Wolff Parkinson White syndrome (WPW) – 8 Interesting Facts

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Synopsis

Key Points

  1. Wolff Parkinson White syndrome (WPW) is a ventricular preexcitation syndrome associated with an extranodal accessory pathway 1 2
  2. Patients may be asymptomatic or present with paroxysmal supraventricular tachycardia or atrial fibrillation. Rarely, atrial fibrillation degenerating to ventricular fibrillation and sudden cardiac death may be the initial presentation
  3. In sinus rhythm, the ECG may be diagnostic. Antegrade conduction down the accessory pathway results in characteristic ECG pattern: 3
    • Slurred upstroke of the QRS complex (delta wave)
    • Short PR interval (less than 120 milliseconds)
    • Wide QRS complex (more than 120 milliseconds)
  4. Most patients with asymptomatic Wolff-Parkinson-White syndrome should have noninvasive testing for risk stratification, including ambulatory ECG monitoring and an exercise stress test. Those at high risk should proceed to electrophysiologic testing
  5. Electrophysiologic testing is indicated for symptomatic patients, for patients with high-risk occupations or moderate- to high-level athletic activity, and for children weighing more than 15 kg who cannot perform an exercise stress test
  6. Catheter ablation of accessory pathways is first line therapy in symptomatic patients with Wolff-Parkinson-White syndrome, especially if hemodynamic instability occurs during arrhythmia 2
  7. Acute episode of supraventricular tachycardia or atrial fibrillation resulting in clinically unstable status is managed with synchronized direct current cardioversion
  8. Most patients with Wolff-Parkinson-White syndrome have good prognosis

Pitfalls

  • Do not use IV adenosine, amiodarone, digoxin, β-blockers, or nondihydropyridine calcium channel blockers in the acute treatment of patients with Wolff-Parkinson-White syndrome who have preexcited atrial fibrillation, because these drugs can accelerate the ventricular rate 6 7
  • In symptomatic patients who elect ongoing medical management instead of ablation, do not use oral digoxin. Digoxin shortens the refractory period of the accessory pathway, such that atrial fibrillation, if it occurs, may induce ventricular fibrillation 3

Terminology

Clinical Clarification

  • Wolff Parkinson White syndrome is an arrhythmia characterized by documented supraventricular tachycardia or atrial fibrillation (or symptoms consistent with it) in a patient with a ventricular preexcitation pattern on ECG during sinus rhythm 3
  • Caused by an extranodal accessory pathway between atria and ventricle
  • Classic preexcitation ECG findings during sinus rhythm include a delta wave and a short PR interval of less than 120 milliseconds 3
  • These findings may not be present if the accessory pathway conducts exclusively in retrograde direction; this path is called a concealed accessory pathway
  • When there are ECG findings of an accessory pathway but the patient has not experienced supraventricular tachycardia or atrial fibrillation, the condition is asymptomatic preexcitation (Wolff-Parkinson-White pattern), which is not technically Wolff-Parkinson-White syndrome (because the syndrome is defined by documented arrhythmia or symptoms thereof) 3

Diagnosis of Wolff Parkinson White syndrome

Clinical Presentation

History

  • Patient with Wolff Parkinson White syndrome may have known history of ECG-diagnosed preexcitation, history of paroxysmal supraventricular tachycardia, or both
  • Patient may be asymptomatic or report intermittent symptoms of abrupt onset
    • Infants may be irritable with difficulty feeding; if there is structural heart disease or prolonged tachycardia, symptoms of heart failure may be present
    • Young children may describe episodes of altered breathing or respiratory distress rather than describing rapid heart beat
    • Older children and adults may describe rapid or irregular heartbeat or intermittent dizziness
    • During sustained tachyarrhythmia, symptoms may include:
      • Chest discomfort
      • Pounding or fluttering palpitations
      • Anxiety
      • Dyspnea
      • Lightheadedness
      • Syncope
    • Rarely, presentation is with sudden cardiac death due to atrial fibrillation progressing to ventricular fibrillation 1

Physical examination

  • Examination findings may be normal if patient presents while in sinus rhythm
  • With supraventricular tachyarrhythmias, heart rate is usually regular (unless atrial fibrillation is present) and at a rate higher than 150 beats per minute
    • Patient may be cool, hypotensive, and diaphoretic with rapid heart rate
  • Cardiac examination findings are usually normal unless there is underlying structural heart disease, which is rare in an adult presenting with Wolff-Parkinson-White syndrome
    • Infants and young children are more likely to have a symptomatic cardiac anomaly (eg, Ebstein anomaly)
    • Adults may have a loud or accentuated S₁

Causes and Risk Factors of Wolff Parkinson White syndrome

Causes

  • Accelerated anomalous atrioventricular conduction pathway
    • Composed of myocardial tissue, which allows nondecremental conduction (unlike atrioventricular nodal tissue)
    • May be located in left free wall, right free wall, or septum; a minority of patients have multiple accessory tracts

Risk factors and/or associations

Age
  • Wolff Parkinson White syndrome occurs more often in younger populations (younger than 30 years) than in elderly populations; this may be because speed of conduction through the accessory pathway tends to decline with age and patient becomes less likely to be symptomatic 2
  • People younger than 30 years are at higher risk of life-threatening arrhythmias 2
  • Asymptomatic children are at higher risk of sudden cardiac death than asymptomatic adults
  • In infants and neonates, this syndrome is the most common cause of supraventricular tachycardia and atrial fibrillation
Sex
  • More common in males
  • Male sex is associated with greater risk of life-threatening arrhythmias 2
Genetics
  • Familial incidence is 5.5/1000 in first-degree relatives 2
  • Familial form of syndrome is associated with increased risk of sudden cardiac death 2
  • Caused by missense mutation in PRKAG2 at locus 7q34-q36, which encodes the gamma-2 subunit of 5′-adenosine monophosphate–activated protein kinase 8
Other risk factors/associations
  • Congenital heart disease involving tricuspid valve, especially Ebstein anomaly
  • Mitral valve prolapse
  • Ventricular septal defect
  • Structural heart disease (conveys higher risk of life-threatening arrhythmias) 2

Diagnostic Procedures

Primary diagnostic tools

  • Initially use history and the presence of classic ECG findings to confirm Wolff Parkinson White syndrome (symptomatic) or Wolff-Parkinson-White pattern (asymptomatic) 3 9
  • Symptomatic patients
    • For infants and young children, consult a pediatric cardiologist about additional diagnostic testing 10
      • Cardiologist performs echocardiography to look for underlying structural heart disease (eg, Ebstein anomaly)
      • Patients in this age group typically do not undergo exercise testing or ambulatory ECG monitoring
      • Risks of electrophysiologic testing are higher in this age group, and tachyarrhythmias may resolve spontaneously, so electrophysiologic testing leading to catheter ablation is less likely to be recommended
    • Adults and older children usually proceed to electrophysiologic testing after discussion of risks and benefits
  • Asymptomatic patients
    • There are no specific recommendations for diagnostic work-up in infants and young children with Wolff-Parkinson-White pattern but without history of paroxysmal tachyarrhythmias; consult pediatric cardiologist about additional testing 10
    • Adults and older children usually undergo noninvasive evaluation to stratify risk for rapid conduction leading to malignant arrhythmias; patients who are not clearly at low risk will have electrophysiologic testing recommended 3
      • Pediatric guidelines for older children and adolescents (aged 8-21 years), in particular, emphasize utility of noninvasive testing 11 12
      • Cardiologist should review ECG for findings that suggest higher versus lower risk; ambulatory ECG provides additional recording data to examine, if needed 3
        • Low-risk finding: intermittent preexcitation during sinus rhythm
        • High-risk findings: persistent (rather than intermittent) preexcitation during sinus rhythm and sinus tachycardia
      • Perform an exercise stress test if ECG evaluation does not clearly place patient in low-risk category; children who cannot perform an exercise stress test should proceed directly to electrophysiologic testing 3 11
        • Abrupt loss of preexcitation during exercise testing is associated with low risk
        • Persistent (or uncertain) loss of preexcitation at physiologic heart rates is associated with high risk
  • Consider invasive electrophysiologic testing (and ablation of identified high-risk accessory pathways) for the following asymptomatic patients after discussion of risks and benefits:
    • Asymptomatic adult patients, to identify accessory pathways at increased risk of arrhythmic events (with ablation if such a pathway is identified) 3 12
    • Patients in risky occupations (eg, pilots, bus drivers, divers) and for athletes participating in moderate- to high-level competitive sports 3 12
    • Asymptomatic children and adolescents aged 8 to 21 years 11
      • Electrophysiologic testing is recommended if there is persistent (or uncertain) loss of preexcitation on exercise stress testing; if stress testing cannot be performed, asymptomatic children should proceed to electrophysiologic testing based on ECG findings

