Cardiac amyloidosis

Definition

Cardiac amyloidosis is a disorder caused by deposits of an abnormal protein in the heart tissue, which make it hard for the heart to work properly.

Alternative Names

Causes

Amyloidosis is the accumulation of clumps of proteins called amyloids in body tissues and organs. The proteins slowly replace normal tissue. There are many forms of amyloidosis.

Cardiac amyloidosis usually occurs during primary amyloidosis, also called AL type amyloidosis. Primary amyloidosis is often seen in persons with multiple myeloma, a type of cancer.

Cardiac amyloidosis, also called "stiff heart syndrome," occurs when amyloid deposits take the place of normal heart muscle. It is the most typical type of restrictive cardiomyopathy. Patients with cardiac amyloidosis may have changes in the way electrical signals move through the heart. This is called a conduction disturbance.

Secondary amyloidosis, also called AA type, rarely affects the heart. However, a subtype of secondary amyloidosis, called senile amyloidosis, involves the heart and blood vessels. Senile amyloidosis is caused by overproduction of a protein different from both the AA and AL types. Senile cardiac amyloidosis is becoming more common as the average age of the population increases.

Cardiac amyloidosis is more common in men than in women. The disease is rare in people under age 40.

References

Hare M. In: Libby P, ed. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo: WB Saunders; 2007.

Arora S, Arora A, Makkar RP, Monga A. Stiff heart syndrome. CMAJ. 2003 Jun 24;168(13):1690-1.

Kholova I, Kautzner J. Current treatment in cardiac amyloidosis. Curr Treat Options Cardiovasc Med. 2006 Dec;8(6):468-73.