Left Ventricular Non-Compaction (LVNC)

What is left ventricular non-compaction cardiomyopathy?

Left ventricular non-compaction (LVNC) cardiomyopathy is a condition where your lower left heart chamber (left ventricle) doesn’t develop properly. Instead of being firm and smooth, the left ventricle is spongy and thick.

LVNC cardiomyopathy is typically a congenital heart condition, meaning you’re born with it. Without treatment, LVNC cardiomyopathy can lead to serious heart problems.

How does left ventricular non-compaction cardiomyopathy affect heart function?

Usually, your left ventricle pumps oxygen-rich blood from your lungs to the rest of your body. In LVNC cardiomyopathy, your left ventricle can’t contract and relax as it’s supposed to. As a result, your heart can’t efficiently pump blood throughout your body.

How rare is left ventricular non-compaction cardiomyopathy?

Experts estimate that up to 12 in 1 million people are diagnosed with LVNC cardiomyopathy each year. The actual number of people with the condition may be higher because people who don’t have symptoms may not have a diagnosis.

What are the symptoms of left ventricular non-compaction cardiomyopathy?

Some people have no symptoms of LVNC cardiomyopathy. Others develop severe heart problems, including sudden cardiac death, when your heart suddenly stops beating. About 2 in 3 people with LVNC cardiomyopathy will develop heart failure.

If you have LVNC cardiomyopathy, you may have:

• Blood clots.
• Difficulty lying flat.
• Fainting.
• Heart palpitations.
• Inability to exercise (dyspnea).
• Irregular heartbeats (arrhythmia).
• Leg swelling (lymphedema).
• Shortness of breath (dyspnea).

What causes left ventricular non-compaction cardiomyopathy?

Usually, you develop LVNC cardiomyopathy because of a gene change (mutation) that develops before you’re born. This gene change may occur randomly or you may inherit it from your parents.

A small percentage of women develop LVNC cardiomyopathy for no known reason during their first pregnancy. Experts think this may occur because pregnancy can put extra stress on your heart muscle. In some women, symptoms disappear after pregnancy. Others deal with LVNC cardiomyopathy long-term.

How is left ventricular non-compaction cardiomyopathy diagnosed?

Your healthcare provider uses several tests to diagnose LVNC cardiomyopathy, including:

• Echocardiograms to assess how your heart pumps blood.
• Electrocardiograms to measure your heart’s electrical activity.
• Imaging tests, including cardiac CT scans and MRIs, to look at your heart’s structure.
• Coronary angiograms to look at how blood flows through your heart’s arteries.

How is left ventricular non-compaction cardiomyopathy treated?

Treatment for LVNC cardiomyopathy varies depending on what symptoms you have. If you have heart failure, your healthcare provider may prescribe medications such as:

• Angiotensin-converting enzyme (ACE) inhibitors such as benazepril (Lotensin®) or lisinopril (Prinivil®, Zestril®), or angiotensin-neprolysin inhibitors such as sacubitril-valsartan (Entresto®) to relax and open your veins and arteries.
• Beta-blockers, such as metoprolol (Lopressor®, Toprol XL®) or atenolol (Tenormin®), to block certain hormones and lower blood pressure.
• Digoxin (Digox®, Lanoxin®) to control your heartbeat and improve blood circulation.
• Diuretics to help rid your body of excess salt and water.

Your provider may recommend an implantable cardioverter defibrillator (ICD) if you’re at risk for cardiac arrest. An ICD delivers an electrical shock to the heart to terminate fast, fatal rhythms. Patients may require heart transplantation, in severe cases.

Can LVNC be reversed?

No. There’s no cure for LVNC cardiomyopathy. But treatment can increase your quality of life and lower your risk of heart complications. There also may be instances (in severe cases) where heart transplantation is required.

What is the outlook for LVNC cardiomyopathy?

The outlook for LVNC cardiomyopathy varies. Treatment can help control symptoms, and some people with the condition live a typical life expectancy.

LVNC cardiomyopathy can be fatal, but it often isn’t. Most people who have LVNC cardiomyopathy do not die from the disease. It’s important to have regular visits with a heart specialist (cardiologist). The cardiologist helps you manage your heart health to lower your risk of complications.

How can I prevent left ventricular non-compaction cardiomyopathy?

There’s no way to prevent LVNC cardiomyopathy. If you have the gene mutation that causes the condition or think you could, you may benefit from genetic testing. A genetic counselor reviews your test results with you and your family. They can help you understand what it means to have LVNC cardiomyopathy, including the risk of passing it to your children.

Are there other conditions that put me at higher risk of LVNC cardiomyopathy?

Yes. LVNC cardiomyopathy may be part of other conditions, such as:

• Barth syndrome.
• Noonan syndrome.
• Roifman syndrome.
• Toriello Carey syndrome.

What questions should I ask my doctor?

If you or your child has LVNC cardiomyopathy, or you think you could, you may want to ask your healthcare provider:

• What is the most likely cause of LVNC cardiomyopathy?
• Do you recommend genetic testing for LVNC cardiomyopathy?
• What tests diagnose LVNC cardiomyopathy?
• What are the treatment options for LVNC cardiomyopathy?
• What are the signs that my child or I need urgent care for a heart problem?