We present the case of a nine-year-old boy afflicted with Ondine's curse, w
ho developed complete atrioventricular heart block after a single bolus of
propofol for induction of anaesthesia for strabismus surgery. Ondine's curs
e, the other name for congenital central hypoventilation syndrome, is chara
cterized by a generalized disorder of autonomic function. Propofol has no e
ffect on the normal atrioventricular conduction system in humans but it red
uces sympathetic activity and can highly potentiate other vagal stimulation
factors. Heart block has been documented after propofol bolus use in adult
s but, to our knowledge, not in children. It would appear that propofol is
not a good choice for anaesthesia in congenital central hypoventilation syn
drome.