Treatment of symptomatic arrhythmias associated with the Wolff-Parkinson-White syndrome
- Luigi Di Biase, MD, PhD, FHRS, FACC
Luigi Di Biase, MD, PhD, FHRS, FACC
- Cardiologist, Electrophysiologist, Section Head Electrophysiology, Director of Arrhythmia Services, Associate Professor, Albert Einstein College of Medicine at Montefiore Hospital, New York
- Senior Researcher, Texas Cardiac Arrhythmia Institute at St. David's Medical Center
- Edward P Walsh, MD
Edward P Walsh, MD
- Chief, Cardiac Electrophysiology, Boston Children’s Hospital
- Professor of Pediatrics, Harvard Medical School
- Section Editors
- Samuel Lévy, MD
Samuel Lévy, MD
- Section Editor — Cardiac Arrhythmias
- Professor of Cardiology
- University of Marseille, France
- Bradley P Knight, MD, FACC
Bradley P Knight, MD, FACC
- Section Editor — Cardiac Arrhythmias
- Professor of Medicine
- Feinberg School of Medicine, Northwestern University
Conduction from the atria to the ventricles normally occurs via the atrioventricular node (AV)-His-Purkinje system. Patients with a preexcitation syndrome have an additional pathway, known as an accessory pathway, which directly connects the atria and ventricles, bypassing the AV node. Normal conduction through the AV node is slower than conduction over the accessory pathway. Thus, when there is conduction over an accessory pathway, the ventricles are activated earlier than if the impulse had traveled through the AV node. This early activation, referred to as preexcitation, is responsible for the classic electrocardiographic (ECG) findings of a shortened PR interval and, in most patients, a delta wave (waveform 1).
Symptoms, ranging from mild palpitations to syncope and, rarely, even sudden cardiac death, are the result of tachycardia, usually due to a macroreentrant circuit involving the AV node, the ventricles, the accessory pathway, and the atria. This classic supraventricular tachycardia associated with WPW syndrome is called AV reentrant or reciprocating tachycardia (AVRT). However, preexcited atrial fibrillation or atrial flutter with a rapid ventricular response may also result in symptoms. Fortunately, the incidence of sudden death in patients with the WPW syndrome is quite low, ranging from 0 to 0.39 percent annually in several large case series, with the lowest risk seen in asymptomatic patients.
Patients with the WPW syndrome are usually treated because of symptomatic arrhythmias. Treatment may sometimes be extended to asymptomatic patients with a WPW pattern if certain "high-risk" features are present. However, most asymptomatic patients with the WPW electrocardiographic pattern are not treated. Treatment options for persons with arrhythmias and the WPW syndrome include nonpharmacologic therapies (ie, catheter ablation of the accessory pathway) as well as pharmacologic therapy (to slow ventricular heart rates or to prevent arrhythmias). The choice of the optimal therapy depends on the acuity of the arrhythmia(s) and the risk of sudden cardiac death, with pharmacologic agents being the treatment of choice for most acute arrhythmias, while catheter ablation is nearly always preferred for the long-term prevention of recurrent arrhythmias involving the accessory pathway.
This topic will review the available therapeutic options for the treatment of arrhythmias in the WPW syndrome. The clinical manifestations, approach to diagnosis, and the types of arrhythmias which can occur in persons with an accessory pathway and the WPW pattern are discussed separately. (See "Epidemiology, clinical manifestations, and diagnosis of the Wolff-Parkinson-White syndrome" and "Atrioventricular reentrant tachycardia (AVRT) associated with an accessory pathway".)
ACUTE TREATMENT OF SYMPTOMATIC ARRHYTHMIAS
While the preferred long-term treatment approach for patients with an accessory pathway, preexcitation, and symptomatic arrhythmias is catheter-based radiofrequency ablation, patients who present with an acute arrhythmia often require initial pharmacologic therapy for ventricular rate control or restoration of sinus rhythm. However, because of the electrophysiologic differences between AV nodal tissue and tissue comprising an accessory pathway, standard therapy for heart rate control may actually worsen symptoms and lead to clinical deterioration in patients with a tachycardia involving an accessory pathway. Knowledge of the presence of an accessory pathway is critical in choosing the correct initial pharmacologic therapy. (See 'Treatment to prevent recurrent arrhythmias' below and "Overview of the acute management of tachyarrhythmias".)
- Page RL, Joglar JA, Caldwell MA, et al. 2015 ACC/AHA/HRS Guideline for the Management of Adult Patients With Supraventricular Tachycardia: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation 2016; 133:e506.
- Mehta D, Wafa S, Ward DE, Camm AJ. Relative efficacy of various physical manoeuvres in the termination of junctional tachycardia. Lancet 1988; 1:1181.
- Belardinelli L, Linden J, Berne RM. The cardiac effects of adenosine. Prog Cardiovasc Dis 1989; 32:73.
- diMarco JP, Sellers TD, Lerman BB, et al. Diagnostic and therapeutic use of adenosine in patients with supraventricular tachyarrhythmias. J Am Coll Cardiol 1985; 6:417.
- DiMarco JP, Sellers TD, Berne RM, et al. Adenosine: electrophysiologic effects and therapeutic use for terminating paroxysmal supraventricular tachycardia. Circulation 1983; 68:1254.
- Exner DV, Muzyka T, Gillis AM. Proarrhythmia in patients with the Wolff-Parkinson-White syndrome after standard doses of intravenous adenosine. Ann Intern Med 1995; 122:351.
- Dougherty AH, Gilman JK, Wiggins S, et al. Provocation of atrioventricular reentry tachycardia: a paradoxical effect of adenosine. Pacing Clin Electrophysiol 1993; 16:8.
- DiMarco JP, Miles W, Akhtar M, et al. Adenosine for paroxysmal supraventricular tachycardia: dose ranging and comparison with verapamil. Assessment in placebo-controlled, multicenter trials. The Adenosine for PSVT Study Group. Ann Intern Med 1990; 113:104.
- Rinkenberger RL, Prystowsky EN, Heger JJ, et al. Effects of intravenous and chronic oral verapamil administration in patients with supraventricular tachyarrhythmias. Circulation 1980; 62:996.
- Sung RJ, Elser B, McAllister RG Jr. Intravenous verapamil for termination of re-entrant supraventricular tachycardias: intracardiac studies correlated with plasma verapamil concentrations. Ann Intern Med 1980; 93:682.
- Jackman WM, Friday KJ, Fitzgerald DM, et al. Use of intracardiac recordings to determine the site of drug action in paroxysmal supraventricular tachycardia. Am J Cardiol 1988; 62:8L.
- Kowey PR, Friehling TD, Marinchak RA. Electrophysiology of beta blockers in supraventricular arrhythmias. Am J Cardiol 1987; 60:32D.
- Anderson S, Blanski L, Byrd RC, et al. Comparison of the efficacy and safety of esmolol, a short-acting beta blocker, with placebo in the treatment of supraventricular tachyarrhythmias. The Esmolol vs Placebo Multicenter Study Group. Am Heart J 1986; 111:42.
- Mandel WJ, Laks MM, Obayashi K, et al. The Wolff-Parkinson-White syndrome: pharmacologic effects of procaine amide. Am Heart J 1975; 90:744.
- Wellens HJ. The wide QRS tachycardia. Ann Intern Med 1986; 104:879.
- Kang KT, Potts JE, Radbill AE, et al. Permanent junctional reciprocating tachycardia in children: a multicenter experience. Heart Rhythm 2014; 11:1426.
- Dorostkar PC, Silka MJ, Morady F, Dick M 2nd. Clinical course of persistent junctional reciprocating tachycardia. J Am Coll Cardiol 1999; 33:366.
- January CT, Wann LS, Alpert JS, et al. 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation 2014; 130:2071.
- Glatter KA, Dorostkar PC, Yang Y, et al. Electrophysiological effects of ibutilide in patients with accessory pathways. Circulation 2001; 104:1933.
- Bianconi L, Boccadamo R, Pappalardo A, et al. Effectiveness of intravenous propafenone for conversion of atrial fibrillation and flutter of recent onset. Am J Cardiol 1989; 64:335.
- Suttorp MJ, Kingma JH, Jessurun ER, et al. The value of class IC antiarrhythmic drugs for acute conversion of paroxysmal atrial fibrillation or flutter to sinus rhythm. J Am Coll Cardiol 1990; 16:1722.
- Suttorp MJ, Kingma JH, Lie-A-Huen L, Mast EG. Intravenous flecainide versus verapamil for acute conversion of paroxysmal atrial fibrillation or flutter to sinus rhythm. Am J Cardiol 1989; 63:693.
- Krahn AD, Klein GJ, Yee R. A randomized, double-blind, placebo-controlled evaluation of the efficacy and safety of intravenously administered dofetilide in patients with Wolff-Parkinson-White syndrome. Pacing Clin Electrophysiol 2001; 24:1258.
