[Isolated cardiac amyloidosis. Presentation of a clinical case]

A 48-year old patient affected by congestive heart failure came to our observation for cardiac arrest due to ventricular fibrillation. After cardiopulmonary resuscitation and defibrillation he underwent complete evaluation. Echo Doppler findings were consistent with restrictive cardiomyopathy. Laboratory findings revealed monoclonal gammopathy and plasma cells dyscrasia. Diagnosis of amyloidosis was then suspected and biopsies of different organs and tissues were performed. Presence of amyloid deposits was found only in myocardial specimens from the right ventricle. Medical treatment with drugs of various classes, administered during hemodynamic invasive monitoring, was uneffective in improving the hemodynamic and clinical status of the patient and he entered in a heart transplantation list. He died six months later, while awaiting for transplantation. Although isolated cardiac amyloidosis is quite rare, we believe that this condition has ever to be kept in mind during differential diagnosis of restrictive cardiomyopathies and we remark that endomyocardial biopsy was mandatory and necessary for certain diagnosis in this case; in addition, the unefficacy of drugs nowadays available for treatment of congestive heart failure in amyloid cardiomyopathy is confirmed.