Isolated non-compaction of the ventricular myocardium is a rare cardiomyopathy characterized by numerous prominent trabeculations and deep intertrabecular recesses.This rare disease is due to an arrest of myocardial morphogenesis. Most cases, when seen in children, are associated with obstructive malformations. We noticed that the diagnosis of this disease is very rare in fetal life.
In this report we present a case of right ventricular non compaction diagnosed by fetal echocardiography at 33 weeks of gestation
Diagnosis of ventricular noncompaction was made according to the characteristic appearance of a two-layered structure of the myocardial wall consisting of a thin compacted epicardial layer and a thick noncompacted endocardial layer (ratio of noncompacted to compacted layers>2) with numerous, excessively prominent trabeculations and deep intertrabecular recesses that filled with blood from the ventricular cavity visualized on color Doppler imaging on echocardiography.
In contrast with postnatal experience, we found isolated non-compaction in the right ventricle.We discuss prenatal diagnosis and natural history of isolated non-compaction of right ventricle.