IDIOPATHIC MYOCARDIAL VASCULITIS PRESENTING AS RESTRICTIVE CARDIOMYOPATHY

A previously unreported case of small-vessel myocardial vasculitis pre senting as restrictive cardiomyopathy and congestive heart failure is described. The hemodynamic study, showing severely increased and equal ized diastolic pressures in atrial and ventricular chambers, and cardi ac MRI, showing normal pericardium and ventricular endomyocardial biop sy, 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 disea se, became available and improved the clinical profile and the diastol ic dysfunction at two-dimensional echocardiographic Doppler analysis.