Heart block following propofol in a child

Area of Science:

• Pediatric Anesthesiology
• Autonomic Nervous System Disorders
• Cardiology

Background:

• Congenital central hypoventilation syndrome (CCHS), also known as Ondine's curse, is a rare disorder affecting autonomic nervous system function.
• Patients with CCHS exhibit impaired responses to hypoxia and hypercapnia, impacting respiratory and cardiovascular regulation.
• Anesthesia management in CCHS patients requires careful consideration due to potential autonomic instability.

Observation:

• A nine-year-old boy with CCHS received a single bolus of propofol for anesthesia induction during strabismus surgery.
• Following propofol administration, the patient developed complete atrioventricular (AV) heart block.
• This adverse cardiac event occurred despite propofol's typical minimal effect on normal human AV conduction.

Findings:

• Propofol, while generally safe for cardiac conduction, can reduce sympathetic activity and potentiate vagal effects.
• The case highlights a potential risk of AV heart block with propofol in pediatric patients with CCHS.
• This is the first documented instance of propofol-induced heart block in a child with CCHS.

Implications:

• Propofol may not be a suitable anesthetic agent for children diagnosed with congenital central hypoventilation syndrome.
• Anesthesiologists should exercise caution and consider alternative anesthetic strategies for CCHS patients.
• Further research is warranted to elucidate the specific mechanisms and risks associated with propofol in CCHS.