Cardiac amyloidosis (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Jinhui Wu, MD

Associate Editor-In-Chief: Ujjwal Rastogi, MBBS [2]

What is 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.

What are the symptoms of cardiac amyloidosis?

Most patients with cardiac amyloidosis may experience symptoms restrictive cardiomyopathy. While other may show no symptoms.

• Palpitations
• Shortness of breath, orthopnea
• Fatigue
• Increased nocturia
• Swelling of legs, ankles, or other part of the body

Diseases with similar symptoms

• Coronary artery disease
• Constrictive pericarditis
• Dilated cardiomyopathy
• Hypertrophic cardiomyopathy
• Restrictive cardiomyopathy
• Sarcoidosis cordis

Who is at risk for cardiac amyloidosis?

Amyloidosis refers to a buildup of clumps of amyloids in body tissues and organs. These proteins called amyloids slowly replace normal tissue and resuly in organ function failure. Amyloidosis often occurs on 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 either of the following symptoms, seeking urgent medical care as soon as possible:

• Palpitations
• Severen shortness of breath
• Orthopnea

Treatment

Your physician may run the following test to confirm the diagnosis

• ECG: 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, 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.
• Thoracoabdominal CT scan
• Magnetic resonance imaging (MRI)
• Nuclear heart scans
• Coronary angiography
• Cardiac biopsy

Treatment effect of cardiac amyloidosis is not good. The purpose of treatment is to decrease the synthesis and deposition of the abnormal protein and protect heart function.

• Diet: A diet of salt and fluid restrictions 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 decomposition of the abnormal protein and relieve patients' side effects. Prednisone is usually used with 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. Usual drugs include dilthiazem, metoprolol, bisoprolol, and carvedilol. These drugs should be monitored 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 gets worse. 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

Sources

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

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