UNIVERSITY OF MINNESOTA | Amyloidosis Program
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AMYLOIDOSIS & THE HEART: Cardiac Amyloidosis
AMYLOIDOSIS & THE HEART: Cardiac Amyloidosis
Cardiac amyloidosis occurs when abnormal protein deposits - called amyloid - build up in the heart muscle. Over time, these deposits make the heart stiff and less able to fill and pump blood effectively.
Amyloid forms when certain proteins fold incorrectly and accumulate in tissues. The body cannot easily break down these misfolded proteins, allowing them to build up in organs such as the heart.
Our Advanced Heart Failure and Transplant Cardiology team provides specialized evaluation and treatment for patients with cardiac involvement from amyloidosis.
What Happens to the Heart?
What Happens to the Heart?
Amyloid deposits can affect:
The heart muscle
Heart valves
The heart’s electrical system
Blood flow within the heart
Over time, this may lead to heart failure or abnormal heart rhythms.
How is Cardiac Amyloidosis Diagnosed?
How is Cardiac Amyloidosis Diagnosed?
Blood and Urine Tests
Evaluate kidney, liver, and heart function and detect abnormal light chains associated with AL (light chain) amyloidosisBlood and Urine Tests
Imaging
Tests such as an echocardiogram, cardiac MRI, and PYP scan evaluate heart structure and help diagnose transthyretin (ATTR) amyloidosis.Imaging
Biopsy
A biopsy is not always needed, but may be required if imaging and blood test results are unclear.Biopsy
Genetic Testing
All patients with ATTR amyloid should have genetic testing to determine whether the disease is inherited.Genetic Testing
Symptoms of Cardiac Amyloidosis
Symptoms of Cardiac Amyloidosis
Common symptoms:
Shortness of breath
Fatigue
Swelling in legs or abdomen
Irregular heartbeat
Chest discomfort
Dizziness or fainting
Other symptoms:
Weight loss
Weakness
Numbness or tingling (especially in ATTR)
Types of Cardiac Amyloidosis
Types of Cardiac Amyloidosis
There are many forms of amyloid, but over 95% of cardiac amyloidosis is caused by one of these two:
ATTR (Transthyretin Amyloidosis)
ATTR (Transthyretin Amyloidosis)
Caused by instability of a protein called transthyretin (TTR)
May be inherited or non-inherited
Often affects the heart and nerves
AL (Light Chain Amyloidosis)
AL (Light Chain Amyloidosis)
Caused by abnormal plasma cells in the bone marrow
Produces harmful light chain proteins
Often affects the heart, kidneys, nerves, and liver
The type of amyloidosis determines which treatments are recommended.
Treatment of Cardiac Amyloidosis
Treatment of Cardiac Amyloidosis
Treatment has two goals:
Reducing amyloid production
Managing heart failure symptoms
Symptom Treatment
Symptom Treatment
Diuretics (“water pills”) to reduce swelling
Careful medication adjustments
Some standard heart medications may not be tolerated
Amyloid-specific Treatment
Amyloid-specific Treatment
Transthyretin Amyloidosis (ATTR)
Transthyretin Amyloidosis (ATTR)
ATTR happens when the transthyretin protein becomes unstable and forms amyloid.
Two forms of ATTR:
Two forms of ATTR:
Wild-Type ATTR: not inherited, usually seen in older adults, mostly affects the heart
Hereditary ATTR: caused by a genetic mutation, can affect heart, nerves, kidneys, and digestion, runs in families
Genetic testing is the only way to tell the difference.
ATTR Treatments:
ATTR Treatments:
Acoramidis (Attruby) or Tafamidis (Vyndamax) stabilizes the TTR protein so it cannot form amyloid.
Taken by mouth
Slows heart damage
Reduces hospitalizations and death
Vutrisiran is an injectable RNA therapy that lowers TTR production.
Given every 3 months
Approved for hereditary ATTR with nerve disease
Being studied for heart involvement
Light Chain Amyloidosis (AL)
Light Chain Amyloidosis (AL)
AL amyloidosis comes from abnormal plasma cells in the bone marrow that make harmful light chains.
AL Treatment:
AL Treatment:
Chemotherapy to stop light chain production
Sometimes stem cell (bone marrow) transplant
Close care with a hematologist/oncologist
Early treatment improves survival.
Most patients with cardiac amyloidosis are treated with medications and careful management of heart symptoms. In certain situations, additional advanced therapies may be considered. These options are evaluated on an individual basis by our multidisciplinary team.
Heart transplant (selected patients)
Liver transplant (rare hereditary ATTR cases)
Supportive care (nutrition, physical therapy, symptom control) is also important.
Cardiac Amyloidosis Clinic
Cardiac Amyloidosis Clinic
Advanced Heart Failure & Transplant Cardiology
Clinic Location: M Health Fairview Clinics and Surgery Center
Clinic: 612-365-5000
Fax: 612-676-5051
After Hours: 612-273-3000
Support and Resources
Support and Resources
Amyloidosis Support Groups: https://www.amyloidosissupport.org
One Amyloid Voice: https://www.oneamyloidosisvoice.com
Mackenzie’s Mission: https://mm713.org
Amyloid Research Consortium: https://arci.org
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