What is Cardiac Amyloidosis?

Cardiac amyloidosis (CA) is an uncommon, but potentially deadly, heart condition. Also called “stiff heart syndrome,” this is a disorder in which abnormal proteins, called amyloids, accumulate in the tissues of the heart. As these protein deposits build up over time, the heart tissue deteriorates, which can cause heart failure if the condition is not treated. It may also cause arrhythmias, because the amyloids disrupt the heart's electrical signals.

This condition tends to affect more men than women, particularly those over the age of 40. While some patients may not notice any symptoms at all, others experience fatigue and reduced stamina, as well as swelling of the legs and frequent nighttime urination. Shortness of breath may occur, particularly with activity, and patients may have difficulty breathing normally while they are lying down. Heart palpitations can also occur, which are rapid or skipped heartbeats.

A doctor may suspect cardiac amyloidosis during a physical examination that reveals hypotension, or low blood pressure, particularly when the patient stands up. Neck veins may also be enlarged. The doctor may hear a heart murmur or unusual sounds in the lungs. If cardiac amyloidosis is suspected, a number of tests may be ordered for a diagnosis. These can include an echocardiogram, a nuclear heart scan, and imaging tests, as well as a tissue biopsy.

Patients who are diagnosed with cardiac amyloidosis must taken action immediately to enlist a specialist, as the prognosis is almost always grim. Since this condition is rare, seeing a specialist with a great deal of experience treating this disorder is highly recommended. The doctor will likely prescribe drugs for immediate management of cardiac amyloidosis, such as beta blockers, digoxin, and calcium channel blockers. Diuretics, or water pills, can help get rid of extra fluid. Patients may also take anti-inflammatory medications, such as prednisone.

The doctor may also recommend chemotherapy medications. Those who have disturbed electrical signals may need a pacemaker. An implantable cardioverter-defibrillator is also an option. Some patients may be candidates for heart transplant surgery. In addition to these treatment options, the doctor may recommend psychological counseling for patients coping with this diagnosis.

Cardiac amyloidosis may also cause other medical problems. Patients should be aware that potential complications can include atrial fibrillation, heart failure, and excessive fluid in the abdomen. A heart rhythm disorder and arrhythmias may occur. Often, patients experience heightened side effects from the medications, which can include dizziness and low blood pressure.