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WPW in chidren

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    Life-Threatening Event Risk in Children
    With Wolff-Parkinson-White Syndrome
    A Multicenter International Study

    ABSTRACT
    OBJECTIVES This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by
    comparing those who had experienced a life-threatening event (LTE) with a control population.
    BACKGROUND Children with WPW syndrome are at risk of sudden death.
    METHODS This retrospective multicenter pediatric study identified 912 subjects #21 years of age with WPW syndrome,
    using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or
    atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of #250 ms or with hemodynamic
    compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects.
    RESULTS Case subjects (n ¼ 96) were older and less likely than subjects (n ¼ 816) to have symptoms or documented
    tachycardia. Mean age at LTE was 14.1 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of
    rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk
    components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced
    cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In
    multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP,
    SPERRI, or SPPCL #250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had $2
    EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and
    15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia.
    CONCLUSIONS Young patients may experience LTE from WPW syndrome without prior symptoms or markers of highrisk on EPS. (J Am Coll Cardiol EP 2017;-:-–-) © 2017 by the American College of Cardiology Foundation.
    From the aDivision of Cardiology, Department of Pediatrics, Primary Children’s Hospital, University of Utah, Salt Lake City, Utah;
    bDivision of Cardiology, Department of Pediatrics, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada;
    cDivision of Pediatric Cardiology, Department of Pediatrics, Cohen Children’s Medical Center of New York, Hofstra-Northwell
    School of Medicine, New Hyde Park, New York; dDepartment of Pediatrics, Division of Cardiology, Stead Family Children’s
    Hospital, University of Iowa, Iowa City, Iowa; eDivision of Cardiology, Department of Pediatrics, University of Michigan Children’s
    Hospital, University of Michigan, Ann Arbor, Michigan; fLabatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario,
    Canada; gDivision of Pediatric Cardiology, Department of Pediatrics, Lucile Packard Children’s Hospital, Stanford University, Palo
    Alto, California; hGreenlane Paediatric and Congenital Cardiac Service, Starship Children’s Hospital, University of Auckland,
    Auckland, New Zealand; iCardiology Division, Department of Pediatrics, Rady Children’s Hospital, University of California San
    Diego, San Diego, California; jDivision of Cardiology, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado;
    kDivision of Pediatric Cardiology, Department of Pediatrics, Seattle Children’s Hospital, Seattle, Washington; lCardiocentro
    Pediatrico William Soler, Havana, Cuba; mDepartment of Paediatric Cardiology, University Hospital of Wales, Cardiff, Wales,
    United Kingdom; nDepartment of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; oDivision
    of Pediatric Cardiology, Cleveland Clinic Foundation, Cleveland, Ohio; pChildren’s Heart Centre, Charles University and Motol
    J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7
    ª 2 0 1 7 B Y T H E A M E R I C A N C O L L E G E O F C A R D I O L O G Y F O U N D A T I O N
    P U B L I S H E D B Y E L S E V I E R
    I S S N 2 4 0 5 - 5 0 0 X / $ 3 6 . 0 0
    h t t p s : / / d o i . o r g / 1 0 . 1 0 1 6 / j . j a c e p . 2 0 1 7 . 1 0 . 0 0 9
    S udden death in Wolff-Parkinson- White (WPW) syndrome is a rare but potentially preventable problem
    affecting young, otherwise healthy people.
    Sudden death is usually a consequence of
    atrial fibrillation with rapid conduction over
    an accessory pathway resulting in ventricular fibrillation. Because WPW patients
    develop atrial fibrillation more frequently
    than the general population, an important
    question is whether there is a risk of ventricular fibrillation should atrial fibrillation
    occur. Assessing accessory pathway conduction properties by using electrophysiology
    study (EPS) is advocated as a preventive
    strategy against sudden death, as noninvasive risk stratification tools are imperfect
    (1–4). Inducible atrioventricular re-entrant
    tachycardia (AVRT) or EPS data suggesting
    a pathway capable of rapid anterograde conduction are identified as predictors of malignant arrhythmia (5–8). Because catheter
    ablation can cure WPW syndrome and eliminate
    risk (9), the small long-term risk of a lifethreatening event (LTE) must be balanced with the
    immediate albeit low risk of an ablation.
