Isolated non-compaction of the ventricular myocardium: prenatal diagnosis and natural history.

Isolated non-compaction of the ventricular myocardium (NCVM) is a rare cardiomyopathy characterized by the persistence of numerous marked ventricular trabeculations and deep intertrabecular recesses with direct vascular supply by the ventricular cavities. We report two cases diagnosed by fetal echocardiography at 27 and 30 weeks' gestation, respectively. Postnatal echocardiography verified the presence of the NCVM seen prenatally. Diagnosis was confirmed at postmortem following neonatal demise in the first case. Surgical intervention for exomphalos and extrahepatic biliary atresia was required in the second case, but there is no clinical abnormality of the cardiovascular system a year after delivery. The uncertainty of prognosis and the familial recurrence described elsewhere indicate the difficulty of counseling and the value of prenatal diagnosis, which is feasible using currently available ultrasonographic equipment. Copyright 2002 ISUOG. Published by John Wiley & Sons, Ltd.