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Idiopathic Myocardial Vasculitis Presenting as Restrictive Cardiomyopathy

Andrea Frustaci; Cristina Chimenti; Maurizio Pieroni
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From the Istituto di Cardiologia, Universitá Cattolica del Sacro Cuore, Rome, Italy


1997 by the American College of Chest Physicians


Chest. 1997;111(5):1462-1464. doi:10.1378/chest.111.5.1462
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Abstract

A previously unreported case of small-vessel myocardial vasculitis presenting as restrictive cardiomyopathy and congestive heart failure is described. The hemodynamic study, showing severely increased and equalized diastolic pressures in atrial and ventricular chambers, and cardiac MRI, showing normal pericardium and ventricular endomyocardial biopsy, not including myocardial vascular component, were insufficient to make a diagnosis. This made a thoracotomy and surgical cardiac biopsy necessary. Steroids and cyclophosphamide, introduced after histologic evidence of necrotizing vasculitis, unassociated with a systemic disease, became available and improved the clinical profile and the diastolic dysfunction at two-dimensional echocardiographic Doppler analysis.


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