Aborted Sudden Cardiac Death and Ventricular Fibrillation in Patients with Wolff–Parkinson–White Syndrome

Background: Wolff–Parkinson–White (WPW) syndrome is characterized as antegrade conduction via an accessory pathway connecting atrial and ventricular muscle resulting in preexcitation pattern (PE) on the ECG. Patients with WPW also have a higher incidence of atrial fibrillation (AF), an otherwise rare condition in childhood and structural normal hearts. Life-threatening arrhythmias due to AF induced ventricular tachycardia (VT) or ventricular fibrillation (VF), and sudden cardiac death (SCD) may occur as first manifestation of WPW syndrome.

Method: Present study is a retrospective analysis of all patients with PE administered to our pediatric cardiology unit between 2001 and 2021. Identification of those WPW patients with the diagnosis of near SCD, VT, or VF was also made in this study.

Results: A total of 468 patients were referred to our hospital from 2001 to 2021 with PE pattern on the ECG or WPW syndrome: n = 124 were referred for noninvasive risk stratification with transesophageal electrophysiological study (TEPS), in 352 invasive patients, risk stratification was performed, 324 patients were ablated. Seven of 468 patients (1.49%) with WPW or PE were referred with aborted sudden cardiac death or VT/VF, 6/7 without prior symptoms or known PE. Mean age of the patients was 11.6 years, one of them was resuscitated in the first month of life. Of them, 86% were male. VF was documented in six of seven patients, requiring an AED discharge. Other causes for possible VF (chanellopathies, coronary artery anomalies, structural heart disease, and myocarditis) were excluded. Permanent WPW syndrome was present in only five of the seven patients: one patient with documented VF requiring defibrillation had a fasciculoventricular fiber but no AP. The other patient showed only intermittent PE with a negative TEPS 4 years before she presented at aged 13 years with documented VF, complete AV block, and still intermittent WPW pattern. All patients with WPW syndrome were ablated successfully, two patients received an ICD and neurological outcome was favorable in all but one patient.

Conclusion: AF-induced VF/VT and SCD in WPW patients are rare but life-threatening condition, affecting 1.28% of our WPW patient cohort during up to 20 years of follow-up. In our series, noninvasive risk stratification failed to prevent one patient from VF, although here the mechanism of VF induction remains unclear. Individual risk assessment in each patient with WPW/PE is warranted.

Publication History

Article published online:
12 February 2022