Amyloidosis is a family of rare diseases that occur when an abnormal protein called amyloid builds up inside the organs. Amyloid is commonly created in the bone marrow, allowing it to be deposited into any tissue or organ. Cardiac amyloidosis occurs when the amyloid proteins deposit into the cardiac tissues, making it difficult for the heart to function properly.
Types
There are three types of amyloidosis; primary, secondary and familial. Primary amyloidosis is the most common type of amyloidosis and can affect many areas including the heart, lungs, kidneys, liver, spleen, nerves, intestines, blood vessels, skin and tongue. The MayoClinic.com explains that primary amyloidosis is not often associated with another disease, with the exception of multiple myeloma, a form of bone marrow cancer.
Secondary amyloidosis occurs in conjunction with a long-term infectious or inflammatory disease such as osteomyelitis or tuberculosis. According to the University of Maryland Medical Center, secondary amyloidosis rarely affects the heart with the exception of a form of secondary amyloidosis known as senile amyloidosis that affects the heart and blood vessels. Familial amyloidosis is an inherited form of amyloidosis that often affects the liver, nerves, heart and kidneys.
Causes
Cardiac amyloidosis occurs when amyloid deposits take the place of normal heart muscle, causing a disease known as restrictive cardiomyopathy. Cardiac amyloidosis may also occur with senile amyloidosis. Senile amyloidosis causes deposits in the heart and blood vessels due to a different protein, according to the University of Maryland Medical Center.
Symptoms
People with cardiac amyloidosis may exhibit symptoms such as excessive nighttime urination, fatigue related to activity and palpitations. Other symptoms may include shortness of breath with activity and trouble breathing while lying down. The National Institutes of Health explains that swelling of legs, ankles and possibly the abdomen may also indicate cardiac amyloidosis.
Diagnosis
Cardiac amyloidosis can mimic many other diseases and so physicians may conduct a number of tests. Physicians will commonly look for abnormal sounds in the lungs or an unusual swishing sound in the heart, low blood pressure or pressure that drops from standing, enlarged neck veins and a swollen liver. Patients may undergo a computed tomography scan of the chest and abdomen, an echocardiogram, magnetic resonance imaging or nuclear heart scan. In some cases, cardiac biopsy may be necessary to confirm diagnosis.
Treatment
Patients may need to take medications to help control the symptoms associated with cardiac amyloidosis. Physicians may prescribe water pills to remove excess fluid as well as digoxin, calcium channel blockers and beta blockers to help the heart function properly. Other possible treatments may include chemotherapy, a pacemaker, corticosteroids and an implantable cardioverter-defibrillator, according to the University of Maryland Medical Center.
Prognosis
Cardiac amyloidosis is a chronic condition that will only worsen over time. The National Institutes of Health explains that the average life expectancy of people with cardiac amyloidosis is less than a year.
