Cardiac Amyloidosis

Submitted by Jim Weeks and reviewed by editors 12-27-2011

Cardiac amyloidosis refers to buildup of amyloids in the tissue of the heart. Amyloids are proteins produced in excess that are deposited in different organs and slowly replace normal tissue.  This is also known as "stiff heart syndrome". It is a fairly rare disease with only around 3000 people diagnosed each year. More men are affected than women. Cardiac amyloidosis may change the way the cardiac electrical impulse is transmitted through the heart effecting the hearts rhythm. It can caused the heart to become hard or stiffening.

Cardiac amyloidosis usually occurs during primary amyloidosis. Primary amyloidosis usually accompanies multiple myeloma, a blood disorder in which too much of a certain type of protein is produced. This excess protein is deposited in the heart and in other organs such as the kidney.

Secondary amyloidosis seldom effects the heart. However there is a type of secondary amyloidosis which does involve the heart called senile amyloidosis. As its name suggests it is a form of amyloidosis that effects older people usually in their eighties or nineties. This particular protein is deposited mainly in the heart, but it can also infiltrate blood vessels. Senile cardiac amyloidosis is becoming more common as the average age of the population increases.


Symptoms of Cardiac amyloidosis

• Palpitations (sensation of feeling heart beat)

• Swelling of legs, ankles, or other portion of the body

• Excessive urination at night

• Fatigue, reduced activity tolerance

• Shortness of breath with activity

• Breathing difficulty while lying down

Diagnosis of Cardiac Amyloidosis

It is difficult to diagnosis of cardiac amyloidosis since early symptoms can resemble other forms of cardio myopathy.

The initial exam usually reveals lung crackles, heart murmurs, or other abnormal sounds. Durring the exam the doctor may notice that the liver may be enlarged and neck veins may be distended. Sometimes the doctor may do a blood pressure test both sitting and standing because blood pressure may be low or may drop when rising to a standing position.

Besides the symptoms you may notice the main diagnostic symptoms are heart enlargement, congestion of the lungs or the veins in the lungs, decreased movement and/or functioning of the heart, heart failure.

Your doctor may use a number of test in the diagnostic process.

• Echocardiogram

• Chest x-ray

• Chest and or abdomen CT scan

• Coronary angiography

• Nuclear heart scans

• Magnetic resonance imaging (MRI)

Typically a doctor will ask for an Electrocardiography test  when Cardiac amyloidosis is suspected. This test may reveal conduction disturbances, arrhythmias such as atrial fibrillation, ventricular tachycardia, or premature and ectopic beats.

An echocardiogram may be used to assess the thickness of the heart wall, the size of the chambers in the heart, and the ability of the heart to fill and pump blood. Sometimes an unusual texture of the heart muscle or the endocardium (the lining of the heart) can be seen as well.

Since many of the symptoms can be found in other heart conditions you doctor may have a cardiac biopsy performed. If this biopsy reveals amyloids it will confirm the diagnosis.

Treatment of Cardiac Amyloidosis

Cardiac amyloidosis can not be cured and there are limited treatments. Life span when diagnosed is usually under 3 years.

Physical activity is good for any one with any form of cardio myopathy and should continue as long as the patient can tolerate it. With any type of heart condition that limits the amount of blood flowing through the heart water builds up in your system. You doctor will ask you to limit your salt intake. To help remove excess water from your body your doctor will probably recommend you take a diuretics(water pill). Digoxin may be used cautiously to improve heart control in patients with atrial fibrillation. As a precaution you doctor may ask you to weigh yourself daily. A weight gain of 3 or 4 pounds or more over 1 or 2 days can indicate excessive fluid accumulation.

Some people benefit from chemotherapy or prednisone. Chemotherapy is used to attack fast growing cells. There has been some success in slowing down cardiac amyloidosis with different chemo drugs. Prednisone is used to reduce swelling and inflammation associated with this condition.

When cardiac amyloidosis effects the heart beat then a pacemaker is needed to keep the heart beat regular.

Depending on the type of amyloidosis that is causing the damage to the heart a heart transplant may be suggested. This is only available for the types if amyloidosis that do not affect other organs.

Complications of Cardiac Amyloidosis

• Congestive heart failure

• Atrial fibrillation or ventricular arrhythmias

• Sick sinus syndrome

• Symptomatic cardiac conduction system disease (irregular beating of the heart)

• Low blood pressure and dizziness from excessive urination

• Increased sensitivity to digoxin with the potential for digoxin toxicity and related arrhythmias

• Ascites (fluid accumulation in the abdomen)

More Information on Amyloidosis

Primary	Symptoms	lichen	macular
systemic	kidney	familial	coughing
picture	secondary	cardiac	hereditary