Cardiac amyloidosis (patient information)

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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]

Cardiac amyloidosis is a disorder caused by deposits of an abnormal protein (amyloid) in the heart tissue, which makes it hard for the heart to work properly. Involvement of the heart can result in loss of consciousness, abnormal heart rhythms, shortness of breath, fatigue, swelling of the legs, weight loss, nausea, dizziness, and disrupted sleep.

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

• Skipped heart beats or extra heart beats also known as Palpitations
• Shortness of breath, or difficulty breathing while lying flat also known as orthopnea
• Fatigue
• Increased urination at night also known as nocturia
• Swelling of the ankles, the legs, or other part of the body

• Diseases with similar symptoms:

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

• 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 amyloidosis) 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.

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 diseases or characteristics:

• Multiple myeloma: Cardiac amyloidosis usually occurs in patients with multiple myeloma.
• Age over 40: The disease is rare in people under age 40.

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:

• Skipped heart beats or extra heart beats also known as palpitations
• Severe shortness of breath, or difficulty breathing while lying flat also known as orthopnea

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 irregular heart beats also known as arrhythmia such as extra beats from the larger chambers of the heart also known as ventricular premature beats, extra beats from the smaller chamber of the heart also known as atrial fibrillation or abnormal conduction of the impulses through the heart also known as heart block.
• Echocardiogram: This is also a painless test for patients which can be used to assess both the structure and function of the heart. Patients with cardiac amyloidosis may have a thickening of the heart muscle a condition known as left ventricular hypertrophy and stiffness of the heart, areas that light up showing amyloid deposition in the heart called an echogenic focus, and decreased heart pumping also known as a reduced ejection fraction.
• Chest x-ray: On chest x-ray, patients with cardiac amyloidosis may show an enlarged heart and fluid in the lungs also known as pulmonary congestion.
• Thoraco-abdominal CT scan
• Magnetic resonance imaging (MRI)
• Nuclear heart scans
• Coronary angiography
• Cardiac biopsy

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.

Directions to Hospitals Treating cardiac amyloidosis

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

• Irregular heart beating in the small chamber of the heart also known as atrial fibrillation or irregular heart beating in the large chamber of the heart also known as ventricular arrhythmias
• Failure of the heart to push blood forward to the body also known as congestive heart failure
• Fluid buildup in the abdomen also known as ascites
• Alternating slow and fast heart beating also known as Sick sinus syndrome

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

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