Functional testing

  • ECG 3
    • Critical diagnostic test for all patients; classic findings in sinus rhythm include:
      • Delta wave: slurred, slowly rising deflection at onset of QRS complex in sinus rhythm, which is definitive evidence of preexcitation
      • QRS complex is widened (more than 120 milliseconds) owing to fusion of preexcited ventricular activation with later ventricular activation via atrioventricular node
      • Short PR interval less than 120 milliseconds
        • Not always seen
        • When seen, usually denotes right-sided accessory pathway
      • ST-T wave abnormalities may be seen, characterized by deflections that are directed negatively with respect to direction of dominant QRS deflection
    • Additional information that may be obtained from ECG
      • Intermittent preexcitation (QRS normalization and abrupt loss of delta wave) during sinus rhythm indicates low risk of sudden cardiac death
  • Ambulatory ECG monitoring
    • Noninvasive, continuous, ambulatory ECG obtained via device worn by patient over a period of 24 to 48 hours (eg, Holter monitor)
    • Indicated for most asymptomatic patients as part of risk stratification 3
      • May document a variety of paroxysmal tachyarrhythmias
        • Most common
          • Orthodromic atrioventricular reciprocating tachycardia: due to antegrade conduction through atrioventricular node and retrograde conduction through accessory pathway
            • Atrioventricular reciprocating tachycardia is the most common arrhythmia in Wolff-Parkinson-White syndrome (representing 95% of reentrant tachycardias in patients with an accessory pathway) 1
            • Most are orthodromic, as follows:
              • Regular narrow-complex tachycardia with ventricular rate of 150 to 250 or more
              • Inverted P waves
              • RP interval that is constant and typically less than half of the RR interval (short RP tachycardia)
          • Atrial fibrillation: originates independently of the accessory pathway (and does not require accessory pathway to perpetuate itself) but may conduct via accessory pathway
            • Irregularly irregular rhythm with ventricular rate more than 180 to 200 beats per minute
            • Beat-to-beat QRS morphology changes; QRS width depends on refractory period of accessory pathway (shorter refractory period results in wider QRS owing to more conduction over the accessory pathway)
            • Ventricular rate more than 180 to 200 beats per minute, which may increase to more than 300 beats per minute with degeneration to ventricular fibrillation
        • Less common
          • Antidromic atrioventricular reciprocating tachycardia: due to antegrade conduction through accessory pathway and retrograde conduction through atrioventricular node (occurs in only 5%-10% of patients with Wolff-Parkinson-White syndrome 1)
            • Regular wide-complex tachycardia with ventricular rate of 150 to 250 or more beats per minute
            • Inverted P waves
            • RP interval that is constant and typically more than half of the RR interval (long RP tachycardia)
      • Assists with risk stratification
        • Loss of preexcitation, as evidenced by abrupt disappearance of delta wave during sinus tachycardia, suggests that accessory pathway has relatively long effective refractory period and is unlikely to precipitate ventricular fibrillation, even during sympathetic stimulation
  • Exercise stress test
    • Patient exercises on a bicycle or treadmill while being continuously monitored with ECG
    • Indicated for most asymptomatic patients as part of risk stratification 3
      • Loss of preexcitation, as evidenced by abrupt disappearance of delta wave during exercise, suggests that accessory pathway has relatively long effective refractory period and is unlikely to precipitate ventricular fibrillation, even during sympathetic stimulation 2
    • Arrhythmias or other ECG changes that are not evident at rest can manifest as increased heart rate during stress test

Procedures

Cardiac electrophysiologic testing
General explanation
  • Cardiac electrical impulses are recorded using several catheters placed in strategic locations
    • Usually (and ideally) catheter tips are within the heart (via advancement from femoral veins)
    • May be performed with transesophageal catheters instead, which are less effective 2
  • Arrhythmias are induced by electrical stimuli delivered to the heart; conduction patterns and presence of accessory pathways are assessed 2
  • Electrophysiologic testing aids in:
    • Determining antegrade/retrograde conduction properties of accessory pathways and atrioventricular node 1 2
    • Establishing inducibility of arrhythmias
    • Measuring accessory pathway effective refractory period 2
    • Identifying locations of accessory pathways 1 2
    • Measuring shortest preexcited R-R interval during induced atrial fibrillation 2
    • Establishing presence of multiple accessory pathways 2
  • Catheter radiofrequency ablation may be performed simultaneously
  • Antiarrhythmic drugs can alter findings of electrophysiologic testing; therefore, all antiarrhythmic drugs are usually stopped 5 half-lives before testing begins
Indication
  • Symptomatic patients with Wolff-Parkinson-White syndrome being considered for ablative procedure of accessory pathway 13
  • Patients with preexcitation pattern who are undergoing heart surgery for another problem 13
  • Strongly consider in asymptomatic adults as follows:
    • Those with family history of sudden cardiac death 13
    • Those who have preexcitation pattern without arrhythmias and who are involved in high-risk activities, such as high-risk occupation (eg, bus driver, pilot, diver) or moderate- to high-level competitive sports 2 13
    • Those who desire the procedure to determine amount of risk presented by their accessory pathway
  • Consider for the following asymptomatic children and adolescents, aged 8 to 21 years: 2
    • Those who are unable to perform an exercise stress test and have persistent preexcitation on ECG
    • Those who have persistent preexcitation, or equivocal or uncertain loss of preexcitation, during exercise stress test
    • Those whose results on noninvasive tests fail to clearly demonstrate low risk for sudden cardiac death
Contraindications
  • No plan for ablation
  • Cardiac thrombus
  • Acute heart failure
  • Pericardial effusion/tamponade
  • Coexisting infection
  • Valvular defects preventing passage of a catheter through tricuspid valve
Complications
  • Unusual (less than 1%) 14
    • Myocardial ischemia
    • Thrombosis and/or embolism
    • Perforation of a blood vessel or the heart
    • Serious or lethal arrhythmias
Interpretation of results
  • Presence of accessory pathways confirms diagnosis of Wolff-Parkinson-White syndrome
  • Test result is positive if arrhythmia is induced
  • Some findings indicate high risk of sudden cardiac death: shortest preexcited R-R interval of 250 milliseconds or less, presence of multiple accessory pathways, septal location of accessory pathway, and inducible arrhythmias 2
  • Some findings indicate lower risk of sudden cardiac death: intermittent preexcitation and absence of retrograde conduction through accessory pathway during testing 2

