Cardiac Amyloidosis

Cardiac amyloidosis is a progressive heart disease that causes the heart muscle to stiffen, creating difficulty in pumping blood throughout the body. This is often a precursor to other serious conditions like restrictive cardiomyopathy and heart failure. At Houston Methodist, our heart specialists and amyloidosis experts collaborate to offer world-class cardiac amyloidosis care. The team provides a thorough evaluation and designs a treatment plan specific to your condition and unique needs. Our specialists have access to the latest technology and clinical trials, meaning that we can often offer cardiac amyloidosis treatment options not available at most hospitals.

What Is Cardiac Amyloidosis?

Cardiac amyloidosis is a heart condition that occurs when abnormal proteins deposit in the heart muscle. These deposits affect heart function and can lead to restrictive cardiomyopathy and, ultimately, heart failure.

 

In addition, these deposits can cluster together and make it difficult for electrical signals to move through your heart. This can lead to abnormalities in the heartbeat, known as arrhythmia, and faulty heart signals, also called heart block.

Early diagnosis of cardiac amyloidosis can lead to treatment of the underlying amyloid process and prevent worsening heart failure.

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Types of Cardiac Amyloidosis

There are two types of cardiac amyloidosis, differentiated by the specific protein being deposited in the heart tissue.

AL Amyloidosis

Also called immunoglobulin light-chain amyloidosis, AL cardiac amyloidosis is the primary form of the disease, in which plasma cells in the bone marrow produce misfolded proteins. This can cause fibrils to be deposited in different organs, including the heart, and is sometimes found in multiple myeloma patients. AL amyloidosis can develop quickly, causing significant organ damage and requiring an immediate course of chemotherapy.

ATTR Amyloidosis

ATTR amyloidosis occurs when the protein transthyretin is misfolded and then deposited in the heart. ATTR amyloidosis is further divided into two sub-types:

  • Wild-type ATTR: This is commonly found in men over the age of 65, and was formerly known as “senile systemic amyloidosis.” It happens when normal protein cells misfold due to age.
  • Hereditary ATTR: This is a genetic mutation that produces unstable transthyretin protein cells. 
Why Choose Houston Methodist for Cardiac Amyloidosis Care

Why Choose Houston Methodist for Cardiac Amyloidosis Care

For those who have extensive amyloid deposition in the heart, our specialists are experts in amyloidosis care and, when required, heart transplant. In fact, we are one of the few hospitals that offer heart transplant for patients with end-stage cardiac amyloidosis, and we have some of the best outcomes after transplant for this disease.

Our multidisciplinary healthcare team of cardiologists, hematologists and cardiac surgeons have the expertise and compassion needed to walk beside you on your treatment journey.

Signs & Symptoms of Cardiac Amyloidosis

The particular cardiac amyloidosis symptoms you experience will often depend on the type of amyloid your body is producing, as well as whether these amyloids are being deposited in organs other than your heart. Common symptoms may include:

Fatigue

Extreme tiredness and fatigue, along with reduced stamina and tolerance for physical activity, usually accompany the different forms of amyloidosis. Patients report feeling severe weakness and a diminished ability to perform daily activities.

Shortness of Breath

This is often the first and most noticeable symptom of amyloid heart disease. Patients note that they frequently feel out of breath when walking or moving around, but they also report the same shortness of breath when lying down or resting.

Swelling

Edema, or swelling of the feet, ankles, legs or abdomen is another common sign of amyloidosis. This is a symptom associated with several types of cardiovascular disease, due to the heart’s reduced ability to adequately pump blood through the body, allowing fluid to build up.

Heart Palpitations

Arrhythmias or atrial fibrillation is common in patients with amyloidosis. These palpitations can feel like a racing, fluttering or pounding heartbeat.

Neuropathy

Amyloidosis can cause peripheral neuropathy, or nerve pain, numbness and tingling felt in the hands or feet. This is due to blood flow being limited throughout the body, which then causes oxygen deprivation (hypoxia) that leads to nerve damage.

Carpal Tunnel Symptoms

Symptoms of carpal tunnel syndrome is one of the early signs of cardiac amyloidosis, especially wild-type TTR found in older men. Symptoms may be felt in one or both hands, and include tingling, burning and pain in some or all of the fingers.

How Cardiac Amyloidosis Is Diagnosed

There are various types and causes of amyloidosis, which can make this condition difficult to diagnose and treat.

At Houston Methodist, heart specialists use several imaging procedures to diagnose cardiac amyloidosis:

 

 

We also perform genetic testing to help diagnose TTR amyloidosis, and often use a cardiac tissue biopsy to confirm a cardiac amyloidosis diagnosis.

 

With an early diagnosis, we can often treat the source of cardiac amyloidosis, limiting further damage to your heart. When diagnosis occurs later during disease development, when there is already extensive amyloid deposition in the heart, treatment options are more limited.

Treatment Options for Cardiac Amyloidosis

There are various types of amyloidosis, and the treatment options differ considerably depending on the underlying cause and whether organs other than the heart are involved. 

  • If you have AL amyloidosis, your cardiologist will work closely with your hematologist to treat the underlying cause of your condition.
  • If you have TTR amyloidosis, your cardiologist may recommend a medication that either helps block the protein involved from sticking to your heart or decreases its production altogether.

  • Non-Surgical Treatment

    While the specific treatment course will vary based on the type of cardiac amyloidosis, there are several medications and drug therapies used to treat the disease, including:

    • Stabilizers: Medications like Tafamidis (Vyndamax/Vyndaqel) that bind with the TTR protein to prevent it from misfolding.
    • Silencers: Drugs like vutrisiran (Amvuttra) that keep the liver from producing the TTR protein.
    • Chemotherapy: Drug therapy that targets the plasma cells involved in AL amyloidosis.
    • Diuretics: Medication used to reduce swelling and treat heart failure symptoms.
    • Blood thinners: Anticoagulation drugs used to prevent blood clots, an ongoing risk for amyloidosis patients.
    • Stem cell transplant: Similar to a blood transfusion, in which healthy stem cells are placed into the bloodstream.

  • Surgical Treatment

    When conservative, medication-based treatment is not an option, your cardiologist may recommend one of the following surgical procedures:

     

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