Wolff-Parkinson-White syndrome
Encyclopedia
Wolff–Parkinson–White syndrome (WPW) is a disorder of the heart
in which the ventricles
of the heart
contract prematurely due to an accessory pathway known as the bundle of Kent. This accessory pathway is an abnormal electrical communication from the atria
to the ventricles. WPW is a type of atrioventricular re-entrant tachycardia.
The incidence of WPW syndrome is between 0.1% and 0.3% of the general population.
While the majority of individuals with a bundle of Kent remain asymptomatic
throughout their entire lives, there is a risk of sudden death associated with the syndrome. Sudden death
due to WPW syndrome is rare (incidence of less than 0.6%), and is due to the accessory pathway disrupting the flow of electricity during tachyarrhythmias.
the individual may have symptoms of palpitations (sudden onset and termination of a fast heart rate), dizziness, shortness of breath, and occasionally fainting/near fainting during episodes of supraventricular tachycardia
. An ECG will show the telltale "Delta wave".
(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
).
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, which lasts slightly less than 200 milliseconds, 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 (see below). 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 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
.
WPW may be associated with PRKAG2
.
pathway between the atria
and ventricle
s in the heart. It is an abnormal pathway that is present in a small percentage of the general population. This pathway is a bundle of connecting tissue that may be either between the left atrium
and the left ventricle
, in which case it is termed a type A pre-excitation, or the right atrium
and the right ventricle
, in which case it is termed a type B pre-excitation. Problems arise when this pathway creates an electrical circuit that bypasses the AV node. The AV node has rate-slowing electrical (regulation) properties, whereas the pathway via the Bundle of Kent does not. When an aberrant electrical connection is made via this bundle, tachyarrhythmia occurs.
In order to treat persons with WPW, destruction of the Bundle of Kent is accomplished by radiofrequency catheter ablation
. This procedure is performed almost exclusively by cardiac electrophysiologists.
in an asymptomatic
individual. In this case it is manifested as a delta wave, which is a slurred upstroke in the QRS complex that is associated with a short PR interval. The short PR interval and slurring of the QRS complex is actually the impulse making it through to the ventricles prematurely (across the accessory pathway) without the usual delay experienced in the AV node.
If the patient experiences episodes of atrial fibrillation, the ECG will show a rapid polymorphic wide-complex tachycardia (without torsades de pointes
). This combination of atrial fibrillation and WPW is considered dangerous, and most antiarrhythmic drugs are contraindicated.
When an individual is in normal sinus rhythm, the ECG characteristics of WPW syndrome are a short PR interval, widened QRS complex (greater than 120ms in length) with slurred upstroke of the QRS complex, and secondary repolarization changes reflected in ST segment-T wave changes.
In individuals with WPW syndrome, electrical activity that is initiated in the SA node travels through the accessory pathway as well as through the AV node to activate the ventricles via both pathways. Since the accessory pathway does not have the impulse slowing properties of the AV node, the electrical impulse first activates the ventricles via the accessory pathway, and immediately afterwards via the AV node. This gives the short PR interval and slurred upstroke to the QRS complex known as the delta wave.
In case of type A pre-excitation (left atrioventricular connections), a positive R wave will be seen in V1 ("positive delta") on the precordial leads of the electrocardiogram
, while in type B pre-excitation (right atrioventricular connections), a predominantly negative delta wave will be seen in lead V1 ("negative delta").
Patients with WPW often exhibit more than one accessory pathway, and in some patients as many as eight additional abnormal pathways can be found. This has been seen in individuals with Ebstein's anomaly
.
Wolff–Parkinson–White syndrome is sometimes associated with Leber's hereditary optic neuropathy
(LHON), a form of mitochondrial disease
.
A good history should be taken to determine whether an individual has factors suggestive of a previous episode of unexplained syncope
(fainting) or palpitations (sudden awareness of one's own, usually irregular, heartbeat). These may be due to earlier episodes of a tachycardia associated with the accessory pathway.
Individuals with WPW syndrome in whom the delta waves disappear with increases in the heart rate are considered at lower risk of SCD. This is because the loss of the delta wave shows that the accessory pathway cannot conduct electrical impulses at a high rate (in the anterograde direction). These individuals will typically not have fast conduction down the accessory pathway during episodes of atrial fibrillation.
