Pediatric Heart Expert Forum
WPW
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WPW

I have a 3 year old little girl named Rylee. At 35 weeks i have an emergency c-section due to elevated fetal heart rate (234-245). shortly after we were told the Rylee had Wolf-Parkinson-White Syndrome. Since we have visited her doctor on a regular basis. Unfortunatly they have not been very helpful in telling us how she developed this or what it might mean for our little girl. she has had SVTs several times. She is no longer on any kind of Beta-Blocker (taken off at 17mts old) What can we expect. and is there a chance that i might have other children, in the futher, with this.
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Dear Kbender,

Wolff-Parkinson-White syndrome (WPW) is a heart condition in which there is an extra electrical connection between the top and the bottom of the heart that exists in addition to the normal connection (the atrioventricular, or A-V, node).  WPW further demonstrates changes on the ECG to show the electrical presence of this extra connection, or pathway.  WPW is congenital, meaning that she was born with it.  Early on, there are numerous electrical connections between the top and the bottom of the heart.  However, by the time the heart is fully formed, by 7-8 weeks of gestation, there is only the A-V node.  Rarely, the extra connection can persist.  It can allow for supraventricular tachycardia (SVT), which is a tachyarrhythmia, or an abnormal fast heart beat.  Most likely, Rylee had SVT in utero, with the heart rates that you’re reporting.

As long as the ECG demonstrates the changes associated with WPW, she is at risk for a couple of issues.  One is SVT, which can’t exist without a second connection between the top and the bottom of the heart.  However, the second is the potential for more life-threatening arrhythmias later in life.  There are some studies that suggest that the risk of sudden death associated with untreated WPW is up to 1% per year.  Not all patients with WPW are at risk for this, though.  It depends on whether the pathway is able to conduct electricity really well, or not.  The way that this can be evaluated is by doing an electrophysiology study, or placing several special catheters with electrodes inside the heart to assess the electrical conduction properties of the heart.  One of the benefits of doing this study, besides assessing the conduction speed of the pathway, is also being able to perform a radiofrequency ablation, in which a small burn is placed on the pathway that eliminates it.  However, this is usually done when the kids are older children or young adolescents.  At our institution, we have been routinely performing ablations on patients with WPW, even if they are asymptomatic.  Meanwhile, if she is still having SVT, we typically do give medicines such as beta blockers to prevent its occurrence.  There are some studies that suggest that up to 1/3 of patients with WPW can have spontaneous disappearance of the pathway by age 18, although this may be overestimated.  

Finally, there is a risk of WPW occurring in other family members.  Usually, it occurs in isolated people in a family, but it has been reported to occur in multiple family members.  This is less common, though.
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