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and the latter can be the first presentation of the disease&#46; Although risk factors for fatal arrhythmic events are not well established&#44; EPS can be a useful tool in risk stratification&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> A short accessory pathway anterograde effective refractory period &#40;AP-AERP&#41;&#44; inducibility of sustained tachyarrhythmias &#40;atrioventricular reciprocating tachycardia &#91;AVRT&#93; and&#47;or atrial fibrillation &#91;AF&#93;&#41; and the presence of multiple accessory pathways are the strongest predictors of life-threatening arrhythmias and SCD&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case report</span><p id="par0075" class="elsevierStylePara elsevierViewall">A 17-year-old male with no history of cardiovascular disease or familial SCD presented to the emergency department &#40;ED&#41; with palpitations&#46; No medication or drug abuse was reported&#46; Symptoms had started three hours earlier at rest&#46; The physical examination revealed normal blood pressure &#40;130&#47;70<span class="elsevierStyleHsp" style=""></span>mmHg&#41; and an irregular pulse approaching 200<span class="elsevierStyleHsp" style=""></span>bpm&#46; The rest of the physical evaluation was unremarkable&#44; with no cardiac murmurs or signs of pulmonary edema&#46; An electrocardiogram &#40;ECG&#41; showed a wide-complex irregular tachycardia with rapid ventricular rate &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#44; suggesting pre-excited AF&#46; Continuous heart monitoring was initiated and two venous lines were inserted&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0080" class="elsevierStylePara elsevierViewall">A few minutes after admission to the ED&#44; the rhythm degenerated into ventricular fibrillation &#40;VF&#41; &#40;checked on the monitor&#41; and the patient collapsed without pulse&#46; Cardiopulmonary resuscitation was promptly started&#46; Recovery of regular pulse and rhythm was confirmed after defibrillation with two electrical shocks &#40;2&#215; 150<span class="elsevierStyleHsp" style=""></span>J&#44; biphasic&#41;&#46; The ECG then obtained revealed sinus rhythm with ventricular pre-excitation &#40;shortened PR interval&#44; widened QRS complex with delta wave and secondary ventricular repolarization abnormalities&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46; The patient was admitted to the coronary care unit &#40;CCU&#41;&#46; No rhythm abnormalities were recorded during CCU monitoring&#46; Serum potassium and magnesium levels were normal and transthoracic echocardiography excluded structural heart disease&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0085" class="elsevierStylePara elsevierViewall">EPS was scheduled and performed within 12<span class="elsevierStyleHsp" style=""></span>hours of admission&#46; Two catheters were positioned via the right femoral vein&#58; a deflectable decapolar catheter in the coronary sinus&#44; and a nondeflectable quadripolar catheter in the right ventricle for His activity tracing and ventricular stimulation&#46; Atrial stimulation was performed using the decapolar catheter&#46; A short AP-AERP was recorded &#40;210<span class="elsevierStyleHsp" style=""></span>ms&#41; using programmed atrial stimulation&#44; indicating a high-risk AP&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">A 4-mm ablation catheter &#40;RF Mariner&#8482;&#44; Medtronic Inc&#46;&#44; Minneapolis&#44; USA&#41; was advanced retrogradely via the right femoral artery and placed in the mitral ring&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Radiofrequency energy &#40;50<span class="elsevierStyleHsp" style=""></span>W&#47;70<span class="elsevierStyleHsp" style=""></span>&#176;C&#41; was applied for 60<span class="elsevierStyleHsp" style=""></span>s to the atrial side of the left lateral mitral ring&#46; Conduction over the AP was successfully interrupted within 3<span class="elsevierStyleHsp" style=""></span>s of energy delivery &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; During the procedure&#44; AF without pre-excitation was triggered&#44; and sinus rhythm was spontaneously recovered&#46; Total procedure time was 90<span class="elsevierStyleHsp" style=""></span>min&#44; and total fluoroscopy time was 17<span class="elsevierStyleHsp" style=""></span>min&#46; The ECG tracing after catheter ablation showed PR and QRS intervals within normal limits &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41;&#46; No procedural complications ensued and the patient was discharged three days after admission&#46; Six months after catheter ablation the patient was asymptomatic with a normal ECG tracing&#46; No tachyarrhythmias were documented during this period&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0100" class="elsevierStylePara elsevierViewall">WPW is a cardiac conduction disorder characterized by the presence of one or multiple APs that predispose patients to frequent episodes of arrhythmia&#46; A Wolff&#8211;Parkinson&#8211;White pattern is present in 0&#46;1&#8211;0&#46;2&#37; of the general population&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> most of whom will never be aware of the issue unless it is discovered incidentally&#46; Symptomatic patients generally experience a good outcome&#44; with either no recurrent arrhythmias or only benign recurrences&#46; Risk of SCD is low&#44; with annual estimates of 0&#46;1&#37; for asymptomatic<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> and 