Rhythm abnormalities in children with isolated ventricular noncompaction

Background: Isolated ventricular noncompaction (IVNC) is a serious cardiomyopathy with a generally poor prognosis. It is characterized by the presence of prominent ventricular myocardial trabeculations and deep intertrabecular recesses, in the absence of other structural heart defects, This cardiomyopathy is usually associated with ventricular dysfunction, thromboembolic events, and rhythm problems. Methods and Results: This article describes 11 children who have rhythm abnormalities associated with IVNC. On admission, eight children had complaints including palpitation, syncope, heart failure, and bradycardia. Ventricular arrhythmias were diagnosed in four children, sinus node and atrioventricular conductance disturbances in six children, and Wolff-Parkinson-White syndrome and associated tachycardia in one child. Three children with ventricular arrhythmias received an automotic implantable cardioverter defibrillator (ICD). Three patients died of cardiac problems during the follow-up period. Conclusion: Since many of rhythm abnormalities, including life-threatening ventricular arrhythmias, may be seen in patients with IVNC, children with IVNC should be screened for arrhythmias. An ICD may be the best treatment for some of these patients.