Differential Diagnosis of Wolff Parkinson White syndrome

Most common

  • Lown-Ganong-Levine syndrome
    • Preexcitation syndrome caused by atriohisian pathway bypassing the atrioventricular node
    • Patients may develop paroxysmal atrial fibrillation or supraventricular tachycardia with rapid ventricular response
    • Characterized by normal QRS complex, short PR interval, and absence of delta wave on ECG
    • Electrophysiologic testing confirms diagnosis
  • Supraventricular tachycardia or atrial fibrillation not due to Wolff-Parkinson-White syndrome (Related: )Atrial fibrillation
    • Specific causes are excluded on the basis of suggestive history and physical examination findings, imaging (eg, chest radiograph, echocardiogram, and, if indicated, CT), or laboratory tests (eg, electrolyte levels, serial troponin levels, digoxin concentration, thyroid hormone levels; as appropriate)
      • Acute coronary syndrome
      • Structural heart disease
      • Chronic obstructive pulmonary disease
      • Pulmonary embolus
      • Electrolyte abnormalities
      • Digitalis/digoxin toxicity
      • Hyperthyroidism

Treatment of Wolff Parkinson White syndrome

Goals

  • Terminate arrhythmia, if present 1
  • Prevent or reduce recurrence of arrhythmias 1
  • Reduce risk of sudden cardiac death 1

Disposition

Admission criteria

In patients with Wolff Parkinson White syndrome presenting with symptomatic tachycardia, especially if it is recurrent, consider admission in consultation with  cardiologist

Criteria for ICU admission
  • Patients requiring urgent cardioversion and presenting with:
    • Cardiac arrest
    • Shock
    • Hemodynamic compromise
    • Congestive heart failure
    • Chest pain
    • Syncope

Recommendations for specialist referral

  • Consult cardiologist for all patients with suspected Wolff-Parkinson-White syndrome
  • Refer to an electrophysiologist for:
    • Further evaluation of symptomatic patients with preexcitation ECG pattern
    • Management decisions (medical or ablative) for symptomatic patients
    • Further evaluation of asymptomatic patients with preexcitation ECG pattern and:
      • High-risk features on ECG or stress testing
      • High-risk occupation (eg, pilot, bus driver, diver)
      • Moderate- to high-level competitive sports (lifestyle or occupation)
      • Family history of Wolff-Parkinson-White syndrome or sudden cardiac death
      • Functional or structural heart disease
      • Desire for definitive (ablative) therapy

Treatment Options

Acute management of a patient with Wolff Parkinson White syndrome presenting with tachyarrhythmia

  • Typical presentations include:
    • Regular narrow-complex tachycardia with antegrade conduction through atrioventricular node (orthodromic atrioventricular reciprocating tachycardia) 3
    • Irregular, polymorphic wide-complex tachycardia (atrial fibrillation)
    • Regular, monomorphic wide-complex tachycardia with antegrade conduction through accessory pathway (antidromic atrioventricular reciprocating tachycardia) (least common presentation)
  • If patient is hemodynamically unstable (with a pulse) and has tachycardia of presumed supraventricular origin: 3
    • Treat with urgent electrical cardioversion
      • For children: start at 0.5 to 1 J/kg; if not effective, increase to 2 J/kg 15
      • For adults: start at 50 to 100 J 5
  • If patient is hemodynamically unstable with a wide-complex tachycardia and ventricular tachycardia cannot be ruled out: 2 16
    • Treat with urgent defibrillation (after pulseless ventricular tachycardia/ventricular fibrillation protocol)
      • For children: start with defibrillation at 2 to 4 J/kg 4
      • For adults: start with defibrillation at 200 to 360 J
  • Management of hemodynamically stable patient:
    • For a regular narrow-complex tachyarrhythmia
      • For both adults and children: vagal maneuvers and/or IV adenosine are first line treatment(s) if there are no contraindications 3 4
        • Very important to record ECG during medication administration or vagal maneuvers; this provides valuable information to the consulting cardiologist
        • Because adenosine may precipitate atrial fibrillation that may then conduct rapidly to the ventricle and cause ventricular fibrillation, ensure that electrical cardioversion is readily available
      • If unresponsive to adenosine or vagal maneuvers
        • In adults
          • Consider an IV β-blocker or IV diltiazem or verapamil (weak recommendation) before synchronized cardioversion 3
            • Exercise extreme caution while using IV β-blocker or calcium channel blocker concomitantly with adenosine
            • Risk of enhancing conduction over the accessory pathway if rhythm converts to atrial fibrillation during administration of medication
            • If rhythm remains unresponsive, administer synchronized cardioversion after adequate sedation or anesthesia 3
        • In children 4
          • Consider treatment with procainamide; amiodarone may be tried if procainamide is ineffective. Given by a slow IV infusion with careful hemodynamic monitoring; consult cardiologist
          • Verapamil may be considered as alternative therapy in older children but should not be routinely used in infants
          • If rhythm remains unresponsive, administer synchronized cardioversion after adequate sedation or anesthesia
    • For a wide-complex tachyarrhythmia
      • If rhythm is regular:
        • Antidromic (wide-complex) atrioventricular reciprocating tachycardia with antegrade conduction via accessory pathway is possible in a patient with Wolff-Parkinson-White syndrome
        • Consider ventricular tachycardia; patient will have atrioventricular dissociation (with ventricular rate faster than atrial rate) or fusion complexes (representing dissociation of supraventricular impulses from a ventricular rhythm) 3
        • Treat wide-complex tachycardia of confirmed supraventricular origin with IV adenosine 1
        • If mechanism cannot be ascertained with confidence, do not give adenosine. Treat as ventricular tachycardia with the following drugs (which also are effective in antidromic atrioventricular reciprocating tachycardia, as they slow down conduction through accessory pathways): 1
          • Amiodarone or procainamide is recommended for pediatric patients in consultation with a cardiologist 17
          • Procainamide and ibutilide are drugs of choice in adult protocols 1
      • If rhythm is irregular:
        • Usually caused by atrial fibrillation with antegrade conduction via both accessory pathway and atrioventricular node 2
          • Adenosine, nondihydropyridine calcium channel blockers, β-blockers, and digoxin are contraindicated because they may: 6 7
            • Enhance conduction over accessory pathway and result in increased ventricular rate and ventricular fibrillation
            • Paradoxically decrease contractility and enhance accessory pathway conduction
          • For adults, IV procainamide or ibutilide are recommended to terminate irregular wide-complex tachycardia (preexcited atrial fibrillation) 2 6
          • For children, procainamide is recommended
      • If patient is clinically unstable (eg, hypotension, chest pain, pulmonary edema, mental status changes):
        • If preexcitation is known from previous ECG, rhythm is likely atrial fibrillation with aberrant conduction, and immediate synchronized direct current cardioversion is recommended

