title: Feasibility and outcomes of ajmaline provocation testing for Brugada syndrome in children in a specialist paediatric inherited cardiovascular diseases centre.
abstract: Brugada syndrome (BrS) is an inherited arrhythmia syndrome that causes sudden cardiac death in the young. The class Ia antiarrhythmic ajmaline can be used to provoke the diagnostic ECG pattern. Its use has been established in adults, but little data exist on the ajmaline provocation test in children. This study aims to determine the safety and feasibility of ajmaline provocation testing in a large paediatric cohort in a specialist paediatric inherited cardiac diseases centre.
  
  
  
  98 consecutive ajmaline tests were performed in 95 children between September 2004 and July 2012 for family history of BrS (n=46 (48%)); family history of unexplained sudden cardiac death (n=39 (41%); symptoms with suspicious ECG abnormalities (n=9 (10%)). Three patients were retested with age, due to the possibility of age-related penetrance. ECG parameters were measured at baseline and during maximal ajmaline effect.
  
  
  
  The mean patient age was 12.55 years, 43% were female. Nineteen patients (20%) had a positive ajmaline test. There were no arrhythmias or adverse events during testing. Ajmaline provoked significant prolongation of the PR, QRS and QTc in all patients. Mean follow-up was 3.62 years with no adverse outcomes reported in any patients with BrS. There were no predictors of a positive ajmaline provocation test on multivariable analysis. One patient who tested negative at 12 years of age, subsequently tested positive at 15 years of age.
  
  
  
  Ajmaline testing appears safe and feasible in children when performed in an appropriate setting by an experienced team. Test positivity may change with age in individuals, suggesting that the test should be repeated in the late teenage years or early adulthood.
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK.
authors.forename: Merlin Ranald
authors.initials: MR
authors.lastname: McMillan
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK.
authors.forename: Thomas George
authors.initials: TG
authors.lastname: Day
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK.
authors.forename: Margarita
authors.initials: M
authors.lastname: Bartsota
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK.
authors.forename: Sarah
authors.initials: S
authors.lastname: Mead-Regan
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK.
authors.forename: Rory
authors.initials: R
authors.lastname: Bryant
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK.
authors.forename: Jasveer
authors.initials: J
authors.lastname: Mangat
authors.affiliation: Department of Cardiology , Boston Children's Hospital , Boston, Massachusetts , USA.
authors.forename: Dominic
authors.initials: D
authors.lastname: Abrams
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK.
authors.forename: Martin
authors.initials: M
authors.lastname: Lowe
authors.affiliation: Inherited Cardiovascular Diseases Unit, Department of Cardiology , Great Ormond Street Hospital for Children , London , UK ; Institute of Cardiovascular Science, University College London , London , UK.
authors.forename: Juan Pablo
authors.initials: JP
authors.lastname: Kaski