title: Natural history of Wolff-Parkinson-White syndrome diagnosed in childhood.
abstract: Wolff-Parkinson-White (WPW) syndrome carries a risk for symptomatic arrhythmias and sudden death. The aim of this study was to examine the natural history of patients with Wolff-Parkinson-White syndrome diagnosed in childhood followed longitudinally at a single institution. The study population consisted of 446 patients. The median age of diagnosis was 7 years, and 61% were male. Associated heart disease was present in 40 patients (9%). Modes of presentation included supraventricular tachycardia (38%), palpitations (22%), chest pain (5%), syncope (4%), atrial fibrillation (0.4%), sudden death (0.2%), and incidental findings (26%); data were unavailable in 4%. During the study period, a total of 243 patients (54%) had supraventricular tachycardia, and 7 patients (1.6%) had atrial fibrillation. Of patients who presented at ≤3 months of age, 35% had resolution of manifest preexcitation compared with 5.8% who presented at >3 months of age (p <0.0001). There were 6 sudden deaths (1.3%), with an incidence of 2.8 per 1,000 patient-years. Two of these patients had structurally normal hearts (incidence 1.1 per 1,000 patient-years). Four of these patients had associated heart disease (incidence 27 per 1,000 patient-years) (p <0.01). In conclusion, in a large population of patients with Wolff-Parkinson-White syndrome diagnosed in childhood, 64% had symptoms at presentation, and an additional 20% developed symptoms during follow-up. There were 6 sudden deaths (1.3%), with an overall incidence of 1.1 per 1,000 patient-years in patients with structurally normal hearts and 27 per 1,000 patient-years in patients with associated heart disease.
authors.affiliation: Medical University of South Carolina, Charleston, South Carolina. Electronic address: cainn@musc.edu.
authors.forename: Nicole
authors.initials: N
authors.lastname: Cain
authors.forename: Claire
authors.initials: C
authors.lastname: Irving
authors.forename: Steven
authors.initials: S
authors.lastname: Webber
authors.forename: Lee
authors.initials: L
authors.lastname: Beerman
authors.forename: Gaurav
authors.initials: G
authors.lastname: Arora
mesh_terms: D000293:Adolescent; D058070:Asymptomatic Diseases; D001281:Atrial Fibrillation / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D013617:Tachycardia, Supraventricular / Q000209:etiology; D014927:Wolff-Parkinson-White Syndrome / Q000150:complications / Q000401:mortality / Q000503:physiopathology*