Cardiac manifestations of amyloid disease.

Amyloidosis has been defined as an infiltrative disorder primarily caused by extracellular tissue deposition of amyloid fibrils. There are at least twenty five different human and eight different animal proteins precursors of amyloid fibrils. Subtypes are differentiated by means of immunohistochemical and genetic testing, with prognosis and therapeutic strategies different from each other. There are a total of five types of amyloidosis: (1) Primary systemic amyloidosis, (2) secondary systemic amyloidosis, (3) Senile amyloidosis, (4) Hereditary amyloidosis, and (5) Hemodialysis-related. Cardiovascular manifestations can be fourfold and include congestive heart failure due to restrictive cardiomyopathy, vascular abnormalities, autonomic dysfunction, and conduction abnormalities. Tissue diagnosis remains the goal standard for diagnosis ofamyloidosis. Non invasive evaluation includes echocardiogram, cardiac MRI and Serum amyloid P component scintigraphy. These studies will be helpful in the diagnosis as well as follow up of patients with cardiac amyloidosis. Cardiac amyloidosis management will vary depending on the subtype but consist of supportive treatment of cardiac related symptoms and reducing the amyloid fibrils formation attacking the underlying disease.