Cardiac amyloidosis is a rare disease that occurs when proteins called amyloids build up in the heart. Currently, there is no cure for the disease, but there are treatments that aim to slow the disease and improve quality of life.
Factors such as early detection and diagnosis may help improve treatment outcomes. New treatments are also being explored. These new treatments are increasing the likelihood of a better prognosis (outlook) for those with cardiac amyloidosis.
It has been difficult to find effective treatments and potential cures for cardiac amyloidosis for several reasons.
Cardiac amyloidosis is very rare, affecting around 4,000 people every year in the United States. Because the condition is so rare, it’s difficult to study, as there are fewer diagnosed people to participate in clinical trials.
Cardiac amyloidosis damages the heart muscle by making it thick, stiff, and weak. This causes restrictive cardiomyopathy or “stiff heart syndrome,” which can lead to heart failure symptoms, including shortness of breath, fatigue, and swelling.
The overlapping symptoms with different heart failure causes make amyloidosis more difficult to diagnose correctly. Recent research has led scientists to believe that some cases of heart failure from the past may have been caused by cardiac amyloidosis. To distinguish cardiac amyloidosis symptoms from other heart failure causes, doctors must be well aware of the possibility of amyloidosis and then conduct several tests, which may include a biopsy to confirm amyloidosis.
Although there is currently no cure, new treatments have become available over the past decade. Better treatment options have also increased the chances of survival.
The approach to treating cardiac amyloidosis depends on the form of amyloidosis affecting the heart. Two main types of amyloidosis are responsible for 95 percent of cardiac amyloidosis:
AL and ATTR amyloidosis have different root causes, so they are treated and managed slightly differently. However, all treatment aims to slow the production of amyloids and address heart damage.
AL amyloidosis is the most common type of amyloidosis in the United States and can affect the heart, leading to cardiac amyloidosis. In people with AL amyloidosis, their plasma cells (white blood cells in the bone marrow) produce too much of an antibody protein called immunoglobulin light chain. Too much production of these proteins causes them to clump together and form amyloid deposits or fibrils, which may damage affected organs.
AL amyloid deposits in the heart may cause arrhythmias (heart rhythm problems), and in some cases, heart failure. These are serious conditions that need to be treated quickly. Damage to the heart is often difficult to treat and may be irreversible. Early detection and diagnosis of cardiac AL amyloidosis can increase the chances of successful treatment.
There are several options for treating AL amyloidosis, including medications and stem cell transplantation. The cause of AL amyloidosis is abnormal plasma cells, so treatments aim to kill and slow the growth of those cells.
ATTR amyloidosis can be hereditary or wild-type. The hereditary form is caused by a genetic mutation (change) in the transthyretin gene, while the wild-type form occurs without a mutation and typically affects older adults. Cardiac ATTR develops when amyloid deposits build up in the heart and interfere with its ability to pump blood effectively.
In the past, ATTR was considered untreatable, but there have been major breakthroughs that provide options for people with the condition. As with AL amyloidosis, treatment options for ATTR aim to improve damage to the heart and address amyloid abnormalities.
These are the main treatments for AL and ATTR amyloidosis.
Cardiac AL amyloidosis may be treated with different types of medications, including chemotherapy and immunomodulators. In some cases, combinations of different medications may prove to be the most beneficial.
In 2021, the U.S. Food and Drug Administration (FDA) approved a drug to treat people newly diagnosed with AL amyloidosis: daratumumab plus hyaluronidase (Darzalex Faspro). This medication is used in combination with other medications.
Other medications include:
To make treatment more effective, these drugs are usually given in combination with a steroid such as dexamethasone. Some of these drugs are still being studied for AL amyloidosis and may be available through a clinical trial. Talk to your healthcare team about the best treatment if you have cardiac AL amyloidosis.
Autologous stem cell transplantation is another type of therapy to treat AL amyloidosis. An autologous stem cell transplant, or bone marrow transplant, is a procedure in which healthy cells are removed from your body before you undergo chemotherapy. After chemotherapy, the healthy stem cells are infused back into your body to help replace the damaged cells.
This treatment targets the root cause of abnormal protein production and may do more to improve the disease in the long term. However, only a few people with AL amyloidosis are considered eligible for the procedure. For all others, drug treatment is often a better option.
Heart disease and heart failure should be managed carefully, as the usual medications for these conditions in people without amyloidosis (including beta-blockers, ACE inhibitors, and calcium channel blockers) can be harmful to some people with ATTR. Talk to your doctor about which medications will be most helpful and effective for addressing heart damage resulting from ATTR amyloidosis.
Several medications have been approved to treat cardiac ATTR in the last decade:
These medications work to prevent the build up of amyloid deposits in the heart. While some of these treatments share the same indication, they don’t necessarily work in the same way.
Because most ATTR amyloidosis is caused by the production of amyloid deposits in the liver, liver transplantation may also be helpful to reduce or stop amyloid production. Liver transplant isn’t always a favored treatment and doesn’t always stop amyloid buildup, so it’s important to discuss your options with your doctor based on your type of ATTR. Some people with ATTR and a severely damaged heart may need both heart and liver transplantation.
Early detection, diagnosis, and treatment are important for improving outcomes in people with cardiac amyloidosis. Although the condition is severe, new treatments are improving the quality of life and survival in people with cardiac amyloidosis.
Another important consideration for treatment is your team of healthcare providers. You’ll want to assemble a team of specialists who have experience treating amyloidosis, including cardiologists (heart doctors), hematologists (blood doctors), rheumatologists (joint, muscle, and bone doctors), and neurologists (brain and spinal cord doctors). You may also want to seek a referral to an amyloidosis treatment center.
MyAmyloidosisTeam is the social network for people with amyloidosis and their loved ones. On MyAmyloidosisTeam, members come together to ask questions, give advice, and share their stories with others who understand life with amyloidosis.
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Hi I Am Taking Attruby ,I Have Heriditary Amylodosis, Although My Biopsy In April 2025, Wasn't Too Goof, Doctor Suggest Try Amvuttra.
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My cardiologist recommended Lasix, 80 MG daily. Result frequent trips to the bathroom!
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