OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND: Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.

Cardiocentro Pediatrico William Soler Havana Cuba

Cardiology Division Department of Pediatrics Rady Children's Hospital University of California San Diego San Diego California

Children's Heart Centre Charles University and Motol University Hospital Prague Czech Republic

Department of Paediatric Cardiology University Hospital of Wales Cardiff Wales United Kingdom

Department of Pediatrics Benioff Children's Hospital University of California San Francisco San Francisco California

Department of Pediatrics Division of Cardiology Stead Family Children's Hospital University of Iowa Iowa City Iowa

Department of Pediatrics Division of Pediatric Cardiology Mayo Clinic Rochester Minnesota

Department of Pediatrics Division of Pediatric Cardiology UCLA Health System University of California Los Angeles Los Angeles California

Division of Cardiology Children's Hospital Colorado University of Colorado Aurora Colorado

Division of Cardiology Department of Pediatrics British Columbia Children's Hospital Vancouver British Columbia Canada

Division of Cardiology Department of Pediatrics Primary Children's Hospital University of Utah Salt Lake City Utah

Division of Cardiology Department of Pediatrics Stollery Children's Hospital University of Alberta Edmonton Alberta Canada

Division of Cardiology Department of Pediatrics University of Michigan Children's Hospital University of Michigan Ann Arbor Michigan

Division of Pediatric Cardiology Cleveland Clinic Foundation Cleveland Ohio

Division of Pediatric Cardiology Department of Pediatrics Cohen Children's Medical Center of New York Hofstra Northwell School of Medicine New Hyde Park New York

Division of Pediatric Cardiology Department of Pediatrics Lucile Packard Children's Hospital Stanford University Palo Alto California

Division of Pediatric Cardiology Department of Pediatrics Seattle Children's Hospital Seattle Washington

Division of Pediatric Cardiology in the Department of Pediatrics Children's Hospital and Medical Center Omaha Nebraska

Division of Pediatric Cardiology Texas Children's Hospital Baylor College of Medicine Houston Texas

Greenlane Paediatric and Congenital Cardiac Service Starship Children's Hospital University of Auckland Auckland New Zealand

Labatt Family Heart Centre Hospital for Sick Children Toronto Ontario Canada

Nationwide Children's Hospital Columbus Ohio

Phoenix Children's Hospital University of Arizona College of Medicine Phoenix Arizona

JACC Clin Electrophysiol. 2018 Apr;4(4):445-447. doi: 10.1016/j.jacep.2017.11.014. PubMed

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