- Garratt C, Antoniou A, Ward D, Camm AJ. Misuse of verapamil in pre-excited atrial fibrillation. Lancet 1989; 1:367.
- Gulamhusein S, Ko P, Carruthers SG, Klein GJ. Acceleration of the ventricular response during atrial fibrillation in the Wolff-Parkinson-White syndrome after verapamil. Circulation 1982; 65:348.
- McGovern B, Garan H, Ruskin JN. Precipitation of cardiac arrest by verapamil in patients with Wolff-Parkinson-White syndrome. Ann Intern Med 1986; 104:791.
- Boriani G, Biffi M, Frabetti L, et al. Ventricular fibrillation after intravenous amiodarone in Wolff-Parkinson-White syndrome with atrial fibrillation. Am Heart J 1996; 131:1214.
- Simonian SM, Lotfipour S, Wall C, Langdorf MI. Challenging the superiority of amiodarone for rate control in Wolff-Parkinson-White and atrial fibrillation. Intern Emerg Med 2010; 5:421.
- Sellers TD Jr, Bashore TM, Gallagher JJ. Digitalis in the pre-excitation syndrome. Analysis during atrial fibrillation. Circulation 1977; 56:260.
- Jackman WM, Wang XZ, Friday KJ, et al. Catheter ablation of accessory atrioventricular pathways (Wolff-Parkinson-White syndrome) by radiofrequency current. N Engl J Med 1991; 324:1605.
- Kuck KH, Schlüter M, Geiger M, et al. Radiofrequency current catheter ablation of accessory atrioventricular pathways. Lancet 1991; 337:1557.
- Calkins H, Sousa J, el-Atassi R, et al. Diagnosis and cure of the Wolff-Parkinson-White syndrome or paroxysmal supraventricular tachycardias during a single electrophysiologic test. N Engl J Med 1991; 324:1612.
- Chen SA, Tai CT. Ablation of atrioventricular accessory pathways: current technique-state of the art. Pacing Clin Electrophysiol 2001; 24:1795.
- Calkins H, Langberg J, Sousa J, et al. Radiofrequency catheter ablation of accessory atrioventricular connections in 250 patients. Abbreviated therapeutic approach to Wolff-Parkinson-White syndrome. Circulation 1992; 85:1337.
- Scheinman MM, Huang S. The 1998 NASPE prospective catheter ablation registry. Pacing Clin Electrophysiol 2000; 23:1020.
- Aguinaga L, Primo J, Anguera I, et al. Long-term follow-up in patients with the permanent form of junctional reciprocating tachycardia treated with radiofrequency ablation. Pacing Clin Electrophysiol 1998; 21:2073.
- Rodriguez LM, Geller JC, Tse HF, et al. Acute results of transvenous cryoablation of supraventricular tachycardia (atrial fibrillation, atrial flutter, Wolff-Parkinson-White syndrome, atrioventricular nodal reentry tachycardia). J Cardiovasc Electrophysiol 2002; 13:1082.
- Wellens HJ. Should catheter ablation be performed in asymptomatic patients with Wolff-Parkinson-White syndrome? When to perform catheter ablation in asymptomatic patients with a Wolff-Parkinson-White electrocardiogram. Circulation 2005; 112:2201.
- Pappone C, Santinelli V. Should catheter ablation be performed in asymptomatic patients with Wolff-Parkinson-White syndrome? Catheter ablation should be performed in asymptomatic patients with Wolff-Parkinson-White syndrome. Circulation 2005; 112:2207.
- Chevalier P, Cadi F, Scridon A, et al. Prophylactic radiofrequency ablation in asymptomatic patients with Wolff-Parkinson-White is not yet a good strategy: a decision analysis. Circ Arrhythm Electrophysiol 2013; 6:185.
- Todd DM, Klein GJ, Krahn AD, et al. Asymptomatic Wolff-Parkinson-White syndrome: is it time to revisit guidelines? J Am Coll Cardiol 2003; 41:245.
- Pappone C, Vicedomini G, Manguso F, et al. Wolff-Parkinson-White syndrome in the era of catheter ablation: insights from a registry study of 2169 patients. Circulation 2014; 130:811.
- Pediatric and Congenital Electrophysiology Society (PACES), Heart Rhythm Society (HRS), American College of Cardiology Foundation (ACCF), et al. PACES/HRS expert consensus statement on the management of the asymptomatic young patient with a Wolff-Parkinson-White (WPW, ventricular preexcitation) electrocardiographic pattern: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology Foundation (ACCF), the American Heart Association (AHA), the American Academy of Pediatrics (AAP), and the Canadian Heart Rhythm Society (CHRS). Heart Rhythm 2012; 9:1006.