0&#46;15&#8211;0&#46;39&#37; for symptomatic patients&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">The ECG features of WPW include a PR interval of &#60;0&#46;12<span class="elsevierStyleHsp" style=""></span>s&#44; slurring and slow rise of the initial QRS complex &#40;delta wave&#41;&#44; a widened QRS complex with a total duration greater than 0&#46;12<span class="elsevierStyleHsp" style=""></span>s&#44; and secondary repolarization abnormalities that are generally directed in an opposite direction to the major delta and QRS vectors&#46; Diagnosis of WPW syndrome requires typical ECG findings with a documented dysrhythmia&#46; The most frequently encountered dysrhythmia in patients with WPW is atrioventricular reciprocating tachycardia &#40;AVRT&#41;&#44; which occurs in 80&#37; of cases&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> AF is not uncommon&#44; occurring in 15&#8211;30&#37; of patients&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> This is a potentially life-threatening arrhythmia in patients with WPW syndrome and may lead to SCD&#46; If an AP has a short anterograde refractory period&#44; rapid repetitive conduction to the ventricles during AF can result in rapid ventricular response with subsequent degeneration to VF&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">Predicting clinical outcome is one of the major issues in asymptomatic WPW subjects&#46; Risk assessment is not well defined and remains a considerable clinical challenge&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">Risk factors for potentially life-threatening arrhythmic events in WPW<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a> include&#58; &#40;1&#41; short AP-AERP &#40;&#60;250<span class="elsevierStyleHsp" style=""></span>ms&#41; allowing a rapid ventricular response in AF&#59; &#40;2&#41; inducibility of tachyarrhythmia during EPS &#40;AVRT and&#47;or AF&#41;&#59; &#40;3&#41; short pre-excited RR interval during AF &#40;&#60;250<span class="elsevierStyleHsp" style=""></span>ms&#41;&#59; &#40;4&#41; multiple APs&#59; &#40;5&#41; male gender&#59; &#40;6&#41; age&#59; and &#40;7&#41; syncope&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Invasive EPS most accurately assesses the electrophysiological properties of the AP and its role in the patient&#39;s clinical arrhythmia&#44; although no single factor has high sensitivity&#44; specificity and positive predictive value&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Pappone et al&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;5&#44;10</span></a> reported that a particular subgroup of asymptomatic patients may be at risk for a malignant arrhythmic event and demonstrated the value of EPS in stratifying asymptomatic patients into high- and low-risk groups&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Noninvasive markers of lower risk such as intermittent loss of pre-excitation&#44; loss of AP conduction on exercise stress testing&#44; and sudden loss of AP conduction after treatment with the antiarrhythmic drugs procainamide or flecainide are limited by insufficient sensitivity and specificity&#44; and currently play little role in patient management&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0130" class="elsevierStylePara elsevierViewall">In our case of a previously healthy 17-year-old patient there were no past ECG records and thus the existence of an AP was not known&#46; A malignant arrhythmia with degeneration to VF was the first presentation and could have resulted in SCD&#46; The first-line therapeutic option was EPS before hospital discharge&#44; resulting in successful ablation of the high-risk AP&#46; However&#44; had the WPW pattern been incidentally found before symptoms&#44; how should we have proceeded&#63;</p><p id="par0135" class="elsevierStylePara elsevierViewall">The 2003 ESC&#47;ACC&#47;AHA guidelines<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> are restrictive regarding the management of an asymptomatic WPW pattern&#44; and recommend routine invasive EPS and catheter ablation only in symptomatic patients&#46; This may be questionable&#58; catheter ablation is now routinely and safely performed by skilled operators&#44; and asymptomatic patients are more commonly referred for invasive risk stratification and prophylactic AP ablation&#46; Pappone et al&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;10</span></a> reported that prophylactic AP catheter ablation performed at the time of initial EPS improved the long-term outcome of patients at high risk for malignant arrhythmias&#44; and the risk significantly and persistently decreased over time after ablation&#46; Furthermore&#44; the efficacy of catheter AP ablation approaches 100&#37;&#44; and overall procedure-related mortality is less than 0&#46;2&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;12</span></a> Accordingly&#44; radiofrequency catheter ablation &#40;RFA&#41; is now widely accepted as a therapy for WPW and is frequently considered the first-line therapy&#46; However&#44; different AP locations still represent challenges for ablation&#46; Parahisian and midseptal APs&#44; which account for only a minority of cases&#44; pose a significant challenge to RFA due to their proximity to the His bundle and AV node&#44; increasing the risk of AV block&#46; A possibly safer approach for elimination of these challenging APs is cryoablation&#44; which creates homogeneous and smaller lesions and is less thrombogenic than RFA&#44; reducing the risk of inadvertent AV block&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> The