Amyloidosis Cardiomyopathy

What is amyloidosis?

Amyloidosis is an umbrella term used to identify a group of diseases which affect different organs in the body. The condition occurs when deposits of protein, also known as amyloids, are left in tissue around the body. As time progresses, these protein deposits start to replace normal, healthy tissue. Due to this, the affected organ may be prevented from working properly.

What is amyloidosis cardiomyopathy?

Amyloidosis cardiomyopathy refers to amyloids building up in or around the heart. While amyloidosis is a serious condition regardless of which organ it affects, amyloidosis cardiomyopathy is a particularly dangerous form of the condition because of the impact is has on the heart muscle and surrounding tissue.

Although there are numerous different types of proteins which can replace normal tissue around the organs, they don't all affect the heart. The most common proteins involved in cardiomyopathy are light-chain, familial or senile.

Despite amyloids affecting the heart in similar ways, new studies are highlighting the importance of identifying the specific type of amyloid which is present. According to scientists, certain proteins may respond better to specific treatments.

It's essential, therefore, that patients diagnosed with amyloidosis cardiomyopathy undergo further testing in order to determine what type of amyloids are affecting the heart. If the amyloids can be identified, appropriate treatment measures can be taken, thus giving the patient the best chance of survival.

What are the symptoms of amyloidosis cardiomyopathy?

When amyloidosis affects the heart muscle, it is known as a form of restrictive cardiomyopathy. As the level of protein deposits in the heart increases, it is unable to pump blood around the body effectively. Also known as ‘stiff heart syndrome', amyloidosis cardiomyopathy ultimately prevents the heart from working properly.

As a result, electrical signals cannot be conducted through the heart effectively. This can result in abnormal heart rhythms and faulty heart signals. Due to this, people with amyloidosis cardiomyopathy may experience palpitations and shortness of breath.

In addition to this, patients may also suffer from extreme tiredness, swelling of the abdomen and excessive night-time urination.

What causes amyloidosis cardiomyopathy?

Amyloidosis cardiomyopathy may occur when patients are suffering from another medical problem, such as blood or bone cancer. Alternatively, any condition which causes inflammation could result in a presentation of amyloidosis cardiomyopathy.

Familial cardiac amyloidosis can also occur and this indicates a genetic link in the disease. Although the disease normally affects people over the age of 40, if it is inherited it may be symptomatic earlier.

What treatment is available for amyloidosis?

In order to treat amyloidosis, physicians will normally attempt to treat the organ dysfunction caused by the condition, while also slowing the rate of amyloid deposition. If they can successfully prevent additional deposits being formed, this should slow the rate of the disease and prevent symptoms from worsening.

Often, chemotherapy is used in order to kill certain plasma cells as this can then reduce the buildup of amyloids.

In the case of amyloidosis cardiomyopathy, chemotherapy can be used to halt the disease while other measures are taken to deal with the effects the condition has already had on the heart muscle.

For example, arrhythmia management may be used to normalize heart rhythms. Pacemakers may also be used in order to overcome faulty conduction within the heart and, in some cases, a heart transplant may be a viable form of treatment.

What is the prognosis of amyloidosis cardiomyopathy?

As amyloidosis cardiomyopathy is not reversible, a patient's prognosis is dependent on their response to treatment, as well as the effects of any other conditions which may be present.

Although amyloidosis cardiomyopathy has been associated with relatively high mortality rates, new technologies are providing a greater range of treatment option. As a result, the life expectancy of many patients with amyloidosis cardiomyopathy can be viewed in years, rather than weeks or months.

Of course, a patient's prognosis will ultimately depend on the level of damage which has already been done to the heart muscle by the time treatment begins. Thorough testing is crucial to making a full diagnosis and physicians must identify the type of amyloid deposits which are present so that inappropriate treatment options can be excluded.

If physicians are able to diagnose the condition quickly, when limited deposits are present, and implement treatment quickly, the patient should have a relatively good prognosis.

Last Reviewed:
June 14, 2017
Last Updated:
October 10, 2017