Nonacute management

  • Asymptomatic patients with a Wolff-Parkinson-White pattern on sinus rhythm ECG
    • Asymptomatic patients younger than 8 years
      • These patients are generally followed up periodically by a cardiologist, without tachyarrhythmia prophylaxis
    • Asymptomatic patients aged 8 to 21 years
      • If noninvasive initial evaluation confirms low-risk status with abrupt and clear loss of preexcitation, clinical follow-up with counseling regarding symptom awareness is a reasonable strategy 11
      • If electrophysiologic testing is performed and finds either inducible supraventricular tachycardia and/or a shortest preexcited R-R interval (during atrial fibrillation) of 250 milliseconds or less, catheter ablation is warranted; discuss risks and benefits with patient and parent 11
    • Asymptomatic patients older than 21 years
      • Evidence for best management strategy is limited 9
      • If patient is clearly in a low-risk category after noninvasive testing and electrophysiologic testing is not performed, no therapy is recommended 3 12
      • If electrophysiologic testing is performed and finds high-risk features, catheter ablation is recommended; if ablation is refused, medical management is offered 9
      • Patients with high-risk occupations or moderate- to high-level athletic activity are offered electrophysiologic testing and ablation
  • Symptomatic patients with a Wolff-Parkinson-White pattern on sinus rhythm ECG (ie, those with history of paroxysmal supraventricular tachycardia or atrial fibrillation, or of symptoms suggesting those)
    • Catheter ablation is first line therapy for symptomatic patients with preexcitation on ECG, especially if hemodynamic instability occurs during arrhythmia 1
      • For children younger than 8 years, electrophysiologic testing with catheter ablation can be considered, but choice depends on many factors, including how symptomatic the child is and the frequency of the arrhythmia
      • Risk of complications is higher in very small children (ie, those weighing less than 15 kg) 18
    • Medical therapy for accessory pathway–mediated arrhythmias is no longer first line management in Wolff-Parkinson-White syndrome; it has been replaced by catheter ablation 1
      • If patient is not a candidate for catheter ablation or refuses ablation, drug prophylaxis may decrease recurrent tachyarrhythmias
        • For patients without structural or ischemic heart disease: oral flecainide or propafenone is reasonable for ongoing management in those who have atrioventricular reciprocating tachycardia and/or preexcited atrial fibrillation (effective in approximately 85%-90% of patients) 3
          • There is little randomized data for treating recurrent or refractory supraventricular tachycardias in very young children; many pediatricians use a variety of other drugs including β-blockers, sotalol, or amiodarone 19
          • In children younger than 1 year or weighing less than 15 kg, the combination of flecainide and sotalol may be safe and effective 20
        • For patients with structural or ischemic heart disease: oral dofetilide or sotalol may be reasonable for ongoing management in those with atrioventricular reciprocating tachycardia and/or preexcited atrial fibrillation 3
        • Small observational studies support use of amiodarone for preventing recurrent atrioventricular reciprocating tachycardia, but long-term efficacy has not been reported 3
        • Oral β-blockers, diltiazem, or verapamil may be reasonable for ongoing management of orthodromic (narrow-complex) atrioventricular reciprocating tachycardia in patients with preexcitation on resting ECG 3
          • Evidence is limited, but clinical experience suggests that the drugs are effective, with a favorable adverse effect profile
          • Use only with caution. Patients with preexcitation may develop atrial fibrillation during an episode of atrioventricular reciprocating tachycardia and be exposed to increased risk of rapid conduction over the accessory pathway while receiving these drugs

