Isolated left ventricular non-compaction is a rare, morphologically distinct 
primary genetic cardiomyopathy, which was first described in children, but is 
becoming increasingly recognised in adults. The most common clinical 
manifestations include heart failure, arrhythmias and embolic events, and in 
children it may be associated with facial dysmorphisms and Wolff-Parkinson-White 
syndrome. Misdiagnosis may occur through lack of awareness of the condition, but 
with increased vigilance detailed cardiac imaging can reliably differentiate 
isolated left ventricular non-compaction from other pathological causes of left 
ventricular dysfunction. This is important because the condition may be 
associated with significant morbidity and mortality, and genetic counselling and 
echocardiographic screening of first-degree relatives is recommended to detect 
asymptomatic cases.