potential risk of AV block due to RFA of parahisian and midseptal APs must always be discussed with the patient&#44; particularly in the younger&#44; and balanced against the benefits of ablation&#46; In conclusion&#44; the benefits of prophylactic catheter ablation &#40;RFA or cryoablation&#41; can outweigh the procedural risks when performed by a skilled operator&#44; and the issue of the management of asymptomatic WPW patients could be readdressed&#46;</p><p id="par0140" class="elsevierStylePara elsevierViewall">Our case illustrates how a WPW pattern may not be as benign as thought&#46; Sudden cardiac death is a dramatic occurrence&#44; particularly in young healthy subjects&#44; and is a real-life event and not merely an item on the reference list of a report on the natural history of the disease&#46; If our patient had had a previous ECG tracing showing a WPW pattern&#44; and if the guidelines had been followed&#44; his fate would have been SCD in the absence of prompt medical assistance&#46; This scenario must be always taken into consideration in each patient with an incidental WPW pattern&#46;</p><p id="par0145" class="elsevierStylePara elsevierViewall">WPW-associated deaths are preventable given the availability of a permanent treatment that is safe and effective&#44; and the benefits of catheter ablation are likely to outweigh the procedural risks when performed by a skilled operator&#46;</p><p id="par0150" class="elsevierStylePara elsevierViewall">The latest guidelines<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> were published over nine years ago&#46; The authors consider that it is currently unacceptable that even one asymptomatic patient with WPW pattern should die or experience a life-threatening arrhythmic event due to a high-risk accessory pathway&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Ethical disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Protection of human and animal subjects</span><p id="par0155" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Confidentiality of data</span><p id="par0160" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Right to privacy and informed consent</span><p id="par0165" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of interest</span><p id="par0170" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
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        4 => array:2 [
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          "titulo" => "Introduction"
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        5 => array:2 [
          "identificador" => "sec0010"
          "titulo" => "Case report"
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        6 => array:2 [
          "identificador" => "sec0015"
          "titulo" => "Discussion"
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          "titulo" => "Ethical disclosures"
          "secciones" => array:3 [
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              "identificador" => "sec0025"
              "titulo" => "Protection of human and animal subjects"
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            1 => array:2 [
              "identificador" => "sec0030"
              "titulo" => "Confidentiality of data"
            ]
            2 => array:2 [
              "identificador" => "sec0035"
              "titulo" => "Right to privacy and informed consent"
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          ]
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    "fechaRecibido" => "2012-03-25"
    "fechaAceptado" => "2012-08-02"
    "PalabrasClave" => array:2 [
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        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec239318"
          "palabras" => array:3 [
            0 => "Wolff&#8211;Parkinson&#8211;White"
            1 => "Sudden cardiac death"
            2 => "Catheter ablation"
          ]
        ]
      ]
      "pt" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palavras-chave"
          "identificador" => "xpalclavsec239319"
          "palabras" => array:3 [
            0 => "Wolff&#8211;Parkinson&#8211;White"
            1 => "Morte s&#250;bita card&#237;aca"
            2 => "Abla&#231;&#227;o por cat&#233;ter"
          ]
        ]
      ]
    ]
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    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Sudden cardiac death &#40;SCD&#41; can be the first clinical manifestation of Wolff&#8211;Parkinson&#8211;White &#40;WPW&#41; syndrome&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Catheter ablation of accessory pathways is now a safe and effective procedure&#44; and is widely recommended in patients with WPW syndrome&#46; However&#44; management of the asymptomatic WPW patient remains controversial&#46; Recent studies have readdressed the issue of risk stratification and prophylactic catheter ablation&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">We describe a case of malignant arrhythmia and aborted SCD as first presentation of WPW syndrome in a previously asymptomatic 17-year-old patient&#46;</p>"
      ]
      "pt" => array:2 [
        "titulo" => "Resumo"
