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Gaurang Vaidya: Diagnosis of rare underlying condition could change heart patient outcomes

Cardiac amyloidosis is considered rare.

It is estimated to affect less than 200,000 people in the United States. With improvement in diagnostic methods, more heart failure patients are now being realized to have underlying cardiac amyloidosis.

Cardiac amyloidosis occurs when an abnormal protein – called amyloid – builds up in your heart tissue. This build up makes it hard for your heart to work the way it should. The heart muscles become rigid, which prevents the muscles from relaxing between beats. This prevents blood from getting into your heart.

Dr. Gaurang Vaidya

There are two main types of cardiac amyloidosis: Transthyretin (ATTR) amyloidosis or light chain (AL) amyloidosis. ATTR amyloidosis results from mutated deposits of transthyretin, a protein made by the liver. The two subtypes of ATTR are wild-type amyloidosis, which generally affects people in their 60s or older; and hereditary amyloidosis, which runs in families and typically affects people in their 40s and older. AL amyloidosis is associated with blood cancers, like multiple myeloma. It is not a type of cancer, but it is treated with chemotherapy.

Besides medication, a heart transplant is typically the most effective solution; however, most patients are not candidates because of age and/or medical history.

Research is still ongoing and many patients who were previously diagnosed with heart failure are now being realized to have underlying cardiac amyloidosis as the cause of their heart failure. It is important to make this distinction because treatment for cardiac amyloidosis is completely different and some of the regular heart failure medications are actually harmful in amyloidosis patients. On the other hand, the current treatment for cardiac amyloidosis have been shown to prolong life and preserve quality of life. The only stipulation being the requirement to start these medications early in the course of disease.

UK HealthCare’s cardiac amyloidosis clinic is the only one of its kind in Kentucky.

Patients who seek treatment through the UK HealthCare cardiac amyloidosis clinic are cared for by a team of doctors who specialize in treating cardiac amyloidosis as well as kidney, liver and other organ problems that can come with it. Nurse coordinators will support and guide patients through their care. The clinic can also connect patients with clinical trials that test new therapies and study how the disease progresses. Additionally, the clinic has nuclear pyrophosphate scan technology, which is highly accurate to diagnose cardiac amyloidosis.

Dr. Gaurang Vaidya, M.D., is a University of Kentucky HealthCare cardiologist specializing in advanced heart failure and heart transplantation.

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