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Cardiac amyloidosis

• Overview
• Symptoms
• Treatment

Alternative Names:

Amyloidosis - cardiac; Primary cardiac amyloidosis - AL type; Secondary cardiac amyloidosis - AA type; Stiff heart syndrome; Senile amyloidosis; Amyloidosis - senile

Treatment:

You can continue to exercise as long as feel like you can.

Your doctor may tell you to make changes to your diet. This may include salt and fluid restrictions.

Diuretics (water pills) may be given to help the body remove excess fluid. The doctor may tell you to weigh yourself everyday. A weight gain of three or more pounds over 1 or 2 days can mean there is too much fluid in the body.

Digoxin, calcium channel blockers, and beta blockers may be used with caution in patients with atrial fibrillation. However, the dosage must be carefully monitored, since patients with cardiac amyloidosis may be unusually sensitive to any side effects.

Other treatments may include:

• Chemotherapy
• Prednisone, an anti-inflammatory medicine
• Pacemaker , if there are problems with heart signals

A heart transplant may be considered for some patients with very poor heart function. However, it is not done in those with AL type amyloidosis, since the disease weakens many other organs. A liver transplant is needed in those with hereditary amyloidosis.

Expectations (prognosis):

Cardiac amyloidosis is a long-term (chronic) condition that slowly gets worse. On average, persons with cardiac amyloidosis live less than 1 year.

Complications:

• Congestive heart failure
• Atrial fibrillation or ventricular arrhythmias
• Sick sinus syndrome (occasionally)
• Symptomatic cardiac conduction system disease (arrhythmias related to abnormal conduction of impulses through the heart muscle)
• Low blood pressure and dizziness from excessive urination (from medication)
• Increased sensitivity to digoxin with the potential for digoxin toxicity and related arrhythmias
• Ascites (fluid accumulation in the abdomen)

Calling your health care provider:

Call your health care provider if you have this disorder and new symptoms develop, particularly:

• Postural dizziness
• Fainting spells
• Excessive weight (fluid) gain
• Excessive weight loss
• Severe difficulty breathing

References:

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine , 7th ed. St. Louis, Mo: WB Saunders; 2005:1683-1685.

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.

• Review Date: 1/17/2007
• Reviewed By: Larry A. Weinrauch, MD, Assistant Professor of Medicine, Harvard Medical School, and Private practice specializing in Cardiovascular Disease, Watertown, MA. Review provided by VeriMed Healthcare Network.

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