B-AB20-04 PROSPECTIVE ASSESSMENT OF CHILDREN WITH WOLFF-PARKINSON-WHITE SYNDROME: HOW RARE ARE EVENTS, REALLY?

Abstract

Wolff-Parkinson-White Syndrome (WPW) is associated with sudden death. Previous retrospective studies have challenged dogma that risk stratification strategies predict risk. There have been calls for validation with multicenter prospective data that we provide here. Using data collected at diagnosis we sought to better understand children with WPW comparing those with and without a life-threatening event (LTE). Patients were enrolled from 19 participating centers in the US, Canada and India. Demographic, clinical and electrophysiology study (EPS) data were compared between children with and without LTE. LTE was defined as aborted sudden death or pre-excited atrial fibrillation (AF) with: SPERRI ≤ 250, syncope or hemodynamic instability. Among 720 subjects (56% M) mean age at diagnosis was 9.8+/-6.4 y (0-21.8 y), 8% had congenital heart disease (CHD). A LTE occurred in 16 (2.2%), palpitations in 266 (37%), documented SVT in 216 (30%), and as an incidental finding on ECG 223 (31%). The LTE was aborted sudden death in 10/16 (62%) and pre-excited AF in 6/16 (38%). Age at LTE was 10.9 +/-7y (0.2 mo -18 y) and 63% were male. The LTE was the sentinel symptom in 60%, whereas 27% had previous SVT and 20% a previous WPW evaluation. Activity at the time of LTE was competitive 20% or noncompetitive 20% athletics, rest/sleep 13%, routine daily activities 40% or unknown 7%. Preexcitation was intermittent in 8% and 7% of the LTE groups had CHD. The outcome of the event was full recovery in 79%, recovery with neurological deficit 7% and death 14%. Comparing those with and without a LTE there was no difference in age, sex or presence of intermittent preexcitation. EPS performed in 69% of those with LTE and 74% of those without identified only APERP as significantly different (263+/-82 vs 312+/-67, p 0.01). In this prospectively enrolled population of children with WPW, 2.2% suffered a LTE, higher than previously reported. Children were similar in age and sex to those without a LTE. The LTE was often the sentinel symptom and occurred during rest or activities of daily life more often than with exercise. The only EPS derived value that differed was APERP. Although the most common LTE type was aborted sudden death, the most frequent outcome was full recovery.