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.


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 automatic implantable cardioverter defibrillator (ICD). Three patients died of cardiac problems during the follow-up period.


CONCLUSIONS
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.
Celiker
Alpay
A
Department of Pediatric Cardiology, Hacettepe University Faculty of Medicine, Ankara, Turkey. aceliker@hacettepe.edu.tr
Ozkutlu
Süheyla
S
Dilber
Embiya
E
Karagöz
Tevfik
T
D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000209:etiology* / Q000517:prevention & control; D009202:Cardiomyopathies / Q000150:complications* / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006339:Heart Rate; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors