Introduction:

Left Ventricular Non-Compaction (LVNC) is a disease where the left ventricular muscle on echocardiogram appears soft and spongy, instead of looking smooth and compact.

 

VNC is mostly a genetic condition.  The genetic causes can be associated with genetic syndromes, metabolic and mitochondrial disorders.  LVNC can also be associated with the other types of heart muscle diseases (HCM, DCM or 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 cardiologist 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 diagnosis LVNC.  The trabeculations within the left ventricle and the overall squeeze or the heart can be measured.

Additional testing may include ECG, genetic testing, MRI and Holter monitor.

 

Treatment:

The 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 at increased risk for sudden cardiac arrest, preventative treatments such 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 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 are feeling 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 to 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.