Risk stratification is best performed via programmed electrical stimulation (PES) in the cardiac electrophysiology
lab. This is an invasive procedure, in which the rate of impulse propagation via the accessory pathway is determined by stimulating the atria and by inducing transient atrial fibrillation
.
High risk features that may be present during PES include an effective refractory period of the accessory pathway less than 270 ms, multiple pathways, septal location of pathway, and inducibility of supraventricular tachycardia
. Individuals with any of these high risk features are generally considered at increased risk for SCD and should be treated accordingly.
It is unclear whether invasive risk stratification (with programmed electrical stimulation) is necessary in the asymptomatic individual. While some groups advocate PES for risk stratification in all individuals under 35 years old, others only offer it to individuals who have history suggestive of a tachyarrhythmia
, since the incidence of sudden death is so low (less than 0.6 percent in some reports).
if their condition is critical (they are hypotensive or lethargic with altered mental status), or, if more stable, medical treatment may be used.
Patients with atrial fibrillation and rapid ventricular response are often treated with amiodarone
or procainamide
to stabilize their heart rate. Procainamide, amiodarone, and cardioversion are now accepted treatments for conversion of tachycardia found with WPW. Adenosine and other AV node blockers should be avoided in atrial fibrillation and atrial flutter with WPW or history of it; this includes adenosine, diltiazem
, verapamil
, other calcium channel blockers and beta blocker
s. Beta-blockers and other pharmacological agents that slow the conduction through the AV pathway (such as digoxin
) should be avoided, because they will exacerbate the syndrome, by blocking the normal heart's electrical pathway, therefore exaggerating the pre-excitation pathway (Bundle of Kent). Patients with a rapid heart beat with narrow QRS complex
es (circus movement tachycardias) may also be cardioverted, alternatively, adenosine may be administered if equipment for cardioversion is immediately available as a backup.
The definitive treatment of WPW syndrome is a destruction of the abnormal electrical pathway by radiofrequency catheter ablation
. This procedure is performed almost exclusively by cardiac electrophysiologists
. Radiofrequency catheter ablation is not performed in all individuals with WPW syndrome because there are inherent risks involved in the procedure. When performed by an experienced electrophysiologist, radiofrequency ablation has a high success rate. Findings from 1994 indicate success rates of as high as 95% in patients treated with radiofrequency catheter ablation for WPW. If radiofrequency catheter ablation is successfully performed, the patient is generally considered cured. Recurrence rates are typically less than 5% after a successful ablation. The one caveat is that individuals with underlying Ebstein's anomaly
may develop additional accessory pathways during progression of their disease.
, Sir John Parkinson
and Paul Dudley White
described the disorder in 1930.
It had been described before by F.N.Wilson in 1915 and A. M. Wedd in 1921.
The first ever diagnosed sufferer of this disease was a S.Singh in 1942, and was diagnosed by K.Bhainiawala a cardiologist at the time.
Heart
The heart is a myogenic muscular organ found in all animals with a circulatory system , that is responsible for pumping blood throughout the blood vessels by repeated, rhythmic contractions...
in which the ventricles
Ventricle (heart)
In the heart, a ventricle is one of two large chambers that collect and expel blood received from an atrium towards the peripheral beds within the body and lungs. The Atria primes the Pump...
of the heart
Heart
The heart is a myogenic muscular organ found in all animals with a circulatory system , that is responsible for pumping blood throughout the blood vessels by repeated, rhythmic contractions...
contract prematurely due to an accessory pathway known as the bundle of Kent. This accessory pathway is an abnormal electrical communication from the atria
Atrium (anatomy)
In anatomy, the atrium , sometimes called auricle , refers to a chamber or space. For example, the term is used for a portion of the lateral ventricle in the brain and the blood collection chamber of the heart...
to the ventricles. WPW is a type of atrioventricular re-entrant tachycardia.
The incidence of WPW syndrome is between 0.1% and 0.3% of the general population.
