ROCK the HEART and the Pediatric and Congenital Electrophysiology Society (PACES) are working together to better understand the risk factors for sudden death in patients with WPW.

• Supraventricular tachycardia (SVT) is the most common heart rhythm abnormality in children.
• Wolff-Parkinson-White syndrome (WPW) is a type of SVT that is associated with sudden death.
• Risk factors and treatment decisions to reduce sudden death in Wolff-Parkinson-White syndrome are based on very limited information.

WPW is common. It occurs in about 1-3/1,000. This is likely an underestimate as there may be children with WPW who have no symptoms and have never had an ECG. Some of these people may never develop symptoms but others may start to notice palpitations and some may have more serious symptoms such as fainting or sudden death. Sudden death may be the first symptom in WPW.

Unlike many other diseases that can cause sudden death in young people, WPW can be treated and even cured.

A presentation of the data collected as part of this collaboration was presented at the Heart Rhythm Society and PACES Research Meetings in Boston in May 2015. At the time of that presentation there were 12 centers from the US, Canada and New Zealand participating and this included 240 patients, cases with a life threatening event and controls who had not had a life threatening event.

The results of the data analysis were remarkable. In 1/2 of the 48 patients with a life threatening event, the life threatening event was the first symptom. Events were more common at rest and with noncompetitive activity so sports restriction are not life-saving in this population. Outcomes from the life threatening event were favorable, but there were 5 deaths. Thus, even in the current era of catheter ablation sudden death is still seen in children with WPW and a lower threshold for catheter of ablation may be needed to prevent life threatening events in children with WPW.

Since that meeting an additional 3 centers have joined and we have retrospective data on 360 patients.
A manuscript of these data is being written.

Future Goals:
We need more information about risk in children with WPW. We will be starting a prospective arm of this research project where data will be collected and patients monitored over time to better help us understand what factors are associated with risk in children with WPW patient.

Rock for the Heart was presented with a grant to the new Cardiology Department at the top-rated Children’s Hospital of Pittsburgh. This new, state of the art facility has the most advanced cardiology equipment and is staffed by some of the world’s finest cardiologists.

One of many pediatric heart defects is Wolff-Parkinson-White Syndrome (WPW). Thousands of children are born with this particular defect every year. This is the defect that Matthew died from.

This study, vs. the new study, the first granted by Rock for the Heart, is intended to help determine the best treatment methods for children born with WPW and is described as follows by Steven A. Webber, MBChB, MRCP, Chief of Cardiology at Children’s Hospital of Pittsburgh in his article “The Natural History of WPW Syndrome in a Large Pediatric Cohort and Correlation of Non-Invasive and Invasive Electrophysiology Testing:”

Sudden death is a devastating complication of heart disease in children. Children with WPW have extra electrical pathways in the heart that may lead to fatal heart rhythm abnormalities (arrhythmias) in a portion of cases. Most studies to date have been performed on adults. At the present time, it is critical that we increase our knowledge so that we can help determine which children are at risk of these serious heart rhythm abnormalities. In this study, we will define the natural history of WPW in a very large cohort of pediatric patients with very long-term follow-up (up to 40 years) in the Greater Pittsburgh Region; this will allow estimation of the risk of spontaneous life-threatening arrhythmias, and the incidence of sudden death. We will then review the results of all prior cardiac testing to determine which test (invasive and non-invasive) best predict a good versus bad outcome. In the long run, it is anticipated that these studies will help identify the best approach to diagnostic evaluation and treatment of children with this life threatening condition.