Abstract
Noncompaction cardiomyopathy (NCCM) is a heterogenous disorder and third most common cardiomyopathy in children. It is characterized by hyper trabeculation, commonly hypothesized due to an arrest in compaction during fetal development. Since 1984 its first description by Gerecke et al. NCCM has been labeled with several names over 35 years that includes—spongy myocardium, left ventricular noncompaction cardiomyopathy (LVNC), Left ventricular hyper trabeculation (LVHT). In the year 2006, the American Heart Association classified NCCM as a distinct form of cardiomyopathy (CMP). NCCM is a rare cardiac disease with an estimated incidence of 0.12 per 100.000 in children up to the age of 10. In children NCCM is more frequently familial than when diagnosed in adulthood and is associated with other congenital heart diseases (CHDs), other genetic CMPs, and neuromuscular diseases (NMDs). Diagnostic challenges made it underestimated incidence. It may be due to non-uniform diagnostic criteria, unawareness, presumed other CMPs, and presence of CHD. NCCM can be asymptomatic to various presentation like heart failure, arrhythmia thromboembolic events, either as individual or in combination. Specific Treatment strategy has not yet established, nevertheless and, betablocker, ACEI or ARB might lead to remodeling of Left ventricular function (LV). In addition to state-of-the-art review, we discuss the epidemiology, pathogenesis, Phenotypes, aetiology, genetics, clinical presentation, outcome, and advancement in therapeutic options of NCCM in pediatric patients- fetuses to children. Furthermore, we provide a simple classification of different forms of disease. Finally long-term outcomes and future perspective are described.