While the majority of individuals with a bundle of Kent remain asymptomatic
Asymptomatic
In medicine, a disease is considered asymptomatic if a patient is a carrier for a disease or infection but experiences no symptoms. A condition might be asymptomatic if it fails to show the noticeable symptoms with which it is usually associated. Asymptomatic infections are also called subclinical...
throughout their entire lives, there is a risk of sudden death associated with the syndrome. Sudden death
Sudden death
In a sport or game, sudden death is a form of competition where play ends as soon as one competitor is ahead of the others, with that competitor becoming the winner. Sudden death is typically used as a tiebreaker when a contest is tied at the end of the normal playing time or the completion of...
due to WPW syndrome is rare (incidence of less than 0.6%), and is due to the accessory pathway disrupting the flow of electricity during tachyarrhythmias.
Signs and symptoms
Patients are usually asymptomatic. However, during supraventricular tachycardiaSupraventricular tachycardia
Supraventricular tachycardia is a general term that refers to any rapid heart rhythm originating above the ventricular tissue. Supraventricular tachycardias can be contrasted to the potentially more dangerous ventricular tachycardias - rapid rhythms that originate within the ventricular...
the individual may have symptoms of palpitations (sudden onset and termination of a fast heart rate), dizziness, shortness of breath, and occasionally fainting/near fainting during episodes of supraventricular tachycardia
Supraventricular tachycardia
Supraventricular tachycardia is a general term that refers to any rapid heart rhythm originating above the ventricular tissue. Supraventricular tachycardias can be contrasted to the potentially more dangerous ventricular tachycardias - rapid rhythms that originate within the ventricular...
. An ECG will show the telltale "Delta wave".
Pathophysiology
In normal individuals, electrical activity in the heart is initiated in the sinoatrialSinoatrial node
The sinoatrial node is the impulse-generating tissue located in the right atrium of the heart, and thus the generator of normal sinus rhythm. It is a group of cells positioned on the wall of the right atrium, near the entrance of the superior vena cava...
(SA) node (located in the right atrium
Right atrium
The right atrium is one of four chambers in the hearts of mammals and archosaurs...
), propagates to the atrioventricular
Atrioventricular node
The atrioventricular node is a part of the electrical control system of the heart that coordinates heart rate. It electrically connects atrial and ventricular chambers...
(AV) node, and then through the bundle of His
Bundle of His
The bundle of His, known as the AV bundle or atrioventricular bundle, is a collection of heart muscle cells specialized for electrical conduction that transmits the electrical impulses from the AV node to the point of the apex of the fascicular branches...
to the ventricles of the heart. (See electrical conduction system of the heart
Electrical conduction system of the heart
The normal intrinsic electrical conduction of the heart allows electrical propagation to be transmitted from the Sinoatrial Node through both atria and forward to the Atrioventricular Node. Normal/baseline physiology allows further propagation from the AV node to the ventricle or Purkinje Fibers...
).
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
Atrial fibrillation
Atrial fibrillation is the most common cardiac arrhythmia . It is a common cause of irregular heart beat, identified clinically by taking a pulse. Chaotic electrical activity in the two upper chambers of the heart result in the muscle fibrillating , instead of achieving coordinated contraction...
or atrial flutter
Atrial flutter
Atrial flutter is an abnormal heart rhythm that occurs in the atria of the heart. When it first occurs, it is usually associated with a fast heart rate or tachycardia , and falls into the category of supra-ventricular tachycardias. While this rhythm occurs most often in individuals with...
), 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
Pulse
In medicine, one's pulse represents the tactile arterial palpation of the heartbeat by trained fingertips. The pulse may be palpated in any place that allows an artery to be compressed against a bone, such as at the neck , at the wrist , behind the knee , on the inside of the elbow , and near the...
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
Electrocardiogram
Electrocardiography is a transthoracic interpretation of the electrical activity of the heart over a period of time, as detected by electrodes attached to the outer surface of the skin and recorded by a device external to the body...
as the PR interval, which lasts slightly less than 200 milliseconds, the time from activation of the atria (manifest as the P wave
P wave (electrocardiography)
In electrocardiography, during normal atrial depolarization, the main electrical vector is directed from the SA node towards the AV node, and spreads from the right atrium to the left atrium...
) and activation of the ventricles (manifest as the QRS complex
QRS complex
The QRS complex is a name for the combination of three of the graphical deflections seen on a typical electrocardiogram . It is usually the central and most visually obvious part of the tracing. It corresponds to the depolarization of the right and left ventricles of the human heart...
).
