Cardiac amyloidosis (patient information)
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Cardiac amyloidosis |
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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:
- Palpitations
- Shortness of breath, orthopnea
- Fatigue
- Increased nocturia
- Swelling of the legs, ankles, or other part of the body
- 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.
Cardiac amyloidosis usually occurs during 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 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.
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:
- Multiple myeloma: Cardiac amyloidosis usually occurs in patients with multiple myeloma.
- Male gender: Cardiac amyloidosis is more common in men than in women.
- Age over 40: The disease is rare in people under age 40.
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:
- EKG: This is a basic test for cardiovascular diseases that records the heart's electrical activity. During this test, you are asked to lie down. The health care provider will clean several areas on your arms, legs, and chest, and attach electrodes to these areas. Then, the electrical activities of your heart will be recorded. ECG may help your doctor identify the type of your arrhythmia, such as ST-segment depression, ventricualr premature beats, tachycardia or heart block.
- Echocardiogram: This is also a painless and no risk test for patients. Cardiac echo can demonstrate both structure and function of the heart. Patients with cardiac amyloidosis may appear signs of ventricular wall hypertrophy and stiffness, several cardiac echogenic focus, and decreased ejection fraction.
- Chest x-ray: On chest X-ray, patients with cardiac amyloidsis may demonstrate images of enlarged heart and pulmonary congestion.
- Thoraco-abdominal CT scan
- Magnetic resonance imaging (MRI)
- Nuclear heart scans
- Coronary angiography
- Cardiac biopsy
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 function of heart.
- 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 desolution 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
- Atrial fibrillation or ventricular arrhythmias
- Congestive heart failure
- Fluid buildup in the abdomen (ascites)
- Sick sinus syndrome