Left ventricular noncompaction/hypertrabeculation is a condition which is characterized by a highly trabeculated, “spongy” myocardium.
It can present at any age with heart failure, arrhythmia and/or thromboembolic events.
A wide variety of mutations have been found to be a cause of hypertrabeculation and it is possible that there is a continuum of
hypertrophic cardiomyopathy, dilated cardiomyopathy and hypertrabeculation/noncompaction.
We present a case of left ventricular hypertrabeculation which presented as sudden infant death syndrome and we propose that this entity may be a hidden cause of arrhythmic death in some infants presenting as sudden infant death syndrome.