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 (see below). 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 cause hemodynamic instability. In some cases, the combination of an accessory pathway and cardiac arrhythmias can trigger ventricular fibrillation
Ventricular fibrillation
Ventricular fibrillation is a condition in which there is uncoordinated contraction of the cardiac muscle of the ventricles in the heart, making them quiver rather than contract properly. Ventricular fibrillation is a medical emergency and most commonly identified arrythmia in cardiac arrest...
, a leading cause of sudden cardiac death
Sudden Cardiac Death
Sudden cardiac death is natural death from cardiac causes, heralded by abrupt loss of consciousness within one hour of the onset of acute symptoms. Other forms of sudden death may be noncardiac in origin...
.
WPW may be associated with PRKAG2
PRKAG2
5'-AMP-activated protein kinase subunit gamma-2 is an enzyme that in humans is encoded by the PRKAG2 gene.- Function :AMP-activated protein kinase is a heterotrimeric protein composed of a catalytic alpha subunit, a noncatalytic beta subunit, and a noncatalytic regulatory gamma subunit. Various...
.
Bundle of Kent
The Bundle of Kent is an extra or accessory conductionElectrical conduction system of the heart
The normal intrinsic electrical conduction of the heart allows electrical propagation to be transmitted from the Sinoatrial Node through both atria and forward to the Atrioventricular Node. Normal/baseline physiology allows further propagation from the AV node to the ventricle or Purkinje Fibers...
pathway between the atria
Atrium (anatomy)
In anatomy, the atrium , sometimes called auricle , refers to a chamber or space. For example, the term is used for a portion of the lateral ventricle in the brain and the blood collection chamber of the heart...
and ventricle
Ventricle (heart)
In the heart, a ventricle is one of two large chambers that collect and expel blood received from an atrium towards the peripheral beds within the body and lungs. The Atria primes the Pump...
s in the heart. It is an abnormal pathway that is present in a small percentage of the general population. This pathway is a bundle of connecting tissue that may be either between the left atrium
Left atrium
The left atrium is one of the four chambers in the human heart. It receives oxygenated blood from the pulmonary veins, and pumps it into the left ventricle, via the mitral valve.-Foramen ovale:...
and the left ventricle
Left ventricle
The left ventricle is one of four chambers in the human heart. It receives oxygenated blood from the left atrium via the mitral valve, and pumps it into the aorta via the aortic valve.-Shape:...
, in which case it is termed a type A pre-excitation, or the right atrium
Right atrium
The right atrium is one of four chambers in the hearts of mammals and archosaurs...
and the right ventricle
Right ventricle
The right ventricle is one of four chambers in the human heart. It receives deoxygenated blood from the right atrium via the tricuspid valve, and pumps it into the pulmonary artery via the pulmonary valve and pulmonary trunk....
, in which case it is termed a type B pre-excitation. Problems arise when this pathway creates an electrical circuit that bypasses the AV node. The AV node has rate-slowing electrical (regulation) properties, whereas the pathway via the Bundle of Kent does not. When an aberrant electrical connection is made via this bundle, tachyarrhythmia occurs.
In order to treat persons with WPW, destruction of the Bundle of Kent is accomplished by radiofrequency catheter ablation
Catheter ablation
Catheter ablation is an invasive procedure used to remove a faulty electrical pathway from the hearts of those who are prone to developing cardiac arrhythmias such as atrial fibrillation, atrial flutter, supraventricular tachycardias and Wolff-Parkinson-White syndrome.It involves advancing several...
. This procedure is performed almost exclusively by cardiac electrophysiologists.
Diagnosis
WPW syndrome is commonly diagnosed on the basis of the surface ECGElectrocardiogram
Electrocardiography is a transthoracic interpretation of the electrical activity of the heart over a period of time, as detected by electrodes attached to the outer surface of the skin and recorded by a device external to the body...
in an asymptomatic
Asymptomatic
In medicine, a disease is considered asymptomatic if a patient is a carrier for a disease or infection but experiences no symptoms. A condition might be asymptomatic if it fails to show the noticeable symptoms with which it is usually associated. Asymptomatic infections are also called subclinical...
individual. In this case it is manifested as a delta wave, which is a slurred upstroke in the QRS complex that is associated with a short PR interval. The short PR interval and slurring of the QRS complex is actually the impulse making it through to the ventricles prematurely (across the accessory pathway) without the usual delay experienced in the AV node.
