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Published on: December 11, 2017
[Congenital complete atrio-ventricular block]
1Département de pédiatrie, hôpital Necker-Enfants malades, Paris. elisabeth.villain@nck.ap-hop-paris.fr
Insights
Congenital complete atrio-ventricular block (AV block) requires pacemaker implantation for children with symptoms or very slow heart rates. Pediatric pacing offers satisfactory results, enabling most children to lead normal lives despite potential complications.
Area of Science:
- Cardiology
- Pediatrics
- Electrophysiology
Context:
- Congenital complete atrio-ventricular block (AV block) is a rare condition with unclear origins, potentially due to anatomical anomalies or maternal antibodies causing fetal myocarditis.
- The diagnosis of congenital AV block can be challenging when identified later in childhood.
Purpose:
- To outline the indications, treatment strategies, and outcomes of pacemaker implantation in children diagnosed with congenital complete AV block.
- To discuss the surgical approaches (epicardial vs. endocavitary) and pacing modes (dual-chamber vs. ventricular) used in pediatric patients.
Summary:
- Pacemaker implantation is the sole treatment for congenital complete AV block, indicated for cardiac failure, syncope, or asymptomatic children with heart rates below 50 bpm.
- Treatment strategies vary based on age and weight, utilizing epicardial pacing for younger children and endocavitary pacing for older children.
- Pacing modes include dual-chamber pacing to restore sinus node function or ventricular pacing for rate control, with generally satisfactory outcomes despite potential complications.
Impact:
- Pediatric pacemaker implantation for congenital complete AV block leads to satisfactory outcomes, allowing the majority of children to lead normal lives.
- Early identification and appropriate pacing mitigate risks of syncope, sudden death, and cardiac dysfunction in affected children.
- While complications like thrombosis and infection exist, advancements in pacing techniques improve long-term results and quality of life for pediatric patients with AV block.
Abstract:
Congenital complete atrio-ventricular block is a rare condition, the result of an anatomical anomaly of the conduction pathways, or even the trans-placental passage of maternal antibodies causing fetal myocarditis with fibrosis of the conduction tissue. It is not clear whether AV block discovered later in childhood is really "congenital". Whatever the age of the child, the only treatment is pacemaker implantation. Cardiac failure and syncope are absolute indications for implantation. Children whose heart rate is below 50 beats per minute (bpm) represent a group at high risk of syncope or even sudden death and must be paced, even if asymptomatic. Moreover, certain patients with immunological complete AV block have cardiopathy and must also be paced. In our department, the route for pacing is epicardial in younger children, in practice weighing less than 10-15 kg, and endocavitary in older children. The choice is either double chamber pacing, which restores AV conduction initiated by the child's sinus, or ventricular pacing with activity-controlled heart rate. Although worrying complications have been described, such as venous thrombosis, infections related to repeated interventions, and delayed cardiomyopathy, the results of paediatric pacing are generally satisfactory and the great majority of children with congenital complete AV block lead a normal life.
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