Risk of Sudden Death in Wolff-Parkinson-White Syndrome

Abstract

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 Article see p 661A number of risk factors for development of SCD have emerged, 6 including (1) shortest preexcited RR interval (SPRRI) during AF 4 and its surrogate, the antegrade effective refractory period (ERP) of the AP; (2) multiple APs; (3) male gender 7 ; (4) a history of or inducibility of atrioventricular reentrant tachycardia (AVRT); (5) age; and (6) syncope.In a large, early series of patients resuscitated from VF related to WPW syndrome, a SPRRI of Ͼ250 ms was not observed, and a range of 200 ms or less was the norm. 4 Most patients in this series had inducible AVRT, and others reported vulnerability to AF and inducible AVRT as risk factors. 7 Although a SPRRI Ͻ250 ms has been virtually uniformly present in patients with VF, many more patients never destined to have sudden death also have this risk factor, which gives it a poor positive predictive value (PPV) with a high negative predictive value (NPV). 8 Thus, a SPRRI of Ͻ250 ms and its surrogate, an antegrade ERP of Ͻ250 ms, have virtually 100% NPV but poor PPV. 9 A SPRRI Ͻ250 ms remains the pivotal risk factor without which VF related to AF would be rare indeed. Requiring the presence of multiple risk factors in combination would be expected to improve specificity but at the risk of loss of sensitivity.The only uncontestable and meaningful end point for accurate risk assessment is the SCD rate, which fortunately is very low. Nonetheless, this low event rate challenges the accuracy of any predictor, including electrophysiological studies. A high NPV reported for risk stratifiers is not meaningful in this context, and it must be borne in min...

Wolff-Parkinson-White Syndrome in the Era of Catheter Ablation

“…Patients with ventricular pre-excitation on the ECG, regardless of whether they have symptoms, have an unpredictable lifetime risk of sudden cardiac death, but only a minority of them are actually at risk, which poses considerable clinical challenges to physicians for identifying the few at risk, particularly children. [1][2][3] Because many patients who experienced VF have had previous episodes of both AF and paroxysmal supraventricular tachycardia, 9 attention has focused for many decades on symptomatic patients, and guidelines have constantly recommended liberal indication for catheter ablation as a Class IA recommendation only for the symptomatic patients. 1 In the present study, over an 8-year follow-up period, only 2 of 451 symptomatic patients (0.4%) experienced cardiac arrest, whereas as many as 13 of 550 initially asymptomatic patients (2.4%) had cardiac arrest as first clinical manifestation of the syndrome, but none of them died.…”

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[1][2][3][4][5][6][7][8][9][10][11] Among a large series of WPW patients resuscitated from a sudden cardiac death, more than a half had ventricular fibrillation (VF) as the sentinel event, 4 which suggests that the risk of sudden death in the asymptomatic population is indeed underrecognized.9,10 The earliest alarming reports of sudden cardiac death were published in the late 1930s, 6,7 but the clinical manifestations typically range from an abnormal ECG finding without symptoms to cardiac arrest or sudden cardiac death. Anecdotal case series on the asymptomatic Background-The management of Wolff-Parkinson-White is based on the distinction between asymptomatic and symptomatic presentations, but evidence is limited in the asymptomatic population.

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Understanding the ECG. Part 5: Pre-excitation

“…In both cases, the resting ECG was abnormal, with a wide QRS complex. Further sporadic reports were published over the next fifteen years, however it was Dr Louis Wolff, Sir John Parkinson, and Paul Dudley White that first identified it as a syndrome in 1930 (Wolff et al, 1930;Obeyesekere et al, 2012). They described eleven cases in which mostly young, healthy people presented with intermittent palpitations, and an abnormal resting ECG.…”

When can ablation be considered a reasonable option in young asymptomatic patients with a Wolff–Parkinson–White ECG?

“…Short preexcited RR interval (SPRRI) during AF is the pivotal variable without which VF is rare. However, the great majority of individuals with WPW, even with SPRRIs of <250 ms, will not experience sudden cardiac death [2,6]. Therefore, identifying the at-risk patient remains a great challenge.…”

“…Inability to clearly demonstrate absolute and abrupt loss of manifest preexcitation (at rest or with exercise) warrants consideration on an individual basis for further testing to invasively evaluate the pathways. However, we need to acknowledge that such testing lacks specificity for predicting VF [2,6].…”