- Crossen KJ, Lindsay BD, Cain ME. Reliability of retrograde atrial activation patterns during ventricular pacing for localizing accessory pathways. J Am Coll Cardiol 1987; 9:1279.
- Jackman WM, Friday KJ, Yeung-Lai-Wah JA, et al. New catheter technique for recording left free-wall accessory atrioventricular pathway activation. Identification of pathway fiber orientation. Circulation 1988; 78:598.
- Denes P, Wyndham CR, Amat-y-Leon F, et al. Atrial pacing at multiple sites in the Wolff-Parkinson-White syndrome. Br Heart J 1977; 39:506.
- Mitchell LB, Mason JW, Scheinman MM, et al. Recordings of basal ventricular preexcitation from electrode catheters in patients with accessory atrioventricular connections. Circulation 1984; 69:233.
- Chen X, Borggrefe M, Shenasa M, et al. Characteristics of local electrogram predicting successful transcatheter radiofrequency ablation of left-sided accessory pathways. J Am Coll Cardiol 1992; 20:656.
- Scheinman MM. Catheter ablation for cardiac arrhythmias, personnel, and facilities. North American Society of Pacing and Electrophysiology Ad Hoc Committee on Catheter Ablation. Pacing Clin Electrophysiol 1992; 15:715.
- Kay GN, Epstein AE, Dailey SM, Plumb VJ. Role of radiofrequency ablation in the management of supraventricular arrhythmias: experience in 760 consecutive patients. J Cardiovasc Electrophysiol 1993; 4:371.
- Calkins H, Yong P, Miller JM, et al. Catheter ablation of accessory pathways, atrioventricular nodal reentrant tachycardia, and the atrioventricular junction: final results of a prospective, multicenter clinical trial. The Atakr Multicenter Investigators Group. Circulation 1999; 99:262.
- Dagres N, Clague JR, Kottkamp H, et al. Radiofrequency catheter ablation of accessory pathways. Outcome and use of antiarrhythmic drugs during follow-up. Eur Heart J 1999; 20:1826.
- Chen SA, Hsia CP, Chiang CE, et al. Reappraisal of radiofrequency ablation of multiple accessory pathways. Am Heart J 1993; 125:760.
- Huang JL, Chen SA, Tai CT, et al. Long-term results of radiofrequency catheter ablation in patients with multiple accessory pathways. Am J Cardiol 1996; 78:1375.
- Schweikert RA, Saliba WI, Tomassoni G, et al. Percutaneous pericardial instrumentation for endo-epicardial mapping of previously failed ablations. Circulation 2003; 108:1329.
- Langberg JJ, Calkins H, Kim YN, et al. Recurrence of conduction in accessory atrioventricular connections after initially successful radiofrequency catheter ablation. J Am Coll Cardiol 1992; 19:1588.
- Dagres N, Clague JR, Lottkamp H, et al. Impact of radiofrequency catheter ablation of accessory pathways on the frequency of atrial fibrillation during long-term follow-up; high recurrence rate of atrial fibrillation in patients older than 50 years of age. Eur Heart J 2001; 22:423.
- Liu J, Dole LR. Late complete atrioventricular block complicating radiofrequency catheter ablation of a left posteroseptal accessory pathway. Pacing Clin Electrophysiol 1998; 21:2136.
- Seidl K, Hauer B, Zahn R, Senges J. Unexpected complete AV block following transcatheter ablation of a left posteroseptal accessory pathway. Pacing Clin Electrophysiol 1998; 21:2139.
- Kessler DJ, Pirwitz MJ, Horton RP, et al. Intracardiac shunts resulting from transseptal catheterization for ablation of accessory pathways in otherwise normal hearts. Am J Cardiol 1998; 82:391.
- Fitchet A, Turkie W, Fitzpatrick AP. Transeptal approach to ablation of left-sided arrhythmias does not lead to persisting interatrial shunt: a transesophageal echocardiographic study. Pacing Clin Electrophysiol 1998; 21:2070.
- Kocovic DZ, Harada T, Shea JB, et al. Alterations of heart rate and of heart rate variability after radiofrequency catheter ablation of supraventricular tachycardia. Delineation of parasympathetic pathways in the human heart. Circulation 1993; 88:1671.
- Psychari SN, Theodorakis GN, Koutelou M, et al. Cardiac denervation after radiofrequency ablation of supraventricular tachycardias. Am J Cardiol 1998; 81:725.
