Cardiac amyloidosis occurs when abnormal proteins called amyloids build up and form deposits in your heart, essentially taking the place of normal heart muscle. This buildup causes the heart to become stiff, reducing the heart's ability to fill with blood between heartbeats. Cardiac amyloidosis can also affect the kidneys, nerves, blood, bowel, rectum, liver and mouth.

While there is no cure, treatments available at Ochsner can limit the production of amyloid protein and help you manage your symptoms. We have diagnostic equipment for amyloidosis you won’t find elsewhere in the Gulf South, and our specialists are among the most experienced.

Click here to learn more about amyloidosis.

Led by renowned cardiac amyloidosis expert Dr. Clement Eiswirth, Jr., Ochsner is home to an experienced, multidisciplinary team with specialties in cardiology, cardiac pathology, gastroenterology, hematology, nephrology, neurology and cardiac research–Ochsner Medical Center faculty members all with extensive experience in cardiac amyloidosis. Part of the Ochsner Heart and Vascular Center, we are committed to educating patients and physicians and spreading awareness about this disease. There is no reason to travel outside the region for leading-edge care.

Hematology

Andrew Dalavisio, MD
Carter Davis, MD
Laura Finn, MD

Cardiology

Clement Eiswirth, MD
Selim Krim, MD
Hector Ventura, MD

Hidden heart failure can run in families, which is a genetic risk factor. There are other symptoms of amyloidosis, which include:

• An irregular heartbeat
• Shortness of breath
• Numbness, tingling or pain in your wrist (carpal tunnel syndrome), or feet
• Thickening of the skin or easy bruising, including purplish patches around the eye
• Frequent dizziness while change in the position (Orthostatic hypotension)
• Weakness and severe fatigue
• Ligament rupture in arm (Biceps rupture)
• An enlarged tongue
• Severe chronic back pain (Lumbar spinal stenosis)
• Poor functioning heart valve (Aortic stenosis)
• Diarrhea (possibly with blood)
• Difficulty swallowing
• Significant, unintentional weight loss

There are approximately 30 different types of amyloidosis. They include:

AL amyloidosis (immunoglobulin light chain amyloidosis) 
AL amyloidosis can affect your heart, kidneys, skin, nerves and liver. It was previously known as primary amyloidosis.

AA amyloidosis 
This form of amyloidosis mostly affects your kidneys but can also affect your digestive tract, liver or heart. It was previously known as secondary amyloidosis. People with chronic infectious or inflammatory diseases, such as rheumatoid arthritis or inflammatory bowel disease, are at risk.

Hereditary amyloidosis
Also known as familial amyloidosis, this type affects the liver, nerves, heart and kidneys.

Wild-type ATTR (ATTRwt)
Wild-Type was once called Senile Systemic Amyloidosis and Senile Cardiac Amyloidosis, even though it’s not directly related to dementia but simply to aging.It’s prevalent old age. Deposits of TTR amyloid can be found throughout the body, but most commonly in the heart. Carpal tunnel syndrome is an early symptom.

Dialysis-related amyloidosis
People on long-term dialysis are at risk for amyloidosis. Proteins in the blood are deposited in joints and tendons, resulting in pain, stiffness and fluid in the joints, as well as carpal tunnel syndrome.

While it’s true that anyone can develop amyloidosis, risk factors include:

Age

Although early onset does occur, most people with the most common type of amyloidosis (AL amyloidosis) are between 60 and 70 years old

Sex
Nearly 70 percent of people with AL amyloidosis are men.

Certain Health Factors
If you have a chronic infectious or inflammatory disease, this increases your risk of AA amyloidosis.

Genetics
Some forms of amyloidosis are hereditary.

Race
Research shows that people of African descent are at higher risk of carrying a genetic mutation associated with cardiac amyloidosis.

Kidney Dialysis
If you're on dialysis, abnormal proteins can build up in your blood and eventually be deposited in tissue. The good news is this condition is less common with modern dialysis techniques.

Along with consultations with Ochsner amyloidosis specialists, tests for cardiac amyloidosis may include:

• An echocardiogram
• Blood tests
• An MRI
• A cardiac biopsy
• Genetic testing
• Echocardiogram with Strain Imaging
• Technetium Pyrophosphate Scan (PYP Scan)

Ochsner is the only medical center in the region where you can get a PYP scan, an alternative to biopsy for diagnosing cardiac transthyretin (ATTR) amyloidosis in some patients.

Your individualized treatment plan will depend on the type of amyloidosis you have as well as the source of the amyloid production.

AL amyloidosis

• high-dose chemotherapy with stem cell transplant

AA amyloidosis

• anti-inflammatory medication (steroids)

ATTR amyloidosis

• a medication called tafamidis
• heart transplant

Hereditary amyloidosis

• liver transplant
  

Dialysis-related amyloidosis

• changing your mode of dialysis
• kidney transplant

Supportive care may include:

• pain medication
• diuretics
• low-salt diet
• blood-thinning medication
• thickeners to add to fluid if you have trouble swallowing
• medication to control your heart rate
• compression stockings

A new drug, Vyndaqel (tafamidis meglumine), is a breakthrough drug therapy for cardiac amyloidosis, which makes it the first drug in the U.S. to be approved to treat amyloidosis. It's the first to treat cardiomyopathy of wild-type or hereditary transthyretin-mediated amyloidosis (ATTR-CM). In addition to Vyndaqel, the FDA has approved another formulation of tafamidis, Vyndamax (tafamidis). The two drugs are approved for ATTR-CM in adults to reduce cardiovascular mortality and cardiovascular-related hospitalization. This treatment is available at Ochsner.

ONPATTRO & TEGSEDI

• the treatment of polyneuropathy associated with amyloidosis

In 2018, research was done at medical centers, including Ochsner, and it showed that a drug called tafamidis had good results in people with ATTR amyloidosis. Patients on tafamidis lived longer, stayed healthier longer and went to the hospital less frequently as a result of heart problems. Moreover, onpattro and tegsedi are the drugs for the treatment of polyneuropathy associated with this cardiac condition.

The Ochsner team is currently conducting research in a variety of aspects of amyloidosis. Ask your doctor for more information.

Click here for our current clinical trials.

Cardiac amyloidosis tends to be underdiagnosed or misdiagnosed. If your patient isn’t responding to treatment and has carpal tunnel syndrome, shortness of breath, and/or has had an echocardiogram that shows a ventricular wall thickness, refer them to an amyloidosis specialist at Ochsner. Our team will keep you informed every step of the way. For more information, call 504-842-4135.

Diagnosing cardiac amyloidosis is complicated. Symptoms can be vague and similar to those of other common diseases. Since amyloids can lead to life-threatening organ failure, receiving an accurate diagnosis promptly is critical. To make an appointment, call 504-842-4135 or visit my.ochsner.org.