If the patient experiences episodes of atrial fibrillation, the ECG will show a rapid polymorphic wide-complex tachycardia (without torsades de pointes
Torsades de pointes
Torsades de pointes, or simply torsades, is a French term that literally means "twisting of the points". It was first described by Dessertenne in 1966 and refers to a specific, rare variety of ventricular tachycardia that exhibits distinct characteristics on the electrocardiogram .- Presentation...
). This combination of atrial fibrillation and WPW is considered dangerous, and most antiarrhythmic drugs are contraindicated.
When an individual is in normal sinus rhythm, the ECG characteristics of WPW syndrome are a short PR interval, widened QRS complex (greater than 120ms in length) with slurred upstroke of the QRS complex, and secondary repolarization changes reflected in ST segment-T wave changes.
In individuals with WPW syndrome, electrical activity that is initiated in the SA node travels through the accessory pathway as well as through the AV node to activate the ventricles via both pathways. Since the accessory pathway does not have the impulse slowing properties of the AV node, the electrical impulse first activates the ventricles via the accessory pathway, and immediately afterwards via the AV node. This gives the short PR interval and slurred upstroke to the QRS complex known as the delta wave.
In case of type A pre-excitation (left atrioventricular connections), a positive R wave will be seen in V1 ("positive delta") on the precordial leads of the electrocardiogram
Electrocardiogram
Electrocardiography is a transthoracic interpretation of the electrical activity of the heart over a period of time, as detected by electrodes attached to the outer surface of the skin and recorded by a device external to the body...
, while in type B pre-excitation (right atrioventricular connections), a predominantly negative delta wave will be seen in lead V1 ("negative delta").
Patients with WPW often exhibit more than one accessory pathway, and in some patients as many as eight additional abnormal pathways can be found. This has been seen in individuals with Ebstein's anomaly
Ebstein's anomaly
Ebstein anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.-Presentation:...
.
Wolff–Parkinson–White syndrome is sometimes associated with Leber's hereditary optic neuropathy
Leber's hereditary optic neuropathy
Leber’s hereditary optic neuropathy or Leber optic atrophy is a mitochondrially inherited degeneration of retinal ganglion cells and their axons that leads to an acute or subacute loss of central vision; this affects predominantly young adult males...
(LHON), a form of mitochondrial disease
Mitochondrial disease
Mitochondrial diseases are a group of disorders caused by dysfunctional mitochondria, the organelles that are the "powerhouses" of the cell. Mitochondria are found in every cell of the human body except red blood cells...
.
Risk stratification
Treatment is based on risk stratification of the individual. Risk stratification is performed to determine which individuals with WPW syndrome are at risk for sudden cardiac death (SCD). Sudden cardiac death in these individuals is due to the propagation of an atrial arrhythmia to the ventricles at a very high rate.A good history should be taken to determine whether an individual has factors suggestive of a previous episode of unexplained syncope
Syncope (medicine)
Syncope , the medical term for fainting, is precisely defined as a transient loss of consciousness and postural tone characterized by rapid onset, short duration, and spontaneous recovery due to global cerebral hypoperfusion that most often results from hypotension.Many forms of syncope are...
(fainting) or palpitations (sudden awareness of one's own, usually irregular, heartbeat). These may be due to earlier episodes of a tachycardia associated with the accessory pathway.
Individuals with WPW syndrome in whom the delta waves disappear with increases in the heart rate are considered at lower risk of SCD. This is because the loss of the delta wave shows that the accessory pathway cannot conduct electrical impulses at a high rate (in the anterograde direction). These individuals will typically not have fast conduction down the accessory pathway during episodes of atrial fibrillation.
Risk stratification is best performed via programmed electrical stimulation (PES) in the cardiac electrophysiology
Cardiac electrophysiology
Cardiac electrophysiology is the science of elucidating, diagnosing, and treating the electrical activities of the heart. The term is usually used to describe studies of such phenomena by invasive catheter recording of spontaneous activity as well as of cardiac responses to programmed electrical...
lab. This is an invasive procedure, in which the rate of impulse propagation via the accessory pathway is determined by stimulating the atria and by inducing transient atrial fibrillation
Atrial fibrillation
Atrial fibrillation is the most common cardiac arrhythmia . It is a common cause of irregular heart beat, identified clinically by taking a pulse. Chaotic electrical activity in the two upper chambers of the heart result in the muscle fibrillating , instead of achieving coordinated contraction...
