Life-Threatening Events in Wolff-Parkinson-White Occur Without Prior Symptoms

Child EKG
Child EKG
Patients aged 21 years with Wolff-Parkinson White syndrome are at high risk for a life-threatening event.

Children and young adult patients aged ≤21 years with Wolff-Parkinson-White (WPW) syndrome, a condition characterized by a rapid heartbeat, are at a high risk for a life-threatening event (LTE), according to a study in JACC Clinical Electrophysiology. These events often occur without warning or indication on electrophysiology (EPS), researchers reported.

The investigators retrospectively reviewed data of people with WPW and a clinically recorded history of an LTE at ≤21 years of age (n=816). LTEs included sudden death, aborted sudden death, or pre-excited atrial fibrillation “with the shortest pre-excited RR interval (SPERRI) in atrial fibrillation of ≤250 ms, regardless of symptoms or documented pre-excited atrial fibrillation associated with hemodynamic compromise, syncope, or seizure, regardless of the SPERRI.” The investigators compared data from those with WPW who had an LTE with data from age-matched controls with WPW who had not experienced an LTE but who had undergone an EPS (n=96).

The incidence rate of LTE was 65%, which comprised sudden death (6%), aborted sudden death (45%), and rapidly conducted pre-excited atrial fibrillation (49%). Significant risk factors for an LTE were male sex (odds ratio [OR] 2.35; 95% CI, 1.28-4.33; P =.006), Ebstein malformation (OR 4.10; 95% CI, 1.10-15.27; P =.035), inducible atrial fibrillation at EPS (OR 2.85; 95% CI, 1.70-4.76; P <.0005), rapid anterograde conduction (accessory pathway effective refractory period, SPERRI, or shortest paced cycle length with pre-excitation during atrial pacing ≤250 ms; OR 3.85; 95% CI, 2.29-6.45; P <.0005), and multiple pathways (OR 3.83; 95% CI, 1.86-7.90; P <.0005).

Approximately 37% of patients with ≥2 risk stratification components did not have high-risk characteristics on EPS. Additionally, 25% of these patients with risk stratification components did not have any distinguishable pathway characteristics or inducible atrioventricular reciprocating tachycardia.

Limitations of the analysis included its retrospective design, lack of standardized clinical and EPS evaluations, and the lack of information on whether control participants were at low risk for LTEs.

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“Because EPS is an imperfect predictor and the risk of an ablation is low, once an invasive EPS is performed, moving forward to a catheter ablation seems appropriate,” the investigators concluded. “We advocate a low threshold for catheter ablation to cure WPW syndrome in children after careful consideration of age and size-based risks.”


Etheridge SP, Escudero CA, Blaufox AD, et al. Life-threatening event risk in children with Wolff-Parkinson-White syndrome: a multicenter international study. JACC Clin Electrophysiol. 2018;4(4):433-444.