developmental delay/mental retardation, and characteristic facial and skeletal findings in affected males. The phenotype in affected females is far more variable and can include developmental differences, obesity, and characteristic facial and skeletal differences. Cardiac anomalies are reported in less than 20% of affected males, with cardiomyopathy being one of the rare but reported complications of this disorder. However, cardiomyopathy is not well characterized in CLS. Here, we report on a 14-year-old boy with physical and developmental findings consistent with CLS who presented with a relatively sudden onset of signs of congestive heart failure due to a restrictive cardiomyopathy; an endomyocardial biopsy demonstrated non-specific hypertrophic myocyte alterations consistent with cardiomyopathy. This is the first description of the histology and electron microscopy of cardiomyopathy in CLS.- - - - - - - - - - ranking = 0.19951741846193keywords = physical (Clic here for more details about this article) |
8/20. A case of transient left ventricular apical ballooning. A condition simulating an acute myocardial infarction.Transient left ventricular apical ballooning, sometimes associated with intraventricular pressure gradient, is a condition simulating an acute myocardial infarction and may occur in patients presenting with chest pain, electrocardiographic changes and minimal myocardial enzyme release typically without coronary angiographic stenosis. It was originally described in the Japanese population and is often associated with cerebrovascular accidents, surgical procedures and emotional and physical stress. We report the case of a 65-year-old woman presenting with chest pain typical of myocardial ischemia, dyspnea, electrocardiographic abnormalities and signs of hemodynamic instability, occurring after a severe emotional stress. echocardiography and contrast ventriculography showed normokinesis confined to the basal segments of the left ventricle, with a markedly decreased ejection fraction. Scintigraphy was suggestive of a large perfusion defect. The electrocardiographic abnormalities and dyskinesis persisted for many hours. coronary angiography, performed in the acute phase, was completely normal. Five months later, the functional and electrocardiographic abnormalities had totally disappeared.- - - - - - - - - - ranking = 0.19951741846193keywords = physical (Clic here for more details about this article) |
9/20. hypereosinophilic syndrome associated with acute necrotizing myocarditis and cardiomyopathy.We report the rare case of a 55-year-old female with massive eosinophilic myocarditis and severe, however reversible, impairment of left ventricular function. The patient presented with reduced physical condition, progressive dyspnea on exertion and peripheral edema. The white blood count revealed a leukocytosis and markedly elevated peripheral blood eosinophilics (48.8%). An endomyocardial biopsy demonstrated massive myocardial infiltration with eosinophilic granulocytes and necrosis. The symptoms and laboratory parameters indicate the presence of a hypereosinophilic syndrome. The differential diagnosis of a churg-strauss syndrome is discussed. Medical heart failure treatment according to international guidelines and an immunosuppressive treatment with prednisolone (Decortin H) 1.5 mg/kgBW) were initiated. This therapy led to a dramatic reduction of the eosinophilic granulocyte count and normalization of the peripheral blood count, which correlated with a significant improvement of clinical symptoms. Consistently, an increase of left-ventricular function was observed. Upon successive dose reduction to a maintenance dosage of 10 mg prednisolone, the patient's clinical status and peripheral blood count remained stable.- - - - - - - - - - ranking = 0.19951741846193keywords = physical (Clic here for more details about this article) |
10/20. Sudden cardiac death due to physical exercise in male competitive athletes. A report of six cases.In the period of 30 years, i.e. from 1973 to 2002, we noticed in croatia 6 sudden and unexpected cardiac deaths in male athletes during or after training. Two were soccer players, 2 athletic runners, one was a rugby player and one was a basketball player. All of them were without cardiovascular symptoms. At the forensic autopsy, the first athlete, aged 29, had chronic myocarditis and thickened left ventricular wall of 15 mm. The second, aged 21, had an acute myocardial infarction of the posterior wall with normal coronaries and thickened left ventricular wall of 15 mm. The third aged 17, had hypoplastic right coronary artery and narrowed ascending aorta, suppurant tonsillitis and subacute myocarditis. Two athletes, aged 29 and 15, had hypertrophic cardiomyopathy and normal coronaries, and one dilated aorta. The sixth, aged 24, had arrhythmogenic cardiomyopathy of the right ventricle. All the 6 athletes died suddenly, obviously because of malignant ventricular arrhythmias. In croatia the death rate among athletes reached 0.15/100 000, in others who practice exercise reached 0.74/100,000 and the difference is highly significant (c2=14.487, Poisson rates=3.81, P=0.00014) and in physicians-specialists reached 33.6/100,000. Preventive medical examinations are essential, especially in athletes before physical exercise, as are other investigations in every case suspicious of heart disease, including electrocardiogram (ECG), stress ECG, echocardiography and stress-echocardiography and other findings if indicated. Physical exercise is contraindicated in acute respiratory infection: in 2 of those cases had been a cause of death as a trigger.- - - - - - - - - - ranking = 0.99758709230965keywords = physical (Clic here for more details about this article) |
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