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In order to characterize the typical ARVC alterations&#44; a right-sided precordial lead ECG and a modified Fontaine ECG were performed&#44; the latter with the following placement of electrodes&#58; the right arm electrode over the manubrium&#44; the left arm electrode over the xiphoid and the left leg electrode in the area corresponding to V<span class="elsevierStyleInf">4</span>&#44; at a recording speed of 25 mm&#47;s and voltage of 10 mm&#47;mV &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46; Both methods clearly showed epsilon waves&#44; especially the modified Fontaine ECG &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>A and B&#41;&#46; Two- and three-dimensional transthoracic echocardiography at our institution showed severe right ventricular dilatation and hypokinesia&#44; with prominent apical trabeculae and false tendons and saccular dilatations in the ventricular free wall &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>A and B&#41;&#46; Cardiac magnetic resonance imaging revealed small foci of subepicardial fatty infiltration in the right ventricular free and inferior walls&#44; interventricular septum and left ventricular free wall&#59; late enhancement study showed focal enhancement in the interventricular septum consistent with fibrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>C and D&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Screening for mutations in genes coding for desmosomal proteins &#8211; plakophilin 2 &#40;PKP2&#41;&#44; desmoglein 2 and desmoplakin &#8211; revealed a nonsense mutation in exon 12 of the <span class="elsevierStyleItalic">PKP2</span> gene&#44; also present in the affected daughter&#46; This mutation is found in 11&#8211;43&#37; of patients with ARVC&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Since the patient remained asymptomatic and 24-hour Holter monitoring showed only pairs and a triplet of ventricular extrasystoles&#44; therapy with amiodarone 200 mg daily was begun and follow-up was scheduled&#46; Around a year later&#44; following an episode of fainting&#44; nonsustained ventricular tachycardia &#40;VT&#41; was documented&#44; with a pattern of left bundle branch block and superior axis &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>C&#41;&#46; This situation&#44; besides being a major criterion for a diagnosis of ARVC&#44; prompted placement of an ICD&#59; around a month after implantation&#44; an episode of VT was converted to sinus rhythm with an appropriate shock following unsuccessful antitachycardia pacing&#46; Around 18 months after this episode&#44; there has been no recurrence of ventricular arrhythmias&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">ARVC is characterized by ventricular arrhythmias and ventricular disease which is reflected macroscopically by replacement of myocardium by fibrous or fibrofatty tissue&#44; predominantly in the right ventricle but sometimes also involving the left ventricle&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Its estimated prevalence is 1&#58;5000 in the general population<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and it is a leading cause of sudden cardiac death &#40;SCD&#41;&#44; with an estimated incidence of 0&#46;08&#8211;9&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> However&#44; it may be a contributing factor in up to 10&#46;8&#37; of cases of SCD in young adults&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Clinical presentation is usually between the ages of 10 and 50&#44; with a mean age at diagnosis of 30&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The main symptoms are dizziness&#44; palpitations and syncope&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> but most patients are asymptomatic and the diagnosis is suspected following nonspecific ECG alterations&#44; echocardiographic abnormalities or documented ventricular arrhythmias&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">It is estimated that around 30&#37; of cases are familial&#46; Of the two inheritance patterns&#44; autosomal dominant is more common&#44; while in the autosomal recessive form&#44; termed Naxos disease&#44; ARVC is part of a cardiocutaneous syndrome that includes palmoplantar keratoderma and woolly hair&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">ARVC should be considered in patients with symptomatic or asymptomatic VT of left bundle branch block morphology in the absence of apparent heart disease&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> A definitive diagnosis requires histological evidence of replacement of right ventricular &#40;RV&#41; myocardium by fibrofatty tissue&#59; however&#44; assessment of this criterion is not practical in clinical practice&#44; and so other data are used for the purpose&#46; The first consensus document detailing diagnostic criteria for this entity appeared in 1994&#44; and a revised version was published in 2010<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">6</span></a> &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46; A definitive diagnosis is made on the basis of two major criteria&#44; one major and two minor criteria&#44; or four minor criteria in different categories&#46; A borderline diagnosis requires one major and one minor criterion or three minor criteria in different categories&#44; and one major criterion or two minor criteria in different categories indicates a possible diagnosis&#46; In the case presented&#44; the patient met the criteria of a family history of ARVC and epsilon waves on 12-lead ECG&#44; which were sufficient for the diagnosis&#46; The latter ECG finding is found in 30&#37; of patients with ARVC&#44; and reflects low-amplitude potentials due to late activation of some parts of the right ventricle&#46; Typically&#44; the epsilon waves are most easily identified in leads V<span class="elsevierStyleInf">1</span> to V<span class="elsevierStyleInf">3</span>&#44; as well as in the right precordial leads&#44; by doubling the sensitivity of the recording and using a filter setting of 40 Hz instead of 150 Hz to decrease the noise level&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">7</span></a> Nevertheless&#44; they