Transthyretin Amyloid Cardiomyopathy (ATTR-CM)
Transthyretin amyloid cardiomyopathy (ATTR-CM), also called cardiac amyloidosis, is a type of heart disease that causes the heart muscle to become thicker, stiffer and larger than normal. It affects the heart’s ability to pump blood and, in some individuals, leads to heart failure.
The experts at UH Harrington Heart & Vascular Institute offer contemporary treatments and symptom management approaches to help patients with ATTR-CM have a better quality of life and live longer.
Your health is important. Get expert care.
To schedule an appointment with a specialist who treats ATTR-CM, call 216-844-5437.
Find a DoctorWhat Causes ATTR-CM?
The condition occurs when a protein called transthyretin produced by the liver does not form correctly. The abnormal proteins, called amyloids, circulate in the bloodstream and accumulate in the body’s organs and tissues. When amyloid proteins collect in the heart muscle, it causes thickening and stiffness in the left ventricle.
The most common causes of ATTR-CM are aging and/or genetics and there is no evidence to suggest that lifestyle factors are responsible for its development. Older individuals and those with pre-existing heart conditions like left ventricular hypertrophy and heart failure may be at greater risk.
Types of ATTR-CM
There are two types of ATTR-CM:
- Wild (wATTR). The most common form of the disease, which typically occurs as a result of aging and is more common in men.
- Hereditary (hATTR). Caused by a gene mutation that is passed down through families, hATTR affects both men and women. There are over 120 gene mutations that can cause hATTR, some of which only affect certain populations such as those of African or Irish descent.
Symptoms of Transthyretin Amyloid Cardiomyopathy
The most common symptom associated with ATTR-CM is shortness of breath, particularly when at rest. Other symptoms include:
- Swelling in the legs, ankles and feet.
- Irregular or increased heart rate.
- Heart palpitations.
- Numbness and tingling in the hands and feet.
- Fatigue.
- Digestive issues like diarrhea, constipation or nausea.
- Coughing or wheezing.
How Is ATTR-CM Diagnosed?
The symptoms associated with ATTR-CM can mimic other medical conditions and some people have no symptoms. As result, ATTR-CM is often not diagnosed until the condition is advanced. People with symptoms or risk factors should see a heart specialist for a full evaluation. Diagnostic tests may include:
- Nuclear Scintigraphy
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The most effective, non-invasive test for ATTR-CM, this specialized scan creates detailed images of the heart to detect the presence of amyloid proteins.
- Electrocardiogram (ECG)
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A non-invasive test that measures the electrical activity of the heart by recording the signals it produces.
- Transthoracic Echocardiogram
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A non-invasive test that uses ultrasound waves to produce an image of the heart. In addition to diagnosing heart attacks, blood clots and heart valve disease, it can identify heart enlargement and stiffening of the heart muscle.
- Cardiac Magnetic Resonance (CMR) Scan
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A non-invasive test that uses magnetic radio waves to generate a detailed image of the heart and detect the presence of amyloid proteins.
- Biopsy
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A biopsy of heart tissue can diagnose ATTR-CM if a patient isn’t a candidate for less invasive diagnostic tests.
If tests confirm a diagnosis of ATTR-CM, genetic testing will be recommended to determine the type. This is done using blood or saliva samples.
Treatment of ATTR-CM
Treatment of ATTR-CM is most effective when it’s diagnosed early.
The medication tafamidis is approved by the FDA to treat ATTR-CM by preventing the build-up of amyloid proteins in the heart. It can slow the progression of the disease but it can’t reverse or cure it.
Other treatment options to manage symptoms and improve quality of life may include:
- Following a healthy, low-sodium diet.
- Diuretic medication to reduce fluid retention.
- Anti-arrhythmic medication to regulate heart rate.
- Correction of irregular heartbeat with minimally invasive procedures such as catheter ablation.
If hereditary-type ATTR-CM is confirmed, family members may be advised to seek genetic counseling to learn how to best manage their risk factors. Some patients with hereditary-type ATTR-CM may benefit from a liver transplant but it would not be a treatment option for those with wild-type ATTR-CM.
Our ATTR-CM Team
Eiran Gorodeski, MD, MPH
(4.8), - Cardiology-Heart Failure, Cardiology
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Beachwood (7 mi.)
Cleveland (0 mi.)
Cleveland (0 mi.)
Beachwood (7 mi.)
Beachwood (7 mi.)
Mentor (19 mi.)
Monique Robinson, MD, PhD
(4.9), - Cardiology, Cardiology-Heart Failure
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Ravenna (29 mi.)
Cleveland (0 mi.)
Cleveland (0 mi.)
South Euclid (4 mi.)
Beachwood (7 mi.)
Richmond Heights (8 mi.)
Bedford (8 mi.)
Ravenna (29 mi.)
Michael Zacharias, DO
- Cardiology-Heart Failure, Cardiology
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Westlake (18 mi.)
Cleveland (0 mi.)
Cleveland (0 mi.)
Cleveland (0 mi.)
Beachwood (7 mi.)
Thomas O'Neill, MD
- Cardiology, Cardiology-Heart Failure
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Cleveland (0 mi.)
Beachwood (7 mi.)
Beachwood (7 mi.)
Parma (11 mi.)
Fairlawn (25 mi.)
Elyria (28 mi.)
Medina (29 mi.)
Robert Montgomery, MD
- Cardiology-Heart Failure, Cardiology
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Cleveland (0 mi.)
Cleveland (0 mi.)
South Euclid (4 mi.)
Beachwood (7 mi.)
Beachwood (7 mi.)
Mentor (19 mi.)
Chardon (21 mi.)
Concord Twp (22 mi.)
Ravi Ramani, MD
- Cardiology-Heart Failure, Cardiology
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Parma (11 mi.)
Cleveland (0 mi.)
Cleveland (0 mi.)
Beachwood (7 mi.)
Elyria (28 mi.)