- Hamdan MH, Page RL, Wasmund SL, et al. Selective parasympathetic denervation following posteroseptal ablation for either atrioventricular nodal reentrant tachycardia or accessory pathways. Am J Cardiol 2000; 85:875.
- Cox JL, Gallagher JJ, Cain ME. Experience with 118 consecutive patients undergoing operation for the Wolff-Parkinson-White syndrome. J Thorac Cardiovasc Surg 1985; 90:490.
- Lawrie GM, Lin HT, Wyndham CR, DeBakey ME. Surgical treatment of supraventricular arrhythmias. Results in 67 patients. Ann Surg 1987; 205:700.
- Johnson DC, Nunn GR, Richards DA, et al. Surgical therapy for supraventricular tachycardia, a potentially curable disorder. J Thorac Cardiovasc Surg 1987; 93:913.
- Holman WL, Kay GN, Plumb VJ, Epstein AE. Operative results after unsuccessful radiofrequency ablation for Wolff-Parkinson-White syndrome. Am J Cardiol 1992; 70:1490.
- Kim SS, Lal R, Ruffy R. Treatment of paroxysmal reentrant supraventricular tachycardia with flecainide acetate. Am J Cardiol 1986; 58:80.
- Ward DE, Jones S, Shinebourne EA. Use of flecainide acetate for refractory junctional tachycardias in children with the Wolff-Parkinson-White syndrome. Am J Cardiol 1986; 57:787.
- Ludmer PL, McGowan NE, Antman EM, Friedman PL. Efficacy of propafenone in Wolff-Parkinson-White syndrome: electrophysiologic findings and long-term follow-up. J Am Coll Cardiol 1987; 9:1357.
- Musto B, D'Onofrio A, Cavallaro C, Musto A. Electrophysiological effects and clinical efficacy of propafenone in children with recurrent paroxysmal supraventricular tachycardia. Circulation 1988; 78:863.
- Echt DS, Liebson PR, Mitchell LB, et al. Mortality and morbidity in patients receiving encainide, flecainide, or placebo. The Cardiac Arrhythmia Suppression Trial. N Engl J Med 1991; 324:781.
- Rosenbaum MB, Chiale PA, Ryba D, Elizari MV. Control of tachyarrhythmias associated with Wolff-Parkinson-White syndrome by amiodarone hydrochloride. Am J Cardiol 1974; 34:215.
- Wellens HJ, Lie KI, Bär FW, et al. Effect of amiodarone in the Wolff-Parkinson-White syndrome. Am J Cardiol 1976; 38:189.
- Feld GK, Nademanee K, Weiss J, et al. Electrophysiologic basis for the suppression by amiodarone of orthodromic supraventricular tachycardias complicating pre-excitation syndromes. J Am Coll Cardiol 1984; 3:1298.
- Chouty F, Coumel P. Oral flecainide for prophylaxis of paroxysmal atrial fibrillation. Am J Cardiol 1988; 62:35D.
- Antman EM, Beamer AD, Cantillon C, et al. Long-term oral propafenone therapy for suppression of refractory symptomatic atrial fibrillation and atrial flutter. J Am Coll Cardiol 1988; 12:1005.
- Kappenberger LJ, Fromer MA, Steinbrunn W, Shenasa M. Efficacy of amiodarone in the Wolff-Parkinson-White syndrome with rapid ventricular response via accessory pathway during atrial fibrillation. Am J Cardiol 1984; 54:330.
- Feld GK, Nademanee K, Stevenson W, et al. Clinical and electrophysiologic effects of amiodarone in patients with atrial fibrillation complicating the Wolff-Parkinson-White syndrome. Am Heart J 1988; 115:102.
- ACUTE TREATMENT OF SYMPTOMATIC ARRHYTHMIAS
- Initial assessment of hemodynamic stability
- Acute termination of orthodromic AVRT
- - Permanent junctional reciprocating tachycardia
- Acute termination of antidromic AVRT
- Acute treatment of atrial fibrillation with preexcitation
- - Avoidance of AV nodal blockers
- TREATMENT TO PREVENT RECURRENT ARRHYTHMIAS
- Catheter ablation
- - Indications for ablation
- Symptomatic patients
- Asymptomatic patients
- - Localizing the accessory pathway
- - Efficacy
- - Arrhythmia recurrence
- - Complications
- Surgical ablation
- Medical therapy for arrhythmia prevention
- - Prevention of recurrent orthodromic AVRT
- - Prevention of recurrent antidromic AVRT
- - Prevention of recurrent preexcited atrial fibrillation
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- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS