109/Retrospective analysis of WPW syndrome in children in south Wales (1986–2019)

Introduction: Wolf Parkinson White disease is a pre-excitation syndrome with an accessory nodal pathway first described in 1930 by Doctors Wolf, Parkinson and White. This is due to the abnormal accessory pathway connecting the atrium and the ventricle on either side through which anterograde or retrograde transmission of sinus impulses occur. Among general population, incidence is estimated to be 0.1–0.3% more prevalent in males than females.
Aims: A retrospective service evaluation of all WPW cases in paediatric population of South Wales between 1986–2019.
Methods: A retrospective observational study, (time period 1/4/1986–30/4/2019. The project was approved by the clinical audit department, and was registered under the UHB clinical audit database (reference no: 8851). Based on a pre-set inclusion and exclusion criteria, 158 patients were audited. All paediatric patients in south Wales who were diagnosed or evaluated for WPW syndrome between time periods of 1/4/1986–30/4/2019, were included. Patients screened due to family history of WPW, but did not have pre-excitation themselves, were excluded. Patients presenting above 16 years were also excluded.
Results: Of the 158 patients audited, 32 (20.2%) presented in the neonatal period, majority presented after the age of 5 years, 101 (63.9%), between 1 and 5 years there were 26 patients (16.4%). The male (84) female (74) ratio was 1.14:1 with slightly higher male preponderance. The diagnosis was incidental in 30% patients. Acute management according to the APLS protocol was required in 34.9% patients, of which 24.3% required adenosine, of the ones who required adenosine, only 67.6% responded to it, the remaining required cardioversion or further resuscitation. The majority of patients in the age group above 5 years (51.7%) had palpitation at rest which was interesting to note. The total number of children who presented with a life-threatening event was 10 (6.3%), of which two children presented with documented AF and needed cardioversion, one of whom was previously being treated as seizure. One child died (0.6%), who had a background of Danone disease. 150 (95%) of the total children had delta wave, the rest had intermittent pre excitation.
A structural abnormality was detected in 24 (15.2%) of the children, whereas the function was abnormal in 21 (13.3%) of the children. On exercise test delta wave persisted in 36 (70.5%) patients. Flecainide (39%) was the medication of choice in majority of patients managed medically. The rest were either managed with beta blockers or a combination of beta blocker with digoxin. Sotalol was used in 6.2% of the children and Propafenone in 2.6% of children.
EPS was performed in 104 (65.8%) of patients, majority of patients were found to have left sided pathway (32.6%), out of which delta wave was found persisting in nearly 27% of patient after the first ablation.
Conclusion: The most common presentation in the paediatric population of south Wales was at the age of greater than 5 years (63.9%) of patients, diagnosis was incidental in one third of the patients. The most common symptom was palpitation at rest 74 (51.7%).
Even though the death rate was low in our paediatric population, a considerable number 10 (6.3%) presented with a life-threatening event being their first presentation.