Ventricular conduction defect in isolated noncompaction of the ventricular myocardium

Abstract

A 9-month-old boy and 4-month-old girl presented with severe heart failure. The electrocardiogram showed complete and "incomplete" left bundle branch block, respectively. In both infants noncompaction of the ventricular myocardium was diagnosed with two-dimensional echocardiography. These cases are thought to be the first descriptions of the occurrence of left bundle branch block with noncompaction of the ventricular myocardium. Noncompaction of the ventricular myocardium should be added to the list of the causes for left bundle branch block in children.

Familial biventricular myocardial noncompaction associated with Ebstein's malformation

“…Cardiac rhythm disorders, including atrial fibrillation [12] and ventricular tachyarrhythmias [2,12,13], and conduction defects, mainly bundle branch block [20], have been reported in patients with noncompaction of the ventricular myocardium. In our subjects, a prolonged H-V interval and non-sustained ventricular tachycardia were documented in the proband, atrial flutter and LBBB were recorded in his father, and non-sustained atrial tachycardia in his son.…”

Rhythm Abnormalities in Children with Isolated Ventricular Noncompaction

“…Different rhythm abnormalities have been reported in patients with IVNC, and ventricular arrhythmias were the most common form 1,3–5,8–11 . In some cases, it may cause palpitations and syncope as the presenting symptoms.…”

“…7 Different rhythm abnormalities have been reported in patients with IVNC, and ventricular arrhythmias were the most common form. 1,[3][4][5][8][9][10][11] In some cases, it may cause palpitations and syncope as the presenting symptoms. In a previous report, ventricular arrhythmias were documented in five of the eight patients and were the presenting symptoms in two.…”

Clinical features of isolated left ventricular noncompaction in children

“…In addition to baseline ECG abnormalities, patient 2 developed second-degree atrioventricular block requiring pacemaker implantation during follow-up. This suggests that ventricular conduction abnormalities may develop later in life and could be due to progressive endocardial fibrosis in association with IVNC [12,17]. Therefore regular follow-up is required for these patients.…”