These deposits can cause thickening of different sections of the heart, leading to decreased cardiac function.

[4] This relatively rare form of cardiac amyloidosis occurs in an estimated six to ten cases per 1,000,000 people.

[4] Biopsy with histological evaluation can rule out Light chain and Familial subtypes, leaving the diagnosis of Senile.

However, greater use of cardiac magnetic resonance has increased the rate of diagnosis[2] The severity of the disease tends to be less than the Light chain and Familial variants.

[8] Cardiac manifestations include: For patients with light-chain amyloidosis, there can be depositions of amyloid into numerous different organs.

[1] Deposits of the amyloids can occur throughout the body, including the heart, liver, kidneys, spleen, adrenal glands, and bones.

[3] This restriction in ventricular motion results in a decreased ability for the heart to pump efficiently, leading to the various symptoms associated with cardiac amyloidosis.

[1] ECGs of patients with cardiac amyloidosis usually show a low voltage in the limb leads, with an unusual extreme right axis.

For patients with light-chain amyloidosis, the QRS complex pattern is skewed,[1] with poor R-waves of the chest leads.

[2] EKG changes may be present, showing low voltage and conduction abnormalities like atrioventricular block or sinus node dysfunction.

[12] Extracardiac biopsies of tissues of the kidney, liver, peripheral nerve, or abdominal fat can be used to confirm the presence of amyloid deposits.

Amyloid deposits in biopsy samples are confirmed through the use of Congo red dye, which produces a green birefringence when viewed under polarized light.

[1] For light-chain amyloidosis patients, bone marrow biopsies could be conducted to determine the baseline percentage of plasma cells and to rule out multiple myeloma.

[1] Cardiac magnetic resonance (CMR) is capable of measuring the thickness of different areas of the heart.

[13] Scintigraphy can be used to measure the extent and distribution of the amyloid throughout the body, including the liver, kidney, spleen, and heart.

[14] In this method of imaging, radiolabeled technetium is injected into the body where it binds to cardiac amyloid deposits.

[1] One of the major routes to decrease the production of these excess light chains is to kill the abnormal cells that are producing them.

[5] Chemotherapeutic agents such as melphalan or bortezomib can be used to kill off the abnormal cell line that is producing the free light chains.

[5] There are newer medications (ixazomib, carfilzomib, daratumumab, elotuzumab) under research for the treatment of multiple myeloma that can help to decrease the production of free light chains.

[5] To treat complications, medications can be prescribed including midodrine for autonomic neuropathy, amiodarone for patients with atrial fibrillation to prevent arrhythmias, and warfarin used after a cardioembolic episode.

[17] In 2012, Craig Lewis, a 55 year old Texan, presented at the Texas Heart Institute with a severe case of amyloidosis.

Worse outcomes have been seen when echocardiography shows left ventricular wall thickness, poor systolic function and severe diastolic dysfunction.

[1] Light chain (AL-CM) Prognosis: For light-chain amyloidosis early detection leads to best possibility of therapies prolonging the period of remission.

[5] In comparison to light chain amyloidosis, the familial subtype is slower to progress and has a more favorable prognosis.

[5] However, the Val 122lle mutation (most common cause of familial cardiac amyloidosis) has a 4-year survival rate of 16% with an average length of 26 months.