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.
Amyloidosis – cardiac; Primary cardiac amyloidosis – AL type; Secondary cardiac amyloidosis – AA type; Stiff heart syndrome; Senile amyloidosis
Causes, incidence, and risk factors
Cardiac amyloidosis usually occurs during primary amyloidosis (AL type amyloidosis). Primary amyloidosis is often seen in people with
Cardiac amyloidosis (“stiff heart syndrome”) occurs when amyloid deposits take the place of normal heart muscle. It is the most typical type of
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.
Some patients may have no symptoms. Those who do may experience:
Excessive urination at night Fatigue, reduced activity tolerance Palpitations(sensation of feeling heart beat) Shortness of breathwith activity Swelling of legs, ankles, or other part of the body (See: abdominal swelling) Trouble breathing while lying down
Signs and tests
It can be difficult to diagnose cardiac amyloidosis, because the signs can be related to a number of different conditions.
Signs may include:
Abnormal sounds in the lung (lung crackles) or a heart murmur
Blood pressure that is low or drops when you stand up
Enlarged neck veins
The following tests may be performed to help diagnose cardiac amyloidosis:
Chest or abdomen CT scan(this is considered the “gold standard”)
Coronary angiography Echocardiogram Magnetic resonance imaging(MRI) Nuclear heart scans(MUGA, RNV)
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.
Other treatments may include:
Implantable cardioverter-defibrillator (AICD)
Pacemaker, if there are problems with heart signals
Prednisone, an anti-inflammatory medicine
Cardiac amyloidosis is a long-term (chronic) condition that slowly gets worse. On average, persons with cardiac amyloidosis live less than 1 year.
Atrial fibrillationor ventricular arrhythmias Congestive heart failure
- Fluid buildup in the abdomen (
- Increased sensitivity to digoxin
- Low blood pressure and dizziness from excessive urination (due to medication)
Sick sinus syndrome Symptomaticcardiac conduction system disease (arrhythmias related to abnormal conduction of impulses through the heart muscle)
Calling your health care provider
Call your health care provider if you have this disorder and new symptoms develop, particularly:
Dizzinesswhen you change position
- Excessive weight (fluid) gain
- Excessive weight loss