are not specific for ARVC since they may be found in cases of RV abnormalities arising from myocardial infarction or cardiac sarcoidosis&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">7</span></a> The usefulness of the Fontaine ECG resides in its greater ability to unmask epsilon waves compared to standard 12-lead ECG and right-sided precordial lead ECG&#59; according to Wang et al&#46;&#44; the modified Fontaine ECG doubles or triples the rate of detection of epsilon waves compared to standard 12-lead ECG&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">8</span></a> One explanation for this may be that it records potentials developed in the right ventricle from the infundibulum to the area of the diaphragm&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">8</span></a> This method is thus particularly useful in cases of suspected ARVC&#44; particularly in the presence of right bundle branch block&#44; in which the detection of epsilon waves is even more important since a QRS duration &#62;110 ms does not allow other ECG criteria that are potentially useful in diagnosing this entity to be assessed&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">7</span></a> Although in this particular case the Fontaine ECG was not crucial to the diagnosis&#44; its ability to detect epsilon waves was the main reason for using this simple but little known electrocardiographic technique&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0050" class="elsevierStylePara elsevierViewall">While magnetic resonance imaging was not mandatory in the case presented following transthoracic echocardiography&#44; we decided to perform it due to its feasibility and its value in characterization of the imaging features of this cardiomyopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">9</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Although mutations in the genes coding for desmosomal proteins are relatively common in patients with suspected ARVC&#44; the usefulness of genetic study remains the subject of debate&#46; The prognostic implications of early identification of affected individuals are unclear given its low penetrance and highly variable expression according to age&#46; A minority of patients suffer arrhythmic events in the absence of previous symptoms or clinical signs of the disease&#46; Many do not in fact develop clinically significant disease&#44; and most of those that do have a relatively benign course&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">6&#44;10</span></a> In the present case&#44; genetic study was performed mainly due to the patient&#39;s family history&#44; and its results did not affect the therapeutic approach adopted&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">With regard to therapeutic management&#44; it is debatable whether antiarrhythmics should be used in asymptomatic patients with ventricular ectopic activity&#46; According to the ACC&#47;AHA&#47;ESC guidelines&#44; amiodarone and sotalol are only indicated &#40;class IIa recommendation&#44; level of evidence C&#41; to treat VT or ventricular fibrillation &#40;VF&#41; when an ICD is not possible&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> a fact that was taken into account during internal review of the approach to adopt in the present case&#46; ICD implantation for secondary prevention following documented VT or VF is a class I recommendation&#44; level of evidence B&#44; while implantation for primary prevention in patients with high-risk alterations such as extensive RV involvement&#44; left ventricular involvement or unexplained syncope assumed to be due to tachyarrhythmia is a class IIa recommendation&#44; level of evidence C&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The case presented highlights the importance of information on family history and of the data provided by widely available diagnostic exams such as ECG and echocardiography&#46; Different ECG techniques that are simple to perform can be useful for initial assessment by demonstrating the presence of a major diagnostic criterion for this cardiomyopathy&#46; Thus&#44; one of the main points of interest in this case report is the potential role of modified Fontaine ECG leads in the diagnosis of ARVC&#59; another is the natural history of the disease&#44; with the patient suffering potentially fatal VT&#44; a major criterion for the diagnosis&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Ethical disclosures</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Protection of human and animal subjects</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Confidentiality of data</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Right to privacy and informed consent</span><p id="par0080" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conflicts of interest</span><p id="par0085" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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    "fechaRecibido" => "2013-06-12"
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            0 => "Arrhythmogenic right ventricular cardiomyopathy"
            1 => "Modified Fontaine ECG"
            2 => "Epsilon waves"
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            0 => "Miocardiopatia arritmog&#233;nica do ventr&#237;culo direito"
            1 => "Eletrocardiograma modificado de Fontaine"
            2 => "Ondas &#233;psilon"
