Living with ATTR-CM
- When your heart becomes too weak to effectively pump blood throughout the body, you will need to make a number of lifestyle changes.
- Shifting to a heart-healthy diet that includes limited sodium intake and fats will help you achieve and maintain a healthy weight.
- Be sure to discuss your exercise limits and concerns with your doctor or a cardiac rehab specialist.
A diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM) is a serious, life-changing event, in that it confirms you have a chronic heart condition that will require significant lifestyle changes and the likely prospect of medications and procedures for the rest of your life. However, the diagnosis also means there is finally an explanation for your symptoms and that your doctor now has a specific condition to target with treatments moving forward.
What is ATTR-CM?
ATTR-CM can be a precursor to heart failure. It occurs when the protein transthyretin, which transports thyroid hormone and vitamin A in the bloodstream, becomes misshapen and forms amyloid fibrils that settle in the heart. As a result, the left ventricle—the heart’s primary pumping chamber—stiffens and becomes unable to relax sufficiently to fill with blood or pump enough blood out of the heart to meet the body’s needs.
Amyloid may also build up in other organs and the nervous system, creating systemic health problems. ATTR-CM is considered a rare condition, though its actual prevalence is hard to estimate. “ATTR cardiomyopathy is, unfortunately, an underdiagnosed condition,” says New York cardiologist Aeshita Dwivedi, MD, who adds that greater awareness of ATTR-CM is leading to more diagnoses, which is also resulting in people starting treatment earlier in the disease process.
Prognosis of ATTR-CM
Because ATTR-CM is often misdiagnosed or diagnosed long after symptoms first appear, it has been difficult to establish a reliable life expectancy for the condition if it is diagnosed and treated early in the disease progression. A 2019 study on the natural history and outcomes of ATTR-CM, suggest that life expectancy may be anywhere from three to 10 years after diagnosis, and that a late diagnosis often corresponds to poor quality of life measures.
But those numbers may be improving. Until recently, ATTR-CM treatment was largely focused on managing heart failure symptoms and associated complications, such as arrhythmia. But in 2019, the FDA approved tafamidis (Vyndamax), a once-daily oral medication that blocks the breaking up of transthyretin proteins and the subsequent formation of amyloid fibrils.
While tafamidis doesn’t cure the disease, evidence suggests that it can significantly slow its progress. A 2018 study of tafamidis suggests that the drug helped reduce the risk of death by 30 percent over the 30-month study period and cut the risk of hospital admission by nearly one-third. The study also found that tafamidis helped slow the decline in everyday functioning associated with ATTR-CM.
And a 2021 study suggests that a timely diagnosis and treatment with tafamidis could extend life expectancy by 5.46 years for people with wild-type (non-hereditary) ATTR-CM and by 7.76 for individuals the hereditary form of ATTR-CM.
Living with a Weakened Heart
While tafamidis and other treatments may help extend life and improve quality of life for people with ATTR-CM, there are still nothing that can reverse the impact of the condition on the heart and anywhere else the amyloid fibrils have settled.
And when your heart becomes too weak to effectively pump blood throughout the body, you will need to make a number of lifestyle changes. Some of what you will need to know can be learned by participating in a cardiac rehabilitation program. If you have been diagnosed with ATTR-CM ask your doctor about prescribing you rehab to learn about diet, exercise, medication adherence and other aspects of living with a heart condition.
Dr. Dwivedi explains that when confronted with the signs of ATTR-CM, like those of heart failure, it’s essential that you begin working closely with your cardiologist and follow the doctor’s advice about medication, checkups, lifestyle, and give thoughtful consideration to discussions of procedures, such as having a pacemaker or pump implanted.
“Number one would be to see your doctor regularly,” Dr. Dwivedi says. “It is important to seek medical care for this condition, because we have good options and can change lives. Second would be talk to your doctor if any of your symptoms change. You would rather know sooner rather than later, before things get worse and when you may need hospitalization.”
She also recommends shifting to a heart-healthy diet that includes limited sodium intake and fats will help you achieve and maintain a healthy weight. Exercise is also important, though your capacity for physical activity will be limited. Be sure to discuss your exercise limits and concerns with your doctor or a cardiac rehab specialist.
“Getting your steps in for the day and doing moderate activities very important,” Dr. Dwivedi says. If you like your doctor can also refer you to cardiac rehabi, which can be a supervised exercise program for you to get comfortable being active. It’s also so important to take your medications. The medications have been shown to benefit this patient population significantly and can change lives.”
End-stage Heart Failure
Treating the heart failure-like symptoms of ATTR-CM usually involve medications that help the burden on the heart and lower blood pressure or boost the pumping strength of the heart.
Over time, devices such as an implantable cardioverter defibrillator and a ventricular assist device (a mechanical pump that helps improve circulation) may be necessary to support healthy heart function. But for some people medications and devices still can’t do enough.
When the heart has reached this point, the condition is known as end-stage heart failure, and for many people with ATTR-CM this is a likely destination. This stage can be made more complicated if ATTR-CM has resulted in amyloid in the liver or other organs.
“In the worst case scenario, you might be looking at a heart transplant,” says New York cardiologist Philip Weintraub. “But it becomes more difficult to enroll someone in a heart transplant that has multi-organ diseases because the heart transplant surgeon has to then be certain that if I transplant the heart, that I’m not going to run into difficulties with the other organ systems because of the fact that one needs to be immunosuppressed.”
Questions to Ask Your Doctor
- Should I join a cardiac rehabilitation program?
- Are there specific changes in diet and exercise I need to make?
- How will my ATTR-CM be monitored?
Learn more about SurvivorNet's rigorous medical review process.
