Cardiac Amyloidosis

The University of Kansas Health System offers leading expertise in the diagnosis and treatment of cardiac amyloidosis. We provide the region’s first and only multidisciplinary amyloid program, which includes a dedicated cardiac amyloid program.

Amyloidosis can take several different forms. It is diagnosed in only about 200,000 people in the United States each year, which can make it challenging to identify. Yet, as each disease variation requires specialized care, an accurate diagnosis is crucial to achieving a positive outcome.

Cardiac amyloidosis is a heart disorder caused by deposits of an abnormal protein called amyloid. When abnormal proteins are deposited into the heart, it reduces the heart’s normal function and ability to pump blood. Signs of cardiac amyloidosis may include:

• Bilateral carpal tunnel syndrome
• Diarrhea or constipation
• Dizziness
• Fatigue
• Heart failure symptoms, such as shortness of breath and swelling in hands, arms, legs and feet
• Joint pain
• Kidney dysfunction
• Loss of appetite
• Low blood pressure
• Nausea or vomiting
• Tingling or pain in hands and feet
• Weakness

Cardiac amyloidosis can be inherited. It is more common in men than women. It is rare in people under age 40. Cardiac amyloidosis can also occur because of other conditions, such as blood disorders and bone cancer.

The types of amyloidosis that can affect the heart include:

Each disease type affects different body parts, causing distinct symptoms. Many are common symptoms often associated with other conditions. Symptoms of cardiac amyloidosis may include:

• Diarrhea or malnutrition, caused by amyloid deposits in the gut
• Fatigue
• Heart palpitations
• Shortness of breath
• Swelling of the abdomen, legs, ankles or other part of the body
• Thickened heart tissue
• Trouble standing, tingling in the arms and hands or the development of carpal tunnel syndrome, caused by amyloid protein deposits in the nerves

Amyloid accumulation in the heart can lead to heart failure and heart rhythm problems, including fast, irregular or slow heart rates. Other cardiac risks may include:

• Atrial fibrillation, an irregular and rapid heart rate that increases the risk of strokes, heart failure and other heart-related complications
• Congestive heart failure, which occurs when the heart doesn’t pump blood as well as it should
• Heart conduction disorders, such as symptomatic cardiac conduction system disease
• Inability to tolerate cardiac medications for heart failure treatment
• Sick sinus syndrome, or the inability of the heart’s natural pacemaker to create a normal heart rate
• Ventricular arrhythmias, or abnormal heartbeats that originate in the lower heart chambers

Our cardiovascular experts provide the latest testing and diagnostics. Diagnostic tests may include:

• Cardiac MRI
• Echocardiography with strain imaging
• Electrodiagnostic testing to test the function of muscles and nerves
• Genetic testing
• Scintigraphy, an imaging technique to identify amyloid proteins
• Tissue pathology of the nerves, muscle, heart, liver or abdominal fat pad

Our specialists perform more than 280 scintigraphy tests each year to diagnose and treat cardiac amyloidosis. They have the expertise and highest experience in the region to recognize and treat this rare disease.

Our highly experienced team has administered the most specialized treatments in the region. We offer all FDA-approved treatment options for both wild-type ATTR amyloidosis and hereditary amyloidosis. Treatment options may include:

• Changes to your diet, such as limiting salt and fluids
• Gene silencer therapies for hereditary ATTR amyloidosis
• Implantable cardioverter defibrillator or pacemaker
• Transthyretin protein stabilizer for wild-type ATTR amyloidosis

Because we are an academic health system, our team participates in many national clinical trials for advanced treatments. Patients can benefit from our knowledge about the latest cardiac amyloidosis treatment options. They may even have the potential to take part in a clinical trial.

The cardiac amyloidosis program at The University of Kansas Health System is one of the nation’s top programs for treatment of this disease. We offer a team of cardiology experts, physicians, advanced practice providers, nurses and pharmacists with unparalleled amyloidosis treatment experience. Amyloid education, as well as early diagnosis and treatment initiation, are the top goals of our care team. We also work with the experts at The University of Kansas Cancer Center to treat other types of amyloidosis.

Our program has expertise in working with insurance providers to obtain authorization prior to medication treatment. Our team has helped 100% of our patients receive insurance approval for specialty medications. Our team also helps patients who cannot afford specialty medication access available grants and resources for copayment assistance.

Our team includes a disease-specific nurse navigator, 2 clinical pharmacists, a medication assistance coordinator, social workers, clinical nurse coordinators and advanced practice providers.