Diagnosis of rare heart disease could change the outcome | Columns

Cardiac amyloidosis is considered rare.

It is estimated to affect fewer than 200,000 people in the United States. With the improvement of diagnostic methods, more and more patients with heart failure suffer from underlying cardiac amyloidosis.

Cardiac amyloidosis occurs when an abnormal protein – called amyloid – builds up in heart tissue. This buildup makes it difficult for your heart to function as it should. The heart muscles become rigid, which prevents the muscles from relaxing between beats. This prevents blood from entering your heart.

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

Besides drugs, a heart transplant is usually the most effective solution; however, most patients are not candidates due to their age and / or medical history.

Research is still ongoing and many patients who were previously diagnosed with heart failure now realize that underlying heart amyloidosis is the cause of their heart failure. It is important to make this distinction because the treatment for heart amyloidosis is completely different and some of the usual heart failure medications are actually harmful in patients with amyloidosis. On the other hand, current treatment for cardiac amyloidosis has been shown to prolong life and preserve quality of life. The only condition being the obligation to start these drugs at the onset of the disease.

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

Patients seeking treatment at the UK HealthCare Cardiac Amyloidosis Clinic are cared for by a team of doctors who specialize in the treatment of cardiac amyloidosis as well as problems with the kidneys, liver and other organs that may cause it. ‘to accompany. The nurse coordinators will support and guide patients throughout their care. The clinic can also connect patients with clinical trials that test new therapies and study disease progression. In addition, the clinic has nuclear pyrophosphate scanning technology, which is very accurate in diagnosing cardiac amyloidosis.

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