Abstract
Cardiac amyloidosis is associated with progressive myocardial amyloid infiltration causing heart failure and arrhythmias. In the stage of manifest heart failure, without treatment the prognosis is less than one year.
The estimated yearly incidence is 8 to10 per million. The main diagnostic tool is echocardiography, showing thick and rigid ventricle walls leading to diastolic dysfunction.
Serum estimation of κ/λ light chains and magnetic resonance both yield highly specific findings. The histological evidence of the amyloid confirms the diagnosis.
The aim of treatment is to suppress the production of the amyloidogenic protein. Early diagnosis of the amyloidosis may improve its highly adverse prognosis.