    The low event rate of WPW syndrome, reduced
    further by catheter ablation, makes risk assessment a
    challenge. Data investigating possible risk factors for
    LTE in children with WPW syndrome, however,
    remain critical. In this study, we compared children
    with WPW syndrome who had experienced an LTE
    with a control population (WPW syndrome without
    LTE) to identify characteristics associated with sudden death risk.
    METHODS
    This multicenter, international, retrospective casecontrol study involved 22 centers from 6 countries
    (United States, Canada, New Zealand, Cuba,
    Czech Republic, and Wales [United Kingdom]) solicited through the Pediatric and Congenital
    Electrophysiology Society (PACES). Data collected
    encompassed the era of catheter ablation in children,
    from January 1990 through June 2016. All centers
    obtained local investigational review board approval,
    and institutional databases were searched to identify
    children with WPW syndrome. De-identified data
    were managed using Research Electronic Data Capture (REDCap), hosted at the University of Utah.
    REDCap is a secure, Web-based application designed
    to support data capture for research (10). All data
    were reviewed by the data coordinating center and
    statistician for appropriateness for inclusion.
    CASE SUBJECTS. Case subjects were children with
    WPW syndrome who had experienced an LTE at #21
    years of age. An LTE was defined as sudden death,
    aborted sudden death, or a clinical episode of preexcited atrial fibrillation with the shortest preexcited RR interval (SPERRI) in atrial fibrillation
    of #250 ms, regardless of symptoms or documented
    pre-excited atrial fibrillation associated with hemodynamic compromise, syncope, or seizure, regardless
    of the SPERRI. Subjects who experienced pre-excited
    atrial fibrillation without associated hemodynamic
    compromise, syncope, or seizure and a SPERRI >250
    ms were excluded. Cases of sudden death were
    included if a pre-mortem electrocardiogram (ECG)
    and/or EPS proving WPW syndrome was available.
    CONTROL SUBJECTS. Control subjects were #21
    years of age with WPW syndrome who had not experienced an LTE or clinical pre-excited atrial fibrillation
    and had undergone an EPS. For each case subject,
    4 age-matched subjects (24 months of age at EPS or
    LTE if no EPS was performed) and 4 non–age-matched
    subjects were selected by each center. Two sets of
    subjects were selected to potentially mitigate and
    investigate influences of age and size on ablation
    outcomes and risk. Matched subjects were selected
    from the same institution when possible or from other
    participating centers. Except for analyses involving
    age, subjects were evaluated as a single control group.
    Congenital heart disease (CHD) was noted, but cases
    and subjects were not matched for this variable.
    A B B R E V I A T I O N S
    A N D A C R O N Y M S
    APERP = accessory pathway
    effective refractory period
    ART = antidromic reciprocating
    tachycardia
    AVRT = atrioventricular
    reciprocating tachycardia
    CHD = congenital heart disease
    EPS = electrophysiology study
    LTE = life-threatening event
    ORT = orthodromic
    reciprocating tachycardia
    SPERRI = shortest pre-excited
    RR interval in atrial fibrillation
    SPPCL = shortest pre-excited
    paced cycle length with atrial
    pacing
    SVT = supraventricular
    tachycardia
    WPW = Wolff-Parkinson-White
    syndrome
    University Hospital, Prague, Czech Republic; qDepartment of Pediatrics, Benioff Children’s Hospital, University of California San
    Francisco, San Francisco, California; rDivision of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine,
    Houston Texas; sNationwide Children’s Hospital, Columbus, Ohio; tDivision of Pediatric Cardiology in the Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, Nebraska; uDepartment of Pediatrics, Division of Pediatric Cardiology,
    UCLA Medical Center, University of California Los Angeles, Los Angeles, California; vPhoenix Children’s Hospital, University of
    Arizona College of Medicine, Phoenix, Arizona; and the wDivision of Cardiology, Department of Pediatrics, British Columbia
    Children’s Hospital, Vancouver, British Columbia, Canada. Dr. Kubus is supported by the Ministry of Health, Czech Republic
    (MHCZ-DRO), University Hospital Motol, Prague, Czech Republic 00064203. All other authors have reported that they have no
    relationships relevant to the contents of this paper to disclose.
    Manuscript received August 16, 2017; revised manuscript received October 3, 2017, accepted October 12, 2017.