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        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Arrhythmogenic right ventricular cardiomyopathy&#44; also known as arrhythmogenic right ventricular dysplasia&#44; is a condition in which myocardium is replaced by fibrous or fibrofatty tissue&#44; predominantly in the right ventricle&#46; It is clinically characterized by potentially lethal ventricular arrhythmias&#44; and is a leading cause of sudden cardiac death&#46; Its prevalence is not known exactly but is estimated at approximately 1&#58;5000 in the adult population&#46; Diagnosis can be on the basis of structural and functional alterations of the right ventricle&#44; electrocardiographic abnormalities &#40;including depolarization and repolarization alterations and ventricular arrhythmias&#41; and family history&#46; Diagnostic criteria facilitate the recognition and interpretation of non-specific clinical features of this disease&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">The authors present a case in which the diagnosis of arrhythmogenic right ventricular cardiomyopathy was prompted by the suspicion of right ventricular disease on transthoracic echocardiography&#46; This was confirmed by detection of epsilon waves on analysis of the ECG&#44; which generally go unnoticed but in this case were the key to the diagnosis&#46; Their presence was also shown by non-conventional ECG techniques such as modified Fontaine ECG&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The course of the disease culminated in the occurrence of ventricular tachycardia&#44; which prompted placement of an implantable cardioverter-defibrillator&#46;</p>"
      ]
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        "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A miocardiopatia arritmog&#233;nica do ventr&#237;culo direito &#8211; tamb&#233;m denominada de displasia arritmog&#233;nica do ventr&#237;culo direito &#8211; &#233; uma patologia em que se verifica a substitui&#231;&#227;o do mioc&#225;rdio por tecido fibroso ou fibroadiposo predominantemente no ventr&#237;culo direito e que se caracteriza clinicamente por arritmias ventriculares potencialmente letais&#44; sendo uma das causas mais relevantes de morte s&#250;bita card&#237;aca&#46; A sua preval&#234;ncia exata &#233; desconhecida&#44; no entanto&#44; estima-se que seja de cerca de 1&#58; 5&#46;000 na popula&#231;&#227;o adulta&#46; O diagn&#243;stico pode ser efetuado mediante a constata&#231;&#227;o de altera&#231;&#245;es estruturais e funcionais do ventr&#237;culo direito&#44; altera&#231;&#245;es eletrocardiogr&#225;ficas &#8211; da condu&#231;&#227;o em ECG basal&#44; arritmias ventriculares &#8211; e da hist&#243;ria familiar&#46; A exist&#234;ncia de crit&#233;rios de diagn&#243;stico facilita o reconhecimento e interpreta&#231;&#227;o das caracter&#237;sticas cl&#237;nicas n&#227;o espec&#237;ficas desta entidade&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Apresenta-se um caso cl&#237;nico em que o diagn&#243;stico de miocardiopatia arritmog&#233;nica do ventr&#237;culo direito foi desencadeado a partir da suspeita de patologia ventricular direita suscitada por ecocardiograma transtor&#225;cico&#46; A an&#225;lise do ECG serviu para o confirmar&#44; tendo-se detetado ondas &#233;psilon que em geral passam despercebidas&#44; mas que neste caso foram a chave para o diagn&#243;stico tendo-se tamb&#233;m aferido a sua exist&#234;ncia em t&#233;cnicas de ECG n&#227;o convencional como o ECG modificado de Fontaine&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">A evolu&#231;&#227;o subsequente do quadro cl&#237;nico culminou com a ocorr&#234;ncia de taquicardia ventricular o que motivou a implanta&#231;&#227;o de cardiodesfibrilhador implant&#225;vel &#40;CDI&#41;&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Please cite this article as&#58; Moreira D&#44; Delgado A&#44; Marmelo B&#44; et al&#46; Miocardiopatia arritmog&#233;nica do ventr&#237;culo direito&#46; Contribui&#231;&#227;o de diferentes t&#233;cnicas de eletrocardiografia&#46; Rev Port Cardiol&#46; 2014&#59;33&#58;243&#46;e1&#8211;243&#46;e7&#46;</p>"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Standard 12-lead ECG&#59; &#40;B&#41; detail of leads V<span class="elsevierStyleInf">1</span> to V<span class="elsevierStyleInf">3</span>&#46; Arrows indicate epsilon waves&#46;</p>"
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        "fuente" => "Adapted from Marcus FI et al&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">7</span></a>&#46;"
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          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Placement of electrodes in Fontaine ECG&#46; LA&#58; left arm electrode&#59; LL&#58; left leg electrode&#59; RA&#58; right arm electrode&#46;</p>"
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          "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Epsilon waves in some leads &#40;arrows&#41;&#58; &#40;A&#41; in right precordial leads&#59; &#40;B&#41; in modified Fontaine leads&#46; &#40;C&#41; Ventricular tachycardia with left bundle branch block morphology and superior axis&#46;</p>"
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          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Alterations documented by imaging studies&#58; &#40;A&#41; two-dimensional transthoracic echocardiogram&#59; &#40;B&#41; three-dimensional transthoracic echocardiogram&#59; &#40;C&#41; and &#40;D&#41; cardiac magnetic resonance&#46; Arrows indicate saccular dilatations in the right ventricular free wall&#46;</p>"
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        "fuente" => "Adapted from the 2010 revised Task Force criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy&#44; in the article &#8220;Clinical manifestations and diagnosis of arrhythmogenic right ventricular cardiomyopathy&#8221;&#44; available at <a class="elsevierStyleInterRef" id="intr0005" href="http://www.uptodate.com/">http&#58;&#47;&#47;www&#46;uptodate&#46;com&#47;</a>&#44; accessed October 1&#44; 2013&#46;"
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          "leyenda" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">ARVC&#58; arrhythmogenic right ventricular cardiomyopathy&#59; BSA&#58; body surface area&#59; MRI&#58; magnetic resonance imaging&#59; PLAX&#58; parasternal long-axis view&#59; PSAX&#58; parasternal short-axis view&#59; RV&#58; right ventricular&#59; RVOT&#58; right ventricular outflow tract&#59; SAECG&#58; signal-averaged ECG&#46;</p>"
