Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White (WPW) syndrome is a cardiac conduction disorder that presents with potentially life-threatening consequences. Wolff-Parkinson-White syndrome-induced dysrhythmias account for 20% of all supraventricular tachycardias that occur in the general population. Clinical presentations range from no symptoms to a sudden cardiac arrest. The risk of sudden death is always present with WPW syndrome, and it is the motivating force in the evaluation and treatment of this syndrome. Current diagnostic modalities are accurate in identifying patients with WPW syndrome, but lack the sensitivity to predict sudden cardiac death. This article reviews the history of WPW syndrome, as well as its general characteristics, diagnostic criteria, treatment modalities, and nursing implications.     

Heredity in Wolff-Parkinson-White syndrome

Medical genetic consultation was provided for 35 patients with Wolff-Parkinson-White syndrome (WPWS). The investigators ascertained the family history, inquired and examined patients, referred the patients' relatives for ECG and echocardiographic investigations. A newly diagnosed WPWS was reported in 3, Clerc-Levy-Cristesco (CLC) syndrome in 17, CLC phenomenon in 45 out of 132 grade I and II relatives of the proband. The study confirmed autosomal dominant inheritance of WPWS. More frequent occurrence of the syndrome was noted in the proband's relatives in case the disease occurred in both parents, less frequent in families with the history of one WPWS-affected parent. It is suggested that the disease should be considered associated with hereditary predisposition.     

History of Wolff-Parkinson-White syndrome

While Drs. Wolff, Parkinson, and White fully described the syndrome that bears their names in 1930, prior case reports had already described the essentials. Over the ensuing century this syndrome has captivated the interest of anatomists, clinical cardiologists, and cardiac surgeons. Stanley Kent described lateral muscular connections over the atrioventricular (AV) groove, which he felt were the normal AV connections. The normal AV connections were, however, clearly described by His and Tawara. True right-sided AV connections were initially described by Wood et al., while Ohnell first described left free wall pathways. David Scherf is thought to be the first to describe our current understanding of the pathogenesis of the Wolff-Parkinson-White (WPW) syndrome in terms of a reentrant circuit involving both the AV node--His axis as well as the accessory pathway. This hypothesis was not universally accepted and many theories were applied to explain the clinical findings. The basics of our understandings were established by the brilliant work of Pick, Langendorf, and Katz who by using careful deductive analysis of ECGs were able to define the basic pathophysiological processes. Subsequently, Wellens and Durrer applied invasive electrical stimulation to the heart in order to confirm the pathophysiological processes. Sealy and his colleagues at Duke University Medical Center were the first to successfully surgically divide an accessory pathway and ushered in the modern area for curative therapy for these patients. Morady and Scheinman were the first to successfully ablate an accessory pathway (posteroseptal) using high-energy direct-current shocks. Subsequently, Jackman, Kuck, Morady, and a number of groups proved the remarkable safety and efficiency of catheter ablation for pathways in all locations using radiofrequency energy. More recently, Gallob et al. first described the gene responsible for a familial form of WPW. The current ability to cure patients with WPW is due to the splendid contributions of individuals from diverse disciplines from throughout the world.     

Wolff-Parkinson-White syndrome concomitant with asymptomatic Brugada syndrome

A 29-year-old man was referred for electrophysiological testing and radiofrequency ablation because of repeated episodes of palpitation over 2 years. A 12-lead electrocardiogram during sinus rhythm showed manifest Wolff-Parkinson-White syndrome and during palpitation showed narrow QRS tachycardia at a rate of 213 beats/min. Following successful radiofrequency ablation of the left anterolateral accessory pathway, sustained atrial fibrillation was induced by atrial extrastimulation. Cibenzoline (2 mg/kg body weight) was injected to terminate atrial fibrillation. ST-T segment elevation in the right precordial leads was observed following cibenzoline administration. Ventricular fibrillation was reproducibly induced by ventricular extrastimuli (S1: 600 ms, S2: 220 ms, S3: 210 ms).     

Electrocardiographic features of Wolff-Parkinson-White syndrome

Wolff-Parkinson-White syndrome is not uncommon in the emergency department. Its early recognition and initial treatment allows rapid restoration to sinus rhythm. Prompt referral to cardiology is essential for risk stratification through electrophysiological studies.     

Transesophageal evaluation of asymptomatic Wolff-Parkinson-White syndrome

Background: Risk stratification for Wolff‐Parkinson‐White (WPW) by intracardiac electrophysiology study (ICEPS) carries risks related to catheterization. We describe an alternative approach by using transesophageal electrophysiology study (TEEPS). Methods: The pediatric electrophysiology database was reviewed for patients with WPW and no documented clinical supraventricular tachycardia (SVT) who underwent risk stratification by TEEPS from October 2005 to November 2010. Of those who underwent subsequent ICEPS, only those with data available to compare accessory pathway (AP) conduction during ICEPS and TEEPS were included. Results: Of 65 patients who underwent TEEPS, 42 were found to have an indication for ablation. The most common indication for ICEPS was inducible SVT, which was induced in 67% of patients. Of 42 patients who underwent subsequent ICEPS, 23 had sufficient data for comparison of AP conduction between ICEPS and TEEPS. There was no difference between the baseline minimum 1:1 antegrade conduction through the accessory pathway found at TEEPS versus ICEPS (312 ± 51 ms vs 316 ± 66 ms, P = 0.5). There was no significant difference between the baseline antegrade AP‐effective refractory period found at TEEPS versus ICEPS (308 ± 34 ms vs 297 ± 37 ms, P = 0.07). There were no complications related to TEEPS or ICEPS. Conclusion: TEEPS is a safe and feasible alternative to ICEPS for risk stratification in patients with asymptomatic WPW and should be considered before ICEPS and ablation. (PACE 2012; 1–5)     

The Wolff-Parkinson-White Syndrome

Typical cases of ventricular preexcitation by conduction through an accessory pathway are easily recognized on electrocardiograms, but atypical cases require electrophysiologic studies. Recent investigations cast doubt on the long-held assumption that Kent bundles invariably form the accessory pathway.     

Wolff-Parkinson-White syndrome: illustrative case and brief review

The Wolff-Parkinson-White syndrome (WPWS) is a pre-excitation syndrome manifested on the 12-lead ECG by a short PR interval (less than .12 sec), a prolonged QRS interval (greater than .10 sec), and an initial slurring of the QRS complex called a delta wave. The anatomical etiology is the presence of accessory atrioventricular conduction fibers called the Kent bundle. Patients with the WPWS may present to the emergency department with a wide spectrum of symptoms, ranging from mild palpitations, to unstable tachydysrhythmias and sudden cardiac death. WPWS should be suspected in any patient presenting with a tachydysrhythmia with a rate exceeding 200/minute. Cardioversion is indicated in the unstable patient. It is impossible to predict the effect of any particular drug without prior electrophysiologic studies in a patient with WPWS. Procainamide is the safest drug for the stable WPWS patient with a tachydysrhythmia, including wide-complex and irregular rhythms. Verapamil is a useful drug in narrow-complex, regular rhythms, although complications have been reported. Atrial fibrillation may be difficult to diagnose in a very rapid tachycardia, and the use of verapamil in WPWS with atrial fibrillation is contraindicated. Electrophysiologic studies are indicated in the WPWS patient to maximize prophylactic therapy. An illustrative case as well as pathophysiology and management of WPWS are discussed.