Overview

What is Cardiac Amyloidosis in adults?

Cardiac amyloidosis is a heart disease in which misshaped proteins become lodged in and around different parts of the heart. As these proteins build up, the heart struggles to pump blood and has to pump harder. Eventually, the extra effort weakens and damages the heart, leading to heart failure.

There are various potential causes of cardiac amyloidosis, including inheritance from one's parents. For others, the condition may occur naturally or be caused by other diseases, such as cancer. There is no cure for amyloidosis, but some types are treatable.

What are the symptoms of Cardiac amyloidosis?

Some people may not experience symptoms. If you do have symptoms, they may include:

Excessive need to urinate at night
Fatigue
Palpitations
Shortness of breath during activities
Swelling of the abdomen, ankles, and legs, or other parts of the body
Difficulty in breathing when lying down

What are the causes of cardiac amyloidosis?

A disorder called amyloidosis is characterized by the accumulation of amyloid protein. These proteins eventually replace healthy tissue, causing the affected organ to fail. Amyloidosis appears in several forms.

Amyloid deposits replace healthy heart muscle in patients with cardiac amyloidosis, often known as "stiff heart syndrome." This is the most common form of restrictive cardiomyopathy. The conduction system, which carries electrical signals across the heart, may be impacted by cardiac amyloidosis. Heart blocks and irregular heartbeats, or arrhythmias, may result from this.

It is possible to inherit the illness, which is called familial cardiac amyloidosis. It may also arise as a consequence of another illness that causes inflammation, such as a certain kind of blood or bone malignancy. Men are more frequently affected by cardiac amyloidosis than women, and those under 40 are not likely to get the condition.

How is cardiac amyloidosis diagnosed?

Doctors first suspect cardiac amyloidosis based on a combination of factors, including symptoms, a physical exam (doctors see, feel, and hear signs of disease), and family history. They then use diagnostic, imaging, and laboratory tests to confirm or rule out cardiac amyloidosis.

If you have familial amyloidosis in your family, you may not need to undergo specific tests, or you may need to undergo others.

What are the tests to diagnose cardiac amyloidosis?

Signs of cardiac amyloidosis can be associated with a variety of different diseases. This can make it difficult to diagnose the problem.

The following tests may be performed:

Blood and urine protein tests
CT scan of the chest or abdomen (considered the "gold standard" for diagnosing this condition)
Coronary angiogram
Echocardiogram
Electrocardiogram (ECG)
Magnetic Resonance Imaging (MRI)
An ECG may show problems with the heart rhythm or heart signal. It may also show a weak signal ("low voltage").
A cardiac biopsy is performed to confirm the diagnosis. Biopsies of other areas, such as the abdomen, kidneys, and bone marrow, are also often done.

What is the treatment of cardiac amyloidosis?

Your doctor may tell you you must change your diet and limit your salt and water intake.

You may need to take a diuretic (water tablets) to help eliminate excess water from your body. Your doctor may have you weigh yourself daily. A weight gain of 3 pounds (more than 1 kg) in 1-2 days may mean too much water in your body.

Medications such as digoxin, calcium channel blockers, and beta-blockers may be used for people with atrial fibrillation. However, medications should be used cautiously and dosages closely monitored. People with cardiac amyloidosis may be susceptible to the side effects of these medications.

Other treatments include:

Chemotherapy
Drugs that target the abnormal protein (tafamidis)
Implantable cardioverter defibrillators (AICDs)
Pacemakers: if there are problems with heart signals
Prednisone, an anti-inflammatory drug

In some types of amyloidosis, if the heart is not functioning very well, a heart transplant may be considered. Individuals with hereditary amyloidosis may require a liver transplant.

What is the prognosis for treatment?

The prognosis for cardiac amyloidosis depends mainly on two factors: the type of amyloidosis and the severity of the symptoms. That's why early detection makes a big difference. People who have successful heart, kidney, and liver transplants tend to do well, but the majority of people who need a transplant are not suitable for one or will have to wait a very long time for a transplant (which can affect survival).

General survival times for AL and ATTR amyloidosis (there is limited data on survival for dialysis-associated amyloidosis) are as follows:

AL amyloidosis- In the early stages, almost 70% of people survive for more than 5 years. In later stages, this value drops to 14%.
ATTR Amyloidosis- In early stages, median survival (similar to the average but not biased by unusually high or low values) was more than 7 years. In later stages, median survival was 18 months to 2 years.

Living with

How can I care for myself or manage my symptoms?

Unfortunately, once you develop amyloidosis, there isn't much you can do to mitigate it directly. If you have a family history of the disease, you should talk to your doctor about genetic testing. Not everyone who has a mutation associated with cardiac amyloidosis will develop the disease, but it's important to know if you are at risk of developing it. Start treatment promptly.

If you do indeed have cardiac amyloidosis, you must follow your doctor's instructions regarding medication and treatment. Many of these medications work in specific ways, so you must take them as your doctor directs for them to work properly.

Why Tender Palm Super-Speciality Hospital for Cardiac Amyloidosis?

Tender Palm Hospital has the most experienced team of Cardiologists, Cardiac Surgeons, and diagnostics with the latest and International standard infection control measures in Lucknow, India. The Cardiac Science Centre team has decades of experience in successfully treating Cardiac Amyloidosis.