Navigate Up

Cancer Center - A-Z Index

#
Q
Y

Print This Page

Cardiac amyloidosis

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.

Alternative Names

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

Causes, incidence, and risk factors

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.

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

Cardiac amyloidosis ("stiff heart syndrome") occurs when amyloid deposits take the place of normal heart muscle. It is the most typical type of restrictive cardiomyopathy . Cardiac amyloidosis may affect the way electrical signals move through the heart (conduction system). This can lead to arrhythmias and conduction disturbances (heart block).

Secondary amyloidosis (AA type) rarely affects the heart. However, a form of secondary amyloidosis called senile amyloidosis involves 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.

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

Symptoms

Some patients may have no symptoms. Those who do may experience:

Signs and tests

It can be difficult to diagnose cardiac amyloidosis, because the signs can be related to a number of different conditions.

Signs may include:

  • Abnormal sounds in the lung (lung crackles) or a heart murmur
  • Blood pressure that is low or drops when you stand up
  • Enlarged neck veins
  • Swollen liver

The following tests may be performed to help diagnose cardiac amyloidosis:

Click to download

An ECG may show problems with the heart beat or heart signals (conduction disturbance).

A cardiac biopsy is used to confirm the diagnosis. A biopsy of another area, such as the abdomen, kidney, or bone marrow, is often done to confirm the diagnosis.

Treatment

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

You may need to take water pills (diuretics) to help your body remove excess fluid. The doctor may tell you to weigh yourself every day. A weight gain of 3 or more pounds over 1 - 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. Patients with cardiac amyloidosis may be unusually sensitive to any side effects.

Other treatments may include:

  • Chemotherapy
  • Implantable cardioverter-defibrillator (AICD)
  • Pacemaker, if there are problems with heart signals
  • Prednisone, an anti-inflammatory medicine

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, because the disease weakens many other organs. People with hereditary amyloidosis will need a liver transplant.

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

Calling your health care provider

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

References

Hare JM. The dilated, restrictive, and infiltrative cardiomyopathies. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa:Saunders Elsevier; 2011:chap 68.

McKenna W. Diseases of the myocardium and endocardium. In:Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 60.

Updated: 6/7/2012

Glenn Gandelman, MD, MPH, FACC Assistant Clinical Professor of Medicine at New York Medical College, and in private practice specializing in cardiovascular disease in Greenwich, CT. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.


©  UPMC | Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by A.D.A.M. Health Solutions. All rights reserved.

For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.

UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.

Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.

For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.

© UPMC
Pittsburgh, PA, USA UPMC.com