Epidemiology, clinical manifestations, and diagnosis of 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
In 1930, Louis Wolff, Sir John Parkinson, and Paul Dudley White published a seminal article describing 11 patients who suffered from attacks of tachycardia associated with a sinus rhythm electrocardiographic (ECG) pattern of bundle branch block with a short PR interval . This was subsequently termed the Wolff-Parkinson-White (WPW) syndrome, although earlier isolated case reports describing similar patients had been published. In 1943, the ECG features of preexcitation were correlated with anatomic evidence for the existence of anomalous bundles of conducting tissue that bypassed all or part of the normal atrioventricular (AV) conduction system (figure 1).
This topic will review the definition and prevalence of the WPW syndrome. The treatment options for patients with tachyarrhythmias and the WPW syndrome are discussed separately. (See "Treatment of symptomatic arrhythmias associated with the Wolff-Parkinson-White syndrome".)
Normal AV conduction versus accessory AV pathway conduction — In the normal heart, the atria and the ventricles are electrically isolated, with conduction of electrical impulses from the atria to the ventricles normally occurring via the atrioventricular node (AV) and the His-Purkinje system. Patients with a preexcitation syndrome have an additional pathway, known as an accessory pathway (AP), which directly connects the atria and ventricles, thereby allowing electrical activity to bypass the AV node (table 1). Tissue in the accessory pathways, which are congenital in origin and result from failure of resorption of the myocardial syncytium at the annulus fibrosis of the atrioventricular valves during fetal development, typically conducts electrical impulses more quickly than the AV node, resulting in the shorter PR interval seen on the surface ECG. (See 'Electrocardiographic (ECG) findings' below and "Lown-Ganong-Levine syndrome and enhanced atrioventricular nodal conduction" and "Mahaim fiber tachycardias".)
It has been estimated that most accessory pathways (60 to 75 percent) are capable of bidirectional conduction (anterograde and retrograde) between the atrium and ventricle. However, some accessory pathways (17 to 37 percent) are only capable of conduction in a retrograde fashion from ventricle to atrium . When accessory pathways conduct exclusively in the retrograde direction (so-called "concealed" accessory pathways), they do not generate a delta wave and the WPW pattern on the surface ECG but are still capable of supporting reentrant tachycardia. Retrograde conduction can occur following ventricular pacing or premature beats, and it can form the retrograde arm of an orthodromic atrioventricular reentrant tachycardia (AVRT) circuit. The vast majority of concealed accessory pathways are left-sided . (See 'Anatomy' below and "Atrioventricular reentrant tachycardia (AVRT) associated with an accessory pathway", section on 'Orthodromic AVRT'.)
Less commonly (5 to 27 percent), an accessory pathway is only capable of conduction in the anterograde direction; in such cases, it can form the antegrade arm of an antidromic atrioventricular reentrant tachycardia (AVRT) circuit. The mechanism responsible for unidirectional conduction along an accessory pathway (anterograde only or retrograde only) remains undetermined. (See "Atrioventricular reentrant tachycardia (AVRT) associated with an accessory pathway", section on 'Antidromic AVRT'.)
- Wolff L, Parkinson J, White PD. Bundle-branch block with short P-R interval in healthy young people prone to paroxysmal tachycardia. 1930. Ann Noninvasive Electrocardiol 2006; 11:340.
- Miller JM. Therapy of Wolff-Parkinson-White syndrome and concealed bypass tracts: Part I. J Cardiovasc Electrophysiol 1996; 7:85.
- Kuck KH, Friday KJ, Kunze KP, et al. Sites of conduction block in accessory atrioventricular pathways. Basis for concealed accessory pathways. Circulation 1990; 82:407.
- Colavita PG, Packer DL, Pressley JC, et al. Frequency, diagnosis and clinical characteristics of patients with multiple accessory atrioventricular pathways. Am J Cardiol 1987; 59:601.
- Zachariah JP, Walsh EP, Triedman JK, et al. Multiple accessory pathways in the young: the impact of structural heart disease. Am Heart J 2013; 165:87.
- Vidaillet HJ Jr, Pressley JC, Henke E, et al. Familial occurrence of accessory atrioventricular pathways (preexcitation syndrome). N Engl J Med 1987; 317:65.
- Milstein S, Sharma AD, Guiraudon GM, Klein GJ. An algorithm for the electrocardiographic localization of accessory pathways in the Wolff-Parkinson-White syndrome. Pacing Clin Electrophysiol 1987; 10:555.
- Fitzpatrick AP, Gonzales RP, Lesh MD, et al. New algorithm for the localization of accessory atrioventricular connections using a baseline electrocardiogram. J Am Coll Cardiol 1994; 23:107.