James Roland has been a health journalist since 2007, not long after his father died of non-Hodgkin’s lymphoma. Read More
Living with ATTR-CM
- When your heart becomes too weak to effectively pump blood throughout the body, you will need to make a number of lifestyle changes.
- Shifting to a heart-healthy diet that includes limited sodium intake and fats will help you achieve and maintain a healthy weight.
- Be sure to discuss your exercise limits and concerns with your doctor or a cardiac rehab specialist.
A diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM) is a serious, life-changing event, in that it confirms you have a chronic heart condition that will require significant lifestyle changes and the likely prospect of medications and procedures for the rest of your life. However, the diagnosis also means there is finally an explanation for your symptoms and that your doctor now has a specific condition to target with treatments moving forward.
What is ATTR-CM?
ATTR-CM can be a precursor to heart failure. It occurs when the protein transthyretin, which transports thyroid hormone and vitamin A in the bloodstream, becomes misshapen and forms amyloid fibrils that settle in the heart. As a result, the left ventricle—the heart’s primary pumping chamber—stiffens and becomes unable to relax sufficiently to fill with blood or pump enough blood out of the heart to meet the body’s needs.
Read More Amyloid may also build up in other organs and the nervous system, creating systemic health problems. ATTR-CM is considered a rare condition, though its actual prevalence is hard to estimate. “ATTR cardiomyopathy is, unfortunately, an underdiagnosed condition,” says New York cardiologist
Aeshita Dwivedi, MD, who adds that greater awareness of ATTR-CM is leading to more diagnoses, which is also resulting in people starting treatment earlier in the disease process.
Prognosis of ATTR-CM
Because ATTR-CM is often misdiagnosed or diagnosed long after symptoms first appear, it has been difficult to establish a reliable life expectancy for the condition if it is diagnosed and treated early in the disease progression. A 2019 study on the natural history and outcomes of ATTR-CM, suggest that life expectancy may be anywhere from three to 10 years after diagnosis, and that a late diagnosis often corresponds to poor quality of life measures.
But those numbers may be improving. Until recently, ATTR-CM treatment was largely focused on managing heart failure symptoms and associated complications, such as arrhythmia. But in 2019, the FDA approved tafamidis (Vyndamax), a once-daily oral medication that blocks the breaking up of transthyretin proteins and the subsequent formation of amyloid fibrils.
While tafamidis doesn’t cure the disease, evidence suggests that it can significantly slow its progress. A 2018 study of tafamidis suggests that the drug helped reduce the risk of death by 30 percent over the 30-month study period and cut the risk of hospital admission by nearly one-third. The study also found that tafamidis helped slow the decline in everyday functioning associated with ATTR-CM.
And a 2021 study suggests that a timely diagnosis and treatment with tafamidis could extend life expectancy by 5.46 years for people with wild-type (non-hereditary) ATTR-CM and by 7.76 for individuals the hereditary form of ATTR-CM.
Living with a Weakened Heart
While tafamidis and other treatments may help extend life and improve quality of life for people with ATTR-CM, there are still nothing that can reverse the impact of the condition on the heart and anywhere else the amyloid fibrils have settled.
And when your heart becomes too weak to effectively pump blood throughout the body, you will need to make a number of lifestyle changes. Some of what you will need to know can be learned by participating in a cardiac rehabilitation program. If you have been diagnosed with ATTR-CM ask your doctor about prescribing you rehab to learn about diet, exercise, medication adherence and other aspects of living with a heart condition.
Dr. Dwivedi explains that when confronted with the signs of ATTR-CM, like those of heart failure, it’s essential that you begin working closely with your cardiologist and follow the doctor’s advice about medication, checkups, lifestyle, and give thoughtful consideration to discussions of procedures, such as having a pacemaker or pump implanted.
“Number one would be to see your doctor regularly,” Dr. Dwivedi says. “It is important to seek medical care for this condition, because we have good options and can change lives. Second would be talk to your doctor if any of your symptoms change. You would rather know sooner rather than later, before things get worse and when you may need hospitalization.”
She also recommends shifting to a heart-healthy diet that includes limited sodium intake and fats will help you achieve and maintain a healthy weight. Exercise is also important, though your capacity for physical activity will be limited. Be sure to discuss your exercise limits and concerns with your doctor or a cardiac rehab specialist.
“Getting your steps in for the day and doing moderate activities very important,” Dr. Dwivedi says. If you like your doctor can also refer you to cardiac rehabi, which can be a supervised exercise program for you to get comfortable being active. It’s also so important to take your medications. The medications have been shown to benefit this patient population significantly and can change lives.”
End-stage Heart Failure
Treating the heart failure-like symptoms of ATTR-CM usually involve medications that help the burden on the heart and lower blood pressure or boost the pumping strength of the heart.
Over time, devices such as an implantable cardioverter defibrillator and a ventricular assist device (a mechanical pump that helps improve circulation) may be necessary to support healthy heart function. But for some people medications and devices still can’t do enough.
When the heart has reached this point, the condition is known as end-stage heart failure, and for many people with ATTR-CM this is a likely destination. This stage can be made more complicated if ATTR-CM has resulted in amyloid in the liver or other organs.
“In the worst case scenario, you might be looking at a heart transplant,” says New York cardiologist Philip Weintraub. “But it becomes more difficult to enroll someone in a heart transplant that has multi-organ diseases because the heart transplant surgeon has to then be certain that if I transplant the heart, that I’m not going to run into difficulties with the other organ systems because of the fact that one needs to be immunosuppressed.”
Questions to Ask Your Doctor
- Should I join a cardiac rehabilitation program?
- Are there specific changes in diet and exercise I need to make?
- How will my ATTR-CM be monitored?
Learn more about SurvivorNet's rigorous medical review process.
James Roland has been a health journalist since 2007, not long after his father died of non-Hodgkin’s lymphoma. Read More
