Risk of sudden death in Wolff-Parkinson-White syndrome: how high is the risk?

In 1930, Dr Louis Wolff, Sir John Parkinson, and Paul Dudley White described a case series of 11 patients with a syndrome that now bears their name.1 The first patient with a short PR interval, ventricular preexcitation, and supraventricular tachycardia was described by Cohn and Fraser in 1913.1 Wood et al postulated the accessory pathway (AP) as its anatomic substrate in 1942, and a large population series reported the prevalence of preexcitation to be 0.15% in 1962.2 Reports in 1971 and 1979 described sudden cardiac death (SCD) in patients with Wolff-Parkinson-White (WPW) syndrome related to atrial fibrillation (AF) that was conducted rapidly over the AP with a short refractory period that deteriorated into ventricular fibrillation (VF).3,4 The first operative ablation of an AP was performed by Sealy in 1967,1 whereas Weber and Schmitz described the first endocardial catheter ablation of an AP in 1983.1 The evolution of curative catheter ablation has clearly become the treatment of choice in the patient with substantive symptoms. A continuing controversy has been the use of this therapy in the asymptomatic or less symptomatic individual, and the central looming theme is the incidence of SCD as part of the natural history of this entity and our ability to predict it. The incidence of SCD in symptomatic patients with WPW syndrome was initially reported in the late 1960s and is estimated to be in the range of 0.25% per year, or 3% to 4% over a lifetime.5