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                  <table border="0" frame="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">I&#46; Global and&#47;or regional dysfunction and structural alterations</span></td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">By 2D echocardiography&#58;- Regional RV akinesia&#44; dyskinesia or aneurysm- and one of the following &#40;end diastole&#41;&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; PLAX RVOT &#8805;32 mm &#40;corrected for body size &#91;PLAX&#47;BSA&#93; &#8805;19 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; PSAX RVOT &#8805;36 mm &#40;corrected for body size &#91;PSAX&#47;BSA&#93; &#8805;21 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or fractional area change &#8804;33&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">By MRI&#58;- Regional RV akinesia&#44; dyskinesia or dyssynchronous RV contraction- and one of the following&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; Ratio of RV end-diastolic volume to BSA &#8805;110 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;male&#41; or &#8805;100 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;female&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or RV ejection fraction &#8804;40&#37;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By RV angiography&#58;- Regional RV akinesia&#44; dyskinesia or aneurysm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By 2D echocardiography&#58;- Regional RV akinesia or dyskinesia- and one of the following &#40;end diastole&#41;&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; PLAX RVOT &#8805;29 to &#60;32 mm &#40;corrected for body size &#91;PLAX&#47;BSA&#93; &#8805;16 to &#60;19 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; PSAX RVOT &#8805;32 to &#60;36 mm &#40;corrected for body size &#91;PSAX&#47;BSA&#93; &#8805;18 to &#60;21 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or fractional area change &#62;33&#37; to &#8804;40&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By MRI&#58;- Regional RV akinesia or dyskinesia or dyssynchronous RV contraction- and one of the following&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; Ratio of end-diastolic volume to BSA &#8805;100 to &#60;110 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;male&#41; or &#8805;90 to &#60;100 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;female&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or fractional area change &#62;40&#37; to &#8804;45&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">II&#46; Tissue characterization of ventricular wall</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Residual myocytes &#60;60&#37; by morphometric analysis &#40;or &#60;50&#37; if estimated&#41;&#44; with fibrous replacement of the RV free wall myocardium in &#8805;1 sample&#44; with or without fatty replacement of tissue on endomyocardial biopsy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Residual myocytes 60&#37; to 75&#37; by morphometric analysis &#40;or 50&#37; to 65&#37; if estimated&#41;&#44; with fibrous replacement of the RV free wall myocardium in &#8805;1 sample&#44; with or without fatty replacement of tissue on endomyocardial biopsy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">III&#46; Ventricular repolarization abnormalities</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Inverted T waves in right precordial leads &#40;V<span class="elsevierStyleInf">1</span>&#44; V<span class="elsevierStyleInf">2</span> and V<span class="elsevierStyleInf">3</span>&#41; or beyond in individuals &#62;14 years of age &#40;in the absence of complete right bundle branch block with QRS &#8805;120 ms&#41;&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Inverted T waves in leads V<span class="elsevierStyleInf">1</span> and V<span class="elsevierStyleInf">2</span> in individuals &#62;14 years of age &#40;in the absence of complete right bundle branch block with QRS &#8805;120 ms&#41; or in V<span class="elsevierStyleInf">4</span>&#44; V<span class="elsevierStyleInf">5</span> or V<span class="elsevierStyleInf">6</span>- Inverted T waves in leads V<span class="elsevierStyleInf">1</span>&#44; V<span class="elsevierStyleInf">2</span>&#44; V<span class="elsevierStyleInf">3</span> and V<span class="elsevierStyleInf">4</span> in individuals &#62;14 years of age in the presence of complete right bundle branch block with QRS &#8805;120 ms&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">IV&#46; Depolarization&#47;conduction abnormalities</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Epsilon wave in the right precordial leads &#40;V<span class="elsevierStyleInf">1</span> to V<span class="elsevierStyleInf">3</span>&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Late potentials by SAECG in &#8805;1 of 3 parameters in the absence of a QRS duration &#8805;110 ms on the standard 12-lead ECG<span class="elsevierStyleHsp" style=""></span>&#8211; Filtered QRS duration &#40;fQRS&#41; &#8805;114 ms<span class="elsevierStyleHsp" style=""></span>&#8211; Duration of terminal QRS &#60;40 &#956;V &#40;low-amplitude signal duration&#41; &#8805;38 ms<span class="elsevierStyleHsp" style=""></span>&#8211; Root-mean-square voltage of terminal 40 ms &#8804;20 &#956;V<span class="elsevierStyleHsp" style=""></span>&#8211; Terminal activation duration of QRS &#8805;55 ms measured from the nadir of the S wave to the end of the QRS&#44; including R&#8242;&#44; in V<span class="elsevierStyleInf">1</span>&#44; V<span class="elsevierStyleInf">2</span>&#44; V<span class="elsevierStyleInf">3</span>&#44; in the absence of complete right bundle branch block&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">V&#46; Arrhythmias</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Nonsustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis &#40;negative or indeterminate QRS in leads II&#44; III&#44; and aVF and positive in lead aVL&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Nonsustained or sustained ventricular tachycardia of RV outflow configuration&#44; left bundle-branch block morphology with inferior axis &#40;positive QRS in leads II&#44; III&#44; and aVF and negative in lead aVL&#41; or of unknown axis- &#62;500 ventricular extrasystoles per 24 hours &#40;Holter&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">VI&#46; Family history</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- ARVC confirmed in a first-degree relative who meets Task Force criteria- ARVC confirmed pathologically at autopsy or surgery in a first-degree relative- Identification of a pathogenic mutation<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> categorized as associated or probably associated with ARVC in the patient under evaluation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- History of ARVC in a first-degree relative in whom it is not possible or practical to determine whether the family member meets Task Force criteria- Premature sudden death &#40;&#60;35 years of age&#41; due to suspected ARVC in a first-degree relative- ARVC confirmed pathologically or by current Task Force criteria in second-degree relative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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            0 => array:3 [