    Etheridge et al. J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7
    Life-Threatening Event Risk in Children With WPW Syndrome - 2 0 1 7 : - – -
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    CLINICAL DATA
    Demographic data included age at presentation,
    EPS information, and last follow-up examination.
    Symptoms, documented supraventricular tachycardia (SVT), and hemodynamically significant CHD
    were noted. Hemodynamically insignificant ventricular and atrial septal defects, ductus arteriosus,
    mitral valve prolapse, isolated left superior vena
    cava, and bicuspid aortic valve without stenosis or
    insufficiency were not considered significant CHD.
    Details of the LTE were collected, including activity
    at the time (rest, active but noncompetitive,
    competitive activity, as determined by the contributing center), type of event (pre-excited atrial
    fibrillation, aborted sudden death or ventricular
    fibrillation, or sudden death), and outcome (death,
    full or near full recovery, or recovery with a neurological deficit). All cases were reviewed by the
    coordinating center.
    ELECTROPHYSIOLOGY STUDIES. By design, an EPS
    was performed in all subjects. If case subjects had
    >1 EPS performed, the earliest study with risk
    stratification data was included for analysis. EPS
    data collected included determination of conduction properties, location of pathway(s), and induction of tachycardia including orthodromic
    reciprocating tachycardia (ORT), antidromic reciprocating tachycardia (ART), atrioventricular node
    reentrant tachycardia, atrial flutter, or atrial fibrillation. We considered risk stratification as performing at least one of the following studies:
    accessory pathway effective refractory period
    (APERP), shortest paced cycle length with preexcitation during atrial pacing (SPPCL) or SPERRI.
    If the atrial effective refractory period (AERP) was
    reached before APERP, the AERP was used in place
    of APERP. An APERP, SPPCL, or SPERRI value
    of #250 ms was considered high-risk. The use of
    anesthesia was noted. Data for isoproterenol were
    reported when available. Ablation success and
    complications were reported.
    STATISTICAL ANALYSIS. Frequency tables were
    generated for all categorical variables (SPSS version
    20.0, IBM Corp., Armonk, New York), with chi-square
    or Fisher exact analyses used to detect differences
    between case and control subjects. Mean and SD were
    reported for continuous variables. Univariate analysis
    of variance (ANOVA) was used to compare means for
    continuous variables between cases and subjects.
    Binomial logistic regression analyses were used to
    predict risk of LTE (i.e., cases vs. subjects) based on
    sex and at least one of following variables: APERP,
    SPERRI, or SPPCL #250 ms; presence of Ebstein
    malformation; inducible atrial fibrillation at EPS; or
    the presence of >1 accessory pathway. The EPSderived data were combined for logistic regression
    analysis because too few subjects had all 3 measurements determined. EPS data for univariate and
    multivariate analyses were obtained in the baseline
    state. Receiver-operating characteristic (ROC) curves
    were constructed to determine the sensitivity and
    specificity of different cutoff values of APERP,
    SPERRI, and SPPCL. For some analyses of tachycardia
    induction, ORT and ART were combined and designated AVRT. Significance was set at a p value
    of #0.05.
    RESULTS
    DEMOGRAPHIC DATA, ENTIRE COHORT. A total of
    108 cases and 864 subjects were initially entered
    into the database, but 12 cases were excluded for not
    meeting LTE criteria. Thus, a total of 912 subjects (96
    cases and 816 subjects) were analyzed (the 48 subjects age-matched to excluded subjects were
    omitted, but the non–age-matched subjects were
    TABLE 1 Characteristics of the Study Cohort
    Case Subjects
    (n ¼ 96)
    Control Subjects
    (n ¼ 816) p Value
    Values are mean SD, %, or n (%). *Non–age-matched subjects were used for analysis.
    CHD ¼ congenital heart disease; EPS ¼ electrophysiology study; LTE ¼ life-threatening event;
    NS ¼ not significant; SVT ¼ supraventricular tachycardia; WPW ¼ Wolff-Parkinson-White
    syndrome.
    Age at WPW presentation, yrs 11.5 6.1 9.7 5.3* 0.003
    Males (%) 78 58 <0.0005
    Race (%)
    Caucasian 67.7 73.2 NS
    African American 9.4 6.5 NS
    Native American, Alaskan Native,
    First Nations
    1.0 0.4 NS
    Asian (%) 3.1 2.1 NS
    Hawaiian/Pacific Islander 1.0 1.7 NS
    >1 ethnic group 0 0.9 NS
    Symptoms before LTE/EPS (%) 60.0 83.9 <0.0005
    chest pain 3.2 6.5 NS
    Syncope 15.1 10.8 NS
    Pre-syncope 5.3 5.8 NS
    Palpitations 47.4 68.4 <0.0005
    Documented SVT (%) 25.5 44.2 0.001
    CHD (%) 7.3 3.2 0.042
    Follow-up, yrs
    From diagnosis 5.0 6.1 4.2 4.5 NS
    From LTE/EPS 2.3 3.6 1.4 1.8 <0.0005
    Number alive at last follow-up (%) 87 (90.6) 100 (100.0) <0.0005
    Death 9 (9) 0 (0)
    J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7 Etheridge et al.
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    3
    retained). The 2 control groups did not differ except
    in age and were combined into a single group for all
    analyses except for age. Demographic and clinical
    data are summarized in Table 1. Case subjects were
    more likely to be male and were older at presentation than non–age-matched subjects. Case subjects
    were less likely to have experienced symptoms
    before the LTE than subjects and were more likely to
    have CHD. The most common lesion was Ebstein
    malformation (n ¼ 15), more prevalent in case subjects (5.2% vs. 1.2%, respectively; p ¼ 0.004). At last
    follow-up examination, there were 9 deaths, all in
    case subjects.
    CLINICAL DATA, CASE SUBJECTS. Table 2 outlines
    the characteristics of case subjects and LTE details.
    Mean age at LTE was 14.1 3.9 years of age with 3
    subjects <5 years of age (Table 3). The LTE occurred
    most often at rest or with noncompetitive activity
    and was equally likely to be rapidly conducted
    pre-excited atrial fibrillation and aborted sudden
    death. There were 6 subjects (6%) who presented
    with sudden death who had a pre-mortem ECG and/or
    EPS demonstrate WPW syndrome, and post-mortem
    evaluation did not reveal an identifiable alternative
    cause of death. Three case subjects experienced 2
    LTEs, and 1 had stable pre-excited atrial fibrillation
    before experiencing an LTE. Among case subjects, age
    <12 years (n ¼ 16) was associated with aborted sudden
    death as the LTE (88% vs 36%, p <0.0005) and age
    $12 years (n ¼ 80) was associated with pre-excited
    atrial fibrillation as the LTE (56% vs 13%, p ¼ 0.002).
    Clinical SPERRI was reported in 46 cases with preexcited atrial fibrillation as their LTE (1 was classified
    as aborted sudden death due to degeneration to
    ventricular fibrillation). The mean SPERRI was 202
    33 ms (range 150 to 320 ms). Two case subjects had
    pre-excited atrial fibrillation and an unknown
    SPERRI, 1 with recurrent seizures requiring intubation and a second subject with poor perfusion and
    hypotension. An additional case subject had syncope
    while running and a clinical SPERRI upon presentation of 320 ms.
    Sudden death or aborted sudden death was the
    presenting symptom in 31 case subjects, and preexcited atrial fibrillation was the presenting symptom
    in 31 subjects. There were 38 (40%) of case subjects
    who had not complained of previous symptoms
    before the LTE. There were 56 case subjects (58%)
    who were not known to have WPW syndrome before
    the LTE. Of these, 32 (33%) were symptom free. The
    remaining 24 reported prior symptoms including
    documented SVT (n ¼ 1), palpitations alone (n ¼ 11),
    palpitations and syncope (n ¼ 5), palpitations and
    near syncope (n ¼ 4), and syncope alone (n ¼ 3).
    Full or near full recovery was likely in case subjects
    after LTE. Five subjects recovered with a neurological
    deficit; of these, 1 subject had acute kidney failure,
    and 1 had right leg compartment syndrome after
    extracorporeal membrane oxygenation support. The
    LTE was sudden death in 6 cases. Three additional
    cases were removed from life support due to devastating neurological injury. One death occurred in a
    child who became a heart transplant donor, and WPW
    syndrome was diagnosed in the recipient subsequent
    to transplantation.
