[Sudden cardiac death due to primary cardiac ion-channel diseases].
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Biomedical subjects
Publications and source records attributed to U C Hoppe.
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Elevated heart rate is associated with increased cardiovascular mortality. Heart rate reduction optimises myocardial oxygen consumption and decreases angina pectoris symptoms. Thus, heart rate control is an important therapeutic strategy in coronary artery disease and, for example, chronic heart failure. The pacemaker current I(f) plays a central role in determining spontaneous activity of the sinus node. Ivabradine, a selective inhibitor of the I(f) channel, reduces heart rate without any effect on cardiac contractility and without lowering blood pressure. While beta-blockers remain the first choice for heart rate reduction, in cases of adverse effects ivabradine may be used to treat stable angina pectoris. Studies evaluating possible further uses, for example in heart failure or after acute myocardial infarction, are still warranted.
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The characteristic leading symptoms of heart failure are a reduction in physical performance, dyspnea and the development of edema. At this point, such examinations as an ECG and determination of various laboratory parameters become mandatory. For the differential diagnostic exclusion of a pulmonary cause of respiratory distress, lung function tests and chest X-rays are needed. If heart failure is suspected clinically, echocardiography is the most important noninvasive method for identifying a cardiac functional disorder. With the aid of this examination, systolic function, diastolic filling, myocardial thickness and valve function can be evaluated. Cardiac catheterization is emportant to determine the underlying causes of heart failure and to plan therapy.
HISTORY AND CLINICAL FINDINGS: We present a 38-year-old male patient with progressive exertional dyspnoea and intermittent palpitations. The initial examination detected a regular tachycardia with a heart rate of 160 bpm. EXAMINATIONS, DIAGNOSIS: In association with intermittent palpitations the 12-lead ECG detected a typical type of atrial flutter. A transthoracic echocardiography and cardiac catheterization revealed a left ventricular dilation and marked systolic dysfunction (EF 39 %) with normal coronaries. The diagnosis of dilated cardiomyopathy was made on these findings. TREATMENT AND COURSE: On the basis of this diagnosis, we treated our patient by radiofrequency catheterablation, which successfully terminated atrial flutter. Three month after ablation the echocardiography was normal with an increase of the shortening fraction from 18 to 37 % and the patient was asymptomatic. Retrospectively the diagnosis of tachycardia-induced cardiomyopathy was made. CONCLUSION: Tachycardia-induced cardiomyopathy is a rare, but reversible form of dilated cardiomyopathy. The cornerstone in the management of these patients is to achieve a normal ventricular beating rate. Radiofrequency catheterablation is a curative therapeutic option that can terminate tachyarrhythmias and lead to significant improvement of left ventricular diameters, systolic function and symptoms in patients with tachycardia-induced cardiomyopathy.
In patients with heart failure and atrial fibrillation cardiac glycosides, generally in combination with beta-blockers, are indicated to control ventricular rate. In systolic heart failure and sinus rhythm, however, the use of digitalis continues to be debated. There are special concerns that cardiac glycosides might lead to an increased mortality rate in women. Retrospective analyses, however, do not indicate any sex-based differences in the effectiveness of cardiac glycosides. Beneficial effects of cardiac glycosides in heart failure seem to be related to the attenuation of sympathetic activation and neurohumoral alterations, which is already obtained at low digoxin serum concentrations, while high serum levels are associated with increased mortality. Therefore, in patients with sinus rhythm who remain symptomatic under an optimized therapy with ACE inhibitors, beta-blockers and diuretics in addition to digitalis should be considered regardless of the gender. However, target serum digoxin concentrations should be low in a range of 0.5 to 0.8 ng/ml.
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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.
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HISTORY AND ADMISSION FINDINGS: A 35-year-old patient presented with a non-penetrating chest trauma due to an automobile accident. Examinations showed a trauma of the left shoulder (reversed Hill-Sachs lesion). After diagnostic procedures, he underwent surgery four weeks later. During the hospital stay, the patient developed angina pectoris, dizziness and syncopes. INVESTIGATIONS AND DIAGNOSIS: When the patient was transferred to our institution a 12-leads electrocardiogram and blood analysis were unremarkable. Cardiac catheterization revealed a relaxation disorder of the left ventricular anterior wall with normal coronaries. The Holter-ECG detected with occasional attacks of dizziness and recurrent syncopes concurrent with intermittent episodes of high-degree atrioventricular block and supraventricular tachycardias. This led to the diagnosis of myocardial contusion with long term symptomatic arrhythmias. TREATMENT AND COURSE: Since syncopes and arrhythmias persisted over several weeks following myocardial contusion a combined therapy with a dual chamber pacemaker and beta-blocker was initiated. CONCLUSION: ECG monitoring after blunt chest trauma in the early period and after several days is mandatory to screen and prevent potentially life threatening posttraumatic arrhythmias.
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