Wolff-Parkinson-White syndrome pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

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. (See electrical conduction system of the heart).

Pathophysiology

• The AV node acts as a gatekeeper, limiting the electrical activity that reaches the ventricles of the heart.
• This function of the AV node is important, because if the signals generated in the atria of the heart were to increase in rate (as they do during atrial fibrillation or atrial flutter), the AV node will limit the electrical activity that conducts to the ventricles. For instance, if the atria are electrically activated at 300 beats per minute, half those electrical impulses are blocked by the AV node, so that the ventricles are activated at 150 beats per minute (giving a pulse of 150 beats per minute).
• Another important property of the AV node is that it slows down individual electrical impulses. This is manifest on the ECG as the PR interval, the time from activation of the atria (manifest as the P wave) and activation of the ventricles (manifest as the QRS complex).
• Individuals with WPW syndrome have an accessory pathway that connects the atria and the ventricles, in addition to the AV node. This accessory pathway is known as the bundle of Kent. This accessory pathway does not share the rate-slowing properties of the AV node, and may conduct electrical activity at a significantly higher rate than the AV node.
• For instance, in the example above, if an individual had an atrial rate of 300 beats per minute, the accessory bundle may conduct all the electrical impulses from the atria to the ventricles, causing the ventricles to activate at 300 beats per minute.
• Extremely fast heart rates are potentially dangerous, and can cause hemodynamic instability. In some cases, the combination of an accessory pathway and cardiac arrhythmias can trigger ventricular fibrillation, a leading cause of sudden cardiac death.

A atrioventricular tachycardia through the accessory bundle. The electrical signal travels from the ventricles via the accessory bundle to the atria and returns to the ventricles via the AV node

Genetics

A small percentage of all cases of WPW syndrome are caused by mutations in the gene PRKAG2. PRKAG2 gene encodes a protein that is a part of the enzyme AMP-activated protein kinase (AMPK), which plays an important role in the energy processes of the cell. This enzyme plays an unknown role in the development of heart before birth. Studies have shown that mutations in the PRKAG2 gene lead to alteration in the AMPK enzyme activity causing a build up of abnormal levels of glycogen in the heart muscle cells. Other studies have found that altered AMPK activity is related to changes in the regulation of certain ion channels in the heart which play an important role in maintaining the heart rhythm.