- Reddy GV, Schamroth L. The localization of bypass tracts in the Wolff-Parkinson-White syndrome from the surface electrocardiogram. Am Heart J 1987; 113:984.
- Epstein AE, Kirklin JK, Holman WL, et al. Intermediate septal accessory pathways: electrocardiographic characteristics, electrophysiologic observations and their surgical implications. J Am Coll Cardiol 1991; 17:1570.
- 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.
- Deal BJ, Keane JF, Gillette PC, Garson A Jr. Wolff-Parkinson-White syndrome and supraventricular tachycardia during infancy: management and follow-up. J Am Coll Cardiol 1985; 5:130.
- SCHIEBLER GL, ADAMS P Jr, ANDERSON RC. The Wolff-Parkinson-White syndrome in infants and children. A review and a report of 28 cases. Pediatrics 1959; 24:585.
- LEV M, GIBSON S, MILLER RA. Ebstein's disease with Wolff-Parkinson-White syndrome; report of a case with a histopathologic study of possible conduction pathways. Am Heart J 1955; 49:724.
- Cappato R, Schlüter M, Weiss C, et al. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly. Circulation 1996; 94:376.
- Attenhofer Jost CH, Connolly HM, O'Leary PW, et al. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 2005; 80:361.
- Gallagher JJ, Pritchett EL, Sealy WC, et al. The preexcitation syndromes. Prog Cardiovasc Dis 1978; 20:285.
- Josephson ME. Preexcitation syndromes. In: Clinical Cardiac Electrophysiology, 4th, Lippincot Williams & Wilkins, Philadelphia 2008. p.339.
- Krahn AD, Manfreda J, Tate RB, et al. The natural history of electrocardiographic preexcitation in men. The Manitoba Follow-up Study. Ann Intern Med 1992; 116:456.
- Kobza R, Toggweiler S, Dillier R, et al. Prevalence of preexcitation in a young population of male Swiss conscripts. Pacing Clin Electrophysiol 2011; 34:949.
- Munger TM, Packer DL, Hammill SC, et al. A population study of the natural history of Wolff-Parkinson-White syndrome in Olmsted County, Minnesota, 1953-1989. Circulation 1993; 87:866.
- Klein GJ, Yee R, Sharma AD. Longitudinal electrophysiologic assessment of asymptomatic patients with the Wolff-Parkinson-White electrocardiographic pattern. N Engl J Med 1989; 320:1229.
- Mah DY, Sherwin ED, Alexander ME, et al. The electrophysiological characteristics of accessory pathways in pediatric patients with intermittent preexcitation. Pacing Clin Electrophysiol 2013; 36:1117.
- Kiger ME, McCanta AC, Tong S, et al. Intermittent versus Persistent Wolff-Parkinson-White Syndrome in Children: Electrophysiologic Properties and Clinical Outcomes. Pacing Clin Electrophysiol 2016; 39:14.
- Klein GJ, Gulamhusein SS. Intermittent preexcitation in the Wolff-Parkinson-White syndrome. Am J Cardiol 1983; 52:292.
- SMITH RF. THE WOLFF-PARKINSON-WHITE SYNDROME AS AN AVIATION RISK. Circulation 1964; 29:672.
- Fitzsimmons PJ, McWhirter PD, Peterson DW, Kruyer WB. The natural history of Wolff-Parkinson-White syndrome in 228 military aviators: a long-term follow-up of 22 years. Am Heart J 2001; 142:530.
- Chiu SN, Wang JK, Wu MH, et al. Cardiac conduction disturbance detected in a pediatric population. J Pediatr 2008; 152:85.
- Obeyesekere MN, Leong-Sit P, Massel D, et al. Risk of arrhythmia and sudden death in patients with asymptomatic preexcitation: a meta-analysis. Circulation 2012; 125:2308.
- Leitch JW, Klein GJ, Yee R, Murdock C. Prognostic value of electrophysiology testing in asymptomatic patients with Wolff-Parkinson-White pattern. Circulation 1990; 82:1718.
- 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, Santinelli V, Rosanio S, et al. Usefulness of invasive electrophysiologic testing to stratify the risk of arrhythmic events in asymptomatic patients with Wolff-Parkinson-White pattern: results from a large prospective long-term follow-up study. J Am Coll Cardiol 2003; 41:239.
- Chen SA, Chiang CE, Tai CT, et al. Longitudinal clinical and electrophysiological assessment of patients with symptomatic Wolff-Parkinson-White syndrome and atrioventricular node reentrant tachycardia. Circulation 1996; 93:2023.
- 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.
- 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.
- Farshidi A, Josephson ME, Horowitz LN. Electrophysiologic characteristics of concealed bypass tracts: clinical and electrocardiographic correlates. Am J Cardiol 1978; 41:1052.
