Wolff-Parkinson-White syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Rim Halaby, M.D. [3]

Overview

Wolff-Parkinson-White (WPW) syndrome patients who are hemodynamically unstable, as reflected by the presence of hypotension, cold extremities, mottling or peripheral cyanosis, or those who present with ischemic chest pain or decompensated heart failure should urgently undergo direct current cardioversion. The medical therapy of hemodynamically stable patients with WPW syndrome depends on the type of the tachycardia. When the ECG findings suggest orthodromic AVRT, the patient should be managed similarly to patients with supreventricular tachycardia followed by the sequential administration of adenosine, verapamil and procainamide in case of failure to improve. Among patients with antidromic AVRT, AV nodal blocking agents should be avoided and patients should be treated with either procainamide, ibutilide or flecainide. In case of WPW syndrome with atrial fibrillation in hemodynamically stable patients, procainamide, ibutilide or flecainide can be administered. The long term treatment of patients with WPW syndrome depends on the presence or absence of symptoms and their severity. Patients who have poorly tolerated symptomatic WPW syndrome should undergo [[catheter ablation.

Acute Treatment

Atrioventricular Reentrant Tachycardia (AVRT)

• AVRT is one of the type of tachycardia that can occur in patients with WPW pattern. AVRT can be either orthodromic or antidromic, and the distinction between the two types is important because it dictates the choice of treatment.

Hemodynamically Unstable Patients

• WPW syndrome patients with AVRT who are hemodynamically unstable, as reflected by the presence of hypotension, cold extremities, mottling or peripheral cyanosis, or those who present with ischemic chest pain or decompensated heart failure should urgently undergo direct current cardioversion. The shocks should be delivered as follows:
  • Narrow regular rhythm: synchronized electrical cardioversion, 50-100 Joules
  • Narrow irregular rhythm: synchronized electrical cardioversion, 120-200 Joules biphasic or 200 Joules monophasic
  • Wide regular rhythm: synchronized electrical cardioversion, 100 Joules
  • Wide irregular rhythm: unsynchronized electrical cardioversion, 200-360 Joules monophasic, or 100-200 Joules biphasic

Orthodromic AVRT in Hemodynamically Stable Patients

• The management of WPW syndrome patients who are hemodynamically stable depends on the type of AVRT. When the ECG findings suggest orthodromic AVRT, the patient should be managed similarly to patients with supreventricular tachycardia. The management should begin with vagal maneuvers such as carotid sinus massage and valsalva maneuver. If the patient's tachycardia does not resolve, the patient should be administered IV adenosine. In case of failure to improve, administration of verapamil must be considered followed by procainamide.

The sequence of therapeutic decisions is summarized below.

Recommendations for acute treatment of orthodromic AVRT

Vagal maneuver (Class I, Level of Evidence B):

❑ Carotid sinus massage for 5-10 seconds in the absent of bruit ❑ Valsalva maneuver for 10-30 seconds by bearing down against closed glottis,more successful technique ❑ Applying ice-cold wet towel to the face

Adenosin(Class I, Level of Evidence B) :

❑ Effective in conversion of AVRT in 90-95% patients ❑ Episode of AVRT may be induced again by PAC or PVC after termination of tachyarrhythmia by adenosin ❑ AF may be induced by adenosin, rapidly passing through accessory pathway Contraindications: asthma, second degree AV block or third degree AV block unless a pacemaker is present

Synchronized cardioversion in unstable hemodynamic or stable hemodynamic and ineffectiveness of vagal maneuver or adenosin: (Class I, Level of Evidence B)

❑ Highly effective in termination of AVRT ❑ Avoidance of complications associated antiarrhythmic drugs ❑ In the presence of PVC or PAC just after cardioversion, antiarrhythmic drugs is recommended for prevention of restarting AVRT

If verapamil is 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 OR ❑ Administer propranolol, metoprolol, and [[esmolol]

