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Wolff-Parkinson-White syndrome

Highlights
Summary Overview

Basics
Definition Epidemiology Aetiology Pathophysiology Classification

Prevention
Screening

Diagnosis
History & examination Tests Differential Step-by-step Criteria Guidelines Case history

Treatment
Details Step-by-step Guidelines

Follow Up
Recommendations Complications Prognosis

Resources
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History & exam


Key factors
presence of risk factors atrioventricular re-entrant tachycardia (AVRT)

Other diagnostic factors


palpitations dizziness

SOB chest pain atrial fibrillation atrial flutter sudden cardiac death (SCD) syncope and presyncope tachycardia in pregnancy congenital cardiac abnormalities

History & exam details

Diagnostic tests
1st tests to order
12-lead ECG

Tests to consider
echocardiogram treadmill exercise test pharmacological testing with procainamide or ajmaline electrophysiology study Diagnostic tests details

Treatment details
Presumptive
unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF direct current (DC) cardioversion

Acute
stable: narrow complex (orthodromic AV reciprocating) tachycardia carotid sinus massage or Valsalva manoeuvre IV adenosine or AV nodal blocking drugs or anti-arrhythmics rapid atrial pacing direct current (DC) cardioversion stable: wide complex (antidromic AV reciprocating) tachycardia IV adenosine or anti-arrhythmics rapid atrial pacing direct current (DC) cardioversion

stable: pre-excited tachycardia due to atrial fibrillation or atrial flutter anti-arrhythmics + consider anticoagulation rapid atrial pacing (for atrial flutter) DC cardioversion stable: pre-excited tachycardia due to atrial tachycardia anti-arrhythmics rapid atrial pacing DC cardioversion

Ongoing
following acute treatment: asymptomatic risk stratification and monitoring catheter ablation following acute treatment: minimally symptomatic monitoring + vagal manoeuvre education (for orthodromic AVRT) catheter ablation pharmacological therapy following acute treatment: symptomatic catheter ablation anti-arrhythmics beta-blockers Treatment details

Summary
Myocardial fibres from the atrium to the ipsilateral ventricle across the mitral or tricuspid annulus (accessory pathway) cause the ventricle to become pre-excited. WPW syndrome is usually restricted to symptomatic patients with a typical ECG abnormality; WPW pattern signifies an asymptomatic patient with typical ECG abnormalities. death. Asymptomatic patients, except for those with specialised jobs (e.g., airline pilot, school bus driver), should not be treated but can be monitored for symptoms. Minimally symptomatic patients may be treated with catheter ablation or medical therapy or only during the rare symptomatic episodes. Symptomatic patients usually undergo catheter ablation as a first-line therapy. Patients often present with AV re-entrant tachycardia or atrial fibrillation and, rarely, sudden cardiac

Catheter ablation has a high efficacy and low risk and can be used either as initial therapy or for patients experiencing side effects or arrhythmia recurrence during drug therapy.

Definition
Occurs when one or more strands of myocardial fibres capable of conducting electrical impulses (known as accessory pathways [APs] or bypass tracts) connect the atrium to the ipsilateral ventricle across the mitral or tricuspid annulus. [1] Conduction from the atrium reaches the adjacent ventricle earlier via the AP, which normally has no conduction delay, and a part of the ventricle is pre-excited. The term "Wolff-Parkinson-White (WPW) syndrome" is usually restricted to symptomatic patients with a typical ECG abnormality, whereas the term "WPW pattern" signifies an asymptomatic patient with typical ECG abnormalities. [2]

Epidemiology
The exact prevalence of pre-excitation syndrome is difficult to estimate because most patients are asymptomatic. Intermittent pre-excitation and loss of pre-excitation over time also precludes accurate estimation of its prevalence. The prevalence of WPW-pattern ECG in the general population is 0.1% to 0.3%. [3] [4] The yearly incidence is 0.004% to 0.1% (50% of these are asymptomatic). The male-to-female ratio is 2:1. [5] [6] Signs of preexcitation were absent in the initial ECG in 22% of subjects with WPWpattern ECG and 40% of these lost pre-excitation in subsequent ECG recordings. [5] The prevalence of WPW syndrome varies with the population studied. In a review of 22,500 healthy aviation personnel, the WPW pattern was seen in 0.25%; however, only 1.8% of these patients had documented arrhythmia. It can be encountered at any age, but the highest incidence is in the third and fourth decades of life. [7]In a report of 228 subjects with WPW syndrome, the overall incidence of arrhythmia was 1% per year during a 22year follow-up.

Aetiology
WPW pattern on ECG arises from a developmental cardiac defect in the AV electrical insulation at the AV groove due to the presence of an AP. These APs are usually single epicardial strands of tissue that travel across the AV groove to connect the atrium and the adjacent ventricular myocardium. View image Less commonly, they are multiple hair-like strands or a broad band of tissues. Rarely, these muscle bands extend over coronary sinus diverticula or may connect right atrial appendage to the anterior right ventricle. The incidence of an AP is 40% to 60% on the left free wall, 20% on the right or left

posteroseptal area, 13% to 21% on the right free wall, and 2% to 10% on the anteroseptal area.

Common locations of accessory pathways (APs) across the mitral and tricuspid annuliFrom the collection of Dr Mithilesh K. DasUnlike the AV node, where

conduction slows at faster rates of stimulation or rapid atrial rates (decremental conduction), approximately 90% of these APs are rapidly conducting (presumably because of INa+ dependent conduction), resulting in circus movement tachycardia involving the atrium and ipsilateral ventricle with the AP as one limb and AV node as another limb of the circuit (AV re-entrant tachycardias). Approximately 10% of the APs in WPW syndrome are slowly conducting (AV node-like property, perhaps ICa+ dependent). The presence of these APs can be associated with various disorders. Ebstein's anomaly is a malformation of the tricuspid valve and right ventricle characterised by adherence of the septal and posterior tricuspid leaflets to the underlying myocardium, apical displacement of the tricuspid annulus and dilation of the atrialised portion of the right ventricle. View image Other rare associations include hypertrophic cardiomyopathy, mitral valve prolapse, atrial septal defect, ventricular septal defect, transposition of the great vessels, coarctation of the aorta, dextrocardia, coronary sinus diverticula, right and left atrial aneurysms, cardiac rhabdomyomas (as seen in patients with tuberous sclerosis), Marfan's syndrome, and Friedreich's ataxia. View image The incidence of multiple APs (2 to 5) in patients with WPW syndrome is 5% to 13%. [8] The frequency of multiple APs in left lateral, right lateral, and septal areas are 44%, 33%, and 22%, respectively. It is more common in patients with a family history of WPW syndrome and in patients with Ebstein's anomaly or other congenital heart diseases (10% to 20%). [9] Multiple APs

are associated with a higher incidence of antidromic reciprocating tachycardia (ART) and a higher incidence of sudden cardiac death (SCD).

Pathophysiology
Normal conduction from the atria to the ventricles occurs via the AV node (where minor physiological delay occurs) and through the His-Purkinje system, resulting in a normal PR interval and a narrow QRS complex. In WPW syndrome, because of the physiological delay of conduction in the AV node, conduction from the atrium reaches the adjacent ventricle earlier via the AP, which normally has no conduction delay, and a part of the ventricle is pre-excited, resulting in a slurred upstroke at the initiation of the QRS complex, known as the delta wave. Therefore, the QRS complexes are the fusion of the normal ventricular depolarisation via the AV node-His bundle and the depolarisation of a part of the ventricle via the AP. The degree of pre-excitation depends upon the relative contribution of the impulse via the AP versus the AV node. Conduction over the AP results in shortening of the PR interval (<120 ms) and widening of the QRS complex (>110 ms) with secondary ST-T wave changes. View image These APs in the WPW syndrome are usually capable of conducting bidirectionally (atrium to ventricle and ventricle to atrium). When an AP is capable of retrograde conduction only (from the ventricle to the atria), the ECG is normal and no delta wave is seen (concealed AP). View image These APs serve as a substrate for re-entry and can cause a supraventricular tachycardia (SVT) involving the AV node as the other connection between the atrium and the ventricle. This type of SVT is termed AVRT. During atrial fibrillation (AF), which occurs in up to one third of WPW syndrome patients, the AP can conduct rapidly to the ventricle, rarely causing ventricular fibrillation (VF) that may result in sudden cardiac death (SCD).
AVRT: AVRT is the most common arrhythmia, which occurs in approximately 70% to 80% of the symptomatic patients with WPW syndrome. The common form of AVRT involves impulse conduction via the AV node, down the His-Purkinje system to the ventricles anterogradely, and the AP retrogradely, resulting in rapid atrial activation after ventricular depolarisation. This is known as orthodromic reciprocating tachycardia (ORT). Less commonly (5% to 10%), AVRT results from impulse conduction in a direction opposite to ORT: that is, conduction from atrium to ventricle via the AP and then from the ventricle to the atrium via the His-Purkinje system and the AV node. Therefore, it is a regular wide complex tachycardia (WCT), because ventricular activation is transmyocardial rather than via the specialised conduction tissue. This type of AVRT is known as antidromic reciprocating tachycardia (ART). View image ORT: AVRT occurs in 70% to 80% of patients with WPW syndrome. During ORT, the impulses reach the ventricles via the normal conduction system (AV node and His-Purkinje system) and then return to the atrium via

the AP as soon as ventricular depolarisation reaches the valve annuli. Tachycardia more commonly starts after a premature atrial complex that blocks at the refractory AP but is able to conduct down the AV node to the ventricles. By this time, the AP recovers and the impulse reaches the atrium via the AP and then travels to the ventricles via the AV node, to continue as ORT. View image During ORT the ventricle is depolarised via the normal conduction system; ORT is a narrow complex tachycardia with a short RP` interval (short RP` tachycardia). View image ART: ART is a less common arrhythmia in WPW syndrome patients. The common mechanism of initiation of the ART is by a premature atrial complex. View image The impulse is blocked at the AV node if it is refractory but conducts down the AP to the ventricles and then back to the atrium via the AV node, and then the circus movement tachycardia is initiated. The baseline ECG (upper panel) of a patient with minimum preexcitation and the WCT (lower panel) is an ART with negative delta wave in inferior leads and lead V1 suggestive of a right inferoseptal AP as shown here. View image The ART is a wide QRS tachycardia and therefore can be confused with a ventricular tachycardia (VT) or an SVT with aberrancy. The baseline ECG is the key in most cases. If there is positive QRS concordance (QRS polarity positive in all precordial leads due to annular location of the APs) during ART, then this may mimic a VT. However, if there is negative concordance (QRS polarity negative in all precordial leads), then an ART is ruled out - except for a rare AVRT involving an atriofascicular pathway, which has AV node-like property that connects the tricuspid annulus to the distal right bundle and results in a left bundle branch block configuration WCT. AF: AF is encountered in 10% to 35% of patients with WPW syndrome. AF with rapid conduction via the AP is recognised by an irregular WCT with a varying degree of ventricular pre-excitation. Patients with a rapidly conducting AP or multiple APs are at a risk for VF due to rapid ventricular stimulation. View image Atrial flutter and atrial tachycardia: Atrial flutter and atrial tachycardia can result in a regular pre-excited WCT. Atrial flutter is encountered in 5% to 10% of patients with WPW syndrome. If the conduction is rapid, especially during atrial flutter with 1:1 AV conduction, then rapid stimulation of ventricle can also result in VF. AV nodal Wenckebach pattern over the AV node or the AP in atrial flutter and AF can result in an irregular WCT. View image

Classification
Manifest accessory AP versus concealed AP APs in WPW syndrome are usually capable of conducting bidirectionally (atrium to ventricle and ventricle to atrium). When an AP is capable of conducting retrogradely only (from the ventricle to the atria), the ECG is normal and no delta wave is seen (concealed AP). View image These APs serve as a substrate for re-entry and can cause a supraventricular tachycardia (SVT) involving the AV node as the other connection between the atrium and the ventricle. This type of SVT is termed atrioventricular re-entrant tachycardia (AVRT). Asymptomatic/minimally symptomatic/symptomatic patients

Minimally symptomatic patients are those with brief, well-tolerated episodes of symptoms (usually fewer than 5 episodes per year of non-pre-excited SVT). Symptomatic patients can be considered as those with episodes less well tolerated by the patient. Tolerance is subjective by the patient. AV re-entrant tachycardia (AVRT) AVRT is the most common arrhythmia, which occurs in approximately 70% to 80% of the symptomatic patients with WPW syndrome. Orthodromic reciprocating tachycardia (ORT): during ORT the ventricle is depolarised via the normal conduction system; ORT is a narrow complex tachycardia with a short RP` interval (short RP` tachycardia). View imageView image Antidromic reciprocating tachycardia (ART): the ART is a wide QRS tachycardia. View image

Screening
Family members are not commonly screened.

