Wolff-Parkinson-White syndrome resident survival guide

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alonso Alvarado, M.D. [2]; Hilda Mahmoudi M.D., M.P.H.[3]; Alejandro Lemor, M.D. [4]

Wolff-Parkinson-White Syndrome Resident Survival Guide Microchapters
Overview
Causes
FIRE
Diagsis
Treatment
Initial
Long-term
Do's
Don'ts

Overview

Wolff-Parkinson-White (WPW) syndrome is a condition of pre-excitation of the ventricles of the heart due to the presence of an accessory pathway known as the Bundle of Kent through which the electrical impulses bypass the AV node. The difference between WPW pattern and WPW syndrome is that WPW pattern is characterized by the presence of characteristic ECG findings, such as a short PR interval and a delta wave, whereas WPW syndrome is the occurrence of tachycardia with or without associated symptoms in a subject with existing WPW pattern.[1] The treatment of WPW syndrome is targeted towards the restoration of the sinus rhythm, usually by the administration of either ibutilide or procainamide. The most common type of arrhythmia in WPW syndrome is AV reentrant tachycardia.[2] Atrial fibrillation in a patient with WPW is life threatening and should be managed urgently. Atrial fibrillation in a patient with WPW should be suspected when there is ECG findings suggestive of atrial fibrillation in the context of a heart rate higher than 220 beats per minute.

Causes

Life Threatening Causes

Wolff-Parkinson-White syndrome can be a life-threatening condition and must be treated as such irrespective of the underlying cause.

Common Causes

FIRE: Focused Initial Rapid Evaluation

A Focused Initial Rapid Evaluation (FIRE) should be performed to identify patients in need of immediate intervention.[2][3]
Boxes in red signify that an urgent management is needed.

Abbreviations: AF: atrial fibrillation; AVRT: AV reentrant tachycardia; BP: blood pressure; ECG: electrocardiography; HF: heart failure; LVH: left ventricular hypertrophy; WPW: Wolff-Parkinson-White pattern

 
 
 
 
 
 
 
Identify cardinal findings that increase the pretest probability of Wolff-Parkinson-White syndrome

❑ Baseline ECG findings suggestive of WPW pattern (pre-excitation)

PR interval (<120 ms)
Delta wave
   WPW EKG leadV2.png

AND
Tachyarrhythmia

ECG findings suggestive of orthodromic AVRT

❑ Ventricular rate usually 200–300 bpm
❑ Regular, narrow QRS complex (usually <120 ms unless pre-existing bundle branch block or aberrant conduction)
P waves may be buried in the QRS or retrograde
Delta wave may be lost during NSR in case of participating concealed bypass tract

ECG findings suggestive of antidromic AVRT

❑ Ventricular rate usually 200–300 bpm
❑ Regular, wide QRS complex
Delta wave is observed during NSR

ECG findings suggestive of AF with WPW

❑ Ventricular rate usually >200 bpm
❑ Irregularly irregular, wide QRS complex with varying morphology
❑ Absence of P waves
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient have any of the following findings that require urgent cardioversion?

❑ Hemodynamic instability

Hypotension
Cold extremities
Peripheral cyanosis
Mottling
Altered mental status

Chest discomfort suggestive of ischemia

Decompensated heart failure
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
YES
 
 
 
NO
 
 
 
 
 
 
 
Perform
electrical cardioversion
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Irregular wide QRS complex

❑ Unsynchronized, biphasic 120–200 J OR
❑ Unsynchronized, monophasic 360 J

Irregular narrow QRS complex

❑ Synchronized, biphasic 120–200 J OR
❑ Synchronized, monophasic 200 J

Regular wide QRS complex

❑ Synchronized, biphasic or monophasic 100 J

Regular narrow QRS complex

❑ Synchronized, biphasic or monophasic 50–100 J
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Complete Diagnostic Approach

A complete diagnostic approach should be carried out after a focused initial rapid evaluation is conducted and following initiation of any urgent intervention.[2]

Abbreviations: AF: atrial fibrillation; AVRT: AV reentrant tachycardia; BP: blood pressure; ECG: electrocardiography; HF: heart failure; LVH: left ventricular hypertrophy

 
 
 
 
Characterize the symptoms:

Asymptomatic
Palpitations
Dyspnea
Fatigue
Chest discomfort
Lightheadedness
Polyuria
Characterize the timing of the symptoms:
❑ Onset

❑ First episode
❑ Recurrent

❑ Duration
❑ Frequency
❑ Termination of the episode

❑ Spontaneous
Medication use
❑ Not terminated
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Examine the patient:

Appearance of the patient
❑ Cool and diaphoretic

Vitals
Heart rate

Tachycardia (150-250 beats per minute)
Rhythm
❑ Regular (most of the cases)
❑ Irregularly irregular (suggestive of AF)

Blood pressure

Hypotension
❑ Normal BP

Cardiovascular
❑ Normal heart examination in most cases
Tricuspid regurgitation characterized by a holosystolic murmur heard best along the left lower sternal border (suggestive of Ebstein's anomaly)
S4 (suggestive of LVH)

Respiratory
Rales (suggestive of HF)

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
WPW with AF

❑ Suspect when AF appears with heart rates of 220 to 360
❑ Irregularly irregular, wide QRS complex with varying morphology

❑ Absence of P waves[4]
Wpw with afib.PNG
 
Orthodromic AVRT

❑ Ventricular rate usually 200–300 bpm
❑ Regular, narrow QRS complex (usually <120 ms unless pre-existing bundle branch block or aberrant conduction)
P waves may be buried in the QRS or retrograde
Delta wave may be lost during NSR in case of participating concealed bypass tract

SVT.jpg
 
Antidromic AVRT

❑ Ventricular rate usually 200–300 bpm
❑ Regular, wide QRS complex
Delta wave is observed during NSR

Wide complex tachy.jpg
 
 

Treatment

Initial Treatment

Shown below is an algorithm summarizing the initial approach to Wolff-Parkinson-White syndrome according to the 2003 ACC/AHA/ESC guidelines for the management of patients with supraventricular arrhythmias.[2][3]

 
 
 
Does the patient have any of the following findings that require urgent cardioversion?

❑ Hemodynamic instability

Hypotension
Cold extremities
Peripheral cyanosis
Mottling
Altered mental status

Chest discomfort suggestive of ischemia
Decompensated heart failure

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Direct current cardioversion (Urgent)
Check FIRE for details
 
 
 
What is the type of tachycardia according to the ECG findings?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
WPW + AF (Stable)
 
Orthodromic AVRT (Stable)
 
Antidromic AVRT (Stable)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Avoid the use of AV node blocking agents such as digoxin, calcium channel blockers, beta blockers and adenosine.

❑ Administer procainamide, 100 mg infusion diluted to 100mg/ml at a rate of 25-50 mg/min every 5 minutes (Class I, Level of Evidence C)[5]

❑ Administer until the arrhythmia is suppressed or until 500 mg has been administered
❑ Wait 10 minutes or longer to administer new dosage
Contraindications: third degree AV block, lupus erythematosus, idiosyncratic hypersensitivity, torsades de pointes
OR

❑ Administer ibutilide 1 mg IV infusion over 10 minutes (Class I, Level of Evidence C)[5]

❑ Repeat the dosage if the tachycardia continues
Contraindications: hypersensitivity to ibutilide or any component of the formulation, QTc >440 msec
OR

❑ Administer flecainide 50 mg every 12 hours (Class IIa, Level of Evidence B)[5]

❑ Increase 50mg BID every four days until efficacy is achieved
❑ Maximum dose recommended for SVT is 300 mg/day
Contraindications: pre-existing second degree AV block or third degree AV block , right bundle branch block associated with a left hemiblock unless a pacemaker is present, cardiogenic shock, hypersensitivity to the drug
 
❑ Use vagal maneuvers (Class I, Level of Evidence B)
Carotid sinus massage
Valsalva maneuver


If not effective initiate IV AV nodal blocking agent

❑ Administer adenosine 6 mg IV (bolus) (Class I, Level of Evidence A)

❑ If initial dose is not effective, administer a second dose of 12 mg, repeated a second time if required
Contraindications: second degree AV block or third degree AV block unless a pacemaker is present

If not effective

❑ Administer verapamil 5 to 10 mg (0.075 to 0.15 mg/kg body weight) IV boluses of over 2 minutes (Class I, Level of Evidence A)

