Cardiac Amyloidosis Program

Consultations with Ochsner Health amyloidosis specialists are complemented by a variety of diagnostic tests for cardiac amyloidosis, which may include:

• An echocardiogram with strain imaging
• An electrocardiogram (EKG) - a recording of the heart's electrical activity
• Blood tests
• An MRI or technetium pyrophosphate scan (PYP Scan)
• A cardiac biopsy
• Genetic testing

A PYP scan, offered exclusively at Ochsner Health in the region, is available as a non-invasive alternative to a biopsy for diagnosing cardiac amyloidosis in certain patients.

At Ochsner Health, our cardiologists and care team will work closely with you to develop a personalized treatment plan tailored to your specific needs and the type of amyloidosis you have. Below are the specialized treatments we offer based on the type of amyloidosis, which may be adjusted based on your personalized care plan:

1. For AL amyloidosis
   1. High-dose chemotherapy combined with a stem cell transplant
2. For Wild-type TTR amyloidosis (TTRwt)
   1. Tafamidis (a protein stabilizer), commercially known as Vyndamax
3. For Hereditary TTR amyloidosis (hTTR)
   1. A medication called tafamidis (a protein stabilizer)
   2. Gene silencers, such as Onpattro or Amvuttra, if polyneuropathy is present
   3. Heart transplant
   4. Liver transplant

Supportive care for all types of amyloidosis may include

To help manage symptoms and improve quality of life, we also offer:

• Diuretics
• Low-salt diet
• Blood-thinning medication
• Medication to control your heart rate
• Compression stockings
• Occupational and physical therapy for polyneuropathy
• ENT care for enlarged tongues or swallowing difficulties

A breakthrough drug therapy, Vyndamax (tafamidis meglumine), is the first FDA-approved treatment in the United States for cardiac amyloidosis. It is specifically approved for treating cardiomyopathy caused by wild-type or hereditary transthyretin-mediated amyloidosis (ATTR-CM) in adults, helping to reduce cardiovascular mortality and hospitalizations. This innovative treatment is available at Ochsner Health.

Your care plan is unique, and treatments may evolve based on your condition and the latest advancements in cardiac amyloidosis care.

Ochsner Health is actively involved in ongoing clinical trials focused on advancing treatments for amyloidosis. Our team conducts research across various aspects of this condition, continually working to improve care and outcomes. Ask your doctor for more information about current clinical trials.

Cardiac amyloidosis is often underdiagnosed or misdiagnosed. If your patient is not responding to treatment and presents with symptoms such as carpal tunnel syndrome, shortness of breath or an echocardiogram showing ventricular wall thickness, consider referring them to an amyloidosis specialist at Ochsner Health. Our team is dedicated to providing expert care and will keep you informed throughout the patient’s treatment journey.

For more information or to make a referral, contact the John Ochsner Heart and Vascular Institute at 504-842-4135 and ask to speak with someone about cardiac amyloidosis.

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.

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.

To learn more about cardiac amyloidosis, visit the amyloidosis foundation webpage.

There are approximately 30 different types of amyloidosis. Hidden heart failure can run in families, which is a genetic risk factor.

The different types and their symptoms are:

1. AL amyloidosis (immunoglobulin light chain amyloidosis) - AL amyloidosis can affect your heart, kidneys, skin, nerves, liver and GI tract. It was previously known as primary amyloidosis. AL is caused by an abnormal protein developed in the bone marrow.
   1. Symptoms of AL amyloidosis:
      1. An irregular heartbeat
      2. Shortness of breath
      3. Numbness, tingling or pain in your wrist (carpal tunnel syndrome), or feet this can lead to a ligament rupture in arm (Biceps rupture) (does occur in AL, but mostly occurs in TTR)
      4. Easy bruising, including purplish patches around the eye
      5. Frequent dizziness while change in the position (Orthostatic hypotension)
      6. Weakness and severe fatigue
      7. An enlarged tongue
      8. Difficulty swallowing
      9. Diarrhea and constipation (possibly with blood as the disease progresses)
      10. Significant, unintentional weight loss
2. TTR amyloidosis - there are two types: hereditary and wild-type. Both types of TTR amyloidosis are caused by an abnormal protein where 95% is developed in the liver and 5% is developed in the eyes.
   1. Hereditary amyloidosis (hTTR) - also known as familial amyloidosis, affects your heart, nerves, GI tract and autonomic system. This type is due to a mutated gene.
      1. Symptoms:
         1. An irregular heartbeat
         2. Shortness of breath
         3. Numbness, tingling or pain in your wrist (carpal tunnel syndrome), or feet this can lead to a ligament rupture in arm (Biceps rupture) (does occur in AL, but mostly occurs in TTR)
         4. Frequent dizziness while change in the position (Orthostatic hypotension)
         5. Weakness and severe fatigue
         6. Severe chronic back pain (Lumbar spinal stenosis)
         7. Poor functioning heart valve (Aortic stenosis)
         8. Diarrhea and constipation (possibly with blood as the disease progresses)
         9. Significant, unintentional weight loss
   2. Wild-type ATTR (ATTRwt) - once called senile systemic amyloidosis and senile cardiac amyloidosis, is related to the aging process. It’s prevalent in older age, seen in ages of 60 and beyond but mostly in 70- to 80-year-olds. Deposits of TTR can be found throughout the body but most commonly in the heart. This type happens spontaneously through old age.
      1. Symptoms:
         1. An irregular heartbeat
         2. Shortness of breath
         3. Numbness, tingling or pain in your wrist (carpal tunnel syndrome), or feet this can lead to a ligament rupture in arm (Biceps rupture) (does occur in AL, but mostly occurs in TTR)
         4. Frequent dizziness while change in the position (Orthostatic hypotension)
         5. Weakness and severe fatigue
         6. Severe chronic back pain (Lumbar spinal stenosis)
         7. Poor functioning heart valve (Aortic stenosis)
         8. Diarrhea and constipation (possibly with blood as the disease progresses)
         9. Significant, unintentional weight loss

While cardiac amyloidosis can develop in anyone, certain factors increase the likelihood of being affected:

1. Age
   1. AL amyloidosis can begin as early as age 35 in both men and women
   2. TTR amyloidosis typically develops around age 60. Men are more likely to develop TTR at a younger age, while women tend to be diagnosed later, though not exclusively.
2. Sex
   1. Nearly 70% of individuals with AL amyloidosis are men
   2. Approximately 75% of those with wild-type amyloidosis are men
3. Genetics
   1. Hereditary amyloidosis is caused by a mutated gene passed down through families
4. Race
   1. Research indicates that individuals of African descent are at a higher risk of carrying a genetic mutation linked to hereditary amyloidosis.