              "identificador" => "tblfn0005"
              "etiqueta" => "a"
              "nota" => "<p class="elsevierStyleNotepara" id="npar0005">A pathogenic mutation is a DNA alteration associated with ARVC that alters or is expected to alter the encoded protein&#44; is unobserved or rare in a large non-ARVC control population&#44; and either alters or is predicted to alter the structure or function of the protein or has demonstrated linkage to the disease phenotype in a conclusive pedigree&#46;</p>"
            ]
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">2010 Revised Task Force diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy&#46;</p>"
        ]
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    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0005"
          "bibliografiaReferencia" => array:10 [
            0 => array:3 [
              "identificador" => "bib0005"
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "ACC&#47;AHA&#47;ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death&#58; a report of the American College of Cardiology&#47;American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines &#40;Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death&#41;"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "D&#46;P&#46; Zipes"
                            1 => "A&#46;J&#46; Camm"
                            2 => "M&#46; Borggrefe"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jacc.2006.07.010"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Am Coll Cardiol"
                        "fecha" => "2006"
                        "volumen" => "48"
                        "paginaInicial" => "e247"
                        "paginaFinal" => "e346"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16949478"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0010"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Arrhythmogenic right ventricular cardiomyopathy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "C&#46; Gemayel"
                            1 => "A&#46; Pelliccia"
                            2 => "P&#46;D&#46; Thompson"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "J Am Coll Cardiol"
                        "fecha" => "2001"
                        "volumen" => "38"
                        "paginaInicial" => "1773"
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                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11738273"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0015"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Screening for hypertrophic cardiomyopathy in young athletes"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "D&#46; Corrado"
                            1 => "C&#46; Basso"
                            2 => "M&#46; Schiavon"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1056/NEJM199808063390602"
                      "Revista" => array:6 [
                        "tituloSerie" => "N Engl J Med"
                        "fecha" => "1998"
                        "volumen" => "339"
                        "paginaInicial" => "364"
                        "paginaFinal" => "369"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9691102"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0020"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Natural history and risk stratification of arrhythmogenic right ventricular dysplasia&#47;cardiomyopathy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "J&#46;S&#46; Hulot"
                            1 => "X&#46; Jouven"
                            2 => "J&#46;P&#46; Empana"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1161/01.CIR.0000143375.93288.82"
                      "Revista" => array:6 [
                        "tituloSerie" => "Circulation"
                        "fecha" => "2004"
                        "volumen" => "110"
                        "paginaInicial" => "1879"
                        "paginaFinal" => "1884"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15451782"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0060"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Electrophysiological characteristics and outcome in patients with idiopathic right ventricular arrhythmia compared with arrhythmogenic right ventricular dysplasia"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "F&#46; Niroomand"
                            1 => "C&#46; Carbucicchio"
                            2 => "C&#46; Tondo"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Heart"
                        "fecha" => "2002"
                        "volumen" => "87"
                        "paginaInicial" => "41"
                        "paginaFinal" => "47"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11751663"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Diagnosis of arrhythmogenic right ventricular cardiomyopathy&#47;dysplasia&#58; proposed modification of the task force criteria"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "F&#46;I&#46; Marcus"
                            1 => "W&#46;J&#46; McKenna"
                            2 => "D&#46; Sherrill"
                          ]
                        ]
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Case report
Arrhythmogenic right ventricular cardiomyopathy: Contribution of different electrocardiographic techniques
Miocardiopatia arritmogénica do ventrículo direito. Contribuição de diferentes técnicas de eletrocardiografia
Davide Moreira
Corresponding author
davidasmoreira@gmail.com

Corresponding author.
