Congenital complete atrioventricular block in the early pediatric population
Abstract
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
Article Information
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
Further Resources
Trending Topic
Moderate-to-severe congenital heart disease, which requires specialist cardiology care, is present in around 6/1,000 live births.1 Patients born with congenital heart defects often require surgery in early life and a consequence of this initial surgical repair is pulmonary regurgitation. The severity of pulmonary regurgitation can worsen over time and present more frequent symptoms, and may […]
Moderate-to-severe congenital heart disease, which requires specialist cardiology care, is present in around 6/1,000 live births.1 Patients born with congenital heart defects often require surgery in early life and a consequence of this initial surgical repair is pulmonary regurgitation. The severity ...
Background: Patients with adult congenital heart disease (ACHD) are more prone to atrial arrhythmia, which increases their morbidity and mortality and risk of hospital admissions when compared with the same population where atrial arrhythmias are absent. Objective: Compare the use ...
First described in 1868 by WS Church, cor triatriatum sinister (CTS) is one of the rarest congenital heart diseases in the world, with an estimated incidence of 0.1%.1,2 It is characterised by the presence of a fibrous membrane that divides the left ...
...
...
...
...
...
...
Short QT syndrome (SQTS) is a cardiac channelopathy characterised by an abnormally short QT interval and an increased risk of atrial and ventricular fibrillation.1 As it often involves young patients with an apparently normal heart, it is imperative for physicians ...
Long QT syndrome (LQTS) is a hereditary disorder characterised by prolongation of the QT interval on electrocardiogram (ECG), syncope and sudden death due to torsade de pointes (Tdp) and/ or ventricular fibrillation.1 To date, 16 genes have been reported as LQTS-causing ...
Advances in surgical technique over the last two decades have heralded a dramatic improvement in survival rates in those born with congenital heart disease (CHD). With current methods, more than 85% of paediatric patients undergoing surgical repair survive into adulthood,1and ...
Log into your Touch Account
Keep track of your clinical interests and newsletter subscriptions.
Sign up with an Email
Or use a .
Register now for FREE access
Already registered? Login below.