        "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A morte s&#250;bita card&#237;aca &#40;MSC&#41; pode ser a primeira manifesta&#231;&#227;o da s&#237;ndrome de Wolff&#8211;Parkinson&#8211;White &#40;WPW&#41;&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A abla&#231;&#227;o por cateter da via acess&#243;ria &#233; atualmente um tratamento seguro e eficaz&#44; estando liberalmente recomendado em doentes sintom&#225;ticos&#46; J&#225; na presen&#231;a de padr&#227;o electrocardiogr&#225;fico de WPW&#44; a orienta&#231;&#227;o terap&#234;utica &#233; alvo de controv&#233;rsia&#46; Alguns estudos vieram reativar a discuss&#227;o relativamente &#224; estratifica&#231;&#227;o de risco e benef&#237;cio da abla&#231;&#227;o profil&#225;tica&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Descrevemos o caso cl&#237;nico de um jovem de 17 anos previamente assintom&#225;tico&#44; com arritmia maligna e morte s&#250;bita card&#237;aca abortada como primeira manifesta&#231;&#227;o da doen&#231;a&#46;</p>"
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      0 => array:2 [
        "titulo" => "<span class="elsevierStyleSectionTitle">Abbreviations</span>"
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            "definicion" => array:13 [
              0 => array:2 [
                "termino" => "AF"
                "descripcion" => "<p id="par0005" class="elsevierStylePara elsevierViewall">atrial fibrillation</p>"
              ]
              1 => array:2 [
                "termino" => "AP"
                "descripcion" => "<p id="par0010" class="elsevierStylePara elsevierViewall">accessory pathway</p>"
              ]
              2 => array:2 [
                "termino" => "AP-AERP"
                "descripcion" => "<p id="par0015" class="elsevierStylePara elsevierViewall">accessory pathway antegrade effective refractory period</p>"
              ]
              3 => array:2 [
                "termino" => "AV"
                "descripcion" => "<p id="par0020" class="elsevierStylePara elsevierViewall">atrioventricular</p>"
              ]
              4 => array:2 [
                "termino" => "AVRT"
                "descripcion" => "<p id="par0025" class="elsevierStylePara elsevierViewall">atrioventricular reciprocating tachycardia</p>"
              ]
              5 => array:2 [
                "termino" => "CCU"
                "descripcion" => "<p id="par0030" class="elsevierStylePara elsevierViewall">coronary care unit</p>"
              ]
              6 => array:2 [
                "termino" => "ECG"
                "descripcion" => "<p id="par0035" class="elsevierStylePara elsevierViewall">electrocardiogram</p>"
              ]
              7 => array:2 [
                "termino" => "ED"
                "descripcion" => "<p id="par0040" class="elsevierStylePara elsevierViewall">emergency department</p>"
              ]
              8 => array:2 [
                "termino" => "EPS"
                "descripcion" => "<p id="par0045" class="elsevierStylePara elsevierViewall">electrophysiological study</p>"
              ]
              9 => array:2 [
                "termino" => "RFA"
                "descripcion" => "<p id="par0050" class="elsevierStylePara elsevierViewall">radiofrequency ablation</p>"
              ]
              10 => array:2 [
                "termino" => "SCD"
                "descripcion" => "<p id="par0055" class="elsevierStylePara elsevierViewall">sudden cardiac death</p>"
              ]
              11 => array:2 [
                "termino" => "VF"
                "descripcion" => "<p id="par0060" class="elsevierStylePara elsevierViewall">ventricular fibrillation</p>"
              ]
              12 => array:2 [
                "termino" => "WPW"
                "descripcion" => "<p id="par0065" class="elsevierStylePara elsevierViewall">Wolff&#8211;Parkinson&#8211;White</p>"
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            ]
          ]
        ]
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        "etiqueta" => "Figure 1"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Presenting 12-lead ECG&#46; A wide-complex irregular tachycardia is shown consistent with pre-excited AF&#46; The ventricular response is very rapid&#44; and the shortest pre-excited RR interval is nearly 200<span class="elsevierStyleHsp" style=""></span>ms&#46;</p>"
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          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Twelve-lead ECG after conversion to sinus rhythm&#46; Shortened PR interval&#44; delta wave&#44; widened QRS complex and secondary repolarization abnormalities are seen&#46; This ECG is indicative of WPW syndrome&#44; and a left lateral AP is suggested &#40;positive delta wave in V1 and inferior leads and negative delta wave in aVL and DI&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p>"
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        "mostrarFloat" => true
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        "figura" => array:1 [
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            "imagen" => "gr3.jpeg"
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          "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Accessory pathway ablation&#46; Surface leads I&#44; II&#44; III&#44; aVF&#44; aVL&#44; aVF and V1&#8211;V6 are shown&#44; with intracardiac recordings from catheters in the right ventricle &#40;HRVp and HRVd&#41;&#44; distal &#40;CS1&#41; and proximal &#40;CS5&#41; coronary sinus&#44; and proximal &#40;RFp&#41; and distal &#40;RFd&#41; ablation catheter&#46; Atrial &#40;A&#41; and ventricular &#40;V&#41; electrical signals are shown&#46; &#40;A&#41; Electrocardiographic and intracardiac signals immediately before catheter ablation&#44; recorded at 200<span class="elsevierStyleHsp" style=""></span>mm&#47;s&#46; &#40;B&#41; Catheter accessory pathway ablation&#44; recorded at 50<span class="elsevierStyleHsp" style=""></span>mm&#47;s&#46; Conduction over the AP disappeared within 3<span class="elsevierStyleHsp" style=""></span>s of radiofrequency energy application&#46; The asterisk represents the beginning of radiofrequency energy application&#46;</p>"
