Cardiac amyloidosis leads to a thick, stiff heart also called a restrictive cardiomyopathy, that requires specialized treatment.
For decades, the team at Emory Healthcare has been at the forefront of cardiac amyloidosis care.
Cardiac Amyloidosis
Comprehensive Care for Cardiac Amyloidosis
Why Choose Emory Healthcare?
Hundreds of patients turn to Emory Healthcare for cardiac amyloidosis treatment. Here, you can expect:
- A program led by a heart failure and transplant cardiologist
- Access to the latest treatments, including clinical trials
- Supportive care from a team of specialists in cardiology, gastroenterology, genetics, hematology, neurology and orthopaedic surgery
What is Cardiac Amyloidosis
Cardiac amyloidosis occurs when a certain type of protein builds up and gets stuck inside the heart. This abnormal protein build-up (called amyloid) causes the heart to thicken and become stiff.
Over time, hearts affected by cardiac amyloidosis stop working properly. This can lead to congestive heart failure and other heart-related conditions like atrial fibrillation and heart valve disease.
Types of Cardiac Amyloidosis
There are two main types of cardiac amyloidosis. They are:
- Light Chain (AL) Amyloidosis: The rarest and most aggressive form of cardiac amyloidosis. AL starts with plasma cells in the bone marrow producing abnormal, toxic proteins called light chains. These light chains deposit in tissues across the body involving the heart in 50-70% of cases. AL has no known cause. It can affect people of any age, but it is most common in people over 50. AL involving the heart is typically co-managed by hematologists to treat the underlying disease and cardiologists to manage heart failure symptoms.
- Transthyretin (ATTR) Amyloidosis: The more common and slower progressing type of cardiac amyloidosis, ATTR starts with misfolding of the transthyretin protein made by the liver. There are two types of ATTR: hereditary and wild-type.
Hereditary (also referred to as Variant) TTR is caused by a mistake in your DNA that impacts your ability to correctly make a certain type of protein (TTR). This genetic type of ATTR generally affects people over age 50.
Wild-type ATTR has no known cause, but it is linked to advanced age. People typically receive a diagnosis of wild-type ATTR when they are over 70. New ATTR treatments help people live longer with fewer symptoms.
Symptoms of Cardiac Amyloidosis
Symptoms of cardiac amyloidosis vary based on your type of amyloidosis and how far your condition has progressed. Most people who experience cardiac amyloidosis report symptoms of congestive heart failure and some other heart conditions. These include:
- Fast or slow heartbeat
- Fluttering, pounding, or racing heartbeat
- Sensations of feeling smothered or suffocated when lying down
- Shortness of breath
- Sudden waking to gasp or catch your breath
- Swelling in the legs that does not go away
The protein build-up caused by amyloidosis can affect more than just the heart. If you have cardiac amyloidosis, you may experience symptoms of other conditions, including:
- Bicep tendon rupture
- Carpal tunnel
- Gastrointestinal problems (including severe constipation or diarrhea)
- Neuropathy
- Spinal stenosis
Diagnosing Cardiac Amyloidosis
A cardiac amyloidosis diagnosis often starts with an abnormal cardiac imaging test. Your doctor may notice signs of amyloidosis during a heart ultrasound (echocardiogram) or heart MRI (magnetic resonance imaging) test, including thickened heart muscle.
If doctors suspect cardiac amyloidosis, they will order a blood test to check for AL. If you have AL, our team will partner with Winship Cancer Institute of Emory University hematologists to conduct additional tests and begin treatment planning.
If blood tests find no signs of AL, your doctor will order a nuclear PYP (pyrophosphate) scan to check for ATTR.
During a nuclear PYP scan, doctors inject safe amounts of radioactive dye into your blood. This dye binds to the type of amyloid found in people with ATTR. Doctors then take pictures of the heart to see if the dye pools there, which indicates the presence of amyloid.
If you have ATTR, doctors will order genetic testing to determine if your ATTR is genetic or wild-type. This will help guide your treatment plan and inform family members of their risk.
Cardiac Amyloidosis Treatments
Emory Healthcare offers the latest treatment options for cardiac amyloidosis, including heart transplantation and access to cutting-edge clinical trials of new medications and therapies.
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