Drug therapy

  • IV drugs used to manage acute tachyarrhythmias in a patient with Wolff-Parkinson-White syndrome (use according to relevant guidelines)
    • Adenosine
      • Adenosine Solution for injection; Neonates: 0.1 mg/kg rapid IV/IO bolus followed immediately by a saline flush. If conversion does not occur within 1 to 2 minutes, give an additional 0.2 mg/kg rapid IV/IO bolus followed by a saline flush.
      • Adenosine Solution for injection; Infants, Children, and Adolescents weighing less than 50 kg: 0.1 mg/kg rapid IV/IO bolus followed immediately by a saline flush. If conversion does not occur within 1 to 2 minutes, give an additional 0.2 mg/kg rapid IV/IO bolus followed by a saline flush.
      • Adenosine Solution for injection; Children and Adolescents weighing 50 kg or more: 6 mg rapid IV/IO bolus followed immediately by a saline flush. If conversion does not occur within 1 to 2 minutes, give an additional 12 mg rapid IV/IO push followed by a saline flush.
      • Adenosine Solution for injection; Adults: 6 mg rapid IV/IO bolus followed immediately by a saline flush. If conversion does not occur within 1 to 2 minutes, give an additional 12 mg rapid IV/IO push followed by a saline flush.
    • β-blocker
      • Esmolol
        • Esmolol Hydrochloride Solution for injection; Infants†, Children†, and Adolescents†: Loading doses of 500 to 1,000 mcg/kg IV given over 1 to 2 minutes followed by initial maintenance infusions of 25 to 300 mcg/kg/minute IV have been used in several small studies (n = 17 to 27). Maintenance infusion rates up to 1,000 mcg/kg/minute IV have been used.
        • Esmolol Hydrochloride Solution for injection; Adults: Initially, 500 mcg/kg IV over 1 minute; start maintenance infusion at 50 mcg/kg/minute IV for 4 minutes. If tachycardia is not controlled, may repeat loading dose and increase maintenance infusion to 100 mcg/kg/minute IV for 4 minute. May repeat loading dose and increase maintenance infusion by 50 mcg/kg/minute increments every 4 minutes up to 200 mcg/kg/minute. Per clinical practice guidelines, maintenance infusions as high as 300 mcg/kg/minute are suggested but are also associated with increased risk of adverse effects and little additional clinical benefit.
    • Calcium channel blocker
      • Diltiazem
        • Diltiazem Hydrochloride Solution for injection; Infants older than 7 months†, Children†, and Adolescents†: 0.25 mg/kg IV over 5 minutes followed by 0.05 to 0.15 mg/kg/hour continuous IV infusion (mean 0.11 mg/kg/hour) has been reported.
          • Off-label use in this age group
        • Diltiazem Hydrochloride Solution for injection; Adults: 0.25 mg/kg IV over 2 minutes. After 15 minutes, 0.35 mg/kg IV over 2 minutes may be given. Individualize as needed. Begin 10 mg/hour continuous IV infusion immediately after IV bolus. Some patients respond to lower doses (e.g., 5 mg/hour). Max: 15 mg/hour.
      • Verapamil (not recommended for children younger than 1 year)
        • Verapamil Hydrochloride Solution for injection; Children and Adolescents: Initially, 0.1 to 0.3 mg/kg (i.e., 2 to 5 mg) IV bolus, given over at least 2 minutes. Max single dose: 5 mg. If no response, repeat dose in 30 minutes. Max total dose: 10 mg.
        • Verapamil Hydrochloride Solution for injection; Adults: Initially, 5 to 10 mg (0.075 to 0.15 mg/kg) IV over at least 2 minutes. If no adequate response after 30 minutes, may give an additional 10 mg (0.15 mg/kg). An optimal interval for subsequent doses has not been determined and should be individualized for each patient. Clinical guidelines recommend 2.5 to 5 mg IV over 2 minutes. If no therapeutic response or adverse reaction is seen, may administer repeated doses of 5 to 10 mg every 15 to 30 minutes up to a total dose of 20 mg. Alternative dosing is 5 mg IV every 15 minutes, up to a total dose of 30 mg.
    • Class Ia antiarrhythmic
      • Procainamide
        • Procainamide Hydrochloride Solution for injection; Neonates: 3.5 to 10 mg/kg IV given over 60 minutes followed by a continuous infusion of 10 to 80 mcg/kg/minute IV has been studied in a small number of neonates with various tachyarrhythmias.
