An unusual case of primary cardiac lymphoma presenting as restrictive cardiomyopathy with arrhythmia is reported in a 72-year-old woman who was admitted for evaluation of exertional dyspnea and palpitations. Electrocardiography (ECG) showed atrioventricular dissociation and right heart cardiac catheterization revealed a typical 'dip-and-plateau' waveform. Restrictive cardiomyopathy was suspecte...

Purpose Retrospective review of 4156 clinical CMR studies (May 2006 to August 2010) was undertaken to identify cases of undifferentiated restrictive cardiomyopathy, confirmed on echocardiography and not otherwise meeting current classification guidelines. CMR had been ordered to further elucidate the etiology of restrictive cardiomyopathy. Obtained metrics were compared to a cohort of age-match...

BACKGROUND Idiopathic restrictive cardiomyopathy is a poorly recognized entity of unknown cause characterized by nondilated, nonhypertrophied ventricles with diastolic dysfunction resulting in dilated atria and variable systolic function. METHODS AND RESULTS Between 1979 and 1996, 94 patients (61% women) 10 to 90 years old (mean, 64 years) met strict morphological echocardiographic criteria f...

OBJECTIVES We present clinical data and heart and skeletal muscle biopsy findings from a series of patients with ultrastructural accumulations of granulofilamentous material identified as desmin. BACKGROUND Desmin cardiomyopathy is a poorly understood disease characterized by abnormal desmin deposits in cardiac and skeletal muscle. METHODS Clinical evaluation, endomyocardial and skeletal mu...

An understanding of the basic principles of diastolic function is important in order to recognize diseases that may result in diastolic dysfunction and diastolic heart failure. Although uncommon, restrictive cardiomyopathy, constrictive pericarditis, and cardiac tamponade are among the disorders that may affect primarily diastolic function with preservation of systolic function. Diastolic heart...

A 61-year-old diabetic woman with a mitochondrial A3243G mutation was hospitalized for evaluation of breathlessness, general fatigue, and leg edema. Chest radiography revealed cardiomegaly with massive pleural effusion. Serum lactate, pyruvate, and brain natriuretic peptide concentrations were elevated. Transthoracic echocardiography revealed a restrictive pattern of transmitral flow, although ...

Background—Idiopathic restrictive cardiomyopathy is a poorly recognized entity of unknown cause characterized by nondilated, nonhypertrophied ventricles with diastolic dysfunction resulting in dilated atria and variable systolic function. Methods and Results—Between 1979 and 1996, 94 patients (61% women) 10 to 90 years old (mean, 64 years) met strict morphological echocardiographic criteria for...

The contractile response of the heart can be altered by disease-related protein modifications to numerous contractile proteins. By utilizing an IAANS labeled fluorescent troponin C, [Formula: see text], we examined the effects of ten disease-related troponin modifications on the Ca(2+) binding properties of the troponin complex and the reconstituted thin filament. The selected modifications are...

Cardiomyopathies are an important cause of congestive heart failure in the elderly, and the magnitude of the problem is compounded by changing population demographics and the frequency of congestive heart failure in the elderly. Although the data are far from complete, differences in the clinical presentations and natural history of the cardiomyopathies in older and younger patients are becomin...

Correspondence to: Dr Siân E Hughes, Department of Histopathology, Royal Free and University College Medical School, University College London, UCL Hospitals NHS Trust, Rockefeller Building, University Street, London WC1E 6JJ, UK; [email protected] _________________________ C ardiomyopathies are defined as diseases of the myocardium, which cause cardiac dysfunction with heart failure, arrhy...