As transthyretin amyloid cardiomyopathy (ATTR-CM) progresses, it can cause more serious problems with the heart and other parts of the body. Talking with your doctor about how the condition may progress is an important step in managing it.
ATTR-CM is an often underrecognized condition that causes atypical proteins to build up in the heart, making it harder for the heart to pump blood. The symptoms and progression of the condition can differ from person to person.
Some people may notice symptoms in their
Regardless of when symptoms start, ATTR-CM is a progressive condition, meaning that it tends to worsen over time.
Learning what can cause ATTR-CM and how it may change over time can help you and your doctor recognize signs of progression earlier, guide treatment decisions, and lower the risk of serious heart complications.
Read on to learn more about how ATTR-CM may progress and what to discuss with your doctor.
To understand the possible causes of ATTR-CM, it’s important to know that there are two different types: hereditary (hATTR-CM) and wild-type (wtATTR-CM).
As the name suggests, hATTR-CM is hereditary. It occurs due to a mutation in the transthyretin (TTR) gene. This change leads to atypical protein deposits that buildup in the heart muscle. These proteins may also collect in other major organs and in the nerves.
Symptoms usually develop in older adults, though they can sometimes begin as early as your
wtATTR-CM, on the other hand, isn’t linked to a gene mutation. It
Regardless of the type, both forms of ATTR-CM cause amyloid proteins to deposit in the heart muscle. Over time, this can lead to cardiomyopathy, which is a stiffening of the heart wall that affects how well the heart pumps blood.
Doctors may also diagnose ATTR-CM in people who
Because ATTR-CM is a serious condition, it’s important to be aware of the key signs and symptoms.
In the early stages, ATTR-CM may not cause any noticeable symptoms. People may also experience other symptoms before or along with heart-related ones, particularly in hereditary ATTR. When symptoms do appear, they can
- tingling in the feet or hands
- shortness of breath, even at rest
- swelling in the legs or feet
- chest congestion
As the condition progresses, additional symptoms may include:
- palpitations or irregular heartbeat (arrhythmias)
- increased heart rate
- lightheadedness and falls, which
may be linked to heart rhythm changes - abdominal bloating
- unexplained coughing or wheezing at rest
- confusion
- a new intolerance to blood pressure medications that previously worked
Some clinical signs of ATTR-CM progression that a doctor may identify include:
- orthostatic hypotension (a drop in blood pressure when standing)
- evidence of amyloidosis from a tissue biopsy
- imaging tests showing increased heart wall thickening or enlargement of heart chambers
ATTR-CM is also considered an underdiagnosed cause of heart failure.
Since ATTR-CM worsens over time, it can help to keep track of your symptoms between doctor’s visits. Writing down how you feel each day may help you and your doctor notice patterns or changes. You can use a notebook, a symptom-tracking app, or a computer file — whichever works best for you.
Weigh yourself daily as well. A sudden increase in weight may signal fluid retention, which can be a sign of worsening heart failure.
Regular checkups with a doctor are also important. Be sure to keep copies of your test results, whether in a journal, an online portal, or a binder of printouts.
Having this information in one place helps you and your doctor see how your ATTR-CM is progressing and decide if your treatment plan needs to change.
There’s currently no cure for ATTR-CM, but several treatments can help slow its progression and manage symptoms. Options
- Food and Drug Administration (FDA)-approved medications, such as tafamidis, acoramidis, and vutrisiran, which can help prevent further amyloid deposits in the heart
- heart or liver transplants, which may be considered for some younger adults
Doctors may prescribe diuretics to manage fluid buildup from heart failure and anticoagulants to lower the risk of stroke in people with atrial fibrillation (an irregular heart rhythm), which is common in ATTR-CM.
They may also recommend medications for nerve pain or arrhythmias. While these treatments may not slow the progression of ATTR-CM, they can improve quality of life.
Researchers are also studying
Life expectancy for someone with ATTR-CM can vary. Because the condition is often underdiagnosed or misdiagnosed, researchers are still learning about long-term outcomes.
In general, hATTR-CM is linked to a shorter life expectancy compared with wtATTR-CM. Research suggests that, on average, without treatment, people with hereditary forms live about 2.5 years after diagnosis. This highlights the importance of early detection and treatment to help slow disease progression.
Studies suggest that hATTR-CM may be more aggressive. On average, people with wtATTR-CM live about 3.5 years after diagnosis, while those with ATTR that affects the nerves, but not the heart, may live 8 to 10 years after diagnosis.
Doctors may also check blood markers, such as NT-proBNP (often elevated in heart failure), since higher levels are linked with faster disease progression.
The overall outlook for ATTR-CM can also depend on other factors, such as age at diagnosis and whether amyloid proteins have spread to other organs. Without treatment, ATTR-CM may progress to heart failure and, in severe cases,
While ATTR-CM is a serious condition, earlier diagnosis and newer treatments are helping improve outcomes and giving people more options for managing the disease.
Getting an accurate diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM) is a crucial step toward accessing treatment. Some therapies can help manage symptoms, while others may directly slow disease progression.
If you’ve been diagnosed with ATTR-CM, knowing the signs of progression can help you and your doctor choose the right treatment plan.
Contact your doctor if you notice changes in your symptoms or think your treatment may need adjusting.