History & examination


Key diagnostic factorshide all
presence of risk factors (common) Key risk factors are congenital cardiac abnormalities. The most common is Ebstein's anomaly. atrioventricular re-entrant tachycardia (AVRT) (common)

The most common arrhythmia is AVRT, which occurs in approximately 70% to 80% of patients.

Other diagnostic factorshide all


palpitations (common) Presenting feature of an acute arrhythmia due to WPW. dizziness (common) Presenting feature of an acute arrhythmia due to WPW. SOB (common) Presenting feature of an acute arrhythmia due to WPW. chest pain (common) Presenting feature of an acute arrhythmia due to WPW. atrial fibrillation (common)

Atrial fibrillation and flutter occur alone in 16% of patients and in 20% of patients with AVRT. Typically the heart rate varies between 150 and 240 bpm.

atrial flutter (common)

Atrial fibrillation and flutter occur alone in 16% of patients and in 20% of patients with AVRT. Typically the heart rate varies between 150 and 240 bpm.

congenital cardiac abnormalities (common)

From 7% to 20% of patients with WPW syndrome have other accompanying congenital abnormalities. The most common is Ebstein's anomaly, which is associated with single or multiple right-sided APs.

sudden cardiac death (SCD) (uncommon)

The following factors are considered high risk for SCD, especially in patients with a history of syncope: fastest rate 225 bpm during atrial fibrillation; anterograde refractory period 270 ms; multiple APs.

syncope and presyncope (uncommon)

Usually with a history of palpitations and dizziness. Syncope is an uncommon presentation (incidence <5%) of WPW syndrome. Most of these episodes are believed to be due to neurocardiogenic syncope. It could rarely be a manifestation of atrial fibrillation with rapid ventricular response or a self-terminating polymorphic ventricular tachycardia and ventricular fibrillation (VF), which occur secondary to the rapid ventricular rates due to anterograde conduction via the AP.

tachycardia in pregnancy (uncommon)

The incidence of symptomatic tachycardia is reported to be higher during pregnancy in women with WPW.

Risk factorshide all


Strong Ebstein's anomaly

The most common congenital abnormality in WPW is Ebstein's anomaly. It is associated with single or multiple right-sided APs.View image

Weak hypertrophic cardiomyopathy

Less commonly, patients with WPW syndrome may have other accompanying congenital

abnormalities.View image mitral valve prolapse

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

atrial septal defect

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

ventricular septal defect

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

transposition of great vessels

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

coarctation of aorta

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

dextrocardia

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

coronary sinus diverticula

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

right and left atrial aneurysms

Less commonly, patients with WPW syndrome may have other accompanying congenital abnormalities.

cardiac rhabdomyomas Seen in patients with tuberous sclerosis. Marfan's syndrome Occasionally associated with WPW syndrome. Friedreich's ataxia Occasionally associated with WPW syndrome. family history

The incidence of familial WPW syndrome among patients with APs is reported to be 3.4% and the prevalence in first-degree relatives is 0.55%. [7] [10]Autosomal dominant inheritance is also reported. Familial forms have a higher incidence of multiple APs, but usually do not have male preponderance or structural cardiac abnormalities. Rare inherited disorders are also associated with familial WPW syndrome. [11]

Diagnostic tests
1st tests to orderhide all
Test

12-lead ECG

ECG should be recorded in any patients with suspected supraventricular arrhythmia. Intermittent pre-excitation is to conduction solely via the AV node (without pre-excitation). Sometimes, pre-excited QRS complexes alternate

by the electrocardiographic presence of QRS complexes with pre-excitation intermingled with narrow QRS comp

narrow QRS complex because of a long refractory period of the AP. View image Intermittent pre-excitation indica

the AP is incapable of conduction to the ventricle fast enough to pose a risk for ventricular fibrillation during rapid tachycardia has been developed. View image re-entrant tachycardia.

arrhythmias such as atrial fibrillation or flutter. An algorithm of delta wave polarity in 12-lead ECG or during antidr

Twelve-lead ECG at baseline shows no delta waves and short RP` tachycardia during the clinical arrhythmia in A

Tests to considerhide all


Test

echocardiogram Risk of associated heart disease is 7% to 20%.

treadmill exercise test

An important indicator of a low risk of sudden cardiac death is the disappearance of pre-excitation during exercis refractory period of the AP and if the refractory period of the AP is reached, then the conduction down the AP is fibrillation. pharmacological testing with procainamide or ajmaline

to a long anterograde refractory period of the AP. [14] Sympathetic stimulation occurring during exercise shorten

blocked. This indicates that during atrial fibrillation, conduction down the AP will not be fast enough to cause ven

Pharmacological testing is performed with IV procainamide (10 mg/kg over 5 minutes) or ajmaline (1 mg/kg body

minutes). Both drugs prolong the refractory period of the AP. If pre-excitation is abolished during or after the infus

most probably indicates a low risk for SCD. These anti-arrhythmic drugs prolong the refractory period of His-Purk

system and may cause complete heart block. Additionally, these drugs can provoke torsades de pointes in 2% to only after 4 to 5 hours of monitoring. electrophysiology study semi-invasive atrial stimulation by trans-oesophageal route. [15] [16] [14] [17]

patients. Therefore, these drugs should be used in a monitored setting and the patients should be discharged ho

Risk stratification is performed invasively by programmed electrical stimulation with intracardiac recordings or wit

Differential diagnosis
Condition Atriofascicular pathway Differentiating signs/symptoms Differentiating tests

There may be no difference in signs and symptoms in acute presentatio n with

Baseline ECG normal. On electrophysiological study, the pathway w lateral tricuspid annulus to right ventricular apex.

Arrhythmias not seen are: orthodromic atrioventricular re-entry tach fibrillation and flutter, and long RP` tachycardia. View image

arrhythmia s.
Lown-Ganong-Levine syndrome

There may be no difference in signs and symptoms in acute presentatio n with arrhythmia s.

Baseline ECG shows short PR interval. On electrophysiological stud bypass or enhanced AV nodal conduction.

Arrhythmias not seen are: antidromic AVRT, atrial fibrillation and flu tachycardia.

Nodofasicular pathway

There may be no difference in signs and symptoms in acute presentatio n with arrhythmia s.

Baseline ECG shows short PR interval, but no delta wave. On elect there will be a pathway from atrioventricular node to the ventricle.

Arrhythmias not seen are: orthodromic AVRT, antidromic AVRT, atr and long RP` tachycardia. View image

Fasciculoventricular pathway

There may be no difference in signs and symptoms in acute presentatio n with arrhythmia

Baseline ECG shows short PR, narrow QRS, and delta wave. On e study, there will be a His bundle or bundle branch to the ventricle.

