Cardiac Amyloidosis

Definition

Definition of 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.

Symptoms

Symptoms of Cardiac Amyloidosis

1. Excessive urination at night
2. Fatigue, reduced activity tolerance
3. Palpitations (sensation of feeling heart beat)
4. Shortness of breath with activity
5. Swelling of legs, ankles, or other part of the body (See: abdominal swelling)
6. Trouble breathing while lying down

Causes

Causes of 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).

Diagnosis

Diagnosis of Cardiac Amyloidosis
The following tests may be performed to help diagnose cardiac amyloidosis:

1. Chest or abdomen CT scan (this is considered the "gold standard")
2. Coronary angiography
3. Echocardiogram
4. Magnetic resonance imaging (MRI)
5. Nuclear heart scans (MUGA, RNV)

Treatment

Treatment of Cardiac Amyloidosis
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.

Prognosis

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

Prevention

Prevention of Cardiac Amyloidosis
Consult with your doctor.