A 30 year old female presented to our ED with pre-syncope. Do you see anything that looks abnormal on this apical four-chamber view of her heart?

 

 

It is fairly easy to see that the systolic function or contractility is decreased, which is consistent with the patient’s history of heart failure, but this patient also has another finding. Look at the left ventricular myocardium more carefully and you will see that it has an abnormal or spongy appearance.  This patient has heart failure from Noncompaction cardiomyopathy.

 

What is Noncompaction Cardiomyopathy?

Glad you asked! Here are some things you should know.

  • It is believed to be genetic entity that causes an abnormality in embryogenesis. If you are like us, you have completely blacked out this part of medical school too, so bare with us for a minute while we explain.
    • The early myocardium has deep recesses that are necessary for nutrition by diffusion of blood over this area because the coronary arteries do not exist yet
    • Between 5-18 weeks gestation, the heart undergoes “compaction” where these recesses or trebeculations come together and close up
    • If this does not occur properly, it is called noncompaction
  • This condition is thought to be fairly uncommon, but actual prevalence is unknown
  • Old and young have been diagnosed with this condition and symptomatology is variable
  • It is arrythmogenic…meaning that it can cause syncope from ventricular tachycardia…meaning you probably do not want to send your patient home if their syncopal event was caused by this
  • There are no specific EKG findings associated with this condition
  • It also causes heart failure and embolic events (clots tend to form in the left ventricle because of the trebeculations)
  • Diagnosis is largely based off of ultrasound findings (2 specifically)
    • Deep recesses in the left ventricular myocardium, some say these recesses should be twice as deep as the thickness of the underlying myocardium
    • Color Doppler that shows flow into these recesses

 

Here is what a normal myocardium should look like in the apical four-chamber view just for comparison. Note that this patient also has severe LV systolic dysfunction.

 

Look back at the images again now that we know what we are looking for.

 

Luckily, this patient already had a defibrillator in place.

 

Bottom Line: Even though Noncompaction Cardiomyopathy is thought to be a rare condition, early diagnosis is felt to be not only very important but also life-saving because a large percentage of people with this condition die from sudden cardiac death. Think about this in your patients presenting to the ED with syncope as this condition will not be identified on EKG. Another win for ultrasound!

 

 

References

  1. Weiford B, Subbarao V and Mulhern K. Noncompaction of the Ventricular Myocardium. Circulation. 2004; 109: 2965-2971.
  2. Ritter M, Oechslin E, Sutsch G, et al. Isolated Noncompaction of the Myocardium in Adults. Mayo Clin Proc. 1997; 72: 26–31. 

 

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