Cardiac Amyloidosis Clinic

What is Cardiac Amyloidosis?

Cardiac amyloidosis is a rare and complex condition characterized by the accumulation of abnormal proteins, known as amyloid fibrils, in the heart tissue. These deposits can disrupt the heart’s normal structure and function, leading to a range of cardiovascular issues. While the condition can affect anyone, it is more prevalent in older adults, particularly those with certain underlying conditions.

Types of Amyloidosis

  1. AL Amyloidosis: This is caused by a disorder of plasma cells, where abnormal light chains are produced and accumulate in various organs, including the heart.
  2. ATTR Amyloidosis: This type arises from transthyretin, a protein made by the liver. ATTR amyloidosis can be hereditary (familial) or occur sporadically in older adults.

Symptoms of Cardiac Amyloidosis
Patients with cardiac amyloidosis may experience a range of symptoms, including:

  • Shortness of breath, especially during physical activity
  • Swelling in the legs and ankles
  • Fatigue and weakness
  • Irregular heartbeat (arrhythmias)
  • Chest pain
  • Dizziness or lightheadedness

If you experience these symptoms, it’s crucial to consult a healthcare professional for an accurate diagnosis.

Diagnosis of Cardiac Amyloidosis
Diagnosing cardiac amyloidosis can be challenging due to the overlap of its symptoms with other cardiac conditions. At our clinic, we utilize a comprehensive approach to diagnosis, which may include:

  • Blood Tests: To check for abnormal proteins and assess kidney function.
  • Electrocardiogram (ECG): To detect arrhythmias and assess heart function.
  • Echocardiogram: An ultrasound of the heart that can reveal thickening of the heart walls.
  • Cardiac MRI: A detailed imaging technique that provides information about the structure and function of the heart.
  • Endomyocardial Biopsy: In some cases, a small tissue sample from the heart may be needed to confirm the presence of amyloid deposits.
  • Cardiac MRI: A detailed imaging technique that provides information about the structure and function of the heart.
  • Endomyocardial Biopsy: In some cases, a small tissue sample from the heart may be needed to confirm the presence of amyloid deposits.
  • PYP scan A PYP scan for amyloidosis, specifically using technetium-99m pyrophosphate (Tc-99m PYP), is a diagnostic imaging technique that helps assess the presence and extent of amyloid deposits in the heart and other tissues.

Treatment Options
The treatment of cardiac amyloidosis focuses on managing symptoms and addressing the underlying cause of the amyloid buildup.

Medications

  1. Chemotherapy: For patients with AL amyloidosis, chemotherapy can help reduce the production of abnormal proteins.
  2. Heart Failure Medications: Diuretics, ACE inhibitors, and beta-blockers may be prescribed to manage heart failure symptoms and improve heart function.
  3. New Therapies: Recent advancements have led to the development of targeted therapies that specifically address the amyloid deposits. These may include monoclonal antibodies and small molecule inhibitors.

Lifestyle Modifications
In conjunction with medical treatments, lifestyle changes can play a significant role in managing cardiac amyloidosis:

  • Diet: A heart-healthy diet low in salt can help manage swelling and blood pressure.
  • Exercise: A tailored exercise program can improve cardiovascular health and overall well-being.
  • Regular Monitoring: Ongoing follow-ups are essential to monitor heart function and adjust treatment as needed.
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