, Anne Delgado, Bruno Marmelo, Emanuel Correia, Pedro Gama, João Pipa, Luís Nunes, Oliveira Santos
Serviço de Cardiologia, Centro Hospitalar Tondela-Viseu, Viseu, Portugal
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    "titulo" => "Arrhythmogenic right ventricular cardiomyopathy&#58; Contribution of different electrocardiographic techniques"
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        "autoresLista" => "Davide Moreira, Anne Delgado, Bruno Marmelo, Emanuel Correia, Pedro Gama, Jo&#227;o Pipa, Lu&#237;s Nunes, Oliveira Santos"
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        "titulo" => "Miocardiopatia arritmog&#233;nica do ventr&#237;culo direito&#46; Contribui&#231;&#227;o de diferentes t&#233;cnicas de eletrocardiografia"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Standard 12-lead ECG&#59; &#40;B&#41; detail of leads V<span class="elsevierStyleInf">1</span> to V<span class="elsevierStyleInf">3</span>&#46; Arrows indicate epsilon waves&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par9010" class="elsevierStylePara elsevierViewall">A 46-year-old woman was referred for cardiology consultation due to dilatation of the right chambers detected on routine transthoracic echocardiography&#46; The patient was asymptomatic and had no personal history of heart disease&#59; however&#44; she had a daughter diagnosed with arrhythmogenic right ventricular cardiomyopathy &#40;ARVC&#41; with left ventricular involvement&#44; who had an implantable cardioverter-defibrillator &#40;ICD&#41; and was being followed in a different hospital&#44; and another daughter without disease&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination showed no abnormalities&#46; Diagnostic exams included 12-lead electrocardiogram &#40;ECG&#41;&#44; which showed a pattern of right bundle branch block&#44; together with epsilon waves and T-wave inversion in leads V<span class="elsevierStyleInf">1</span>&#8211;V<span class="elsevierStyleInf">3</span> &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; In order to characterize the typical ARVC alterations&#44; a right-sided precordial lead ECG and a modified Fontaine ECG were performed&#44; the latter with the following placement of electrodes&#58; the right arm electrode over the manubrium&#44; the left arm electrode over the xiphoid and the left leg electrode in the area corresponding to V<span class="elsevierStyleInf">4</span>&#44; at a recording speed of 25 mm&#47;s and voltage of 10 mm&#47;mV &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46; Both methods clearly showed epsilon waves&#44; especially the modified Fontaine ECG &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>A and B&#41;&#46; Two- and three-dimensional transthoracic echocardiography at our institution showed severe right ventricular dilatation and hypokinesia&#44; with prominent apical trabeculae and false tendons and saccular dilatations in the ventricular free wall &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>A and B&#41;&#46; Cardiac magnetic resonance imaging revealed small foci of subepicardial fatty infiltration in the right ventricular free and inferior walls&#44; interventricular septum and left ventricular free wall&#59; late enhancement study showed focal enhancement in the interventricular septum consistent with fibrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>C and D&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Screening for mutations in genes coding for desmosomal proteins &#8211; plakophilin 2 &#40;PKP2&#41;&#44; desmoglein 2 and desmoplakin &#8211; revealed a nonsense mutation in exon 12 of the <span class="elsevierStyleItalic">PKP2</span> gene&#44; also present in the affected daughter&#46; This mutation is found in 11&#8211;43&#37; of patients with ARVC&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Since the patient remained asymptomatic and 24-hour Holter monitoring showed only pairs and a triplet of ventricular extrasystoles&#44; therapy with amiodarone 200 mg daily was begun and follow-up was scheduled&#46; Around a year later&#44; following an episode of fainting&#44; nonsustained ventricular tachycardia &#40;VT&#41; was documented&#44; with a pattern of left bundle branch block and superior axis &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>C&#41;&#46; This situation&#44; besides being a major criterion for a diagnosis of ARVC&#44; prompted placement of an ICD&#59; around a month after implantation&#44; an episode of VT was converted to sinus rhythm with an appropriate shock following unsuccessful antitachycardia pacing&#46; Around 18 months after this episode&#44; there has been no recurrence of ventricular arrhythmias&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">ARVC is characterized by ventricular arrhythmias and ventricular disease which is reflected macroscopically by replacement of myocardium by fibrous or fibrofatty tissue&#44; predominantly in the right ventricle but sometimes also involving the left ventricle&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Its estimated prevalence is 1&#58;5000 in the general population<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and it is a leading cause of sudden cardiac death &#40;SCD&#41;&#44; with an estimated incidence of 0&#46;08&#8211;9&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> However&#44; it may be a contributing factor in up to 10&#46;8&#37; of cases of SCD in young adults&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Clinical presentation is usually between the ages of 10 and 50&#44; with a mean age at diagnosis of 30&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The main symptoms are dizziness&#44; palpitations and syncope&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> but most patients are asymptomatic and the diagnosis is suspected following nonspecific ECG alterations&#44; echocardiographic abnormalities or documented ventricular arrhythmias&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">It is estimated that around 