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
- 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
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.
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.