.
High risk features that may be present during PES include an effective refractory period of the accessory pathway less than 270 ms, multiple pathways, septal location of pathway, and inducibility of supraventricular tachycardia
Supraventricular tachycardia
Supraventricular tachycardia is a general term that refers to any rapid heart rhythm originating above the ventricular tissue. Supraventricular tachycardias can be contrasted to the potentially more dangerous ventricular tachycardias - rapid rhythms that originate within the ventricular...
. Individuals with any of these high risk features are generally considered at increased risk for SCD and should be treated accordingly.
It is unclear whether invasive risk stratification (with programmed electrical stimulation) is necessary in the asymptomatic individual. While some groups advocate PES for risk stratification in all individuals under 35 years old, others only offer it to individuals who have history suggestive of a tachyarrhythmia
Tachycardia
Tachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...
, since the incidence of sudden death is so low (less than 0.6 percent in some reports).
Treatment
Acutely, people with WPW who are experiencing tachyarrhythmias may require synchronized electrical cardioversionCardioversion
Cardioversion is a medical procedure by which an abnormally fast heart rate or cardiac arrhythmia is converted to a normal rhythm, using electricity or drugs. Synchronized electrical cardioversion uses a therapeutic dose of electric current to the heart, at a specific moment in the cardiac cycle...
if their condition is critical (they are hypotensive or lethargic with altered mental status), or, if more stable, medical treatment may be used.
Patients with atrial fibrillation and rapid ventricular response are often treated with amiodarone
Amiodarone
Amiodarone is an antiarrhythmic agent used for various types of tachyarrhythmias , both ventricular and supraventricular arrhythmias. Discovered in 1961, it was not approved for use in the United States until 1985...
or procainamide
Procainamide
Procainamide INN is a pharmaceutical antiarrhythmic agent used for the medical treatment of cardiac arrhythmias, classified by the Vaughan Williams classification system as class Ia.-History:...
to stabilize their heart rate. Procainamide, amiodarone, and cardioversion are now accepted treatments for conversion of tachycardia found with WPW. Adenosine and other AV node blockers should be avoided in atrial fibrillation and atrial flutter with WPW or history of it; this includes adenosine, diltiazem
Diltiazem
Diltiazem is a non-dihydropyridine member of the class of drugs known as calcium channel blockers, used in the treatment of hypertension, angina pectoris, and some types of arrhythmia....
, verapamil
Verapamil
Verapamil is an L-type calcium channel blocker of the phenylalkylamine class. It has been used in the treatment of hypertension, angina pectoris, cardiac arrhythmia, and most recently, cluster headaches. It is also an effective preventive medication for migraine...
, other calcium channel blockers and beta blocker
Beta blocker
Beta blockers or beta-adrenergic blocking agents, beta-adrenergic antagonists, beta-adrenoreceptor antagonists or beta antagonists, are a class of drugs used for various indications. They are particularly for the management of cardiac arrhythmias, cardioprotection after myocardial infarction ,...
s. Beta-blockers and other pharmacological agents that slow the conduction through the AV pathway (such as digoxin
Digoxin
Digoxin INN , also known as digitalis, is a purified cardiac glycoside and extracted from the foxglove plant, Digitalis lanata. Its corresponding aglycone is digoxigenin, and its acetyl derivative is acetyldigoxin...
) should be avoided, because they will exacerbate the syndrome, by blocking the normal heart's electrical pathway, therefore exaggerating the pre-excitation pathway (Bundle of Kent). Patients with a rapid heart beat with narrow QRS complex
QRS complex
The QRS complex is a name for the combination of three of the graphical deflections seen on a typical electrocardiogram . It is usually the central and most visually obvious part of the tracing. It corresponds to the depolarization of the right and left ventricles of the human heart...
es (circus movement tachycardias) may also be cardioverted, alternatively, adenosine may be administered if equipment for cardioversion is immediately available as a backup.