30&#37; of cases are familial&#46; Of the two inheritance patterns&#44; autosomal dominant is more common&#44; while in the autosomal recessive form&#44; termed Naxos disease&#44; ARVC is part of a cardiocutaneous syndrome that includes palmoplantar keratoderma and woolly hair&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">ARVC should be considered in patients with symptomatic or asymptomatic VT of left bundle branch block morphology in the absence of apparent heart disease&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> A definitive diagnosis requires histological evidence of replacement of right ventricular &#40;RV&#41; myocardium by fibrofatty tissue&#59; however&#44; assessment of this criterion is not practical in clinical practice&#44; and so other data are used for the purpose&#46; The first consensus document detailing diagnostic criteria for this entity appeared in 1994&#44; and a revised version was published in 2010<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">6</span></a> &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46; A definitive diagnosis is made on the basis of two major criteria&#44; one major and two minor criteria&#44; or four minor criteria in different categories&#46; A borderline diagnosis requires one major and one minor criterion or three minor criteria in different categories&#44; and one major criterion or two minor criteria in different categories indicates a possible diagnosis&#46; In the case presented&#44; the patient met the criteria of a family history of ARVC and epsilon waves on 12-lead ECG&#44; which were sufficient for the diagnosis&#46; The latter ECG finding is found in 30&#37; of patients with ARVC&#44; and reflects low-amplitude potentials due to late activation of some parts of the right ventricle&#46; Typically&#44; the epsilon waves are most easily identified in leads V<span class="elsevierStyleInf">1</span> to V<span class="elsevierStyleInf">3</span>&#44; as well as in the right precordial leads&#44; by doubling the sensitivity of the recording and using a filter setting of 40 Hz instead of 150 Hz to decrease the noise level&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">7</span></a> Nevertheless&#44; they are not specific for ARVC since they may be found in cases of RV abnormalities arising from myocardial infarction or cardiac sarcoidosis&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">7</span></a> The usefulness of the Fontaine ECG resides in its greater ability to unmask epsilon waves compared to standard 12-lead ECG and right-sided precordial lead ECG&#59; according to Wang et al&#46;&#44; the modified Fontaine ECG doubles or triples the rate of detection of epsilon waves compared to standard 12-lead ECG&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">8</span></a> One explanation for this may be that it records potentials developed in the right ventricle from the infundibulum to the area of the diaphragm&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">8</span></a> This method is thus particularly useful in cases of suspected ARVC&#44; particularly in the presence of right bundle branch block&#44; in which the detection of epsilon waves is even more important since a QRS duration &#62;110 ms does not allow other ECG criteria that are potentially useful in diagnosing this entity to be assessed&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">7</span></a> Although in this particular case the Fontaine ECG was not crucial to the diagnosis&#44; its ability to detect epsilon waves was the main reason for using this simple but little known electrocardiographic technique&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0050" class="elsevierStylePara elsevierViewall">While magnetic resonance imaging was not mandatory in the case presented following transthoracic echocardiography&#44; we decided to perform it due to its feasibility and its value in characterization of the imaging features of this cardiomyopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">9</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Although mutations in the genes coding for desmosomal proteins are relatively common in patients with suspected ARVC&#44; the usefulness of genetic study remains the subject of debate&#46; The prognostic implications of early identification of affected individuals are unclear given its low penetrance and highly variable expression according to age&#46; A minority of patients suffer arrhythmic events in the absence of previous symptoms or clinical signs of the disease&#46; Many do not in fact develop clinically significant disease&#44; and most of those that do have a relatively benign course&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">6&#44;10</span></a> In the present case&#44; genetic study was performed mainly due to the patient&#39;s family history&#44; and its results did not affect the therapeutic approach adopted&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">With regard to therapeutic management&#44; it is debatable whether antiarrhythmics should be used in asymptomatic patients with ventricular ectopic activity&#46; According to the ACC&#47;AHA&#47;ESC guidelines&#44; amiodarone and sotalol are only indicated &#40;class IIa recommendation&#44; level of evidence C&#41; to treat VT or ventricular fibrillation &#40;VF&#41; when an ICD is not possible&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> a fact that was taken into account during internal review of the approach to adopt in the present case&#46; ICD implantation for secondary prevention following documented VT or VF is a class I recommendation&#44; level of evidence B&#44; while implantation for primary prevention in patients with high-risk alterations such as extensive RV involvement&#44; left ventricular involvement or unexplained syncope assumed to be due to tachyarrhythmia is a class IIa recommendation&#44; level of evidence C&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The case presented highlights the importance of information on