Elsevier

American Heart Journal

Volume 138, Issue 3, September 1999, Pages 403-413
American Heart Journal

Clinical features of Wolff-Parkinson-White syndrome☆,☆☆,★,★★,♢

https://doi.org/10.1016/S0002-8703(99)70140-7Get rights and content

Abstract

Wolff-Parkinson-White syndrome is the most common form of ventricular preexcitation. Understanding this syndrome is fundamental for anyone interested in learning about arrhythmias. This review addresses (1) the historic sequence of events that led to the understanding of this syndrome; (2) the pathologic, embryologic, and electrophysiologic properties of accessory pathways; (3) the epidemiology and genetics of this syndrome; (4) the clinical diagnosis of this syndrome, with special emphasis on the arrhythmias that patients with ventricular preexcitation are predisposed to; and (5) the therapy for patients with Wolff-Parkinson-White syndrome. (Am Heart J 1999;138:403-13.)

Section snippets

Embryology and pathology

Accessory pathways normally consist of thin strands of morphologically normal myocardium that may be located either in the subendocardium or the subepicardium, although accessory pathways made up of specialized conduction tissue have been described.8, 9 During early cardiogenesis there is direct continuity between atrial and ventricular myocardium that is normally lost along the lateral cardiac aspects by ingrowth of the annulus fibrosis (Figure 1).10

. Loss of direct continuity between atrial and

Electrophysiologic properties of accessory pathways

Most accessory pathways have electrophysiologic properties that are different from those of the normal atrioventricular conduction system. Compared with the normal conduction system, these accessory pathways exhibit faster conduction of impulses that is not subject to decremental conduction properties. The presence of decremental conduction in the atrioventricular node but not in the accessory pathway in patients with Wolff-Parkinson-White syndrome is responsible for the electrocardiographic

Epidemiology and genetics

The prevalence of electrocardiographic preexcitation has been reported to be between 0.1 and 3.1 per 1000 persons.18, 19, 20, 21 It should be kept in mind, however, that the intermittent nature of preexcitation in some patients (6.7 in 100 of men and 16 of 100 in women) may have led to the underestimation of the true prevalence of this disease.18 A recent cross-sectional study showed that the prevalence of ventricular preexcitation in Japanese school children was significantly higher in

Clinical diagnosis

Wolff-Parkinson-White syndrome is said to be present when, along with electrocardiographic evidence of preexcitation, the patient has symptoms to suggest recurrent tachyarrhythmias. The 3 main electrocardiographic features of preexcitation are a short PR interval (<0.12 seconds), a prolonged QRS complex (>0.12 seconds), and a slurred, slow rising onset of the QRS complex that is known as the delta wave (Figures 2 and 3).42

. Resting electrocardiogram showing preexcitation. Note short PR interval,

Arrhythmias

Cardiac arrhythmias occur in approximately 50% of individuals who have ventricular preexcitation.18, 20, 23, 29, 53 Diagnostic clues that suggest the participation of an accessory pathway in a tachyarrhythmia are rate of >200 beats/min, slowing down of a narrow QRS complex tachycardia with the occurrence of bundle branch block indicating the utilization of an accessory atrioventricular connection ipsilateral to the bundle branch block (Coumel-Slama effect), initiation of a tachycardia showing

Management

Treatment of patients with Wolff-Parkinson-White syndrome depends on the types of arrhythmias that have been documented, their frequency, and the severity of associated symptoms. Patients with symptomatic arrhythmias may be managed in the long term with antiarrhythmic drugs or with catheter ablation of the accessory pathways. No controlled clinical trials have been done that compare antiarrhythmic drugs with placebo specifically in patients with this syndrome, although patients with this

Conclusions

Wolff-Parkinson-White syndrome is the most common form of the preexcitation syndromes, and it has a good overall prognosis. This prognosis has been improved further by better diagnostic tools such as electrophysiologic studies that are very effective in the localization of accessory pathways and by effective therapy with radiofrequency ablation. Despite the low prevalence of Wolff-Parkinson-White syndrome, it will remain one of the most interesting syndromes in the field of cardiac

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    • Cited by (0)

    ☆

    From the Divisions of Clinical Pharmacology and Cardiology, Department of Medicine, Duke University Medical Center.

    ☆☆

    Dr Pritchett’s research in supraventricular arrhythmias was supported by grant M01-RR00030 from the National Center for Resources, National Institutes of Health, Bethesda, Md.

    ★

    Guest Editor for this manuscript was Gervasio A. Lamas, MD, Mt Sinai Medical Center, Miami Beach, Fla.

    ★★

    Reprint requests: Edward Pritchett, MD, Room 04280, Duke South Hospital, DUMC-3477, Durham, NC 27710.E-mail: [email protected]

    ♢

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