Search
Cardiac amyloidosis can present along a spectrum from asymptomatic to severe CHF w/conduction abnormalities
ECG with low voltage + echocardiogram with thickened myocardium should heighten suspicion
Definitive Dx. is myocardial biopsy identifying the infiltrative lesion (MRI w/gad is also supportive)
AL (light chain) amyloidosis is an acquired disease from improperly functioning plasma cells
¨ Rapidly progressive and life threatening
¨ Tx. w/chemotherapeutic agents (+/- BMT)
Transthyretin-related (TTR) amyloidosis is produced by the liver (2 types)
Familial transthyretin-related amyloidosis (ATTR)
Senile systemic amyloidosis (SSA)
¨ Both are slowly progressive
¨ Tx liver transplant (ATTR) and supportive care (SSA)
References
Quarta C, Kruger J, Falk R. Cardiac Amyloidosis. Circulation. Sept 2012;126(2)178-182