Arrhythmias not seen are: orthodromic AVRT, antidromic AVRT, atr and long RP` tachycardia. View image

s.
Other APs with atypical course

There may be no difference in signs and symptoms in acute presentatio n with arrhythmia s.

Baseline ECG shows delta wave. Electrophysiology shows a corona atrial appendage to ventricle. View image

Step-by-step diagnostic approach


It is common to have WPW pattern as an incidental finding in healthy people who have ECG recording done for a routine health check-up. A 12-lead ECG showing delta waves signifies APs capable of conducting anterograde. In the majority of patients these APs are capable of retrograde conduction as well. View image

Acute presentations
Patients with an acute arrhythmia due to WPW may present with palpitations, dizziness, shortness of breath, and chest pain. The most common arrhythmias diagnosed are atrioventricular re-entrant tachycardia (AVRT), atrial fibrillation, and atrial flutter. Sudden cardiac death and syncope and presyncope are associated outcomes but rare. ECG should be recorded in any patients with suspected supraventricular arrhythmia.

Congenital cardiac abnormalities


From 7% to 20% of patients with WPW syndrome have other accompanying congenital abnormalities, the most common being Ebstein's anomaly, which is associated with single or multiple right-sided APs. View image Other rare associations include hypertrophic cardiomyopathy, View image mitral valve prolapse, atrial septal defect, ventricular septal defect, transposition of the great vessels, coarctation of the aorta, dextrocardia, coronary sinus diverticula, right and left atrial aneurysms, cardiac rhabdomyomas (as seen in

patients with tuberous sclerosis), Marfan's syndrome, and Friedreich's ataxia. Echo can be used to diagnose these.

Locating APs
The precise localisation of these APs helps in assessing the success rate and risks for catheter ablation, and in planning the ablation strategy. For example, left-sided APs are often ablated via a transseptal approach, and paraseptal APs, which are close to the His bundle, are associated with a high risk for complete AV block during catheter ablation (some electrophysiologists now prefer cryoablation for APs close to the His bundle; see the treatment section). Several algorithms have been developed, but, as a general rule, a positive delta wave (first 40 ms of QRS complex) in lead V1 suggests a left-sided AP and a negative delta wave in V1 suggests a right-sided AP. View image A positive delta wave in inferior lead with inferior axis represents anteroseptal AP. View image A left axis (with a negative delta wave in lead V1) suggests a right lateral AP. If the delta waves in the inferior leads are negative, then it denotes a posteroseptal AP right or left, depending on the delta wave vector in lead V1 as described above. View image A negative delta wave in lead I, aVL, and V6 (rightward QRS axis) with a positive delta wave in lead V1 suggests a left lateral pathway. It is prudent to recognise that the delta wave vector can only be determined with precision when the ECG shows maximum pre-excitation. A right-sided AP with minimal pre-excitation but with a posteroseptal location cannot be determined with a high probability until ART occurs. View image

Risk stratification
Patients with WPW pattern should be risk stratified with non-invasive and invasive tests, and those who are low risk should be monitored for any future arrhythmia. Since non-invasive tests are considered inferior to invasive electrophysiological assessment for risk of sudden cardiac death, some electrophysiologists feel that some asymptomatic patients warrant invasive risk stratification. Therefore, asymptomatic patients should be referred to an electrophysiologist or a cardiologist with expertise in arrhythmia evaluation for risk stratification. While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13] The risk of sudden cardiac death is determined by the

anterograde refractory period of the accessory pathway. If the refractory period is very short, patients are at risk for developing ventricular fibrillation in the setting of atrial fibrillation conducting rapidly over the accessory pathway. If ventricular pre-excitation is intermittent at rest, then the accessory pathway is low risk for causing sudden cardiac death. If ventricular pre-excitation is always present at rest, patients should undergo exercise treadmill testing looking for abrupt loss of pre-excitation, again indicative of an accessory pathway incapable of dangerously rapid conduction from the atrium to the ventricle. If pre-excitation does not abruptly disappear with exercise, then invasive risk stratification at electrophysiology study should be considered. Accessory pathways capable of rapid anterograde conduction should be ablated to reduce the risk of sudden cardiac death, regardless of whether or not they cause supraventricular tachycardia. Intermittent pre-excitation occurs due to age-related degeneration of the APs, and usually these pathways have a longer refractory period. It is also important to note that left lateral pathways may not be able to pre-excite the left ventricle earlier than the arrival of the impulse via the normal route due to its distance from the sinus node. Therefore, the baseline ECG may be unremarkable with a normal PR interval and no delta wave on the ECG. These pathways become manifest only when AV nodal conduction is relatively slow due to altered autonomic tone, rapid atrial rate due to sinus tachycardia (such as during exercise), or atrial arrhythmias (latent WPW syndrome). The ECG of a patient with minimal pre-excitation who developed an AVRT is shown here. View imageView image A treadmill exercise test can be used as an indicator of risk of sudden death. View imageView imageView image
Click to view diagnostic guideline references.

Diagnostic criteria

WPW syndrome versus WPW-pattern ECG


WPW syndrome or pre-excitation syndrome occurs when one or more strands of myocardial fibres capable of conducting electrical impulses (known as APs or bypass tracts) connect the atrium to the ipsilateral ventricle across the mitral or tricuspid annulus. [1] A patient who is asymptomatic but with typical WPW ECG abnormalities is referred to as having WPW pattern.

Asymptomatic/minimally symptomatic/symptomatic patients


Minimally symptomatic patients are those with brief well-tolerated episodes of symptoms (usually fewer than 5 episodes per year of non-pre-excited SVT). Symptomatic patients can be considered as those with episodes less well tolerated by the patient. Tolerance is subjective by the patient.

Case history #1
A 42-year-old man complains of palpitations following dizziness and a brief loss of consciousness. His wife reports that he is pale and short of breath. Emergency medical services were called and found him pulseless. The ECG revealed a rapid, irregular wide complex tachycardia. Later he was successfully resuscitated with two successive DC shocks.

Case history #2
A 35-year-old man presents to an ER with palpitation, SOB, dizziness, and chest pain of 4 hours' duration. An ECG demonstrated narrow-complex short RP` tachycardia that responded to IV adenosine. The ECG during sinus rhythm revealed signs of pre-excitation.

Other presentations
Asymptomatic people are sometimes diagnosed during a routine physical examination. The most common age of the presentation is adolescence and early adulthood (in third and fourth decades); however, it can occur at any age. There can be a long-standing history of palpitations lasting for minutes to hours.

