Wolff-Parkinson-White syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Sara Zand, M.D.[2] Cafer Zorkun, M.D., Ph.D. [3]

Overview

Wolff-Parkinson-White (WPW) syndrome is the most common cause of ventricular pre-excitation and the second common cause of supraventricular tachycardia. There is a muscle fiber that bridges the atrioventricular groove providing electrical continuity between the atrium and ventricle in parallel to the atrioventricular node-His-Purkinje axis. The atrial impulse activates the entire or part of the ventricle or the ventricular impulse activates the entire atrium or part of it, earlier than normally be expected. Patients with WPW syndrome may present with abrupt palpitation, presyncope, syncope, or sudden cardiac death(SCD). In some patients, SCD is the first presentation of WPW syndrome, especially in the setting of atrial fibrillation with a rapid ventricular response. Wolff-Parkinson-White syndrome is named after the cardiologists Louis Wolff, John Parkinson, and Paul Dudley White who gave a definitive description of the conduction disorder of the heart in 1930. The term Wolff-Parkinson-White syndrome was coined in 1940. Bundle of Kent was first discovered by Albert Frank Stanley Kent, a British physiologist following finding the lateral branch in the atrioventricular groove of the monkey heart. Wolff-Parkinson-White (WPW) syndrome is the occurrence of arrhythmia in the presence of an accessory pathway. WPW can be classified according to the site of origin, location in the mitral or tricuspid annulus, type of conduction (antegrade vs retrograde), and characteristics of the conduction (decremental vs nondecremental). In addition, WPW can be classified based of the type of atrioventricular reciprocating tachycardia (AVRT) it causes, which can be either orthodromic (~95% of the cases) or antidromic.In normal individuals, electrical activity in the heart is initiated in the sinoatrial (SA) node (located in the right atrium), propagates to the atrioventricular (AV) node, and then through the bundle of His to the ventricles of the heart. Individuals with Wolf-parkinson-White (WPW) have an accessory pathway, known as the bundle of Kent, that communicates between the atria and the ventricles. The conduction through the accessory pathway can be bidirectional (most commonly), only retrogarde (less common), or only antegrade (least common). The most common type of tachycardia associated with WPW is atrioventricular reciprocating tachycardia (AVRT). The accessory pathway does not share the rate-slowing properties of the AV node; therefore, the combination of an accessory pathway and cardiac arrhythmia can trigger ventricular fibrillation, a leading cause of sudden cardiac death. The prevalence of WPW syndrome is approximately 100-300 per 100000 individuals worldwide. The incidence of tachyarrhythmia was estimated to be 1000 cases per 100000 individuals in the year in patients with WPW pattern. The incidence of sudden cardiac death in patients with Wolff-Parkinson-White syndrome was estimated to be 70-450 per 100000 patient-years. WPW syndrome is more commonly observed among young patients. In one study WPW syndrome was observed in 7% of individuals over 60-year-old. Men are more commonly affected with WPW syndrome than women. The men to women ratio is approximately 2 to 1. There is no racial predilection for WPW syndrome. High-risk criteria for sudden cardiac death in Wolff-Parkinson-White syndrome during electrophysiology study include the presence of multiple accessory pathways, R-R interval <250 milliseconds in antegrade conduction of accessory pathway during inducing atrial fibrillation, sustained atrial fibrillation induced by AV re-entry tachycardia, presence of Structural heart disease such as ebstein anomaly or hypertrophic cardiomyopathy.



Historical Perspective

Wolff-Parkinson-White syndrome is named after the cardiologists Louis Wolff, John Parkinson, and Paul Dudley White who gave a definitive description of the conduction disorder of the heart in 1930. The term Wolff-Parkinson-White syndrome was coined in 1940. Bundle of Kent was first discovered by Albert Frank Stanley Kent, a British physiologist following finding the lateral branch in the atrioventricular groove of the monkey heart.

Classification

Wolff-Parkinson-White (WPW) syndrome is the occurrence of arrhythmia in the presence of an accessory pathway. WPW can be classified according to the site of origin, location in the mitral or tricuspid annulus, type of conduction (antegrade vs retrograde), and characteristics of the conduction (decremental vs nondecremental). In addition, WPW can be classified based on the type of atrioventricular reciprocating tachycardia (AVRT) it causes, which can be either orthodromic (~95% of the cases) or antidromic.

Pathophysiology

In normal individuals, electrical activity in the heart is initiated in the sinoatrial (SA) node (located in the right atrium), propagates to the atrioventricular (AV) node, and then through the bundle of His to the ventricles of the heart. Individuals with Wolf-parkinson-White (WPW) have an accessory pathway, known as the bundle of Kent, that communicates between the atria and the ventricles. The conduction through the accessory pathway can be bidirectional (most commonly), only retrogarde (less common), or only antegrade (least common). The most common type of tachycardia associated with WPW is atrioventricular reciprocating tachycardia (AVRT). The accessory pathway does not share the rate-slowing properties of the AV node; therefore, the combination of an accessory pathway and cardiac arrhythmia can trigger ventricular fibrillation, a leading cause of sudden cardiac death.

Causes

Differentiating Xyz from Other Diseases

Epidemiology and Demographics

The prevalence of WPW syndrome is approximately 100-300 per 100000 individuals worldwide. The incidence of tachyarrhythmia was estimated to be 1000 cases per 100000 individuals in the year in patients with WPW pattern. The incidence of sudden cardiac death in patients with Wolff-Parkinson-White syndrome was estimated to be 70-450 per 100000 patient-years. WPW syndrome is more commonly observed among young patients. In one study WPW syndrome was observed in 7% of individuals over 60-year-old. Men are more commonly affected with WPW syndrome than women. The men to women ratio is approximately 2 to 1. There is no racial predilection for WPW syndrome.


Risk Factors

High-risk criteria for sudden cardiac death in Wolff-Parkinson-White syndrome during electrophysiology study include the presence of multiple accessory pathways, R-R interval <250 milliseconds in antegrade conduction of accessory pathway during inducing atrial fibrillation, sustained atrial fibrillation induced by AV re-entry tachycardia, precence of Structural heart disease such as ebstein anomaly or hypertrophic cardiomyopathy.

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