Noncompaction of the ventricular myocardium in Melnick-Needles syndrome

A 4-year-old asymptomatic boy was referred for evaluation after being diagnosed with Melnick-Needles syndrome. Echocardiography demonstrated noncompaction of the left ventricular myocardium as an isolated cardiac finding. Though other structural cardiac defects were reported previously in this syndrome, this is the first report of this rare cardiac anomaly in this likewise rare syndrome. We postulate that this occurrence represents a primary disorder of early fetal development. Patients with Melnick-Needles syndrome should be evaluated for noncompaction of the ventricular myocardium and its potential cardiovascular dysfunction.