Wolff-Parkinson-White Syndrome Clinical Presentation
- Author: Christopher R Ellis, MD, FACC, FHRS; Chief Editor: Jeffrey N Rottman, MD more...
Patients with Wolff-Parkinson-White (WPW) syndrome may present with anything from mild chest discomfort or palpitations with or without syncope to severe cardiopulmonary compromise or cardiac arrest.
An infant with WPW syndrome may frequently be irritable, may not tolerate feedings, or may demonstrate evidence of congestive heart failure (CHF). Infants often have a history of not behaving as usual for 1-2 days. An intercurrent febrile illness is often observed.
A verbal child with WPW syndrome usually reports chest pain, palpitations, or breathing difficulty. Most children are previously well, and a minority of children have a positive family history of this condition.
Older patients can usually describe the sudden onset of a pounding heartbeat, which is regular and “too rapid to count.” This is typically accompanied by a concomitant change in their tolerance for activity. An irregular rhythm may herald the presence of atrial fibrillation (AF). Occasionally, evidence of disease is discovered on routine electrocardiography (ECG), independent of a concurrent tachydysrhythmia.
In patients with WPW syndrome, the tachycardia that produces symptoms may be a supraventricular tachycardia (SVT), AF, or atrial flutter. In a series of 212 patients with tachyarrhythmias and WPW syndrome, SVT alone occurred in 64%, AF alone occurred in 20%, and both occurred in 16% of patients.
SVT in WPW syndrome may begin in childhood or may not appear clinically until the patient reaches middle age. The clinical course can be unpredictable, as SVT induction depends upon changes in accessory pathway and often AV node EP properties that can vary with time.
SVT due to reentry in WPW is typically orthodromic tachycardia in 95% and antidromic tachycardia in 5% (see Pathophysiology). Orthodromic SVT is usually well tolerated and not a high risk, especially in the pediatric population after young infancy. Antidromic SVT presents more frequently with dizziness and syncope. In addition, it may precipitate ventricular tachycardia and ventricular fibrillation (VF).
Light-headedness and near syncope appear to occur more commonly in persons with WPW syndrome who have paroxysmal SVT (PSVT) or atrial fibrillation than in those with atrioventricular (AV) nodal reentry.
Syncope can occur because of inadequate cerebral circulation due to a rapid ventricular rate or because the tachyarrhythmia is depressing the sinus pacemaker, causing a period of asystole at the point of tachycardia termination.
PSVT can be followed after termination by polyuria, which is due to atrial dilatation and release of atrial natriuretic factor.
Wolff-Parkinson-White (WPW) syndrome has no specific examination features except for those that may accompany symptomatic dysrhythmias. The vast majority of WPW patients have normal cardiac examination findings.
Many young patients appear may present with resting tachycardia on physical examination, with only minimal symptoms (eg, palpitations, weakness, mild dizziness) despite exceedingly fast heart rates. Upon physical examination, the patient may be cool, diaphoretic, and hypotensive. Crackles in the lungs are common because the rapid heart rate may cause pulmonary vascular congestion due to congestive heart failure (CHF).
During supraventricular tachycardia (SVT), the rhythm is unvarying and regular, with constant intensity of the first heart sound. The jugular venous pressure can be elevated, but the waveform generally remains constant.
An infant experiencing an episode of SVT is usually tachypneic and irritable; pallor is common. The pulse is very rapid and diminished in volume. The ventricular rate typically is 200-250 bpm, and the blood pressure is decreased. If the episode has been untreated for several hours, the patient often has poor perfusion, hepatomegaly, and cardiac failure. The child is usually anxious but hemodynamically stable. Tachypnea often accompanies the tachycardia.
Once the arrhythmia has been terminated, the physical examination findings are generally normal.
Clinical features of associated cardiac defects may be present, such as the following:
In the presence of congenital heart defects (CHDs) or cardiomyopathy, findings of the underlying condition often become apparent only after the SVT has been terminated, although the hemodynamic consequences may be poorly tolerated.
In several series, the incidence of associated congenital heart disease is reported to be as high as 30%, most commonly Ebstein anomaly of the tricuspid valve and corrected transposition of the great arteries.
Approximately 10% of patients with Ebstein anomaly of the tricuspid valve have WPW syndrome. They usually have more than 1 accessory pathway (AP), and those are usually on the right side. Patients with corrected transposition of the great arteries and left-side Ebstein anomaly may also have WPW syndrome. In these patients, the AP is on the left side or septal.
Patients with Ebstein anomaly of the tricuspid valve may present with cyanosis, tachypnea, and other signs of congestive heart failure in presence of a rapid heart rate. The electrocardiogram (ECG) may show either wide or narrow QRS, SVT, and, sometimes, QRS with changing morphology if more than one AP is present. Patients with right-side accessory pathways should be screened for the Ebstein anomaly by echocardiography.
Patients with glycogen-storage diseases have muscle weakness with normal or increased muscle bulk, macroglossia and hepatomegaly in the case of Pompe disease, and mental retardation in case of Danon disease.