- Massumi RA. Familial Wolff-Parkinson-White syndrome with cardiomyopathy. Am J Med 1967; 43:931.
- Gollob MH, Green MS, Tang AS, et al. Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med 2001; 344:1823.
- Gollob MH, Seger JJ, Gollob TN, et al. Novel PRKAG2 mutation responsible for the genetic syndrome of ventricular preexcitation and conduction system disease with childhood onset and absence of cardiac hypertrophy. Circulation 2001; 104:3030.
- Campbell RW, Smith RA, Gallagher JJ, et al. Atrial fibrillation in the preexcitation syndrome. Am J Cardiol 1977; 40:514.
- Sharma AD, Klein GJ, Guiraudon GM, Milstein S. Atrial fibrillation in patients with Wolff-Parkinson-White syndrome: incidence after surgical ablation of the accessory pathway. Circulation 1985; 72:161.
- Wellens HJ, Durrer D. Wolff-Parkinson-White syndrome and atrial fibrillation. Relation between refractory period of accessory pathway and ventricular rate during atrial fibrillation. Am J Cardiol 1974; 34:777.
- Sung RJ, Castellanos A, Mallon SM, et al. Mechanisms of spontaneous alternation between reciprocating tachycardia and atrial flutter-fibrillation in the Wolff-Parkinson-White syndrome. Circulation 1977; 56:409.
- Fujimura O, Klein GJ, Yee R, Sharma AD. Mode of onset of atrial fibrillation in the Wolff-Parkinson-White syndrome: how important is the accessory pathway? J Am Coll Cardiol 1990; 15:1082.
- Timmermans C, Smeets JL, Rodriguez LM, et al. Aborted sudden death in the Wolff-Parkinson-White syndrome. Am J Cardiol 1995; 76:492.
- Klein GJ, Bashore TM, Sellers TD, et al. Ventricular fibrillation in the Wolff-Parkinson-White syndrome. N Engl J Med 1979; 301:1080.
- Oren JW 4th, Beckman KJ, McClelland JH, et al. A functional approach to the preexcitation syndromes. Cardiol Clin 1993; 11:121.
- Montoya PT, Brugada P, Smeets J, et al. Ventricular fibrillation in the Wolff-Parkinson-White syndrome. Eur Heart J 1991; 12:144.
- 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.
- Yee, R, Klein, GJ, Sharma, AD, et, al. Tachycardia associated with accessory atrioventricular pathways. In: Cardiac Electrophysiology, Zipes, DP, Jalife, J (Eds), WB Saunders, Philadelphia 1990. p.463.
- Wang K, Asinger R, Hodges M. Electrocardiograms of Wolff-Parkinson-White syndrome simulating other conditions. Am Heart J 1996; 132:152.
- Sternick EB. Familial pseudo-WPW syndrome. J Cardiovasc Electrophysiol 2009; 20:E62; author reply E63.
- 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.
- Mehta D, Wafa S, Ward DE, Camm AJ. Relative efficacy of various physical manoeuvres in the termination of junctional tachycardia. Lancet 1988; 1:1181.
- Pappone C, Santinelli V, Manguso F, et al. A randomized study of prophylactic catheter ablation in asymptomatic patients with the Wolff-Parkinson-White syndrome. N Engl J Med 2003; 349:1803.
- Al-Khatib SM, Arshad A, Balk EM, et al. Risk Stratification for Arrhythmic Events in Patients With Asymptomatic Pre-Excitation: A Systematic Review for the 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:e575.
- Normal AV conduction versus accessory AV pathway conduction
- WPW pattern versus WPW syndrome
- Accessory pathway location
- Associated cardiac abnormalities
- Prevalence of WPW pattern
- Prevalence of WPW syndrome
- Prevalence of concealed accessory pathways
- FAMILIAL WPW
- CLINICAL MANIFESTATIONS
- Arrhythmias associated with WPW
- - Tachycardias requiring an accessory pathway for initiation and maintenance
- - Tachycardias not requiring an accessory pathway for initiation and maintenance
- Atrioventricular nodal reentrant tachycardia
- Atrial fibrillation
- Atrial flutter
- Ventricular tachycardia
- Ventricular fibrillation and sudden death
- Electrocardiographic (ECG) findings
- - WPW pattern on ECG
- - WPW pattern and ECG interpretation for other disorders
- Electrophysiology testing
- DIFFERENTIAL DIAGNOSIS
- Differential diagnosis of ECG findings
- Differential diagnosis of supraventricular tachycardia
- RISK STRATIFICATION OF ASYMPTOMATIC PATIENTS WITH WPW PATTERN
- Mechanism of and risk factors for SCD in WPW
- Approach to risk stratification
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS