Congenital complete atrioventricular block in the early pediatric population

Overview

Congenital complete atrioventricular block (CCAVB) is a rare potentially lethal disease
with an estimated incidence of 1 every 15.000 to 20.000 live born infants.
In structurally normal hearts two kinds of congenital heart block can be identified: one usually diagnosed
in utero associated with the circulating maternal anti-SS-A/Ro and anti-SS-B/La antibodies,
the other kind is detected later in the neonatal period or during the infancy or childhood
and present no clear relation with maternal antibodies. Nowadays, the diagnosis can be made in
utero as early as between week 16 and 28 of gestation by foetal echocardiography.
The mortality in isolated CCAVB is estimated between 8 and 16% and between 4 and 8% in children
and adults. The mortality and morbidity of patients diagnosed outside the neonatal period
is significantly lower than those with a in utero diagnosis.
Risk factors for worse outcome in CCAVB are the foetal diagnosis, the presence of hydrops fetalis,
delivery at 32 weeks gestation, and a ventricular rate <55 beats/min in early pregnancy (13,18).
Aim of this review is to delineate the current knowledge on CCAVB presenting in children without
structural heart disease including aetiology, outcome and management. Also outlined in this
review are some of the problems still debated in this issue. (Heart International 2006; 2: 1-5)

Keywords

Congenital complete heart block, Fetal heart block, Pacing, Anti-SS-A/Ro and anti- SS-B/La antibodies

Correspondence

Riccardo Cappato, MD, Arrhythmias and Electrophysiology Center, Policlinico San Donato, University of Milan, Italy, Via Morandi 30, 20097 San Donato Milanese, (MI) – Italy, rcappato@libero.it