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.; Hilda Mahmoudi M.D., M.P.H.[2]; Alejandro Lemor, M.D. [3]

Wolff-Parkinson-White Syndrome Resident Survival Guide Microchapters
Overview
Causes
FIRE
Diagnosis
Treatment
Initial
WPW with AF
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. The algorithm below is based on the 2003 ACC/AHA/ESC and the ACLS guidelines.[2][3]
Boxes in salmon color signify that an urgent management is needed.

 
 
 
Identify cardinal findings that increase the pretest probability of Wolff-Parkinson-White syndrome
❑ Palpitations
❑ Chest discomfort
❑ Regular rhythm
❑ Rate over 150 bpm
❑ Orthodrome AVRT: narrow QRS complex preceded by a p-wave
❑ Antidrome AVRT: wide QRS complex followed by a retrograde p-wave
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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 urgent synchronized electrical cardioversion
❑ Narrow regular rhythm: 50-100 Joules
❑ Narrow irregular rhythm: 120-200 Joules biphasic or 200 Joules monophasic
❑ Wide regular rhythm: 100 Joules
❑ Wide irregular rhythm: defibrillation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 


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

 
 
 
 
Characterize the symptoms:

❑ Asymptomatic
Palpitations
Dyspnea
Fatigue
Chest discomfort
Lightheadedness
Polyuria
Characterize the timing of the symptoms:
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 rhythm
❑ Rapid ventricular response (over 220 bpm)
Wide QRS complex
❑ No P wave

ref name="FenglerBrady2007">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.</ref>
 
Orthodromic AVRT

❑ Regular rhythm
Narrow QRS complexes
P wave before QRS

 
Antidromic AVRT

❑ Regular rhythm
Wide QRS complexes
P wave after QRS

 
 

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

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
 
 
 
Yes
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
❑ Assess the ECG
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Orthodromic AVRT
 
Antidromic AVRT
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

❑ 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- or third-degree A-V block (except in patients with a functioning artificial pacemaker

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 (systolic pressure less than 90 mm Hg) or cardiogenic shock

If not effective
❑ Administer procainamide, 100 mg infusion diluted to 100mg/ml at a rate of 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: complete heart block, lupus erythematosus, idiosyncratic hypersensitivity, torsades de pointes
 

Avoid the use of av blocking agents such as digoxin, verapamil or diltiazem

❑ 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 procainamide, 100 mg infusion diluted to 100mg/ml at a rate of 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: complete heart block, lupus erythematosus, idiosyncratic hypersensitivity, torsades de pointes

Or
❑ Administer adenosine 6 mg IV (bolus)

❑ If initial dose is not effective, administer a second dose of 12 mg, repeated a second time if required
❑ Should be used with caution because may produce AF
Contraindications: second- or third-degree A-V block (except in patients with a functioning artificial pacemaker

 
 
 

Wolff-Parkinson-White Syndrome with Atrial Fibrillation

Atrial fibrillation in a patient with WPW should be suspected when the the heart rate of a patient with WPW is between 220 and 360 bpm. Shown below is an algorithm summarizing the managment of Wolff-Parkinson-White syndrome with atrial fibrillation according to the ACC/AHA/ESC 2006 Guidelines for the Management of Patients with Atrial Fibrillation.[3][4]

 
 
 
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

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
 
 
 
Yes
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Avoid the use of AV node blocking agents such as digoxin, verapamil or diltizem
❑ Restore sinus rhythm (Class I, Level of Evidence C)

❑ 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 procainamide 100 mg infusion diluted to 100mg/ml at a rate of 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 to administer new dosage
Contraindications: complete heart block, lupus erythematosus, idiosyncratic hypersensitivity, torsades de pointes

Or

❑ Administer amiodarone 15 mg/min in 10 minutes (Class IIb, Level of Evidence B)
❑ Then, 1mg/min for 6 hours
❑ Then 0.5 mg/min for 18 hours
Contraindications: cardiogenic shock, severe sinus-node dysfunction
 
 
 

Long-Term Treatment

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

 
 
 
 
 
Long Term management
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Single or infrequent episodes

❑ No treatment (Class I, Level of Evidence C)
And
Vagal maneuvers (Class I, Level of Evidence B)
Or
❑ Single dose treatment (pill-in-the-pocket): verapamil, dialtizem or beta blockers (Class I, Level of Evidence B)
Or
Catheter ablation (Class IIa, Level of Evidence B)
Or
Sotalol or amiodarone (Class IIb, Level of Evidence B)
Or
Flecainide or propofenone (Class IIb, Level of Evidence C)

Avoid AV blocking agents such as: digoxin, verapamil, dialtizem(Class III, Level of Evidence C)

 
 
 
 
 
 
 

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 "ACC/AHA/ESC Guidelines for the Management of Patients With Supraventricular Arrhythmias—Executive Summary". Retrieved 15 August 2013.
  3. 3.0 3.1 3.2 "Part 8: Adult Advanced Cardiovascular Life Support". Retrieved 3 April 2014.
  4. Fuster, V.; Rydén, LE.; Cannom, DS.; Crijns, HJ.; Curtis, AB.; Ellenbogen, KA.; Halperin, JL.; Le Heuzey, JY.; Kay, GN. (2006). "ACC/AHA/ESC 2006 Guidelines for the Management of Patients with Atrial Fibrillation: 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 Revise the 2001 Guidelines for the Management of Patients With Atrial Fibrillation): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society". Circulation. 114 (7): e257–354. doi:10.1161/CIRCULATIONAHA.106.177292. PMID 16908781. Unknown parameter |month= ignored (help)
  5. 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)
  6. 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)
  7. 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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