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Epidemiology, clinical manifestations, and diagnosis of the Wolff-Parkinson-White syndrome

Authors
Luigi Di Biase, MD, PhD, FHRS, FACC
Edward P Walsh, MD
Section Editors
Samuel Lévy, MD
Bradley P Knight, MD, FACC
Deputy Editor
Brian C Downey, MD, FACC

INTRODUCTION

In 1930, Louis Wolff, Sir John Parkinson, and Paul Dudley White published a seminal article describing 11 patients who suffered from attacks of tachycardia associated with a sinus rhythm electrocardiographic (ECG) pattern of bundle branch block with a short PR interval [1]. This was subsequently termed the Wolff-Parkinson-White (WPW) syndrome, although earlier isolated case reports describing similar patients had been published. In 1943, the ECG features of preexcitation were correlated with anatomic evidence for the existence of anomalous bundles of conducting tissue that bypassed all or part of the normal atrioventricular (AV) conduction system (figure 1).

This topic will review the definition and prevalence of the WPW syndrome. The treatment options for patients with tachyarrhythmias and the WPW syndrome are discussed separately. (See "Treatment of symptomatic arrhythmias associated with the Wolff-Parkinson-White syndrome".)

DEFINITION

Normal AV conduction versus accessory AV pathway conduction — In the normal heart, the atria and the ventricles are electrically isolated, with conduction of electrical impulses from the atria to the ventricles normally occurring via the atrioventricular node (AV) and the His-Purkinje system. Patients with a preexcitation syndrome have an additional pathway, known as an accessory pathway (AP), which directly connects the atria and ventricles, thereby allowing electrical activity to bypass the AV node (table 1). Tissue in the accessory pathways, which are congenital in origin and result from failure of resorption of the myocardial syncytium at the annulus fibrosis of the atrioventricular valves during fetal development, typically conducts electrical impulses more quickly than the AV node, resulting in the shorter PR interval seen on the surface ECG. (See 'Electrocardiographic (ECG) findings' below and "Lown-Ganong-Levine syndrome and enhanced atrioventricular nodal conduction" and "Mahaim fiber tachycardias".)

It has been estimated that most accessory pathways (60 to 75 percent) are capable of bidirectional conduction (anterograde and retrograde) between the atrium and ventricle. However, some accessory pathways (17 to 37 percent) are only capable of conduction in a retrograde fashion from ventricle to atrium [2]. When accessory pathways conduct exclusively in the retrograde direction (so-called "concealed" accessory pathways), they do not generate a delta wave and the WPW pattern on the surface ECG but are still capable of supporting reentrant tachycardia. Retrograde conduction can occur following ventricular pacing or premature beats, and it can form the retrograde arm of an orthodromic atrioventricular reentrant tachycardia (AVRT) circuit. The vast majority of concealed accessory pathways are left-sided [3]. (See 'Anatomy' below and "Atrioventricular reentrant tachycardia (AVRT) associated with an accessory pathway", section on 'Orthodromic AVRT'.)

Less commonly (5 to 27 percent), an accessory pathway is only capable of conduction in the anterograde direction; in such cases, it can form the antegrade arm of an antidromic atrioventricular reentrant tachycardia (AVRT) circuit. The mechanism responsible for unidirectional conduction along an accessory pathway (anterograde only or retrograde only) remains undetermined. (See "Atrioventricular reentrant tachycardia (AVRT) associated with an accessory pathway", section on 'Antidromic AVRT'.)

                                

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Literature review current through: Nov 2016. | This topic last updated: Thu May 26 00:00:00 GMT+00:00 2016.
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References
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