Other congenital heart diseases associated with WPW syndrome include atrial and ventricular septal defects and coronary sinus diverticula.
The abnormal QRS complexes of WPW syndrome, when present, may appear similar to those observed in acute myocardial infarction (MI), left ventricular hypertrophy (LVH), and hypertrophic cardiomyopathy. Repolarization abnormalities are common in patients with WPW syndrome, and thus, acute MI and LVH cannot be diagnosed if a delta wave is present.
Wolff L, Parkinson J, White PD. Bundle-branch block with short P-R interval in healthy young people prone to paroxysmal tachycardia. Am Heart J. 1930 Aug. 5(6):685-704. [Full Text].
Gollob MH, Green MS, Tang AS, et al. Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med. 2001 Jun 14. 344(24):1823-31. [Medline].
Calkins H, Kuck KH, Cappato R, et al, for the Heart Rhythm Society Task Force on Catheter and Surgical Ablation of Atrial Fibrillation. 2012 HRS/EHRA/ECAS expert consensus statement on catheter and surgical ablation of atrial fibrillation: recommendations for patient selection, procedural techniques, patient management and follow-up, definitions, endpoints, and research trial design: a report of the Heart Rhythm Society (HRS) Task Force on Catheter and Surgical Ablation of Atrial Fibrillation. Developed in partnership with the European Heart Rhythm Association (EHRA), a registered branch of the European Society of Cardiology ... Heart Rhythm. 2012 Apr. 9 (4):632-696.e21. [Medline].
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 Jun 6. 324(23):1612-8. [Medline].
Sarubbi B, D'Alto M, Vergara P, et al. Electrophysiological evaluation of asymptomatic ventricular pre-excitation in children and adolescents. Int J Cardiol. 2005 Feb 15. 98(2):207-14. [Medline].
Durrer D, Schuilenburg RM, Wellens HJ. Pre-excitation revisited. Am J Cardiol. 1970 Jun. 25(6):690-7. [Medline].
Pappone C, Vicedomini G, Manguso F, et al. Risk of malignant arrhythmias in initially symptomatic patients with Wolff-Parkinson-White syndrome: results of a prospective long-term electrophysiological follow-up study. Circulation. 2012 Feb 7. 125(5):661-8. [Medline].
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 Nov 6. 349(19):1803-11. [Medline].
Sethi KK, Dhall A, Chadha DS, Garg S, Malani SK, Mathew OP. WPW and preexcitation syndromes. J Assoc Physicians India. 2007 Apr. 55 Suppl:10-5. [Medline].
Katritsis DG, Camm AJ. Atrioventricular nodal reentrant tachycardia. Circulation. 2010 Aug 24. 122 (8):831-40. [Medline].
Stroobandt RX, Barold SS, Sinnaeve AF. Preexcitation and Wolff-Parkinson-White Syndrome (WPW). ECG from Basics to Essentials: Step by Step. Chichester, West Sussex, UK: John Wiley & Sons, Ltd; 2016. chapter 17.
Khairy P, Van Hare GF, Balaji S, et al. PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease: 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 (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Societ... Heart Rhythm. 2014 Oct. 11 (10):e102-65. [Medline].
Ehtisham J, Watkins H. Is Wolff-Parkinson-White syndrome a genetic disease?. J Cardiovasc Electrophysiol. 2005 Nov. 16(11):1258-62. [Medline].
Bowles NE, Jou CJ, Arrington CB, et al for the Baylor Hopkins Centers for Mendelian Genomics. Exome analysis of a family with Wolff-Parkinson-White syndrome identifies a novel disease locus. Am J Med Genet A. 2015 Aug 18. [Medline].
Brembilla-Perrot B, Yangni N'da O, Huttin O, et al. Wolff-Parkinson-White syndrome in the elderly: clinical and electrophysiological findings. Arch Cardiovasc Dis. 2008 Jan. 101(1):18-22. [Medline].
Pappone C, Santinelli V. Electrophysiology testing and catheter ablation are helpful when evaluating asymptomatic patients with Wolff-Parkinson-White pattern: the pro perspective. Card Electrophysiol Clin. 2015 Sep. 7 (3):371-6. [Medline].
Pediatric and Congenital Electrophysiology Society (PACES), Heart Rhythm Society (HRS), American College of Cardiology Foundation (ACCF), American Heart Association (AHA), American Academy of Pediatrics (AAP), Canadian Heart Rhythm Society (CHRS). 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), an... Heart Rhythm. 2012 Jun. 9 (6):1006-24. [Medline].
Zhang Y, Wang L. Atrial vulnerability is a major mechanism of paroxysmal atrial fibrillation in patients with Wolff-Parkinson-White syndrome. Med Hypotheses. 2006. 67(6):1345-7. [Medline].
Deneke T, Mügge A. Atrial fibrillation and Wolff-Parkinson-White syndrome: mechanisms revisited?. J Cardiovasc Electrophysiol. 2012 Mar. 23 (3):287-9. [Medline].
