Evaluation and Management of the Cardiac Amyloidosis

Evaluation and Management of the Cardiac Amyloidosis Joseph B. Selvanayagam, Philip N. Hawkins, Biju Paul, Saul G. Myerson, Stefan Neubauer This review summarizes the current state of evidence for the evaluation and management of cardiac amyloidosis. The electrocardiographic and echocardiographic features that are the best discriminators for disease are discussed and the evidence for this is critically appraised. The recent evidence from cardiovascular magnetic resonance imaging and prognostic implications of elevated biochemical markers are summarized. Intermediate-dose infusional chemotherapy regimes and high-dose chemotherapy with peripheral stem cell rescue remain the mainstay of disease treatment. Future strategies to stabilize the native structures of amyloidogenic proteins, to disrupt established deposits using antibodies, and small-molecule drugs are discussed.