We report 12 patients with ventricular noncompaction who were echocardiographically identified at our institution since 1991. The mean age at presentation was 3.5 years. Five patients had isolated noncompaction. Three of them had subnormal left ventricular systolic function at presentation. Noncompaction was associated with complex congenital heart defect in 3 patients. Four patients had simple...

FAMILIAL COMPLETE HEART BLOCK (CHB) has been described by a number of observers since the report by Morquio' in 1901. Among those reported are thirteen families with multiple cases of well-documented congenital CHB2-14 using the classic criteria of Yater'5 and eliminating those with intracardiac defects. More recently, an association has been noted between congenital CHB and maternal connective...

CriticalCareNurse Vol 29, No. 5, OCTOBER 2009 45 C ongenital complete atrioventricular block (CCAVB) occurs from fetal age to young adulthood in a person who has had no other cardiac arrhythmia detected before the heart block and no other history or signs and symptoms of generalized disease. In this article, I present the etiology of CCAVB, risk factors, electrophysiology, pathophysiology, clin...

Electronic pacemakers have been the standard of care for over 50 years for patients with symptomatic bradycardia caused by conditions such as sick sinus syndrome, complete heart block, and congenital heart block. Effective, electronic devices are subject to hardware malfunction, recalls of generators and leads, implantrelated complications and infections. Although the incidence is not high, dev...

BACKGROUND The neonatal lupus syndrome can be present as congenital heart block (CHB) or as neonatal lupus erythematosus (NLE), both seldom passively acquired autoimmune diseases. CHB starts around week 20 of pregnancy and is a lifelong event, whereas NLE is self limiting and usually starts at the 6th week of the child's age-the maximum recorded up to week 20. CASE REPORT An asymptomatic moth...

The study by Balmer et al. in the present issue of Europace lends support to our clinical notion that early intervention is beneficial even to the young individual. The children in this study that received a pacemaker had signs and/or symptoms such as bradycardia, exercise intolerance, syncope or heart failure that were ameliorated by pacemaker implantation. Three patients received a pacemaker ...