❑ Give 30% of the dose in case of hepatic impairment
❑ Monitor for prolonged PR interval in case of renal impairment
Contraindications: severe left ventricular dysfunction, hypotension or cardiogenic shock

If not effective

❑ Administer procainamide, 100 mg infusion diluted to 100mg/ml at a rate of 25-50 mg/min every 5 minutes (Class I, Level of Evidence B)

❑ Give until the arrhythmia is suppressed or up to 500 mg
❑ Wait 10 minutes or longer to administer new dosage
Dosage should be adjusted for the individual patient in case of renal impairment
Contraindications: third degree AV block, lupus erythematosus, idiosyncratic hypersensitivity, torsades de pointes
 
Avoid the use of AV node blocking agents such as digoxin, calcium channel blockers, beta blockers and adenosine.

❑ Administer procainamide, 100 mg infusion diluted to 100mg/ml at a rate of 25-50 mg/min every 5 minutes (Class I, Level of Evidence B)

❑ Give until the arrhythmia is suppressed or until 500 mg has been administered
❑ Wait 10 minutes or longer to administer new dosage
Contraindications: third degree AV block, lupus erythematosus, idiosyncratic hypersensitivity, torsades de pointes
OR

❑ Administer ibutilide 1 mg IV infusion over 10 minutes (Class I, Level of Evidence B)

❑ Repeat the dosage if the tachycardia continues
Contraindications: hypersensitivity to ibutilide or any component of the formulation, QTc >440 msec
OR

❑ Administer flecainide 50 mg every 12 hours

❑ Increase 50mg BID every four days until efficacy is achieved
❑ Maximum dose recommended for SVT is 300 mg/day
Contraindications: pre-existing second degree AV block or third degree AV block , right bundle branch block associated with a left hemiblock unless a pacemaker is present, cardiogenic shock, hypersensitivity to the drug
 
 
 

Long-Term Treatment

Shown below is an algorithm summarizing the long-term treatment of Wolff-Parkinson-White syndrome.[2]

 
 
 
 
 
Does the patient with pre-excitation have symptomatic arrhythmia?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Is the arrhythmia poorly tolerated, OR
is atrial fibrillation with rapid conduction present?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Do's

Don'ts

References

  1. "Wolff-Parkinson-White Syndrome and Accessory Pathways". Retrieved 1 April 2014.
  2. 2.0 2.1 2.2 2.3 2.4 "ACC/AHA/ESC Guidelines for the Management of Patients With Supraventricular Arrhythmias—Executive Summary". Retrieved 15 August 2013.
  3. 3.0 3.1 "Part 8: Adult Advanced Cardiovascular Life Support". Retrieved 3 April 2014.
  4. Fengler, Brian T.; Brady, William J.; Plautz, Claire U. (2007). "Atrial fibrillation in the Wolff-Parkinson-White syndrome: ECG recognition and treatment in the ED". The American Journal of Emergency Medicine. 25 (5): 576–583. doi:10.1016/j.ajem.2006.10.017. ISSN 0735-6757.
  5. 5.0 5.1 5.2 5.3 American College of Cardiology Foundation. American Heart Association. European Society of Cardiology. Heart Rhythm Society. Wann LS, Curtis AB; et al. (2013). "Management of patients with atrial fibrillation (compilation of 2006 ACCF/AHA/ESC and 2011 ACCF/AHA/HRS recommendations): a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines". Circulation. 127 (18): 1916–26. doi:10.1161/CIR.0b013e318290826d. PMID 23545139.
  6. Garratt, C.; Antoniou, A.; Ward, D.; Camm, AJ. (1989). "Misuse of verapamil in pre-excited atrial fibrillation". Lancet. 1 (8634): 367–9. PMID 2563516. Unknown parameter |month= ignored (help)
  7. Gulamhusein, S.; Ko, P.; Carruthers, SG.; Klein, GJ. (1982). "Acceleration of the ventricular response during atrial fibrillation in the Wolff-Parkinson-White syndrome after verapamil". Circulation. 65 (2): 348–54. PMID 7053894. Unknown parameter |month= ignored (help)
  8. McGovern, B.; Garan, H.; Ruskin, JN. (1986). "Precipitation of cardiac arrest by verapamil in patients with Wolff-Parkinson-White syndrome". Ann Intern Med. 104 (6): 791–4. PMID 3706931. Unknown parameter |month= ignored (help)


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