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.
"Patients with symptomatic arrhythmia might be managed in the long term with antiarrhythmic drugs or with catheter ablation of the accessory pathway. Al-Khatib et al.  concluded that if the patients’ paroxysmal supraventricular tachycardia is satisfactorily controlled by a beta-blocker, no additional evaluation or therapy is required. In our patient population, which includes patients over a long follow-up time span, 6 of the 11 symptomatic patients had undergone catheter ablation, 2 patients planned to undergo catheter ablation in the near future, and 5 patients were controlled satisfactorily with antiarrhythmic drugs. "
[Show abstract][Hide abstract] ABSTRACT: To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff-Parkinson-White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and followed by pediatric cardiologists between 1980 and 2005 in The Netherlands. During a follow-up after EA diagnosis of 13 years 3 months (range: 6 days to 28 years 2 months), 16 (17%) of the 93 pediatric EA patients exhibited rhythm disturbances. Nine patients showed arrhythmic events starting as of the neonatal period. Supraventricular tachycardia was noted in 11 patients. One patient died in the neonatal period due to intractable supraventricular tachycardia resulting in heart failure and one patient died at 5 weeks of age most probably due to an arrhythmic event. The 14 surviving patients all show preexcitation, albeit 4 of them intermittently, and all have a right-sided accessory pathway location. Nine patients underwent catheter ablation of an accessory pathway. Only four patients are currently on antiarrhythmic drugs. The 17% prevalence of rhythm disturbances in pediatric EA patients, most commonly supraventricular arrhythmias, is significantly lower than in adult EA patients. Life-threatening rhythm disturbances are not frequent early in life. Symptomatic patients are well treated with radiofrequency catheter ablation.
"Treatment with beta-adrenergic blockers appears to be an effective therapy (Fisher et al. 1999), but occasionally an implantable cardiac defibrillator is necessary (Swan et al. 1999). Table 2 shows the main clinical features of CPVT."
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