The definitive treatment of WPW syndrome is a destruction of the abnormal electrical pathway by radiofrequency catheter ablation
Catheter ablation
Catheter ablation is an invasive procedure used to remove a faulty electrical pathway from the hearts of those who are prone to developing cardiac arrhythmias such as atrial fibrillation, atrial flutter, supraventricular tachycardias and Wolff-Parkinson-White syndrome.It involves advancing several...
. This procedure is performed almost exclusively by cardiac electrophysiologists
Cardiac electrophysiology
Cardiac electrophysiology is the science of elucidating, diagnosing, and treating the electrical activities of the heart. The term is usually used to describe studies of such phenomena by invasive catheter recording of spontaneous activity as well as of cardiac responses to programmed electrical...
. Radiofrequency catheter ablation is not performed in all individuals with WPW syndrome because there are inherent risks involved in the procedure. When performed by an experienced electrophysiologist, radiofrequency ablation has a high success rate. Findings from 1994 indicate success rates of as high as 95% in patients treated with radiofrequency catheter ablation for WPW. If radiofrequency catheter ablation is successfully performed, the patient is generally considered cured. Recurrence rates are typically less than 5% after a successful ablation. The one caveat is that individuals with underlying Ebstein's anomaly
Ebstein's anomaly
Ebstein anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.-Presentation:...
may develop additional accessory pathways during progression of their disease.
History
Cardiologists Louis WolffLouis Wolff
Louis Wolff was an American cardiologist.Louis Wolff married Alice Muscanto, a flute player born in Vilnius who played with her sisters and brothers in a touring musical ensemble. Louis was a concert-quality violinist who enjoyed accompanying his wife and her siblings in their apartment in...
, Sir John Parkinson
John Parkinson (physician)
Sir John Parkinson was an English cardiologist remembered for describing Wolff-Parkinson-White syndrome.- Biography :...
and Paul Dudley White
Paul Dudley White
Paul Dudley White , American physician and cardiologist, was born in Roxbury, Massachusetts, the son of Herbert Warren White and Elizabeth Abigail Dudley. White's interest in medicine was sparked early in life, when he accompanied his father, a family practitioner, on rounds and house calls in a...
described the disorder in 1930.
It had been described before by F.N.Wilson in 1915 and A. M. Wedd in 1921.
The first ever diagnosed sufferer of this disease was a S.Singh in 1942, and was diagnosed by K.Bhainiawala a cardiologist at the time.
Notable cases
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. - Former World Wrestling EntertainmentWorld Wrestling EntertainmentWorld Wrestling Entertainment, Inc. is an American publicly traded, privately controlled entertainment company dealing primarily in professional wrestling, with major revenue sources also coming from film, music, product licensing, and direct product sales...
wrestler Hassan Assad, also known as Montel Vontavious Porter - NY Rangers forward Mike Rupp.
- Shock Rocker Marilyn MansonMarilyn MansonMarilyn Manson may refer to:* Marilyn Manson , an American rock musician* Marilyn Manson , the American rock band led by the singer of the same name...
See also
- Lown–Ganong–Levine syndrome
- Cardiac electrophysiologyCardiac electrophysiologyCardiac electrophysiology is the science of elucidating, diagnosing, and treating the electrical activities of the heart. The term is usually used to describe studies of such phenomena by invasive catheter recording of spontaneous activity as well as of cardiac responses to programmed electrical...
- Electrical conduction system of the heartElectrical conduction system of the heartThe normal intrinsic electrical conduction of the heart allows electrical propagation to be transmitted from the Sinoatrial Node through both atria and forward to the Atrioventricular Node. Normal/baseline physiology allows further propagation from the AV node to the ventricle or Purkinje Fibers...
- ElectrocardiogramElectrocardiogramElectrocardiography is a transthoracic interpretation of the electrical activity of the heart over a period of time, as detected by electrodes attached to the outer surface of the skin and recorded by a device external to the body...
- Electrophysiology studyElectrophysiology studyAn electrophysiology study is a minimally invasive procedure which tests the electrical conduction system of the heart to assess the electrical activity and conduction pathways of the heart. The study is indicated to investigate the cause, location of origin, and best treatment for various...
- Bundle of HisBundle of HisThe bundle of His, known as the AV bundle or atrioventricular bundle, is a collection of heart muscle cells specialized for electrical conduction that transmits the electrical impulses from the AV node to the point of the apex of the fascicular branches...