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          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Twelve-lead ECG after catheter ablation&#46; PR and QRS intervals are within normal limits&#46;</p>"
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      "titulo" => "References"
      "seccion" => array:1 [
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                            2 => "C&#46;U&#46; Plautz"
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                      "titulo" => "Usefulness of invasive electrophysiologic testing to stratify the risk of arrhythmic events in asymptomatic patients with Wolff&#8211;Parkinson&#8211;White pattern"
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                            0 => "C&#46; Pappone"
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                          "autores" => array:3 [
                            0 => "C&#46; Pappone"
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                          "etal" => true
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                      "autores" => array:1 [
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                          "etal" => true
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                            0 => "V&#46; Santinelli"
                            1 => "A&#46; Radinovic"
                            2 => "F&#46; Mangusto"
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Case report
Aborted sudden cardiac death as first presentation of Wolff–Parkinson–White syndrome
Morte súbita cardíaca abortada como primeira manifestação da síndrome de Wolff–Parkinson–White
Guida Silva
Corresponding author
gmadeira.silva@gmail.com

Corresponding author.
, Gustavo Pires de Morais, João Primo, Olga Sousa, Eulália Pereira, Marta Ponte, Lino Simões, Vasco Gama
Serviço de Cardiologia, Centro Hospitalar de Vila Nova de Gaia/Espinho-EPE, Vila Nova de Gaia, Portugal
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        "titulo" => "Morte s&#250;bita card&#237;aca abortada como primeira manifesta&#231;&#227;o da s&#237;ndrome de Wolff&#8211;Parkinson&#8211;White"
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          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Twelve-lead ECG after conversion to sinus rhythm&#46; Shortened PR interval&#44; delta wave&#44; widened QRS complex and secondary repolarization abnormalities are seen&#46; This ECG is indicative of WPW syndrome&#44; and a left lateral AP is suggested &#40;positive delta wave in V1 and inferior leads and negative delta wave in aVL and DI&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Introduction</span><p id="par0070" class="elsevierStylePara elsevierViewall">WPW syndrome is a disorder characterized by the presence of one or more accessory pathways that predispose patients to frequent episodes of arrhythmias&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The 2003 ESC&#47;ACC&#47;AHA guidelines<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> recommend routine electrophysiological study &#40;EPS&#41; with liberal indications for catheter ablation in symptomatic patients&#46; However&#44; management of asymptomatic subjects with incidentally found pre-excitation patterns remains controversial&#46; Prognosis is usually good&#44; but there is a lifetime risk of malignant arrhythmias and sudden cardiac death &#40;SCD&#41;&#44; and the latter can be the first presentation of the disease&#46; Although risk factors for fatal arrhythmic events are not well established&#44; EPS can be a useful tool in risk stratification&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> A short accessory pathway anterograde effective refractory period &#40;AP-AERP&#41;&#44; inducibility of sustained tachyarrhythmias &#40;atrioventricular reciprocating tachycardia &#91;AVRT&#93; and&#47;or atrial fibrillation &#91;AF&#93;&#41; and the presence of multiple accessory pathways are the strongest predictors of life-threatening arrhythmias and SCD&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case report</span><p id="par0075" class="elsevierStylePara elsevierViewall">A 17-year-old male with no history of cardiovascular disease or familial SCD presented to the emergency department &#40;ED&#41; with palpitations&#46; No medication or drug abuse was reported&#46; Symptoms had started three hours earlier at rest&#46; The physical examination revealed normal blood pressure &#40;130&#47;70<span class="elsevierStyleHsp" style=""></span>mmHg&#41; and an irregular pulse approaching 200<span class="elsevierStyleHsp" style=""></span>bpm&#46; The rest of the physical evaluation was unremarkable&#44; with no cardiac murmurs or signs of pulmonary edema&#46; An electrocardiogram &#40;ECG&#41; showed a wide-complex irregular tachycardia with rapid ventricular rate &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#44; suggesting pre-excited AF&#46; Continuous heart monitoring was initiated and two venous lines were inserted&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0080" class="elsevierStylePara elsevierViewall">A few minutes after admission to the ED&#44; the rhythm degenerated into ventricular fibrillation &#40;VF&#41; &#40;checked on the monitor&#41; and the patient collapsed without pulse&#46; Cardiopulmonary resuscitation was promptly started&#46; Recovery of regular pulse and rhythm was confirmed after defibrillation with two electrical shocks &#40;2&#215; 150<span class="elsevierStyleHsp" style=""></span>J&#44; biphasic&#41;&#46; The ECG then obtained revealed sinus rhythm with ventricular pre-excitation &#40;shortened PR interval&#44; widened QRS complex with delta wave and secondary ventricular repolarization abnormalities&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46; The patient was admitted to the coronary care unit &#40;CCU&#41;&#46; No rhythm abnormalities were recorded during CCU monitoring&#46; Serum potassium and magnesium levels were normal and transthoracic echocardiography excluded structural heart disease&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0085" class="elsevierStylePara elsevierViewall">EPS was scheduled and performed within 12<span class="elsevierStyleHsp" style=""></span>hours of admission&#46; Two catheters were positioned via the right femoral vein&#58; a deflectable decapolar catheter in the coronary sinus&#44; and a nondeflectable quadripolar catheter in the right ventricle for His activity tracing and ventricular stimulation&#46; Atrial stimulation was performed using the decapolar catheter&#46; A short AP-AERP was recorded &#40;210<span class="elsevierStyleHsp" style=""></span>ms&#41; using programmed atrial stimulation&#44; indicating a high-risk AP&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">A 4-mm ablation catheter &#40;RF Mariner&#8482;&#44; Medtronic Inc&#46;&#44; Minneapolis&#44; USA&#41; was advanced retrogradely via the right femoral artery and placed in the mitral ring&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Radiofrequency energy &#40;50<span class="elsevierStyleHsp" style=""></span>W&#47;70<span class="elsevierStyleHsp" style=""></span>&#176;C&#41; was applied for 60<span class="elsevierStyleHsp" style=""></span>s to the atrial side of the left lateral mitral ring&#46; Conduction over the AP was successfully interrupted within 3<span class="elsevierStyleHsp" style=""></span>s of energy delivery &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; During the procedure&#44; AF without pre-excitation was triggered&#44; and sinus rhythm was spontaneously recovered&#46; Total procedure time was 90<span class="elsevierStyleHsp" style=""></span>min&#44; and total fluoroscopy time was 17<span class="elsevierStyleHsp" style=""></span>min&#46; The ECG tracing after catheter ablation showed PR and QRS intervals within normal limits &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41;&#46; No procedural complications ensued and the patient was discharged three days after admission&#46; Six months after catheter ablation the patient was asymptomatic with a normal ECG tracing&#46; No tachyarrhythmias were documented during this period&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0100" class="elsevierStylePara elsevierViewall">WPW is a cardiac conduction disorder characterized by the presence of one or multiple APs that predispose patients to frequent episodes of arrhythmia&#46; A Wolff&#8211;Parkinson&#8211;White pattern is present in 0&#46;1&#8211;0&#46;2&#37; of the general population&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> most of whom will never be aware of the issue unless it is discovered incidentally&#46; Symptomatic patients generally experience a good outcome&#44; with either no recurrent arrhythmias or only benign recurrences&#46; Risk of SCD is low&#44; with annual estimates of 0&#46;1&#37; for asymptomatic<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> and 0&#46;15&#8211;0&#46;39&#37; for symptomatic patients&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">The ECG features of WPW include a PR interval of &#60;0&#46;12<span class="elsevierStyleHsp" style=""></span>s&#44; slurring and slow rise of the initial QRS complex &#40;delta wave&#41;&#44; a widened QRS complex with a total duration greater than 0&#46;12<span class="elsevierStyleHsp" style=""></span>s&#44; and secondary repolarization abnormalities that are generally directed in an opposite direction to the major delta and QRS vectors&#46; Diagnosis of WPW syndrome requires typical ECG findings with a documented dysrhythmia&#46; The most frequently encountered dysrhythmia in patients with WPW is atrioventricular reciprocating tachycardia &#40;AVRT&#41;&#44; which occurs in 80&#37; of cases&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> AF is not uncommon&#44; occurring in 15&#8211;30&#37; of patients&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> This is a potentially life-threatening arrhythmia in patients with WPW syndrome and may lead to SCD&#46; If an AP has a short anterograde refractory period&#44; rapid repetitive conduction to the ventricles during AF can result in rapid ventricular response with subsequent degeneration to VF&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">Predicting clinical outcome is one of the major issues in asymptomatic WPW subjects&#46; Risk assessment is not well defined and remains a considerable clinical challenge&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">Risk factors for potentially life-threatening arrhythmic events in WPW<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a> include&#58; &#40;1&#41; short AP-AERP &#40;&#60;250<span class="elsevierStyleHsp" style=""></span>ms&#41; allowing a rapid ventricular response in AF&#59; &#40;2&#41; inducibility of tachyarrhythmia during EPS &#40;AVRT and&#47;or AF&#41;&#59; &#40;3&#41; short