    ELECTROPHYSIOLOGY STUDY DATA, ENTIRE
    COHORT. Table 4 outlines EPS data and ablation
    outcomes. EPS data were available in all subjects and
    TABLE 2 Clinical Characteristics of Case Subjects (N ¼ 96)
    Values are mean SD or n (%).
    Abbreviations as in Table 1.
    Age at LTE (yrs) 14.1 3.9 (range 0.4
    months-21 yrs)
    Known WPW 40 (42)
    LTE as presenting symptom 62 (65)
    LTE diagnosis
    Pre-excited atrial fibrillation 47 (49)
    Aborted sudden death 43 (45)
    Sudden death 6 (6)
    Activity at time of LTE
    Rest 37 (39)
    Active, noncompetitive 33 (34)
    Active, competitive 10 (10)
    Unknown 16 (17)
    Outcome of the LTE
    Full/near full recovery 82 (85)
    Recovery with neurological injury 5 (5)
    Death 9 (9)
    TABLE 3 LTE in Children <5 Years of Age
    Age Event Details Outcome
    0.6 month Aborted sudden
    death
    Resuscitated cardiac arrest
    after weeks of poor
    feeding
    Seizures, small cerebral
    hemorrhage. Awaiting
    EPS on therapy.
    0.1 month Aborted sudden
    death
    Resuscitated cardiac arrest as
    first symptom
    EPS at 4.3 yrs with inducible
    ORT and successful
    ablation of midseptal AP.
    2.4 yrs Ventricular
    fibrillation
    Known WPW patient
    presented with ventricular
    fibrillation while
    noncompliant with
    amiodarone
    EPS at 5.7 yrs inducible ORT
    and successful ablation
    of left lateral AP.
    AP ¼ accessory pathway; ECG ¼ electrocardiogram; ORT ¼ orthodromic reciprocating tachycardia; other abbre
    viations as in Table 1.
    Etheridge et al. J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7
    Life-Threatening Event Risk in Children With WPW Syndrome - 2 0 1 7 : - – -
    4
    91% of cases. EPS data were not available in 8 case
    subjects, 7 of whom died without a previous EPS, and
    an infant who had yet to undergo EPS (Table 3). In 4
    case subjects, EPS data were from a procedure performed before LTE. One case subject had EPS performed at a nonparticipating institution, and data
    were not available. One case subject was lost to
    follow-up after an acutely successful ablation. He
    presented in ventricular fibrillation with unsuccessful
    resuscitation. He had mild Ebstein malformation with
    trivial tricuspid regurgitation; autopsy findings were
    otherwise unremarkable. In the remaining 83 case
    subjects, EPS data were from a procedure after the
    LTE, including EPS and ablation data from a procedure performed in a recipient of a heart transplanted
    from a donor with WPW syndrome.
    Tachycardia induction. Table 4 outlines tachycardias induced. Case subjects were less likely to have
    ORT but more likely to have ART, atrial flutter, and
    atrial fibrillation.
    A ccessory pathway anterograde functional
    properties. Risk stratification, defined as the determination of accessory pathway anterograde functional property (APERP, SPERRI, or SPPCL) during
    EPS, was undertaken in 89% of case and 94% of
    control subjects. There were 60 of 87 case subjects
    (69%) who underwent an EPS with $2 accessory
    pathway functional properties determined. Case
    subjects had significantly shorter APERP, SPERRI, and
    SPPCL values and were more likely to have multiple
    accessory pathways (Table 4, Figure 1). Case subjects
    were more likely to have at least 1 functional property
    considered high-risk. There were values for EPSderived SPERRI reported in 39 (45%) of case subjects
    (mean 247 61 ms). However, in 14 of 39 (36%), the
    SPERRI was >250 ms. In 13 of these subjects, the
    procedure was performed using general anesthesia;
    in 1 subject, conscious sedation was used. This is not
    different from the group where the SPERRI
    was #250 ms, 18 had general anesthesia and 7 had
    conscious sedation (p ¼ 0.20). Among 60 case subjects who had risk stratification that included $2
    accessory pathway characteristics, 22 of 60 (37%) did
    not have concerning pathway characteristics, and 15
    of 60 (25%) had neither concerning pathway characteristics nor inducible AVRT. Figure 1 shows the proportion with pathway functional characteristics
    considered high-risk (APERP, SPERRI, or SPPCL
    of #250 ms) and with multiple accessory pathways.
