title: Impact of clinical history and electrophysiologic characterization of accessory pathways on management strategies to reduce sudden death among children with Wolff-Parkinson-White syndrome.
abstract: OBJECTIVE
  This study sought to determine whether the clinical and electrophysiologic criteria developed in adults also identify children with Wolff-Parkinson-White syndrome at risk for sudden death.
  
  
  BACKGROUND
  In adults with Wolff-Parkinson-White syndrome, a shortest RR interval <220 ms during atrial fibrillation is a sensitive marker for sudden death. However, because reliance on the shortest RR interval has a low positive predictive value, the clinical history has assumed a pivotal role in assessing risk. This approach has not been evaluated in children.
  
  
  METHODS
  We retrospectively evaluated 60 children </= 18 years old who underwent comprehensive electrophysiologic evaluation between 1979 and 1989 before undergoing operation for Wolff-Parkinson-White syndrome. Clinical and electrophysiologic data were analyzed after patients had been grouped by their clinical presentation: high risk (cardiac arrest), intermediate risk (syncope or atrial fibrillation) or low risk (orthodromic reciprocating tachycardia alone).
  
  
  RESULTS
  Ten children had a clinical cardiac arrest (high risk); only one had a prior history of syncope or atrial fibrillation. Compared with the intermediate (n = 19) and low risk groups (n = 31), there were no differences in age ([mean +/- SD] 14.8 +/- 0.6 vs. 14.7 +/- 0.6 vs. 14.5 +/- 1.7 years), duration of symptoms (1.9 +/- 0.5 vs. 4.1 +/- 1.1 vs. 5.2 +/- .08 years), incidence of congenital heart disease (30% vs 26% vs 32%), presence of multiple pathways (20% vs 16% vs 16%) or accessory pathway location. A shortest pre-excited RR interval <220 ms was found in 7 of 7 high risk patients (sensitivity 100%), 14 of 19 intermediate risk patients and 11 of 31 low risk patients (prevalence 35%).
  
  
  CONCLUSIONS
  Cardiac arrest was the only distinguishing clinical feature between high and low risk groups and the first manifestation in 80% of the children of an accessory pathway that can precipitate a life-threatening arrhythmia. In this series, the largest reported to date of children with Wolff-Parkinson-White syndrome having a cardiac arrest, a shortest pre-excited RR interval <220 ms was more sensitive than clinical history for identifying those at risk for sudden death.
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: B I
authors.initials: BI
authors.lastname: Bromberg
authors.forename: B D
authors.initials: BD
authors.lastname: Lindsay
authors.forename: M E
authors.initials: ME
authors.lastname: Cain
authors.forename: J L
authors.initials: JL
authors.lastname: Cox
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000517:prevention & control*; D004562:Electrocardiography; D004594:Electrophysiology; D006329:Heart Conduction System / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008487:Medical History Taking; D011379:Prognosis; D012189:Retrospective Studies; D012306:Risk; D012307:Risk Factors; D012680:Sensitivity and Specificity; D014927:Wolff-Parkinson-White Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery