Cardiac amyloidosis (patient information)

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 * Associate Editors-In-Chief: Jinhui Wu, MD. Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]

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 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

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,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

Who is at highest risk?
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

Diagnosis
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 options
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