Treatment Options

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group stable: narrow complex (orthodromic AV reciprocating) tachycardia line 1st Treatmenthide all

carotid sinus massage or Valsalva manoeuvre

Since ORT involves the AV node as one limb of the circus movement tachycardia, any manoeuvre or drug that slows or interrupts conduction in the AV node can terminate the tachycardia. In a haemodynamically stable patient, vagal manoeuvres such as a carotid sinus massage should be performed at the bedside, or, alternatively, patients can be instructed to perform a Valsalva manoeuvre to terminate the arrhythmia.

2nd

IV adenosine or AV nodal blocking drugs or antiarrhythmics

Adenosine is preferred because of its ultra-short half life (<18 seconds). It can be used a second

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

time if there is no response. It should be given in the veins closer to the heart.

AV nodal blocking drugs (diltiazem, verapamil, metoprolol) can be used when there is no response or a recurrence after carotid sinus massage and adenosine.

Anti-arrhythmic drugs (procainamide, ibutilide, amiodarone, flecainide) can be used when there is no response or a recurrence after carotid sinus massage, adenosine, and AV nodal blocking drugs. Primary Options adenosine : 6 mg intravenously initially, followed by 12 mg intravenously in 1-2 minutes if required

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

Secondary Options diltiazem : 0.25 mg/kg (average adult dose 20 mg) intravenously initially, followed by 0.35 mg/kg (average adult dose 25 mg) intravenously in 15 minutes if required OR verapamil : 2.5 to 5 mg intravenously initially, followed by 5-10 mg (or 0.15 mg/kg) intravenously in 15-30 minutes if required, maximum 30 mg/total dose OR metoprolol : 2.5 to 5 mg intravenously every 2-5 minutes as needed, maximum 15 mg/total dose

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

Tertiary Options procainamide : 100 mg intravenously every 5 minutes as needed, maximum 1000 mg/total dose OR ibutilide : <60 kg body-weight: 0.01 mg/kg intravenously initially, followed by 0.01 mg/kg intravenously in 10 minutes if required; 60 kg body-weight: 1 mg intravenously initially, followed by 1 mg intravenously in 10 minutes if required OR amiodarone : 150 mg intravenously initially, followed by 0.5 to 1 mg/minute intravenous infusion if needed OR

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

flecainide : 2 mg/kg (maximum 150 mg/dose) intravenously initially, followed by 1.5 mg/kg/hour for 1 hour if needed, then 0.1 to 0.25 mg/kg/hour for up to 24 hours if needed, maximum 600 mg/day
3rd

rapid atrial pacing

Rapid atrial pacing using a temporary pacemaker for overdrive suppression of the tachycardia can be used if previous drug treatments fail.

4th

direct current (DC) cardioversion

In patients whose symptoms persist despite pharmacotherapy or atrial pacing, DC cardioconversion should be used.

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

stable: wide complex (antidromic AV reciprocating) tachycardia

1st

IV adenosine or anti-arrhythmics

If the tachycardia is haemodynamically stable, then management is with IV adenosine or antiarrhythmic drugs.

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

Adenosine is preferred because of its ultra-short half life (<18 seconds). It can be used a second time if there is no response. It should be given in the veins closer to the heart.

Anti-arrhythmic drugs (procainamide, ibutilide, amiodarone, flecainide) can also be used when there is no response or a recurrence after adenosine.

Calcium channel blockers, digoxin, and betablockers are contraindicated in this population, because these drugs slow the conduction via the AV node but do not have any effect on the accessory pathway. This may result in a rapid conduction via the accessory pathway, which may

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

cause ventricular tachycardia and ventricular fibrillation leading to sudden cardiac death. Primary Options adenosine : 6 mg intravenously initially, followed by 12 mg intravenously in 1-2 minutes if required Secondary Options procainamide : 100 mg intravenously every 5 minutes as needed, maximum 1000 mg/total dose OR ibutilide : <60 kg body-weight: 0.01 mg/kg intravenously initially, followed by 0.01 mg/kg intravenously in 10 minutes if required; 60 kg

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

body-weight: 1 mg intravenously initially, followed by 1 mg intravenously in 10 minutes if required OR amiodarone : 150 mg intravenously initially, followed by 0.5 to 1 mg/minute intravenous infusion if needed OR flecainide : 2 mg/kg (maximum 150 mg/dose) intravenously initially, followed by 1.5 mg/kg/hour for 1 hour if needed, then 0.1 to 0.25 mg/kg/hour for up to 24 hours if needed, maximum 600 mg/day
2nd

rapid atrial pacing

Rapid atrial pacing using a temporary pacemaker

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

for overdrive suppression of the tachycardia can be used if previous drug treatments fail.
3rd

direct current (DC) cardioversion

In patients whose symptoms persist despite pharmacotherapy or atrial pacing, DC cardioconversion should be used.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

Conscious sedation (e.g. with IV propofol) is also required.

stable: pre-excited tachycardia due to atrial fibrillation or atrial flutter

1st

anti-arrhythmics + consider anticoagulation

Pre-excited tachycardia results in a rapid irregular wide complex tachycardia with varying duration and amplitude of QRS complexes depending upon the degree of pre-excitation.

IV infusions of anti-arrhythmic drugs such as procainamide, ibutilide, flecainide, or amiodarone, which prevent rapid conduction through the AP, are used, even though they may not be able to terminate the atrial arrhythmia. [15]

Anticoagulation should be considered in atrial

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

fibrillation and atrial flutter depending on presence of co-morbid cardiologic abnormalities and duration of onset. Primary Options procainamide : 100 mg intravenously every 5 minutes as needed, maximum 1000 mg/total dose OR ibutilide : <60 kg body-weight: 0.01 mg/kg intravenously initially, followed by 0.01 mg/kg intravenously in 10 minutes if required; 60 kg body-weight: 1 mg intravenously initially, followed by 1 mg intravenously in 10 minutes if required OR

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

amiodarone : 150 mg intravenously initially, followed by 0.5 to 1 mg/minute intravenous infusion if needed OR flecainide : 2 mg/kg (maximum 150 mg/dose) intravenously initially, followed by 1.5 mg/kg/hour for 1 hour if needed, then 0.1 to 0.25 mg/kg/hour for up to 24 hours if needed, maximum 600 mg/day
2nd

rapid atrial pacing (for atrial flutter)

In patients with atrial flutter, rapid atrial pacing using a temporary pacemaker for overdrive suppression of the atrial flutter can be used if previous drug treatments fail.

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all 2nd

Rapid atrial pacing has no role in atrial fibrillation.

