Cardiac amyloidosis

Abstract

Cardiac involvement can be found in all types of amyloidosis, but is most frequent in AL amyloidosis. Severity of cardiac infiltration is by far the most relevant prognostic determinant. Once the heart is affected, amyloidosis carries a poor prognosis. Diagnosis is based on non-invasive testing such as ECG, echocardiography and cardiac MRI (CMR). However, endomyocardial biopsy is needed to unequivocally confirm cardiac infiltration and for immunohistochemical differentiation. Therapy primarily aims to reduce amyloid precursor proteins and treat end-organ failure. Specific cardiologic therapy is largely restricted to diuretics, anticoagulation and pacemaker implantation. In rare cases urgent heart transplantation followed by high-dose chemotherapy and stem cell transplantation can be considered. © 2012 Springer-Verlag.