        • Procainamide Hydrochloride Solution for injection; Infants, Children, and Adolescents: 3.5 to 15 mg/kg IV given over 20 to 60 minutes followed by a continuous infusion of 10 to 80 mcg/kg/minute IV (Max: 2 g/day IV) has been recommended. Higher infusion rates (up to 120 mcg/kg/minute IV) have been used in a limited number of infants and young children with postsurgical junctional tachycardia.
        • Procainamide Hydrochloride Solution for injection; Adults: 15 to 17 mg/kg as an IV infusion, infused at a rate of 20 to 30 mg/minute. Alternatively, 100 mg IV every 5 minutes given by slow IV push up to 1,000 mg.
    • Class III antiarrhythmic
      • Ibutilide
        • Ibutilide Fumarate Solution for injection; Adults weighing less than 60 kg: 0.01 mg/kg/dose IV over 10 minutes; may repeat 10 minutes after end of initial infusion if arrhythmia is not terminated.
        • Ibutilide Fumarate Solution for injection; Adults weighing 60 kg or more: 1 mg IV over 10 minutes; may repeat 10 minutes after end of initial infusion if arrhythmia is not terminated.
  • Oral drugs used for maintenance of sinus rhythm
    • Class IC antiarrhythmic
      • Flecainide
        • Flecainide Acetate Oral tablet; Infants 6 months and younger: Initially, 50 mg/m2/day PO given in 2 to 3 divided doses is recommended by the manufacturer; increase as needed every 4 days to achieve clinical goals (Max: 200 mg/m2/day PO). Alternatively, 1 to 6 mg/kg/day PO given in 2 to 3 divided doses (Max: 8 mg/kg/day PO) has been recommended.
        • Flecainide Acetate Oral tablet; Infants older than 6 months, Children, and Adolescents: Initially, 100 mg/m2/day PO given in 2 to 3 divided doses is recommended by the manufacturer; increase as needed every 4 days to achieve clinical goals (Max: 200 mg/m2/day PO). Alternatively, 1 to 6 mg/kg/day PO given in 2 to 3 divided doses (Max: 8 mg/kg/day PO) has been recommended.
        • Flecainide Acetate Oral tablet; Adults: Initially, 50 mg PO every 12 hours. Increase dose by increments of 50 mg PO twice daily every 4 days as needed. Max: 300 mg/day.
      • Propafenone
        • Propafenone Hydrochloride Oral tablet; Infants†: Initially, 100 to 200 mg/m2/day PO, in 3 divided doses for 3 days or more; dose may be increased by 100 mg/m2/day every 3 days, if needed, up to 600 mg/m2/day PO, given in 3 divided doses. Average dose: 300 to 500 mg/m2/day.
          • Off-label use in this age group
        • Propafenone Hydrochloride Oral tablet; Children†: Initially, 8 to 10 mg/kg/day PO in 3 divided doses. Increase by 2 to 3 mg/kg/day every 2 to 3 days PRN, up to 15 mg/kg/day PO (or 600 mg/m2/day PO), given in 3 divided doses. Average dosage: 13 to 16 mg/kg/day.
          • Off-label use in this age group
        • Propafenone Hydrochloride Oral tablet; Adults: Very limited data. 300 mg PO every 8 hours for 48 hours has been used.
    • β-blocker
      • Propranolol
        • Propranolol Hydrochloride Oral tablet; Neonates, Infants, Children, and Adolescents: Initially, 0.5 to 1 mg/kg/day PO, divided every 6 to 8 hours. Titrate by 1 mg/kg/day every 3 to 5 days as needed. Usual maintenance is 2 to 4 mg/kg/day PO. Max: 16 mg/kg/day or 60 mg/day. In older adolescents, 10 to 30 mg/dose PO every 6 to 8 hours may be given.
        • Propranolol Hydrochloride Oral solution; Adults: Initially, 10 to 30 mg PO 3 or 4 times daily. Dosage may be increased up to 160 to 320 mg/day PO, given in 3 to 4 divided doses. In geriatric patients, use conservative initial doses and titrate carefully.
    • Calcium channel blocker
      • Diltiazem
        • Diltiazem Hydrochloride Oral tablet; Infants, Children, and Adolescents: Use not established.
        • Diltiazem Hydrochloride Oral tablet, extended-release; Adults: 120 to 360 mg PO once daily.
      • Verapamil
        • Verapamil Hydrochloride Oral tablet; Children† and Adolescents†: Limited data available; 2 to 7 mg/kg/day PO (Max: 480 mg/day) divided 3 times daily has been suggested.
          • Off-label use in this age group
        • Verapamil Hydrochloride Oral tablet; Adults: 240 to 480 mg/day PO, given in 3 to 4 divided doses.