Bunch TJ, May HT, Bair TL, et al. Long-term natural history of adult Wolff-Parkinson-White syndrome patients treated with and without catheter ablation. Circ Arrhythm Electrophysiol. 2015 Oct 19. [Medline].
Szumowski L, Walczak F, Urbanek P, et al. Risk factors of atrial fibrillation in patients with Wolff-Parkinson-White syndrome. Kardiol Pol. 2004 Mar. 60(3):206-16; discussion 217. [Medline].
Bromberg BI, Lindsay BD, Cain ME, Cox JL. Impact of clinical history and electrophysiologic characterization of accessory pathways on management strategies to reduce sudden death among children with Wolff-Parkinson-White syndrome. J Am Coll Cardiol. 1996 Mar 1. 27(3):690-5. [Medline].
Attoyan C, Haissaguerre M, Dartigues JF, Le Metayer P, Warin JF, Clementy J. [Ventricular fibrillation in Wolff-Parkinson-White syndrome. Predictive factors]. Arch Mal Coeur Vaiss. 1994 Jul. 87(7):889-97. [Medline].
Kiger ME, McCanta AC, Tong S, Schaffer M, Runciman M, Collins KK. Intermittent versus Persistent Wolff-Parkinson-White Syndrome in Children: Electrophysiologic Properties and Clinical Outcomes. Pacing Clin Electrophysiol. 2015 Aug 8. [Medline].
Shah A, Hocini M, Haissaguerre M, Jais P. Non-invasive mapping of cardiac arrhythmias. Curr Cardiol Rep. 2015 Aug. 17 (8):60. [Medline].
Mark DG, Brady WJ, Pines JM. Preexcitation syndromes: diagnostic consideration in the ED. Am J Emerg Med. 2009 Sep. 27(7):878-88. [Medline].
Fengler BT, Brady WJ, Plautz CU. Atrial fibrillation in the Wolff-Parkinson-White syndrome: ECG recognition and treatment in the ED. Am J Emerg Med. 2007 Jun. 25(5):576-83. [Medline].
Arruda MS, McClelland JH, Wang X, et al. Development and validation of an ECG algorithm for identifying accessory pathway ablation site in Wolff-Parkinson-White syndrome. J Cardiovasc Electrophysiol. 1998 Jan. 9(1):2-12. [Medline].
Ghosh S, Avari JN, Rhee EK, Woodard PK, Rudy Y. Hypertrophic cardiomyopathy with preexcitation: insights from noninvasive electrocardiographic imaging (ECGI) and catheter mapping. J Cardiovasc Electrophysiol. 2008 Nov. 19(11):1215-7. [Medline]. [Full Text].
Pappone C, Manguso F, Santinelli R, et al. Radiofrequency ablation in children with asymptomatic Wolff-Parkinson-White syndrome. N Engl J Med. 2004 Sep 16. 351(12):1197-205. [Medline].
Skanes AC, Obeyesekere M, Klein GJ. Electrophysiology testing and catheter ablation are helpful when evaluating asymptomatic patients with Wolff-Parkinson-White pattern: the con perspective. Card Electrophysiol Clin. 2015 Sep. 7 (3):377-83. [Medline].
Duszanska A, Lenarczyk R, Kowalski O, Streb W, Kukulski T, Kalarus Z. Evaluation of left ventricular systolic and diastolic function in patients with atrioventricular re-entrant tachycardia treated by radiofrequency current ablation. Acta Cardiol. 2008 Apr. 63(2):221-7. [Medline].
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 Jun 6. 324(23):1605-11. [Medline].
Pappone C, Radinovic A, Santinelli V. Sudden death and ventricular preexcitation: is it necessary to treat the asymptomatic patients?. Curr Pharm Des. 2008. 14(8):762-5. [Medline].
Capone CA, Ceresnak SR, Nappo L, Gates GJ, Schechter CB, Pass RH. Three-catheter technique for ablation of left-sided accessory pathways in Wolff-Parkinson-White is less expensive and equally successful when compared to a five-catheter technique. Pacing Clin Electrophysiol. 2015 Aug 27. [Medline].
Gulletta S, Tsiachris D, Radinovic A, et al. Safety and efficacy of open irrigated-tip catheter ablation of Wolff-Parkinson-White syndrome in children and adolescents. Pacing Clin Electrophysiol. 2013 Apr. 36(4):486-90. [Medline].
Karakoyun S, Tanboga IH, Gokdeniz T, et al. Assessment of left atrial mechanics in patients with preexcitation syndrome scheduled for catheter ablation. Echocardiography. 2015 Aug 24. [Medline].
Maden O, Balci KG, Selcuk MT, et al. Comparison of the accuracy of three algorithms in predicting accessory pathways among adult Wolff-Parkinson-White syndrome patients. J Interv Card Electrophysiol. 2015 Dec. 44 (3):213-9. [Medline].