Learning About Left Ventricular Non-Compaction (LVNC) in Children

Left ventricular non-compaction (LVNC)is a rare heart condition. It happens when the left ventricle doesn’t form as it should. The left ventricle is the lower left chamber of the heart. The left ventricle muscle contracts to pump blood to the body.

The muscle wall of the left ventricle is usually smooth and firm. With LVNC, the muscle wall has a lot of finger-like ridges (trabeculations) and deep grooves. They can make the muscle look sponge-like.

LVNC can affect how the left ventricle pumps blood. It may not pump blood correctly. This can lead to other problems, including an abnormal heart rhythm (arrhythmia), blood clots, or heart failure.

Children diagnosed with LVNC often have other health conditions. These can include:

• Other types of heart muscle diseases (cardiomyopathies).
• Heart defects that are present at birth (congenital defects).
• Conditions related to gene changes (genetic syndromes).
• Issues with the body’s metabolism (metabolic issues).
• Conditions that affect the nerves and related muscles (neuromuscular disorders).

LVNC is a genetic disease. This means it is passed down through families. Children who have LVNC are born with it. Certain problems with genes (gene mutations) can cause the condition.

If your child has LVNC, then other close family members may be at risk for this condition. Talk to your doctor about getting other family members tested.

Some children with LVNC do not have symptoms. The most common symptoms of LVNC are symptoms of heart failure. A child with heart failure may:

• Feel very tired.
• Be short of breath.
• Feel like their heart is pounding or racing (palpitations).
• Feel weak or dizzy.
• Have swelling (edema), especially in the legs, ankles, and feet.
• Gain weight. This may happen over a day or two or more slowly.
• Cough or wheeze, especially when lying down.
• Feel bloated or sick to the stomach.

Heart failure can sometimes cause the heart to stop suddenly, which can be life-threatening.

Your child's doctor will do an exam. The doctor will ask about your child's symptoms, past health, and family history.

Your child may have imaging tests of the heart. These may include a cardiac MRI or a type of ultrasound called an echocardiogram (echo).

LVNC can be diagnosed in a child at any age. Sometimes children are diagnosed during regular screenings or tests for other health problems.

There is no specific treatment for LVNC. Treatment focuses on managing symptoms and other heart problems, like an abnormal heart rhythm or blood clots.

Depending on your child’s symptoms, treatment may include:

• Medicines used for heart and blood vessel problems, such as beta-blockers, ACE inhibitors, or ARBs.
• Anticoagulants. These are medicines used to treat and prevent problems caused by blood clots.
• Close monitoring of other health problems.
• A device called an implantable cardioverter-defibrillator (ICD). It can fix an abnormal heart rate or rhythm.
• A heart transplant. Some children with LVNC need a heart transplant. But this isn’t common.

If your child does not have symptoms or other heart problems, their doctor may recommend follow-up visits and tests every few years.