Cardiac amyloidosis (patient information)

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Cardiac amyloidosis

Overview

What are the symptoms?

What are the causes?

Who is at highest risk?

When to seek urgent medical care?

Diagnosis

Treatment options

Where to find medical care for Cardiac amyloidosis?

What to expect (Outlook/Prognosis)?

Possible complications

Cardiac amyloidosis On the Web

Ongoing Trials at Clinical Trials.gov

Images of Cardiac amyloidosis

Videos on Cardiac amyloidosis

FDA on Cardiac amyloidosis

CDC on Cardiac amyloidosis

Cardiac amyloidosis in the news

Blogs on Cardiac amyloidosis

Directions to Hospitals Treating Cardiac amyloidosis

Risk calculators and risk factors for Cardiac amyloidosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jinhui Wu, MD; Ujjwal Rastogi, MBBS [2]

Overview

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

What are the symptoms of Cardiac amyloidosis?

  • Most patients with cardiac amyloidosis may experience symptoms of restrictive cardiomyopathy, while others may show no symptoms. Common symptoms include:
  • Diseases with similar symptoms:

What causes Cardiac amyloidosis?

Amyloidosis refers to a family of diseases in which there is a buildup of clumps of proteins called amyloids in body tissues and organs. These proteins slowly replace normal tissue, leading to failure of the involved organ. There are many forms of amyloidosis that may involve specific organs like the heart (cardiac amlyoidosis), the neurologic system with a peripheral neuropathy or the kidney.

Cardiac amyloidosis usually occurs as part of primary amyloidosis (AL type amyloidosis). Primary amyloidosis is often seen in people with multiple myeloma and cancer.

Cardiac amyloidosis ("stiff heart syndrome") occurs when amyloid deposits take the place of normal heart muscle. It is the most typical form of restrictive cardiomyopathy. Cardiac amyloidosis may affect the way electrical impulses move through the heart (electrical conduction system). This can lead to irregular heart beating also known as arrhythmias and failure of the electrical impulses to conduct through the heart (heart block).

Secondary amyloidosis (AA type) rarely affects the heart. However, one form of secondary amyloidosis called senile amyloidosis may involve the heart and blood vessels. Senile amyloidosis is caused by overproduction of a different protein. The condition is becoming more common as the average age of the population increases.

There is no difference in the incidence of cardiac amyloidosis between men and women as it affects both genders equally. The disease is rare in people under age 40.

Who is at highest risk?

Amyloidosis refers to abnormal deposition of amyloid protein in the body tissues and organs. These proteins gradually replace the normal tissue resulting in organ dysfunction. Amyloidosis often occurs in patients with the following characteristics:

When to seek urgent medical care?

Call your health care provider if symptoms of cardiac amyloidosis develop. If you experience any of the following symptoms, seek urgent medical care as soon as possible:

Diagnosis

Your physician may run the following test to confirm the diagnosis:

Treatment options

There is no definitive treatment available for cardiac amyloidosis. The purpose of treatment is to decrease the synthesis and deposition of the abnormal protein and to protect the heart's function.

  • Diet: Salt and fluid restriction is recommended to patients with cardiac amyloidosis.
  • Medications
  • Chemotherapy: Drugs such as cyclophosphamide, carmustine and doxorubicin, can be used to inhibit the over-immunoreaction and decrease the synthesis of abnormal protein.
  • Prednisone: Prednisone, an anti-inflammatory medicine, may promote the dissolution of the abnormal protein and relieve patients' symptoms. Prednisone is usually used along with other chemotherapy drugs.
  • Diuretics: Diuretics may help your body remove excess fluid and relieve swelling.
  • Digoxin, calcium channel blockers, and beta blockers: These three types of drugs can be used to control heart rate when atrial fibrillation happens. Digoxin and calcium channel blockers are contraindicated in cardiac amyloidosis due to a potential for toxicity. Usual drugs include metoprolol, bisoprolol, and carvedilol. These drugs should be monitored carefully by the doctors.

Where to find medical care for Cardiac amyloidosis?

Directions to Hospitals Treating cardiac amyloidosis

What to expect (Outlook/Prognosis)?

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

Possible complications

Sources

http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm

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