Wolff-Parkinson-White syndrome

Wolff-Parkinson-White syndrome (WPW) is a syndrome of pre-excitation of the ventricles due to an accessory pathway known as the bundle of Kent. This accessory pathway is an abnormal electrical communication from the atria to the ventricles.

The incidence of WPW syndrome is between 0.1 and 3 percent of the general population.^1-3 While the vast majority of individuals with WPW syndrome remain asymptomatic throughout their entire lives, there is a risk of sudden death associate with WPW syndrome. Sudden death due to WPW syndrome is rare (incidence of ≤0.6%^3,4), and is due to the effect of the accessory pathway on tachyarrhythmias in these individuals.

Contents

1 Pathophysiology 2 Diagnosis 3 Risk stratification 4 Treatment 5 Related topics 6 References

Pathophysiology

In normal individuals, electrical activity in the heart is initiated in the SA node (located in the right atrium), propogates to the AV node, and then through the bundle of His to the ventricles of the heart. (See electrical conduction system of the heart).

The AV node acts as a gatekeeper, limiting the electrical activity that reaches the ventricles of the heart. This is an important function of the AV node, because if the signals generated in the atria of the heart were to increase in rate (such as during atrial fibrillation or atrial flutter), the AV node will limit the electrical activity that conducts to the ventricles. For instance, if the atria are electrically activated at 300 beats per minute, half those electrical impulses are blocked by the AV node, so that the ventricles are activated at 150 beats per minute (giving a pulse of 150 beats per minute). Another important property of the AV node is that it slows down individual electrical impulses. This is manifest on the EKG as the PR interval, the time from activation of the atria (manifest as the P wave) and activation of the ventricles (manifest as the QRS complex).

Individuals with WPW syndrome have an accessory pathway that connects the atria and the ventricles, in addition to the AV node. This accessory pathway is known as the bundle of Kent. This accessory pathway does not share the rate-slowing properties of the AV node, and may conduct electrical activity at a significantly higher rate than the AV node. For instance, in the example above, if an individual had an atrial rate of 300 beats per minute, the accessory bundle may conduct all the electrical impulses from the atria to the ventricles, causing the ventricles to activate at 300 beats per minute. The ventricles are not capable of activating in a uniform manner at rates that fast and will fibrillate instead (ventricular fibrillation). If not corrected rapidly, ventricular fibrillation leads to sudden cardiac death (SCD).

Diagnosis

Missing image WPW_EKG_leadV2.png EKG changes with Wolff-Parkinson-White syndrome.

One beat from lead V2 demonstrating characteristic findings in WPW symdrome. Note the short PR interval of 0.10 seconds (red bar) and the long QRS complex at 0.16 seconds (blue and green bars). The blue bar represents the slurred upstroke of the QRS complex known as the delta wave.

WPW syndrome is commonly diagnosed on the basis of the surface EKG in an asymptomatic individual. In this case it is manifest as a delta wave, which is a slurred upstroke in the QRS complex that is associated with a short PR interval. WPW syndrome may also be noted on an EKG of an individual in cardiac arrest. In this case, the EKG will show a wide-complex tachycardia with widely varying morphologies of the QRS complexes with varying rates. This EKG finding is due to atrial fibrillation with rapid conduction down the accessory pathway.

When an individual is in normal sinus rhythm, the EKG characteristics of WPW syndrome are a short PR interval, widened QRS complex (greater than 120 ms in length) with slurred upstroke of the QRS complex, and secondary repolarization changes reflected in ST segment-T wave changes.

In individuals with WPW syndrome, electrical activity that is initiated in the SA node travels through the accessory pathway as well as through the AV node to activate the ventricles via both pathways. Since the accessory pathway does not have the impulse slowing properties of the AV node, the electrical impulse first activates the ventricles via the accessory pathway, and immediately afterwards via the AV node. This gives the short PR interval and slurred upstroke to the QRS complex known as the delta wave.

Patients with WPW often exhibit more than one accessory pathway, and in some patients as many as eight additional abnormal pathways can be found. This has been seen in individuals with Ebstein's anomaly.

Wolff-Parkinson-White syndrome is sometimes associated with Leber's hereditary optic neuropathy (LHON), a form of mitochondrial disease.⁵

Risk stratification

Missing image Wolff-Parkinson-White_syndrome_12_lead_EKG.png 12 lead EKG of individual with Wolff-Parkinson-White syndrome.

12 lead EKG of an individual with WPW syndrome. The accessory pathway is located in the left posteroseptal region.

