An 11-year-old boy with familial dysautonomia presented with palpitations. Continuous 24-h Holter monitoring revealed intermittent high-grade atrioventricular block and asystole. The unopposed parasympathetic tone in patients with dysautonomia may make them susceptible to bradycardia and atrioventricular block. We recommend routine 24-h Holter monitoring screening and, when indicated, consideration of pacemaker implantation to reduce the high risk of sudden death phenomena in this patient population.
- Familial dysautonomia
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Cardiology and Cardiovascular Medicine