Definition

  Ventricular noncompaction is a primary congenital cardiomyopathy which is characterized by a spongy morphological appearance of the left ventricle.

  In children, ventricular noncompaction is generally accompanied by other congenital heart defects.

  In adults, noncompaction is usually observed as an isolated cardiac disease.



Etiology

  Mutations of several genes are reported in different patients.



Clinical significance

  Supraventricular arrhythmias, ventricular arrhythmias or sudden cardiac death may be seen.

  It has been suggested that among patients with ventricular noncompaction, the following criteria shows those with increased risk for cardiovascular death or heart transplantation:
1. PR (PQ) interval prolongation (>200 msec)
2. At least 0.1 mV ST segment depression or elevation in inferior leads.



The ECG abnormalities that are reported with increased frequency in patients with ventricular noncompaction are

  ST segment depression or elevation

  Left ventricular hypertrophy

  Bradycardia

  T wave negativity

  PR interval prolongation

  QT interval (QTc) prolongation

  Supraventricular arrhythmias

  Ventricular arrhythmias

  Brugada syndrome like appearance



References

  Ann Noninvasive Electrocardiol 2011;16:184-191.

  Circ J 2011;75:1728-1734.

  Cardiol Res Pract 2010 Dec 1;2011:201962.

  Interact Cardiovasc Thorac Surg 2011;12:370-373.

  Cardiol J 2011;18:310-313.

  Circulation 2006;113:1807-1816.

  Am J Cardiol 2011;108:1021-1023.




ECG 1. The ECG above, belongs to a 3 months-old baby who has extensive noncompaction of the left ventricle.

Pediatric cardiologist Dr. Mahmut Gokdemir has donated the above CEG to our website.

Click here for a more detailed ECG