Nondrug and supportive care

Procedures
Radiofrequency catheter ablation

General explanation

  • Catheter ablation of accessory pathways has replaced medical therapy for many patients, owing to high success rate (95%) 1
  • Steerable ablation catheter is introduced into cardiac chambers to identify accessory pathways, assess their conduction properties, and obliterate conduction through them 1
  • Radiofrequency energy is used for performing ablation (cryoablation is also sometimes used)
  • Rhythm abnormalities may occur and death occurs in up to 0.2% of cases 1

Indication

  • Symptomatic patients with Wolff Parkinson White syndrome 1
  • Some asymptomatic patients
    • Adults: those with high-risk occupations, moderate- to high-level athletic activity, or high-risk features
    • Children and adolescents aged 8 to 21 years with preexcitation ECG pattern who have:
      • Shortest preexcited R-R interval of 250 milliseconds or less during atrial fibrillation 2
      • Shortest preexcited R-R interval more than 250 milliseconds during atrial fibrillation and develop symptoms such as palpitations or syncope 2
      • Structural heart disease or ventricular dysfunction 2

Contraindications

  • Intracardiac thrombus
  • Uncontrolled infection or coexisting medical condition
  • Pregnancy, owing to radiation exposure risk

Complications

  • Higher risk of ablation-related complications with accessory pathways in anteroseptal or midseptal anatomic location
Vagal maneuver during supraventricular tachycardia

General explanation

  • Valsalva maneuver: modified breathing
    • Adult patient: patient exhales against closed glottis or bears down as if to defecate after taking a deep breath
    • Pediatric patient: patient blows into an occluded drinking straw
  • Cold applied to the face: covering the patient’s face with a bag of cold water/crushed ice for 15 to 30 seconds
    • Best for children, who are unable to perform Valsalva maneuver
  • Carotid massage: application of digital pressure on carotid sinus
    • 1% risk of neurologic injury ranging from transient ischemic attack to profound hemiplegia 21 22
    • Should not be considered first line vagal maneuver, especially in older patients 22
    • Before performing carotid sinus massage, auscultate on both sides of neck to rule out carotid bruit
    • Never perform carotid sinus massage on both sides simultaneously
    • Valsalva maneuvers may be performed simultaneously to increase carotid sinus sensitivity

Indication

  • To slow down or terminate narrow-complex tachycardia or stable wide-complex supraventricular tachycardia 1

Contraindications

  • Do not perform carotid massage:
    • Unless carotid bruits have been ruled out by auscultation of both carotid arteries
    • If there is history of cerebral infarction or transient ischemic attack within the preceding 3 months
    • If there is possible or known digoxin toxicity
Synchronized direct current cardioversion

General explanation

  • Direct current is applied across the chest wall to cause temporary depolarization of cardiac cells
  • Direct current cardioversion has immediate effect in terminating dysrhythmias
  • Lowest effective energy setting should be used
    • 50 to 100 J for supraventricular tachycardia in adults
    • Start at 0.5 to 1 J/kg for supraventricular tachycardia in children; if not effective, increase to up to 2 J/kg
  • Postconversion dysrhythmias may occur
  • Ventricular fibrillation may be induced by direct current cardioversion, and this requires urgent defibrillation

Indication

  • Hemodynamically unstable patients with Wolff-Parkinson-White syndrome who have tachyarrhythmia and a palpable pulse

Contraindications

  • Atrial fibrillation lasting for longer than 36 to 48 hours, without anticoagulant therapy
  • Sinus tachycardia
  • Junctional/multifocal atrial tachycardia (Related: Multifocal atrial tachycardia)
  • Digoxin toxicity (Related: Digoxin, digitoxin, and other cardiac glycoside toxicity)

Special populations

  • Athletes
    • All patients diagnosed with Wolff-Parkinson-White syndrome should be cleared by a cardiologist before participation in sports
  • Children
    • Congenital heart diseases (eg, Ebstein anomaly) are common in infants with Wolff-Parkinson-White syndrome, and such infants frequently have multiple accessory pathways 2
    • Asymptomatic pediatric patients with Wolff-Parkinson-White syndrome are at increased risk of sudden cardiac death compared with asymptomatic adults 23
    • Irrespective of athletic status, children and adolescents with preexcitation ECG pattern should be referred to a pediatric electrophysiologist for risk stratification 2
    • Children who are at high risk of sudden cardiac death based on electrophysiologic testing and risk stratification should undergo early ablation 23
    • Attention-deficit/hyperactivity disorder medications may be administered to asymptomatic patients with Wolff-Parkinson-White syndrome, if indicated, with close follow-up for any onset of cardiac symptoms 2

Monitoring

  • When starting dofetilide or sotalol for ongoing medical management of patients with atrioventricular reciprocating tachycardia or preexcited atrial fibrillation
    • Inpatient monitoring with serial ECGs is generally performed because of the potential for significant QT prolongation and torsades de pointes

Complications and Prognosis

Complications

  • Symptomatic patients with Wolff-Parkinson-White syndrome are at risk for recurrent supraventricular tachycardias and atrial fibrillation
    • Increased risk of syncope during paroxysmal tachycardias
    • Atrial fibrillation conducting via the accessory tract with a very rapid ventricular response may progress to ventricular fibrillation and sudden cardiac death

Prognosis

  • Most asymptomatic patients with preexcitation have good prognosis; risk of sudden cardiac death in symptomatic patients is about 0.25% per year 24
  • Asymptomatic pediatric patients with Wolff-Parkinson-White syndrome are at increased risk of sudden cardiac death compared with asymptomatic adults 23
  • Multiple accessory pathways, septal location of accessory pathways, and highly inducible arrhythmias indicate poorer prognosis 1
  • Increased risk of sudden death is associated with history of symptomatic tachycardia, multiple accessory pathways, and a shortest preexcited R-R interval of less than 250 milliseconds during atrial fibrillation 3

Screening and Prevention

Screening

Screening tests

  • ECG is a sensitive test for screening with high negative predictive value; 25 however, benefit or cost-effectiveness of random screening for Wolff-Parkinson-White syndrome is not supported by available evidence
Sources

Blomström-Lundqvist C et al: ACC/AHA/ESC guidelines for the management of patients with supraventricular arrhythmias–executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop guidelines for the management of patients with supraventricular arrhythmias) developed in collaboration with NASPE-Heart Rhythm Society. J Am Coll Cardiol. 42(8):1493-531, 2003
Cross Reference  

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