Left Ventricular Non-Compaction (LVNC) in Children

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. 

In LVNC, the normal development of the heart muscle in the womb is interrupted and it does not become compacted and smooth, instead the muscle fibers remain “non compacted” and have a trabeculated appearance.  

LVNC 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).

Signs and Symptoms

Many children with LVNC are asymptomatic. Some may present with nonspecific cardiac symptoms and others may have more severe 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

Diagnosis of LVNC is based on the medical and family history, physical exam, and cardiac testing.

Cardiac Testing:

  • An ECG to evaluate the rhythm of the heart and possible enlargement
  •  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
  • A Holter monitor to screen for abnormal heart rhythms
  • Genetic testing to look for genes associated with LVNC
  • Cardiac MRI may be done. It is a test that uses a magnetic field to obtain very clear pictures of the heart muscle
  • Blood tests such as BNP (B-type natriurectic peptide) which is a blood test that helps show it heart failure is present

 

Treatment

  • The treatment for LVNC is focused on improving cardiac function, preventing/minimizing 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