The prognosis for amyloid heart disease depends on the type of amyloids involved, how widespread the impact is, and how developed the condition is when treatment is started. Depending on these variables, life expectancy with this condition can range from three months to ten years.
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What is cardiac amyloidosis?
Amyloids are faulty proteins made in the bone marrow that can deposit in the organs and impair function. Cardiac amyloidosis occurs when there is a buildup of these abnormal proteins in the heart. Amyloidosis heart conditions can be more dangerous than other forms of amyloidosis, but these proteins can cause significant problems for any organs when they accumulate.
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What are cardiac amyloidosis symptoms?
Not every case will have symptoms, and some may come and go. Severe and progressed cases often have more extreme symptoms. End-stage cardiac amyloidosis symptoms mimic those of heart failure. Potential symptoms of cardiac amyloidosis can include:
- Difficulty breathing while prone
- Excessive urination at night
- Fatigue after minimal exertion
- Heart palpitations
- Cardiomyopathy
- Lightheadedness
- Shortness of breath
- Swelling of the abdomen or extremities
What causes cardiac amyloidosis?
Amyloidosis of the heart occurs from the buildup of faulty proteins within the heart. But the cause lies behind the creation or formation of these abnormal proteins. The creation of these amyloids can either be genetic or acquired.
ATTR amyloidosis
ATTR amyloidosis is an inherited type of cardiac amyloidosis cardiac amyloidosis. In these cases, the liver produces an abnormal form of transthyretin. When the transthyretin protein is malformed in this way, it can develop into fibrous chunks that attach to the organs, impeding their function.
AL amyloidosis
Al amyloidosis is an acquired type. Most cases of cardiac amyloidosis in the United States involve AL amyloidosis. With this type, the plasma cells within your bone marrow create faulty proteins and release them into your body.
Dialysis-related amyloidosis
The other type of acquired case is when your body builds up too much of an otherwise healthy protein and cannot filter it down to a safe level. This type most commonly develops after years of dialysis treatment for other conditions.
What are the risk factors of cardiac amyloidosis?
Cardiac amyloidosis is relatively rare and generally not preventable. Cardiac amyloidosis risk factors are often outside of our control. The more commonly accepted risk factors include.
- Age: People over 40 are more likely to develop cardiac amyloidosis, with most diagnoses occurring in patients 50 or older.
- Country of origin: Some populations have a higher rate of an associated genetic mutation that leads to inherited amyloidosis. Several European countries, Finland, Japan, Portugal, and Sweden are known to have higher rates of this genetic variance.
- Prolonged dialysis treatment: Being treated for dialysis increases the risk of developing cardiac amyloidosis symptoms. However, this often takes years of dialysis before occurring. Specialized dialysis filters can help reduce, but not remove, this risk.
- Race: Because of the inheritable nature of some types of amyloidosis, this condition can be more common in some genetic demographics. Individuals of West African descent are at greater risk of developing inherited amyloidosis.
- Sex: Cardiac amyloidosis is more common in males. Acquired types of amyloidosis are significantly more common in males.
How is cardiac amyloidosis diagnosed?
The primary tests for a cardiac amyloidosis diagnosis are cardiac biopsies and technetium pyrophosphate scans. A biopsy takes a small cutting from the heart to be analyzed and tested. A technetium pyrophosphate scan is an imaging test that uses dye to highlight buildups of transthyretin amyloids. Depending on your case and the type of amyloids behind your condition, the physician may also utilize other tests.
Other common types of cardiac amyloidosis diagnostic tests include the following:
- Blood analysis: Some types of cardiac amyloidosis involve amyloids circulating within the bloodstream in a form that blood tests can detect. Identifying the amyloid at fault can help inform the diagnosis and the treatment.
- Electrocardiogram (ECG): ECGs use electrodes temporarily attached to the chest's surface to detect your heart's electrical signals.
- Echocardiogram: These devices use echolocation to create an image of your heart.
- Genetic testing: Genetic tests can help identify if you carry one of the genetic mutations responsible for inherited amyloidosis.
- Urinalysis: In some cases, amyloids can collect in your urine. Testing the urine can determine which type of amyloids are involved and inform a treatment plan.
How is cardiac amyloidosis treated?
Cardiac amyloidosis treatment can vary between cases, with extreme conditions potentially requiring a heart transplant. Curing this condition is generally not possible, although early detection and appropriate treatments can significantly reduce your associated risks. Depending on the type of amyloid responsible for your cardiac amyloidosis, your physician can recommend several treatments. Often, cardiac amyloidosis requires a multidisciplinary approach to both non-surgical and surgical treatments.
- Anti-arrhythmia medications
- Chemotherapy
- Dialysis with specialized filters
- Diuretic medications
- Fibril inhibitor medications
- Genetic silencer medications
- Immunotherapy
- Stem cell transplant
- Stabilizer medications
- Heart transplant
- Implanted cardioverter defibrillator
- Implanted pacemaker
- Kidney transplant
- Liver transplant
How to prevent cardiac amyloidosis
For the most part, cardiac amyloidosis cannot be prevented. The exception is dialysis-related amyloidosis, which is most likely to develop after prolonged dialysis treatment. Avoiding dialysis may not be viable if you need it to treat another severe condition.
There is still much to learn about acquired cardiac amyloidosis. As such, medical experts are not yet clear on preventing this condition. With inherited amyloidosis, it may be even more complicated since the root cause is within your DNA. As current medical science and technology stand, this inherited condition cannot yet be removed or corrected within the DNA.
Living with cardiac amyloidosis
Cardiac amyloidosis prognoses can be grim. This condition can be treated and managed for a time, but it is often fatal. Depending on the type of cardiac amyloidosis, the average survival length for untreated patients ranges from three months to five years. With proper treatment for cardiac amyloidosis, the survival rate increases, but the illness still significantly impacts life expectancy. The earlier the condition is diagnosed, and the sooner treatment begins, the better your chances of surviving past the first few years with this condition.
FAQs
While cardiac amyloidosis can be treated, it cannot be cured. Sometimes, a heart or liver transplant may effectively remove the condition for a time. However, the condition will likely return unless the underlying cause of amyloids is identified and addressed.
High-intensity exercises may increase some symptoms, including shortness of breath and dizziness. It's important not to strain your system when you have this condition, but physical activity is still important. Finding the right balance can be difficult but may help alleviate some symptoms, like fatigue. Speak with your physician before starting an exercise routine if you have been diagnosed with cardiac amyloidosis.
