ABSTRACT
HISTORY AND CLINICAL FINDINGS: A 51-year-old woman was referred for kidney transplant evaluation. An HIV infection had been diagnosed ten years ago, viral load being negative at present. Renal type AA-amyloidosis was demonstrated by kidney biopsies. The patient had no symptoms. Clinical examination revealed no significant abnormalities with exception of a systolic murmur. A 12-channel electrocardiogram demonstrated peripheral low voltage. Echocardiography showed severe diastolic dysfunction, ventricular hypertrophy and biatrial dilatation. In addition, the aortic valve displayed a calcified mass. TREATMENT AND COURSE: Treatment with an ACE-inhibitor for diastolic heart failure was continued, and the patient was included into the renal transplantation program. CONCLUSIONS: Amyloidosis is characterized by an abnormal folding of certain proteins and their extracellular deposition in several organs. This leads to corresponding dysfunction and can result in organ failure. Cardiac involvement is usually associated with immunoglobulin-light-chain (AL) - or familial transthyretin-associated (ATTR) - amyloidosis and indicates a poor prognosis. This report demonstrates a rare case of pronounced cardiac manifestation of the amyloid protein A (AA) - amyloidosis.