Recommendations for acute treatment of orthodromic AVRT
Perform the following maneuvers (Class I, Level of Evidence B):
❑ Vagal maneuvers ❑ Carotid sinus massage ❑ Valsalva maneuver
If not effective initiate the IV AV nodal blocking agent adenosine:
❑ Administer adenosine 6 mg IV (bolus) (Class I, Level of Evidence A) ❑ If the initial dose is not effective, administer a second dose of 12 mg, repeated a second time if required Contraindications: asthma, second degree AV block or third degree AV block unless a pacemaker is present
If adenosine is 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 verapamil is 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 OR ❑ Administer propranolol, metoprolol, and esmolol	If verapamil is 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	If verapamil is 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	If verapamil is 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

Antidromic AVRT in Hemodynamically Stable Patients

• In antidromic AVRT, the antegrade conduction of the electrical signals occurs through the accessory pathway, while the retrograde conduction occurs through either the AV node or a second accessory pathway. Therefore, among patients with antidromic AVRT, AV nodal blocking agents should be avoided because they are ineffective in the subset of patients among whom the retrograde conduction occurs through a second accessory pathway rather than through the AV node. In this case, the use of digoxin, calcium channel blockers, beta blockers and adenosine should be avoided. Adenosine, in particular, might lead to atrial fibrillation with rapid ventricular response. Patients should be treated with either procainamide, ibutilide, or flecainide.

Treatment of Antidromic AVRT in Hemodynamically Stable Patients
Medication	Dosage	Notes
Procainamide	100 mg infusion diluted to 100mg/ml at a rate of 25-50 mg/min every 5 minutes	❑ 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
Ibutilide	1 mg IV infusion over 10 minutes	❑ Repeat the dosage if the tachycardia continues Contraindications: hypersensitivity to ibutilide or any component of the formulation, QTc >440 msec
Flecainide	50 mg every 12 hours	❑ Increase 50mg BID every four days until efficacy is achieved ❑ Maximum dose recommended for supraventricular tachycardia 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

Atrial Fibrillation

• WPW syndrome with atrial fibrillation should be suspected whenever the ECG reveals an irregular rhythm with absent P wave in the presence of a heart rate more than 220 beats per minute.

Hemodynamically Unstable Patients

In hemodynamically unstable patients, urgent direct current cardioversion should be performed.

Hemodynamically Stable Patients

• Hemodynamically stable patients can be administered any of the following intravenous medications:
  • Procainamide (Class I, Level of evidence C)- in the presence of wide QRS on ECG or a rapid preexcited ventricular response
  • Ibutilide (Class I, Level of evidence C)- in the presence of wide QRS on ECG or a rapid preexcited ventricular response
  • Flecainide (Class IIa, Level of evidence B)
  • Quinidine (Class IIb, Level of evidence B)
  • Procainamide (Class IIb, Level of evidence B)
  • Disopyramide (Class IIb, Level of evidence B)
  • Ibutilide (Class IIb, Level of evidence B)
  • Amiodarone(Class IIb, Level of evidence B)

• AV nodal blocking drugs, such as adenosine, verapamil, digoxin, and beta blockers must be avoided.

Long Term Treatment

The long term management of patients with WPW syndrome depends on the presence or absence of syndrome. Among symptomatic patients, the tolerability of the symptoms guides the choice of the long term treatment.

Asymptomatic Patients

• Asymptomatic patients can either receive no treatment (Class I, Level of Evidence C) or can undergo catheter ablation (Class IIa, Level of Evidence B).

Symptomatic Patients

• Patients who have poorly tolerated symptoms or atrial fibrillation with rapid conduction should be treated with catheter ablation (Class I, Level of Evidence B).

• Patients who have well tolerated symptoms can be treated with any of the following:
  • Catheter ablation (Class I, Level of Evidence B), or
  • Class I C antiarrhythmic agents such as: flecainide, propafenone (Class IIa, Level of Evidence C), or
  • Sotalol, amiodarone or beta blockers (Class IIa, Level of Evidence C)

• AV nodal blocking agents such as digoxin, verapamil and diltiazem should not be administered to patients with WPW syndrome and atrial fibrillation (Class III, Level of Evidence C).

Contraindicated medications

WPW SYNDROME is considered an absolute contraindication to the use of the following medications:

• Eletriptan
• Diltiazem
• Frovatriptan
• Sumatriptan
• Zolmitriptan