Treatment is based on risk stratification of the individual. Risk stratification is performed to determine which individuals with WPW syndrome are at risk for sudden cardiac death (SCD). Sudden cardiac death in these individuals is due to the propogation of an atrial arrhythmia to the ventricles at a very high rate.

A good history should be taken to determine whether an individual has factors suggestive of a previous episode of unexplained syncope or palpitations. These may be due to earlier episodes of a tachycardia associated with the accessory pathway.

Individuals with WPW syndrome in whome the delta waves disappear with increases in the heart rate are considered at lower risk of SCD. This is because the loss of the delta wave shows that the accessory pathway cannot conduct electrical impulses at a high rate (in the anterograde direction). These individuals will typically not have fast conduction down the accessory pathway during episodes of atrial fibrillation.

Risk stratification is best performed via programmed electrical stimulation (PES) in the cardiac electrophysiology lab. This is an invasive procedure, in which the rate of impulse propogation via the accessory pathway is determined by stimulating the atria and by inducing transient atrial fibrillation.

High risk features that may be present during PES include an effective refractory period of the accessory pathway less than 270 ms, multiple pathways, septal location of pathway, and inducibility of supraventricular tachycardia. Individuals with any of these high risk features are generally considered at increased risk for SCD and should be treated accordingly.⁶

It is unclear whether invasive risk stratification (with programmed electrical stimulation) is necessary in the asymptomatic individual.⁷ While some groups advocate PES for risk stratification in all individuals under 35 years old, others only offer it to individuals who have history suggestive of a tachyarrhythmia, since the incidence of sudden death is so low^3,4.

Treatment

The definitive treatment of WPW syndrome is destruction of the abnormal electrical pathway by radiofrequency catheter ablation. This procedure is performed almost exclusively by cardiac electrophysiologists. Radiofrequency catheter ablation is not performed in all individuals with WPW syndrome because there are inherent risks involved in the procedure.

When performed by an experienced electrophysiologist, radiofrequency ablation has a high success rate.⁸ If radiofrequency catheter ablation is successfully performed, the patient is generally considered cured. Recurrence rates are typically less than 5 percent after a successful ablation.⁸ The one caveat is that individuals with underlying Ebstein's anomaly may develop additional accessory pathways during progression of their disease.

Related topics

• Cardiac electrophysiology
• Electrical conduction system of the heart
• Electrocardiogram
• Electrophysiologic study

References

1. Rosner MH, Brady WJ Jr, Kefer MP, Martin ML. Electrocardiography in the patient with the Wolff-Parkinson-White syndrome: diagnostic and initial therapeutic issues. Am J Emerg Med. 1999 Nov;17(7):705-14. (Medline abstract (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10597097))

2. Sorbo MD, Buja GF, Miorelli M, Nistri S, Perrone C, Manca S, Grasso F, Giordano GM, Nava A. The prevalence of the Wolff-Parkinson-White syndrome in a population of 116,542 young males. G Ital Cardiol. 1995 Jun;25(6):681-7. (Medline abstract (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7649416))

3. Munger TM, Packer DL, Hammill SC, Feldman BJ, Bailey KR, Ballard DJ, Holmes DR Jr, Gersh BJ. A population study of the natural history of Wolff-Parkinson-White syndrome in Olmsted County, Minnesota, 1953-1989. Circulation. 1993 Mar;87(3):866-73. (Medline abstract (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8443907))

4. 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 Sep;142(3):530-6. (Medline abstract (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11526369))

5. Mashima Y, Kigasawa K, Hasegawa H, Tani M, Oguchi Y. High incidence of pre-excitation syndrome in Japanese families with Leber's hereditary optic neuropathy. Clin Genet. 1996 Dec;50(6):535-7. (Medline abstract (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9147893))

6. Pappone C, Santinelli V, Manguso F, Augello G, Santinelli O, Vicedomini G, Gulletta S, Mazzone P, Tortoriello V, Pappone A, Dicandia C, Rosanio S. 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 reference (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=14602878))

7. Campbell RM, Strieper MJ, Frias PA, Collins KK, Van Hare GF, Dubin AM. Survey of current practice of pediatric electrophysiologists for asymptomatic Wolff-Parkinson-White syndrome. Pediatrics. 2003 Mar;111(3):e245-7. (Medline abstract (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12612279))

8. Pappone C, Lamberti F, Santomauro M, Stabile G, De Simone A, Turco P, Pannain S, Loricchio ML, Rotunno R, Chiariello M. Ablation of paroxysmal tachycardia in Wolff-Parkinson-White syndrome. Cardiologia. 1993 Dec;38(12 Suppl 1):189-97. (Medline abstract (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8020017))