DC cardioversion

In patients with atrial fibrillation or atrial flutter whose symptoms persist despite anti-arrythmic agents, DC cardioconversion should be used.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

required.

stable: pre-excited tachycardia due to atrial tachycardia

1st

anti-arrhythmics

Pre-excited tachycardia results in a rapid irregular wide complex tachycardia with varying duration and amplitude of QRS complexes depending upon the degree of pre-excitation.

IV infusions of anti-arrhythmic drugs such as procainamide, ibutilide, flecainide, or amiodarone, which prevent rapid conduction through the AP, are used, even though they may not be able to terminate the atrial arrhythmia. [15] Primary Options

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

procainamide : 100 mg intravenously every 5 minutes as needed, maximum 1000 mg/total dose OR ibutilide : <60 kg body-weight: 0.01 mg/kg intravenously initially, followed by 0.01 mg/kg intravenously in 10 minutes if required; 60 kg body-weight: 1 mg intravenously initially, followed by 1 mg intravenously in 10 minutes if required OR amiodarone : 150 mg intravenously initially, followed by 0.5 to 1 mg/minute intravenous infusion if needed OR

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

flecainide : 2 mg/kg (maximum 150 mg/dose) intravenously initially, followed by 1.5 mg/kg/hour for 1 hour if needed, then 0.1 to 0.25 mg/kg/hour for up to 24 hours if needed, maximum 600 mg/day
2nd

rapid atrial pacing

Rapid atrial pacing using a temporary pacemaker for overdrive suppression of the atrial tachycardia can be used if previous drug treatments fail.

3rd

DC cardioversion

In patients with atrial tachycardia whose symptoms persist despite antiarrythmic agents or atrial pacing, DC cardioconversion should be used.

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

A 50- to 360-J biphasic synchronized DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronized mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required. However, the recurrence of atrial arrhythmia after DC cardioversion may be higher in atrial tachycardia, compared to atrial fibrillation/flutter, depending on the mechanism. If the atrial tachycardia is related to abnormal automaticity, DC

Treatment Patient group unstable: BP <90/60 mmHg, signs of systemic hypoperfusion or unstable AF line 1st Treatmenthide all

direct current (DC) cardioversion

Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

A 50- to 360-J biphasic synchronised DC shock can be used (commonly the initial cardioversion is attempted with 50 J for SVT, but up to 360 J may be needed for cardioversion of atrial fibrillation with rapid ventricular response). DC cardioversion without the synchronised mode can induce ventricular fibrillation.

Conscious sedation (e.g. with IV propofol) is also required.

Presumptive
Treatment Patient group line Treatmenthide all

cardioversion may not be effective at all, such as multifocal atrial tachycardia (MAT).

Acute

Treatment Patient group following acute treatment: asymptomatic line 1st Treatmenthide all

risk stratification and monitoring

While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13]

Asymptomatic patients, except for those in specialised jobs with particular safety issues, should not be treated but can be monitored for symptoms by visits to a physician around every 2 years.

adjunct [?]

catheter ablation

Asymptomatic patients in specialised jobs with particular safety issues (e.g., airline pilot, school bus driver) can be considered for catheter ablation. [18]

following acute treatment: minimally symptomatic

1st

monitoring + vagal manoeuvre education (for orthodromic AVRT)

These are patients who have a single, welltolerated, non-pre-excited supraventricular tachycardia (SVT) episode, or those who regularly have fewer than 5 SVT episodes a year, also welltolerated and non-pre-excited.

Patients require monitoring with visits to a physician about every 2 years. Patients with orthodromic AV reciprocating tachycardia (narrow complex tachycardia) can be taught vagal maneuvers (e.g., Valsalva) to stop episodes of SVT.

1st

catheter ablation

Catheter ablation has a high efficacy and low risk, and can be used either as initial therapy or for patients experiencing side effects or arrhythmia recurrence during drug therapy. It is often a matter

Treatment Patient group following acute treatment: asymptomatic line 1st Treatmenthide all

risk stratification and monitoring

While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13]

Asymptomatic patients, except for those in specialised jobs with particular safety issues, should not be treated but can be monitored for symptoms by visits to a physician around every 2 years. of patient choice, and is useful in those with specialised jobs that require consciousness (e.g., school bus driver, airline pilot). [18]

2nd

pharmacological therapy

Those whose episodes are not sufficiently controlled by vagal manoeuvres can carry the pillin-the-pocket (propranolol and diltiazem) to take at the onset of symptoms of a non-pre-excited tachycardia. Other versions include propafenone or flecainide along with metoprolol or diltiazem.

Class I anti-arrhythmic agents (fleicanide or propafenone) are suitable for patients with no additional cardiac disease but cannot be used in people with CAD or structural heart disease.

In patients with CAD or structural heart disease, class III anti-arrhythmic agents (sotalol, amiodarone, or dofetilide) may be used.

Verapamil and diltiazem should not be used as the sole therapy for patients with accessory pathways that might be capable of rapid conduction during AF. This concern also applies to digoxin, which also should not be used in this situation.

QT interval must be determined prior to starting

Treatment Patient group following acute treatment: asymptomatic line 1st Treatmenthide all

risk stratification and monitoring

While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13]

Asymptomatic patients, except for those in specialised jobs with particular safety issues, should not be treated but can be monitored for symptoms by visits to a physician around every 2 years. therapy with dofetilide as it is contraindicated if QTc is >440 msec (>500 msec in patients with ventricular conduction abnormalities). Primary Options pill-in-the-pocket propranolol : 80 mg orally (immediate-release) as a single dose and diltiazem : 120 mg orally (immediate-release) as a single dose OR pill-in-the-pocket propafenone : 450-600 mg orally (immediaterelease) as a single dose or flecainide : 200-300 mg orally as a single dose -- AND -metoprolol : 50 mg orally (immediate-release) as a single dose or diltiazem : 120 mg orally (immediate-release) as a single dose

Treatment Patient group following acute treatment: asymptomatic line 1st Treatmenthide all

risk stratification and monitoring

While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13]

Asymptomatic patients, except for those in specialised jobs with particular safety issues, should not be treated but can be monitored for symptoms by visits to a physician around every 2 years. Secondary Options flecainide : 50-150 mg orally twice daily OR propafenone : 150-300 mg orally (immediaterelease) every 8 hours Tertiary Options sotalol : 80-160 mg orally twice daily OR amiodarone : 600-800 mg/day orally given in 2 divided doses as a loading dose for 2 weeks, followed by maintenance dose of 200-400 mg/day OR dofetilide : 125-500 micrograms orally twice daily

following acute treatment: symptomatic

1st

catheter ablation

All patients should be offered catheter ablation.

2nd

anti-arrhythmics

Used in patients who refuse ablation or in whom ablation is unsuitable. Class I anti-arrhythmic agents (fleicanide or

Treatment Patient group following acute treatment: asymptomatic line 1st Treatmenthide all

risk stratification and monitoring

While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13]

Asymptomatic patients, except for those in specialised jobs with particular safety issues, should not be treated but can be monitored for symptoms by visits to a physician around every 2 years. propafenone) are suitable for patients with no additional cardiac disease but cannot be used in people with CAD or structural heart disease.

In patients with CAD or structural heart disease, class III anti-arrhythmic agents (sotalol, amiodarone, or dofetilide) may be used.

QT interval must be determined prior to starting therapy with dofetilide as it is contraindicated if QTc is >440 msec (>500 msec in patients with ventricular conduction abnormalities). Primary Options flecainide : 50-150 mg orally twice daily OR propafenone : 150-300 mg orally (immediaterelease) every 8 hours OR sotalol : 80-160 mg orally twice daily OR amiodarone : 600-800 mg/day orally given in 2 divided doses as a loading dose for 2 weeks, followed by maintenance dose of 200-400 mg/day OR

Treatment Patient group following acute treatment: asymptomatic line 1st Treatmenthide all

risk stratification and monitoring

While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13]

Asymptomatic patients, except for those in specialised jobs with particular safety issues, should not be treated but can be monitored for symptoms by visits to a physician around every 2 years.

dofetilide : 125-500 micrograms orally twice daily adjunct [?] beta-blockers

Beta-blockers can be given to patients who find that they need additional control of symptoms. Primary Options atenolol : 50-200 mg/day orally given in 1-2 divided doses OR metoprolol : 25-100 mg orally twice daily

Ongoing

Treatment approach
Treatment of acute presentations is based on the nature of the arrhythmia. Ongoing treatment is decided according to the symptoms of the patient, as well as risk stratification for sudden cardiac death.

Asymptomatic
While controversy exists, most experts recommend that all patients with ventricular pre-excitation undergo risk stratification to determine their risk of sudden cardiac death, regardless of the presence of symptoms. [12] [13] The risk of sudden cardiac death is determined by the anterograde refractory

period of the accessory pathway. If the refractory period is very short, patients are at risk for developing ventricular fibrillation in the setting of atrial fibrillation conducting rapidly over the accessory pathway. If ventricular preexcitation is intermittent at rest, then the accessory pathway is low risk for causing sudden cardiac death. If ventricular pre-excitation is always present at rest, patients should undergo exercise treadmill testing looking for abrupt loss of pre-excitation, again indicative of an accessory pathway incapable of dangerously rapid conduction from the atrium to the ventricle. If pre-excitation does not abruptly disappear with exercise, then invasive risk stratification at electrophysiology study should be considered. Accessory pathways capable of rapid anterograde conduction should be ablated to reduce the risk of sudden cardiac death, regardless of whether or not they cause supraventricular tachycardia (SVT).

Minimally symptomatic
These are patients who have a single, well-tolerated, non-pre-excited supraventricular tachycardia episode, or those who regularly have fewer than 5 SVT episodes a year, also well-tolerated and non-pre-excited. Patients require monitoring with visits to a physician about every 2 years. Patients with orthodromic AV reciprocating tachycardia (narrow complex tachycardia) can be taught vagal maneuvers (e.g., Valsalva) to stop episodes of SVT. All minimally symptomatic patients requiring further treatment can carry the pill-in-the-pocket (propranolol and diltiazem) to take at the onset of symptoms of a non-pre-excited tachycardia. Other versions include propafenone or flecainide along with metoprolol or diltiazem. Minimally symptomatic patients may be treated with catheter ablation or medical therapy or only during the rare symptomatic episodes. Decision to treat should be based on patients' concern about their own symptoms. Catheter ablation has a high efficacy and low risk, and can be used either as initial therapy or for patients experiencing side effects or arrhythmia recurrence during drug therapy.

Symptomatic patients
Symptomatic patients usually undergo catheter ablation as a first-line therapy. Anti-arrhythmic drugs are one of the therapeutic options for the management of symptomatic WPW syndrome, but they have been increasingly replaced by

catheter ablation. The choice between drugs and ablation is often down to the preference of the patients and whether they wish to go through the procedure. Although there have been no controlled trials of drug prophylaxis involving symptomatic WPW syndrome, drugs are recommended on the basis of the safety and efficacy of these anti-arrhythmic drugs reported in a number of small, non-randomised trials (each involving <50 patients). Antiarrhythmic drugs include AVN blocking agents and class I as well as class III anti-arrhythmic agents. The efficacy of chronic oral beta-blockers in the treatment of AV re-entrant tachycardia or WPW syndrome has not been examined, probably reflecting the fact that catheter ablation is the therapy of choice for these patients. Despite the absence of data from clinical trials, chronic oral beta-blocker therapy may be used for treatment of patients with WPW syndrome, particularly if the AP has been demonstrated to be incapable of rapid anterograde conduction during an electrophysiological study. The incidence of symptomatic tachycardia is reported to be higher during pregnancy, which may be refractory to drugs that are safe to use in such circumstances. Therefore, a radiofrequency ablation should be considered prior to the next planned pregnancy.

Acute management
Acute management follows a path by whether the patient is stable or unstable; and if stable, by whether they are in orthodromic AV reciprocating tachycardia (narrow complex with short RP` tachycardia), antidromic AV reciprocating tachycardia (wide complex tachycardia), or whether they have atrial fibrillation, flutter, or tachycardia. [15] Patients with an acute tachycardia and who are haemodynamically unstable (BP <90/60 mmHg, with signs of systemic hypoperfusion) or who have atrial fibrillation with >250 bpm, or in whom the atrial fibrillation has degenerated to ventricular fibrillation, require immediate DC cardioversion.

Acute management of supraventricular tachycardia and atrial fibrillation in patients with WPW syndromeFrom the collection of Dr Mithilesh K. Das

Orthodromic AV reciprocating tachycardia (narrow complex tachycardia)


Since ORT involves the AV node as one limb of the circus movement tachycardia, any manoeuvre or drug that slows or interrupts conduction in the AV node can terminate the tachycardia. In a haemodynamically stable patient, vagal manoeuvres such as carotid sinus massage should be performed at the bedside, or, alternatively, patients can be instructed to perform a Valsalva manoeuvre to terminate the arrhythmia. If this fails, pharmacotherapy with IV adenosine, AV nodal blocking drugs, or antiarrhythmic agents is indicated.

Adenosine is preferred because of its ultra-short half-life (<18 seconds). It should be given as a rapid IV injection in a central vein or in the antecubital vein, followed by 10 to 20 mL of rapid normal saline flush. It can be used a second time if there is no response. Adenosine injection may cause chest pain, chest tightness, bronchospasm, dizziness, and a short run of atrial fibrillation (1% to 15%). Although atrial fribrillation is usually transient, it can cause rapid conduction to the ventricle via the AP, and if the conduction is rapid then it can potentially result in ventricular fibrillation. Therefore, resuscitation equipment should be available as a standby. Adenosine should be avoided in patients with severe asthma and in patients with a known hypersensitivity to the drug. In addition, adenosine injection is associated with a higher risk of heart block in patients on carbamazepine therapy. AV nodal blocking drugs (diltiazem, verapamil, metoprolol) can be used when there is no response or a recurrence after carotid sinus massage and adenosine. Anti-arrhythmic drugs (procainamide, ibutilide, amiodarone, flecainide) can be used when there is no response or a recurrence after carotid sinus massage, adenosine, and AV nodal blocking drugs. Rapid atrial pacing using a temporary pacemaker for overdrive suppression of the tachycardia can be used if the previous drug treatments fail. Direct current (DC) cardioversion can then be used when symptoms persist in patients or if the patient becomes haemodynamically unstable.

Antidromic AV reciprocating tachycardia (narrow complex tachycardia)


If the tachycardia is haemodynamically stable, then management is similar to that of the ORT, with IV adenosine or anti-arrhythmic drugs. Calcium channel blockers, digoxin, and beta-blockers are contraindicated in this population, because these drugs slow the conduction via the AV node but do not have any effect on the accessory pathway. This may result in a rapid conduction via the accessory pathway, which may cause ventricular tachycardia and ventricular fibrillation leading to sudden cardiac death. Rapid atrial pacing using a temporary pacemaker for overdrive suppression of the tachycardia can be used if the previous drug treatments fail. DC cardioversion may be needed for refractory tachycardia.

Atrial fibrillation, atrial flutter, and atrial tachycardia

Pre-excited tachycardia results in a rapid irregular wide complex tachycardia with varying duration and amplitude of QRS complexes depending upon the degree of pre-excitation. View image IV infusions of anti-arrhythmic drugs such as procainamide, ibutilide, flecainide, or amiodarone, which prevent rapid conduction through the AP, are used, even though they may not be able to terminate the atrial arrhythmia. [15] Anticoagulation should be considered in atrial fibrillation and atrial flutter depending on presence of co-morbid cardiologic abnormalities and duration of onset. In atrial tachycardia or atrial flutter, rapid atrial pacing using a temporary pacemaker for overdrive suppression of the tachycardia or flutter can be used if the previous drug treatments fail. However, it is more effective in atrial tachycardia than atrial flutter since the atrial rate is slower in atrial tachycardia. Rapid atrial pacing has no role in atrial fibrillation. DC cardioversion can be used when symptoms persist in patients or if the patient becomes haemodynamically unstable. However, the recurrence of atrial arrhythmia after DC cardioversion may be higher in atrial tachycardia, compared to atrial fibrillation/flutter, depending on the mechanism. If the atrial tachycardia is related to abnormal automaticity, DC cardioversion may not be effective at all, such as multifocal atrial tachycardia (MAT).

Monitoring
Patients should visit their physician every 2 to 3 years for monitoring.

Patient Instructions
Patients should visit their physician every 2 to 3 years for monitoring. Patients should call the emergency services if they have any recurrence of symptoms. In minimally symptomatic patients who have a single, well-tolerated, non-preexcited supraventricular tachycardia (SVT) episode, or those who regularly have fewer than 5 SVT episodes a year, also well-tolerated and non-preexcited, these patients can be taught vagal maneuvers (e.g., Valsalva) to stop episodes of SVT. Those requiring further treatment can carry the pill-in-the-pocket (propranolol and diltiazem) to take at the onset of symptoms of a non-pre-excited

tachycardia. Other versions include propafenone or flecainide along with metoprolol or diltiazem.

Complications
Complicationhide all

sudden cardiac death (SCD) Usually with a history of palpitations and dizziness. SCD due to ventricular fibrillation (VF) is a rare manifestation with an incidence of 0.025% to 0.38%. High-risk patients include those with a rapidly conducting accessory pathway (AP) (1:1 AV conduction at >250 bpm) and multiple APs. WPW syndrome accounts for 10% of SCD in the young population. [19] The incidence in patients with WPW syndrome ranges from 0.15% to 0.6%/year over a 3- to 10-year follow-up. [19] [15] The mode of death is thought to be due to AF with a rapid ventricular response via an AP that induces polymorphic ventricular tachycardia and VF resulting in syncope or SCD. Although syncope in patients with WPW syndrome is considered an ominous prognostic sign, in one study of patients with WPW syndrome referred for EP study, the incidence of aborted SCD was 28% and 16% in patients with and without a history of syncope, respectively. However, this did not reach statistical significance. catheter ablation complications Catheter ablation is generally a safe procedure with an overall complication rate of 4.4% and a major complication rate of 1% to 2%. The common minor complications are related to the vascular access, catheter manipulation, transseptal atrial puncture for left-sided AP ablation, application of RF energy, and left-sided ablation: bleeding from vascular sites, haematomas, DVT, arteriovenous fistula, pseudoaneurysm at the arterial access sites, pneumothorax, infection. The risk of major complications is 1.8%: death, related to catheter manipulation, due to valvular damage, perforation of the coronary sinus or myocardial wall, coronary artery dissection, and thrombosis (0.08% to 0.2%), or related to the delivery of radiofrequency energy (AV block, 0.17% to 1.0%), myocardial perforation, coronary artery spasm or occlusion, transient ischaemic attacks and cerebrovascular accidents, or pericardial tamponade (0.13% and 1.1%), mostly occurs during transseptal puncture, but can occur during the application of radiofrequency energy.

Prognosis
Symptomatic patients with WPW syndrome should undergo catheter ablation as a first-line therapy. [14] Minimally symptomatic patients may be treated with catheter ablation or medical therapy or only during the rare symptomatic episodes. Catheter ablation has a high efficacy and low risk and can be used either as initial therapy or for patients experiencing side effects or arrhythmia recurrence during drug therapy. Patient preference is always an important consideration in these scenarios. Subjects with WPW pattern (asymptomatic patients), except for those with specialised jobs, should not be treated but can be monitored for symptoms.