Left Ventricular Non-Compaction (LVNC) in Children

Left ventricular non-compaction (LVNC) is a disease where the left ventricular muscle on the echocardiogram appears soft and spongy instead of smooth and compact.

LVNC is primarily a genetic condition. The genetic causes can be associated with genetic syndromes, metabolic disorders, and mitochondrial disorders. LVNC can also be associated with other types of heart muscle diseases hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), or restrictive cardiomyopathy (RCM). It is important to understand that LVNC does not always represent a disease state. Some people can have LVNC for life with no consequences. Your doctor can help determine if that is the case.

Signs and Symptoms

Many children with LVNC experience no symptoms. For those who develop heart failure or abnormal heart rhythms, symptoms can include:

  • shortness of breath (starts with exercise, but over time occurs at rest)
  • shortness of breath when lying flat
  • fatigue
  • persistent cough
  • swelling in face, abdomen, legs, or feet 
  • unexplained weight gain
  • dizziness or lightheadedness
  • fainting or passing out (syncope)
  • abnormal heartbeats
  • nausea and vomiting
  • decreased appetite

Diagnosis

An echocardiogram is the most common test to diagnose LVNC. Trabeculations within the left ventricle and the overall squeeze or the heart can be measured.

Additional testing may include an electrocardiogram (ECG/EKG), genetic testing, cardiac MRI, and Holter monitor.

Treatment

Treatment for LVNC is focused on improving cardiac function, preventing symptoms, and protecting children from abnormal heart rhythms.

A child with decreased heart function may be started on a blood thinner to reduce the risk of blood clots forming between the trabeculations.  

If a child is identified as at risk for sudden cardiac arrest, preventative treatments such as medications to control the heart rate (antiarrhythmic medications) or an implantable cardioverter defibrillator (ICD) may be recommended.

Lifestyle changes

Activity restrictions may be recommended if your child’s heart function is weak or if there is evidence of abnormal heart rhythms. “Intense” physical activity may be a risk for a child with a weakened heart muscle. Children with LVNC should never “push through” a symptom if they feel tired, dizzy, or have difficulty breathing with an activity. They should remain well hydrated at all times and rest when needed. It will be important to discuss the sports and activities that are safe and appropriate for the child and focus on what can be done and how to stay safe when participating in activities. Children with LVNC should also follow a healthy, well-balanced diet.

 

Keep Exploring

Heart Rhythm Disorders
Millions of people experience irregular or abnormal heartbeats, called arrhythmias, at some point in their lives. Most of the time, they are harmless and happen in healthy people free of heart disease. However, some abnormal heart rhythms can be serious or even deadly. Having other types of heart disease can also increase the risk of arrhythmias.
Pediatrics and Congenital Heart Disease (CHD)
This section is for pediatric patients and families living with heart rhythm disorders and heart rhythm disorders related to congenital heart disease (CHD).
Early Warning Signs
If you are experiencing a racing, pounding, rumbling or flopping feeling in your chest or if you have been fainting, having repeated dizzy spells, feeling lightheaded or you are extremely fatigued, it's time to see a doctor to discuss your heart health.
Common Treatments
Learning about the underlying cause of any heart rhythm disorder provides the basis for selecting the best treatment plan. Information and knowledge about care options, and their risks and benefits help you work with your health care provider to make the best choices.
Lifestyle
Since other heart disorders increase the risk of developing abnormal heart rhythms, lifestyle changes often are recommended. Living a “heart healthy” lifestyle can ease the symptoms experienced with heart rhythm disorders and other heart disorders, and can be beneficial to overall patient health.
The Normal Heart
The heart is a fist-sized muscle that pumps blood through the body 24 hours a day, 365 days a year, without rest. The normal heart is made up of four parts: two atria on the top of the heart (right atrium and left atrium), and two ventricles (right ventricle and left ventricle) which are the muscular chambers on the bottom of the heart that provide the major power to pump blood.