    Figure 2 shows the distribution of EPS data in cases
    and subjects, and although there is overlap between
    case and subjects, a low-risk cutoff can be noted. No
    case subject had a SPERRI >370 ms or an APERP >400
    ms. One case subject had a SPPCL >440 ms. Figure 3
    demonstrates ROC curves for the risk stratification
    maneuvers and demonstrates no significant differences between the areas under the curve for each risk
    stratification maneuver.
    TABLE 4 Baseline EPS Data and Ablation Outcomes
    Case Subjects
    (n ¼ 96)
    Control Subjects
    (n ¼ 816) p Value
    Values are mean SD, n (%), or %. *Non–age-matched controls were included for analysis.
    APERP ¼ accessory pathway effective refractory period; ART ¼ antidromic reciprocating tachycardia; AVNRT ¼
    atrioventricular node reentry tachycardia; AVRT ¼ atrioventricular reciprocating tachycardia; SPERRI ¼ shortest
    pre-excited RR interval in atrial fibrillation; SPPCL ¼ shortest paced cycle length with pre-excitation during atrial
    pacing; other abbreviations as in Table 1.
    Age at EPS, yrs 14.3 3.6 13.4 3.9* 0.042
    EPS performed 87 (91) 816 (100) NS
    General anesthesia used (%) 86 87 NS
    SVT induced (%) 82 74 NS
    AVRT 56 64 NS
    ORT 52 63 0.035
    ART 6 1 0.001
    Atrial fibrillation 52 27 <0.0005
    Atrial flutter 5 1 0.038
    AVNRT 2.3 0.7 NS
    Risk stratification undertaken 77 (89) 766 (94) 0.056
    APERP, ms 271 50 309 55 <0.0005
    SPERRI, ms 247 61 315 77 <0.0005
    SPPCL, ms 279 89 315 74 0.001
    APERP #250 ms (%) 41 12 <0.0005
    SPERRI #250 ms (%) 64 18 <0.0005
    SPPCL #250 ms (%) 46 21 <0.0005
    $1 functional measurement #250 ms 48 (62) 186 (24.5) <0.0005
    >1 accessory pathway (%) 18.4 5.2 <0.0005
    Ablation performed 85 (98) 789 (97) NS
    Ablation successful 71 (82) 353 (93) 0.001
    Procedure complications 8 (9) 23 (3) 0.002
    FIGURE 1 EPS-Derived Accessory Pathway Characteristics
    Percentage of case and control subjects with pathway characteristics suggesting high-risk
    (value #250 ms) is shown. *p # 0.05. AP ¼ accessory pathway; ms ¼ milliseconds;
    APERP ¼ accessory pathway effective refractory period; EPS ¼ electrophysiology study;
    SPERRI ¼ shortest pre-excited RR interval in atrial fibrillation; SPPCL ¼ shortest paced
    cycle length with pre-excitation during atrial pacing. In this graph on the X axis it should be
    >1 AP-I will be attaching a new version of this graph with this correction.
    J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7 Etheridge et al.
    - 2 0 1 7 : - – - Life-Threatening Event Risk in Children With WPW Syndrome
    5
    When EPS data from case subjects with pre-excited
    atrial fibrillation were compared to those of subjects
    with aborted sudden death, there were no significant
    differences in mean APERP, SPERRI, or SPPCL values
    and no differences in the proportion with any of these
    values #250 ms or the presence of multiple accessory
    pathways.
    Isoproterenol. EPS data during isoproterenol infusion were not available for the entire cohort (Table 5).
    Isoproterenol therapy resulted in a shortening of
    pathway functional properties in both groups,
    although case subjects continued to have significantly shorter SPERRI and SPPCL. Only 3 case subjects
    had all 3 functional characteristic studies performed
    while they were receiving isoproterenol therapy, too
    few to make meaningful conclusions.
    PREDICTORS OF LIFE-THREATENING EVENTS. Factors
    associated with LTE were identified by univariate
    analysis (Table 6), including APERP, SPERRI, and
    SPPCL #250 ms; presence of Ebstein malformation;
    inducible atrial fibrillation at EPS; and presence of
    multiple pathways. Logistic regression was statistically significant (chi-square: 5 ¼ 82.94; df ¼ 5, p <
    0.0005). Table 7 outlines the features that were
    independently associated with increased odds of
    having an LTE.
