04/05/03

 

 

 

 

 

 

 

   

Contents | Director | Case 1 | Case 2 | Review 1 | Review 2 | Review 3 | Rad 1

AMR - September 2003

   

 

 

Review -
Wolff-Parkinson-White (WPW) Syndrome


Chris Talluto, MD

A 25-year-old man presents to the emergency room with palpitations, shortness of breath, and a feeling of lightheadedness.  A 12 lead EKG shows a SVT and adenosine is administered.  The tachycardia resolves and a repeat EKG shows a shortened PR interval, a widened QRS complex, and a delta wave. A diagnosis of Wolff-Parkinson-White (WPW) Syndrome is made.

In 1970 Durrer et al clarified that “preexcitation exists, if in relation to atrial events, the whole or some part of the ventricular muscle is activated earlier by the impulse originating from the atrium than would be expected if the impulse reached the ventricles by way of the normal specific conduction system only. “ Of the various preexcitation syndromes, the most common is WPW syndrome which occurs in about 0.15%- 0.2% of the general population with men being affected 60-70% more commonly than women. It can affect people of all ages, but usually is recognized in children and young adults presenting with an arrhythmia.  It is thought that there is a congenital accessory pathway between the atrium and ventricles.  There are many different locations for the bypass tracts including atriofascicular, fasciculoventricular, intranodal, or nodoventricular, but the most common bypass tract is an accessory atrioventricular pathway often referred to as the bundle of Kent.  This is the congenital anomaly seen in WPW.

Typically patients present to the emergency room with mild symptoms of chest discomfort or palpitations, but can also present in full cardiopulmonary arrest secondary to an associated arrhythmia.  Small children often present with irritability, vomiting of feeds, and decreased oral intake.   Typically the heart rates are approximately 250 for older individuals, whereas babies can present with heart rates in the 300’s.  On physical exam the patient may be cool, diaphoretic and hypotensive and crackles in the lungs can be heard because of diastolic heart failure secondary to the tachycardia.  In babies, the physical exam can be completely normal except for the isolated tachycardia. The differential diagnosis at this time includes atrial fibrillation, atrial flutter, ventricular tachycardia, or supraventricular tachycardia. A diagnosis of WPW syndrome can be made when you have both preexcitation of the EKG and paroxysmal tachycardias.

EKG findings are particularly helpful in making an initial diagnosis, although the diagnosis of WPW syndrome can not usually be made until the tachycardia resolves. The classic EKG morphology of WPW is a shortened PR interval of less than 0.12sec., a slurred upstroke of the QRS complex (delta wave), and a wide QRS complex.

                    

This typical pattern is seen with antegrade conduction activating the ventricles from both the bypass tract and AV nodal His-Purkinje system.  Depending upon the location of the accessory pathway in relation to the sinus node, the relative transmission characteristics of the accessory pathway and the AV node, the morphology of the EKG may vary from the classic presentation to near normal.  An accessory pathway that does not have EKG changes is usually revealed when the rate exceeds the refractory period of the AV node.  This “latent” accessory pathway can conduct both anterograde and retrograde.  There is an accessory pathway in which only retrograde transmission of the impulses can occur, and this is called concealed. This concealed accessory pathway can lead to circus movement tachycardias; one of the most common types of arrhythmias seen in WPW syndrome. The other most common arrhythmia is atrial fibrillation.

Patients presenting with paroxysmal supraventricular tachycardia, the impulse is usually conducted antegradely over the normal AV system and retrogradely through the bypass tract.  Rarely (approximately 5% of patients) there is antegrade conduction through the bypass tract and retrograde conduction through the normal AV system. This produces a wide complex QRS complex in which the ventricles are stimulated by the accessory pathway.  If there is a premature atrial beat that occurs during the refractory  period of the accessory pathway, the impulse could travel down the AV nodal pathway, but then return via the accessory pathway resulting in circus movement tachycardias (CMT). This orthodromic tachycardia is a narrow complex and is rate limited by the refractory period of the AV node. Differentiating CMT from PSVT is unnecessary because the treatment for both is IV adenosine. It is important to remember that any regular wide-complex tachycardia should be considered V-Tach until proven otherwise, however a stable patient with a wide complex tachycardia can be treated with adenosine.

Afib in a patient with WPW occurs in 11-40% of patients, and can be fatal because of the anatomy involved.  Patients with WPW have the accessory pathway with a shorter anterograde refractory period allowing for much faster transmission of  impulses.  This can lead to a 1:1 transmission from the atria to the ventricles leading to hypotension and if the rate becomes too high, V fib. The typical EKG pattern of A Fib through an accessory pathway appears as a wide-complex, irregular tachycardia.

Treatment of patients with WPW differs depending upon the presentation.  Patients who are unstable may need cardioversion or ACLS resuscitation. Treatment of A Fib in a patient with WPW is different than a patient with a normal heart.  If treated with conventional drugs, the  prolongation of the refractory period of the AV node would increase the rate of transmission  through the accessory pathway and increase ventricular rate, therefore cardioversion is the preferred method of treatment.  Treating CMT/PSVT is similar and is accomplished by prolonging the refractory period of the AV node.  Adenosine 6mg IV push, should be the first line of treatment. In children, a dose of 0.1mg/kg/dose is used with a max dose of 6mg initially.  If this fails, increase the dose to 12mg via IV push. Adenosine can be used regardless of whether the complex is wide or narrow as long as the patient is stable.

Of importance is the regularity of the QRS.  A regular QRS distinguishes CMT/PSVT  from  Fib, which has irregular QRS patterns.  A regular QRS either narrow or wide can be treated with adenosine. If the QRS is irregular its more likely to be AFib, and adenosine should not be used and either cardioversion or procanamide should be used.  When in doubt it is safer to err on the side of treating for AFib.

REFERENCES

  1. Garratt C, Antoniou A, Ward D, et al. Misuse of verapamil in pre-excited atrial fibrillation. Lancet 1989 Feb 18; 1(8634): 367-9.
  2. Herbert M, Tully G, et al. Wolff-Parkinson-White Syndrome. E-medicine 2001 December; 1-9.
  3. Sharma AD, Klein, GJ, Yee R. Intravenous adenosine triphosphate during wide QRS complex tachycardia: safety, therapeutic efficacy, and diagnostic utility. Am J Med 1990 Apr; 88(4)); 337-43.