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Wolff- Parkinson-White Syndrome
Transcript of Wolff- Parkinson-White Syndrome
Sudden death in young competitive athlete...
Wolf-Parkinson- White Syndrome
1. Wiedermann CJ, Becker AE, Hopferwieser T, Mühlberger V, Knapp E. Sudden death in a young competitive athlete with Wolf—Parkinson—White syndrome. European Heart Journal. 1987 Jun 1;8(6):651-5.
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3. Gollob MH, Green MS, Tang AS, Gollob T, Karibe A, Hassan AS, Ahmad F, Lozado R, Shah G, Fananapazir L, Bachinski LL. Identification of a gene responsible for familial Wolff–Parkinson–White syndrome. New England Journal of Medicine. 2001 Jun 14;344(24):1823-31.
4. Medscape[Internet]. Wolff-Parkinson-White Syndrome Treatment & Management. Available from: http://emedicine.medscape.com/article/159222-treatment#d1
5. Lu CW, Wu MH, Chen HC, Kao FY, Huang SK. Epidemiological profile of Wolff–Parkinson–White syndrome in a general population younger than 50years of age in an era of radiofrequency catheter ablation. International journal of cardiology. 2014 Jul 1;174(3):530-4.
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Catheter ablation of the accessory atria-ventricular pathways by radio-frequency current.
Pharmacotherapy includes antiarrhythmic agents for example: Beta-blockers, Flecainide, Propafenone, Amiodarone etc.
Digoxin and Verapamil appears to be contraindicated for many patients with Wolff-Parkinson-White syndrome because it may enhance antegrade conduction through the accessory pathway.
Why is WPW syndrome such a big deal?
A young athlete suddenly died after being discharged ouf of hospital 10 days earlier. He was admitted earlier for rapid heart beating and left the hospital in a stable condition.
His history stated that he was asymptomatic and occasionally experienced tachycardias.
His ECG showed a short PR interval as well as a Delta wave pattern in the QRS-complex.
Conducting tissue in the heart
Bundle of His
Left and right bundle
WPW syndrome is a birth defect where an accessory conducting pathway is formed.
In a normal heart electrical impulses flow from the SA node - AV node- Bundle of His- L/R bundle branches - Purkinje fibres.
But due to the accessory pathway a short circuit is is created which can exclude the AV node and lead to the pre-excitation of the ventricles.
Wolff-Parkinson-White Syndroem can lead to:
Sudden cardiac death
There are three different types known.
WPW: Sinus rhythm.
The accessory pathway and the AV node both depolarizes the ventricles - thus there is a sinus (regular) heart beat. Although pre-excitation via the accessory band can create the very distinct ECG pattern: a short PR interval and the delta wave in the QRS-complex.
WPW: Orthodromic Tachycardia:
The electrical impulse moves in the normal direction through the AV node but uses the accessory pathway to return to the atria. This can lead to tachycardia.
WPW Atrial Fibrillation:
Ventricles are mainly depolarized via the accessory pathway. This is very dangerous as the accessory pathway does not posses rate-limiting properties. And can lead to tachycardia, syncope, collapse and death.
The prevalence of WPW is 0.68-1.7/1000 globally, but there is a 1.5-3.1/1000 prevalence in Western Countries.
There is a peak in the ages 20-24 years.
The prevalence of Sudden Cardiac Death in symptomatic patients is 0.25% per year or a 3-4% in a lifetime.
Main research and development issues raised by WPW.
Research about treatment options is difficult because some patients' first presentation of WPW syndrome is ventricular fibrillation or Sudden Cardiac Death.
50% of patients with WPW syndrome is asymptomatic.
The ECG pattern varies from person to person and may even appear normal in some individuals.
WPW syndrome is a rare congenital heart disorder and thus not much research could have been done.
The genetic cause of WPW is not yet well understood.