    Of those who underwent EPS, ablation was
    attempted in 98% of cases and 97% of subjects. Success was lower in case subjects (82% vs. 93%,
    respectively; p ¼ 0.001). Table 8 outlines the pathway
    locations. More complications were noted in case
    subjects (Table 4), including atrioventricular block
    FIGURE 2 Distribution of EPS-Derived Data
    Box-and-whisker plots of APERP, SPERRI, and SPPCL in case and control subjects. Shown are mean values (solid line), and the standard
    deviations (thicker solid lines), and all values for data obtained, case subjects (red), and subjects (blue). The dotted line marks 250 ms. There
    were 2 outliers in which SPERRI was >600 ms (2 subjects) and 4 outliers (1 case and 3 subjects) with SPPCL >600 ms, these are not
    represented in the graphs. Abbreviations as in Figure 1.
    Etheridge et al. J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7
    Life-Threatening Event Risk in Children With WPW Syndrome - 2 0 1 7 : - – -
    6
    (2.3% vs. 0.2%, respectively; p ¼ 0.048). There were
    no deaths resulting from EPS or ablation.
    DISCUSSION
    This multicenter, international study is the largest to
    have addressed pediatric WPW syndrome and has
    increased our understanding of risk. Although the
    children were treated in the contemporary era of
    catheter ablation, sudden death still occurred. An LTE
    was the sentinel event in 65%, with sudden death or
    aborted sudden death sentinel in nearly 1 of 3 subjects. Importantly, case subjects were less likely than
    subjects to have experienced previous symptoms.
    In this study, we sought to better characterize LTEs
    in children. Events occurred most often in adolescent males who were not engaged in competition.
    Although competitive athletics are considered to increase risk (11–14), sports restriction would not have
    prevented LTEs in the 73% of our case subjects whose
    FIGURE 3 ROC Curves
    (A) APERP, AUC ¼ 0.702 (95% CI: 0.633 to 0.770; p < 0.0005), and #250 ms has a sensitivity of 41% and specificity of 88% in differentiating
    between cases and subjects. (B) SPERRI, AUC ¼ 0.768 (95% CI: 0.679 to 0.858; p < 0.0005), and #250 ms has a sensitivity of 64% and specificity
    of 82% in differentiating between cases and subjects. (C) SPPCL, AUC ¼ 0.703 (95% CI: 0.629 to 0.778; p<0.0005), and #250 ms has a
    sensitivity of 46% and specificity of 79% in differentiating between cases and subjects. There were no significant differences in AUC among APERP,
    SPERRI, and SPPCL. AUC ¼ area under the curve; ROC ¼ receiver operating characteristic; other abbreviations as in Figure 1.
    J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7 Etheridge et al.
    - 2 0 1 7 : - – - Life-Threatening Event Risk in Children With WPW Syndrome
    7
    events did not occur with competition. Our data are
    consistent with previous series exploring sudden
    death in the young, where most events occurred
    during rest or sleep (15–17). The 10% of events
    occurring with sports, however, remains disproportionately high when one considers the percentage of
    time engaged in sports compared with time spent at
    rest and in noncompetitive activities. Thus, our data
    do not support unrestricted sports participation in
    patients with WPW syndrome but demonstrate that
    sports restriction does not keep children safe.
    Many studies have assessed pacing maneuvers for
    risk stratification. A prospective study found shorter
    APERP and degeneration into atrial fibrillation after
    AVRT were associated with development of
    malignant arrhythmias (9). Other studies have proposed that SPERRI best predicts risk, as patients with
    ventricular fibrillation had a SPERRI #250 ms (7). Our
    data suggest that substantial risk is present even
    without evidence of rapid anterograde pathway conduction at EPS, as commonly defined. Instead, the
    cutoff value at EPS identified in this cohort was
    higher: no case subject had an APERP >400 ms or a
    SPERRI >370 ms.


    KEY WORDS pediatrics, sudden death,
    Wolff-Parkinson-White syndrome
    J A C C : C L I N I C A L E L E C T R O P H Y S I O L O G Y V O L . - , N O . - , 2 0 1 7 Etheridge et al.
    - 2 0 1 7 : - – - Life-Threatening Event Risk in Children With WPW Syndrome
    11
     

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