BACKGROUND: Isolated non-compaction cardiomyopathy (NCCM) was first described in 
1984. This disorder, a primary genetic cardiomyopathy, is now attracting 
increased attention.
METHOD: The current state of the epidemiology, pathogenesis, pathophysiology, 
clinical features, diagnosis, treatment, and prognosis of NCCM are discussed on 
the basis of a review of selected literature as well as the authors' personal 
experience.
RESULTS: The pathogenesis of NCCM is thought to involve a genetically determined 
disturbance of the myocardial compaction process during fetal endomyocardial 
morphogenesis. It is not accompanied by any other cardiac anomalies. 
Echocardiography is the diagnostic method of choice. The diagnosis is based on 
the following echocardiographic criteria: the presence of at least 4 prominent 
trabeculations and deep intertrabecular recesses, blood flow from the 
ventricular cavity into the intertrabecular recesses, and a typical bilaminar 
structure of the affected portion of the left ventricular myocardium. NCCM can 
also be diagnosed with magnetic resonance imaging of the heart. The clinical 
severity of NCCM is variable; its manifestations include heart failure, 
thromboembolic events, and arrhythmias. The treatment is symptom-based. Patients 
with symptomatic NCCM have a poor prognosis.
CONCLUSION: NCCM is a type of cardiomyopathy that was first described 25 years 
ago. Its molecular genetic basis is not yet fully clear, and the same is true of 
its diagnosis, treatment, and prognosis. Further study of these matters is 
needed.