pre-excited RR interval during AF &#40;&#60;250<span class="elsevierStyleHsp" style=""></span>ms&#41;&#59; &#40;4&#41; multiple APs&#59; &#40;5&#41; male gender&#59; &#40;6&#41; age&#59; and &#40;7&#41; syncope&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Invasive EPS most accurately assesses the electrophysiological properties of the AP and its role in the patient&#39;s clinical arrhythmia&#44; although no single factor has high sensitivity&#44; specificity and positive predictive value&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Pappone et al&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;5&#44;10</span></a> reported that a particular subgroup of asymptomatic patients may be at risk for a malignant arrhythmic event and demonstrated the value of EPS in stratifying asymptomatic patients into high- and low-risk groups&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Noninvasive markers of lower risk such as intermittent loss of pre-excitation&#44; loss of AP conduction on exercise stress testing&#44; and sudden loss of AP conduction after treatment with the antiarrhythmic drugs procainamide or flecainide are limited by insufficient sensitivity and specificity&#44; and currently play little role in patient management&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0130" class="elsevierStylePara elsevierViewall">In our case of a previously healthy 17-year-old patient there were no past ECG records and thus the existence of an AP was not known&#46; A malignant arrhythmia with degeneration to VF was the first presentation and could have resulted in SCD&#46; The first-line therapeutic option was EPS before hospital discharge&#44; resulting in successful ablation of the high-risk AP&#46; However&#44; had the WPW pattern been incidentally found before symptoms&#44; how should we have proceeded&#63;</p><p id="par0135" class="elsevierStylePara elsevierViewall">The 2003 ESC&#47;ACC&#47;AHA guidelines<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> are restrictive regarding the management of an asymptomatic WPW pattern&#44; and recommend routine invasive EPS and catheter ablation only in symptomatic patients&#46; This may be questionable&#58; catheter ablation is now routinely and safely performed by skilled operators&#44; and asymptomatic patients are more commonly referred for invasive risk stratification and prophylactic AP ablation&#46; Pappone et al&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;10</span></a> reported that prophylactic AP catheter ablation performed at the time of initial EPS improved the long-term outcome of patients at high risk for malignant arrhythmias&#44; and the risk significantly and persistently decreased over time after ablation&#46; Furthermore&#44; the efficacy of catheter AP ablation approaches 100&#37;&#44; and overall procedure-related mortality is less than 0&#46;2&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;12</span></a> Accordingly&#44; radiofrequency catheter ablation &#40;RFA&#41; is now widely accepted as a therapy for WPW and is frequently considered the first-line therapy&#46; However&#44; different AP locations still represent challenges for ablation&#46; Parahisian and midseptal APs&#44; which account for only a minority of cases&#44; pose a significant challenge to RFA due to their proximity to the His bundle and AV node&#44; increasing the risk of AV block&#46; A possibly safer approach for elimination of these challenging APs is cryoablation&#44; which creates homogeneous and smaller lesions and is less thrombogenic than RFA&#44; reducing the risk of inadvertent AV block&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> The potential risk of AV block due to RFA of parahisian and midseptal APs must always be discussed with the patient&#44; particularly in the younger&#44; and balanced against the benefits of ablation&#46; In conclusion&#44; the benefits of prophylactic catheter ablation &#40;RFA or cryoablation&#41; can outweigh the procedural risks when performed by a skilled operator&#44; and the issue of the management of asymptomatic WPW patients could be readdressed&#46;</p><p id="par0140" class="elsevierStylePara elsevierViewall">Our case illustrates how a WPW pattern may not be as benign as thought&#46; Sudden cardiac death is a dramatic occurrence&#44; particularly in young healthy subjects&#44; and is a real-life event and not merely an item on the reference list of a report on the natural history of the disease&#46; If our patient had had a previous ECG tracing showing a WPW pattern&#44; and if the guidelines had been followed&#44; his fate would have been SCD in the absence of prompt medical assistance&#46; This scenario must be always taken into consideration in each patient with an incidental WPW pattern&#46;</p><p id="par0145" class="elsevierStylePara elsevierViewall">WPW-associated deaths are preventable given the availability of a permanent treatment that is safe and effective&#44; and the benefits of catheter ablation are likely to outweigh the procedural risks when performed by a skilled operator&#46;</p><p id="par0150" class="elsevierStylePara elsevierViewall">The latest guidelines<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> were published over nine years ago&#46; The authors consider that it is currently unacceptable that even one asymptomatic patient with WPW pattern should die or experience a life-threatening arrhythmic event due to a high-risk accessory pathway&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Ethical disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Protection of human and animal subjects</span><p id="par0155" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Confidentiality of data</span><p id="par0160" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Right to privacy and informed consent</span><p id="par0165" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of interest</span><p id="par0170" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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            1 => "Sudden cardiac death"
