Battling Transthyretin (TTR) Cardiac Amyloidosis? Learn More About Your Treatment Options

1 in 25 Blacks is stricken with the Val122Ile mutation in the transthyretin gene that results in an age-dependent, late-onset restrictive/hypertrophic cardiomyopathy that can mimic other forms of heart failure leading to misdiagnosis and underdiagnosis.

The transthyretin (TTR) cardiac amyloidosis type, in particular, is underdiagnosed in elderly Black populations and should be suspected in any adult patient of this descent who has unexplained left wall thickening on echocardiogram with or without macroglossia. Increased awareness of this entity will identify more patients with cardiac amyloidosis, thereby allowing timely medication and treatments.

People who have cardiomyopathy but no signs or symptoms may not need treatment. Sometimes, dilated cardiomyopathy that comes on suddenly may even go away on its own.

In other instances, treatment is needed. Treatment hinges on a few factors: the type of cardiomyopathy, the severity of your symptoms and complications as well as your age and overall health.

According to The American Heart Association, your doctor may prescribe medicines to:

Lower your blood pressure: ACE inhibitors, angiotensin II receptor blockers, beta blockers and calcium channel blockers are examples of medicines that lower blood pressure.

Slow your heart rate: Beta blockers, calcium channel blockers, and digoxin are examples of medicines that slow the heart rate. Beta blockers and calcium channel blockers also are used to lower blood pressure.

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Keep your heart beating with a normal rhythm: These medicines, called

antiarrhythmics, help prevent arrhythmias (irregular heartbeats).

Balance electrolytes in your body: Electrolytes are minerals that help maintain fluid levels and the acid-base balance in your body. Electrolytes also help muscle and nerve tissues work properly. Medicines used to balance electrolytes include aldosterone blockers.

Remove excess fluid and sodium from your body: Diuretics, or “water pills,” are an example of a medicine that helps remove excess fluid and sodium from the body.

Prevent blood clots from forming: Anticoagulants (PDF), or “blood thinners,” help to prevents blood clots. Blood thinners often are used to prevent blood clots from forming in people who have dilated cardiomyopathy.

Reduce inflammation: Medications used to reduce inflammation include corticosteroids.
A range of surgical and nonsurgical procedures can be used to treat cardiomyopathy as well:

Septal Myectomy: Septal myectomy is open-heart surgery considered for people who have obstructive hypertrophic cardiomyopathy and severe symptoms. This surgery generally is reserved for younger patients and for people whose medications aren’t working well. A surgeon removes

part of the thickened septum that’s bulging into the left ventricle. This improves blood flow within the heart and out to the body.

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Surgically Implanted Devices: Surgeons can implant several types of devices in the body to help the heart work better, including:

• Pacemaker: This small device uses electrical pulses to prompt the heart to beat at a normal rate.
• Cardiac Resynchronization Therapy (CRT) Device: This device coordinates contractions between the heart’s left and right ventricles.
• Left Ventricular Assist Device (LVAD): This implantable device helps the heart pump blood to the body. An LVAD can be used for long-term therapy or as an interim treatment for those awaiting a heart transplant.
• Implantable Cardioverter Defibrillator (ICD): An ICD helps to maintain a normal heartbeat by sending an electric shock to the heart if an arrhythmia, or irregular heartbeat, is detected.

Heart Transplant: In a heart transplant surgery, a person’s diseased heart is replaced with a healthy heart from a deceased donor. A heart transplant is a last resort for people who have end-stage heart failure. (“End-stage” means that all other treatment options have been explored, without success.)

Alcohol Septal Ablation (nonsurgical procedure): In this procedure, your doctor injects ethanol (a type of alcohol) through a tube into the small artery that supplies blood to the thickened area of the heart muscle affected by cardiomyopathy. The alcohol kills cells, and the thickened tissue shrinks to more normal size.

When treating transthyretin (TTR) cardiac amyloidosis, the main objectives are to stop the disease from getting worse, manage any conditions that cause or contribute to the disease, reduce complications and the risk of sudden cardiac arrest (SCA), and to control symptoms so that you can live as normally as possible. If you’re suffering from amyloidosis, there are many options out there for you.

For more information on heart health and heart failure, visit our Health Conditions page on BlackDoctor.org.

Tia Muhammad, BS, is an award-winning freelance content & media creative, copywriter, blogger, digital designer, and marketing consultant. She owns the boutique content and digital media company, jackieGLDN|studio.