A case report on a patient with Wolff-Parkinson-White syndrome presenting for scoliosis surgery

Authors: Ee-Ling, Ng; Nanthini, Pillay; Suan-Ling, Lim
KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore

Conflicts of interest: None declared

Background
Wolff-Parkinson-White syndrome (WPW) is a congenital syndrome with an increased risk of malignant tachyarrhythmias and sudden cardiac death. Inappropriate treatment of this syndrome peri-operatively can quickly cause clinical deterioration. Unfamiliarity with this rare condition (0.1–0.3% of population) and difficult resuscitation in the prone position could further worsen outcomes. 

Case report
We report a 16 year-old girl with WPW and Down syndrome who underwent scoliosis surgery. She was subjectively asymptomatic after the first episode of tachyarrhythmia, had no electrophysiologic study done, and was not on antiarrhythmics. Her parents were counseled on the risk of tachyarrhythmias requiring electrical cardioversion and cardiopulmonary resuscitation. Adenosine and procainamide were made readily available pre-operatively. We instituted invasive haemodynamic monitoring post-induction and defibrillation pads were applied prior to prone positioning. Excessive increases in sympathetic or vagal tone were prevented. Surgery proceeded uneventfully. She had an uneventful recovery in the paediatric intensive care unit.

Discussion
There have been case reports describing the peri-operative management of WPW, and ACC-AHA 2015 guidelines on the management of tachyarrhythmias in these patients. However, we were unable to find a succinct guide to intra-operative arrhythmias in WPW. These resources also do not address the unique circumstance of our patient needing prolonged major surgery in the prone position. Unfamiliarity with WPW impacted the anaesthetic plan, a concern mirrored in a survey we conducted in our institution. We thus crystallized information in the literature to formulate an easy reference guide, and find that it would be useful to share our experience.

Learning points
WPW poses a significant risk of adverse cardiac outcome. In a patient undergoing major surgery with limited physical access for resuscitation, conscientious preparation and a clear, succinct treatment algorithm could make the difference.