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            0 => "Wolff&#8211;Parkinson&#8211;White"
            1 => "Morte s&#250;bita card&#237;aca"
            2 => "Abla&#231;&#227;o por cat&#233;ter"
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    "resumen" => array:2 [
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        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Sudden cardiac death &#40;SCD&#41; can be the first clinical manifestation of Wolff&#8211;Parkinson&#8211;White &#40;WPW&#41; syndrome&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Catheter ablation of accessory pathways is now a safe and effective procedure&#44; and is widely recommended in patients with WPW syndrome&#46; However&#44; management of the asymptomatic WPW patient remains controversial&#46; Recent studies have readdressed the issue of risk stratification and prophylactic catheter ablation&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">We describe a case of malignant arrhythmia and aborted SCD as first presentation of WPW syndrome in a previously asymptomatic 17-year-old patient&#46;</p>"
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        "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A morte s&#250;bita card&#237;aca &#40;MSC&#41; pode ser a primeira manifesta&#231;&#227;o da s&#237;ndrome de Wolff&#8211;Parkinson&#8211;White &#40;WPW&#41;&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A abla&#231;&#227;o por cateter da via acess&#243;ria &#233; atualmente um tratamento seguro e eficaz&#44; estando liberalmente recomendado em doentes sintom&#225;ticos&#46; J&#225; na presen&#231;a de padr&#227;o electrocardiogr&#225;fico de WPW&#44; a orienta&#231;&#227;o terap&#234;utica &#233; alvo de controv&#233;rsia&#46; Alguns estudos vieram reativar a discuss&#227;o relativamente &#224; estratifica&#231;&#227;o de risco e benef&#237;cio da abla&#231;&#227;o profil&#225;tica&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Descrevemos o caso cl&#237;nico de um jovem de 17 anos previamente assintom&#225;tico&#44; com arritmia maligna e morte s&#250;bita card&#237;aca abortada como primeira manifesta&#231;&#227;o da doen&#231;a&#46;</p>"
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                "termino" => "AF"
                "descripcion" => "<p id="par0005" class="elsevierStylePara elsevierViewall">atrial fibrillation</p>"
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                "termino" => "AP"
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                "descripcion" => "<p id="par0015" class="elsevierStylePara elsevierViewall">accessory pathway antegrade effective refractory period</p>"
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                "descripcion" => "<p id="par0025" class="elsevierStylePara elsevierViewall">atrioventricular reciprocating tachycardia</p>"
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                "descripcion" => "<p id="par0030" class="elsevierStylePara elsevierViewall">coronary care unit</p>"
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                "termino" => "ECG"
                "descripcion" => "<p id="par0035" class="elsevierStylePara elsevierViewall">electrocardiogram</p>"
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                "termino" => "ED"
                "descripcion" => "<p id="par0040" class="elsevierStylePara elsevierViewall">emergency department</p>"
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                "termino" => "EPS"
                "descripcion" => "<p id="par0045" class="elsevierStylePara elsevierViewall">electrophysiological study</p>"
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              9 => array:2 [
                "termino" => "RFA"
                "descripcion" => "<p id="par0050" class="elsevierStylePara elsevierViewall">radiofrequency ablation</p>"
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              10 => array:2 [
                "termino" => "SCD"
                "descripcion" => "<p id="par0055" class="elsevierStylePara elsevierViewall">sudden cardiac death</p>"
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              11 => array:2 [
                "termino" => "VF"
                "descripcion" => "<p id="par0060" class="elsevierStylePara elsevierViewall">ventricular fibrillation</p>"
              ]
              12 => array:2 [
                "termino" => "WPW"
                "descripcion" => "<p id="par0065" class="elsevierStylePara elsevierViewall">Wolff&#8211;Parkinson&#8211;White</p>"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Presenting 12-lead ECG&#46; A wide-complex irregular tachycardia is shown consistent with pre-excited AF&#46; The ventricular response is very rapid&#44; and the shortest pre-excited RR interval is nearly 200<span class="elsevierStyleHsp" style=""></span>ms&#46;</p>"
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Revista Portuguesa de Cardiologia (English edition)
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