family history and of the data provided by widely available diagnostic exams such as ECG and echocardiography&#46; Different ECG techniques that are simple to perform can be useful for initial assessment by demonstrating the presence of a major diagnostic criterion for this cardiomyopathy&#46; Thus&#44; one of the main points of interest in this case report is the potential role of modified Fontaine ECG leads in the diagnosis of ARVC&#59; another is the natural history of the disease&#44; with the patient suffering potentially fatal VT&#44; a major criterion for the diagnosis&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Ethical disclosures</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Protection of human and animal subjects</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Confidentiality of data</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Right to privacy and informed consent</span><p id="par0080" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conflicts of interest</span><p id="par0085" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Arrhythmogenic right ventricular cardiomyopathy&#44; also known as arrhythmogenic right ventricular dysplasia&#44; is a condition in which myocardium is replaced by fibrous or fibrofatty tissue&#44; predominantly in the right ventricle&#46; It is clinically characterized by potentially lethal ventricular arrhythmias&#44; and is a leading cause of sudden cardiac death&#46; Its prevalence is not known exactly but is estimated at approximately 1&#58;5000 in the adult population&#46; Diagnosis can be on the basis of structural and functional alterations of the right ventricle&#44; electrocardiographic abnormalities &#40;including depolarization and repolarization alterations and ventricular arrhythmias&#41; and family history&#46; Diagnostic criteria facilitate the recognition and interpretation of non-specific clinical features of this disease&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">The authors present a case in which the diagnosis of arrhythmogenic right ventricular cardiomyopathy was prompted by the suspicion of right ventricular disease on transthoracic echocardiography&#46; This was confirmed by detection of epsilon waves on analysis of the ECG&#44; which generally go unnoticed but in this case were the key to the diagnosis&#46; Their presence was also shown by non-conventional ECG techniques such as modified Fontaine ECG&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The course of the disease culminated in the occurrence of ventricular tachycardia&#44; which prompted placement of an implantable cardioverter-defibrillator&#46;</p>"
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        "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A miocardiopatia arritmog&#233;nica do ventr&#237;culo direito &#8211; tamb&#233;m denominada de displasia arritmog&#233;nica do ventr&#237;culo direito &#8211; &#233; uma patologia em que se verifica a substitui&#231;&#227;o do mioc&#225;rdio por tecido fibroso ou fibroadiposo predominantemente no ventr&#237;culo direito e que se caracteriza clinicamente por arritmias ventriculares potencialmente letais&#44; sendo uma das causas mais relevantes de morte s&#250;bita card&#237;aca&#46; A sua preval&#234;ncia exata &#233; desconhecida&#44; no entanto&#44; estima-se que seja de cerca de 1&#58; 5&#46;000 na popula&#231;&#227;o adulta&#46; O diagn&#243;stico pode ser efetuado mediante a constata&#231;&#227;o de altera&#231;&#245;es estruturais e funcionais do ventr&#237;culo direito&#44; altera&#231;&#245;es eletrocardiogr&#225;ficas &#8211; da condu&#231;&#227;o em ECG basal&#44; arritmias ventriculares &#8211; e da hist&#243;ria familiar&#46; A exist&#234;ncia de crit&#233;rios de diagn&#243;stico facilita o reconhecimento e interpreta&#231;&#227;o das caracter&#237;sticas cl&#237;nicas n&#227;o espec&#237;ficas desta entidade&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Apresenta-se um caso cl&#237;nico em que o diagn&#243;stico de miocardiopatia arritmog&#233;nica do ventr&#237;culo direito foi desencadeado a partir da suspeita de patologia ventricular direita suscitada por ecocardiograma transtor&#225;cico&#46; A an&#225;lise do ECG serviu para o confirmar&#44; tendo-se detetado ondas &#233;psilon que em geral passam despercebidas&#44; mas que neste caso foram a chave para o diagn&#243;stico tendo-se tamb&#233;m aferido a sua exist&#234;ncia em t&#233;cnicas de ECG n&#227;o convencional como o ECG modificado de Fontaine&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">A evolu&#231;&#227;o subsequente do quadro cl&#237;nico culminou com a ocorr&#234;ncia de taquicardia ventricular o que motivou a implanta&#231;&#227;o de cardiodesfibrilhador implant&#225;vel &#40;CDI&#41;&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Please cite this article as&#58; Moreira D&#44; Delgado A&#44; Marmelo B&#44; et al&#46; Miocardiopatia arritmog&#233;nica do ventr&#237;culo direito&#46; Contribui&#231;&#227;o de diferentes t&#233;cnicas de eletrocardiografia&#46; Rev Port Cardiol&#46; 2014&#59;33&#58;243&#46;e1&#8211;243&#46;e7&#46;</p>"
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        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Alterations documented by imaging studies&#58; &#40;A&#41; two-dimensional transthoracic echocardiogram&#59; &#40;B&#41; three-dimensional transthoracic echocardiogram&#59; &#40;C&#41; and &#40;D&#41; cardiac magnetic resonance&#46; Arrows indicate saccular dilatations in the right ventricular free wall&#46;</p>"
        ]
      ]
      4 => array:8 [
        "identificador" => "tbl0005"
        "etiqueta" => "Table 1"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "fuente" => "Adapted from the 2010 revised Task Force criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy&#44; in the article &#8220;Clinical manifestations and diagnosis of arrhythmogenic right ventricular cardiomyopathy&#8221;&#44; available at <a class="elsevierStyleInterRef" id="intr0005" href="http://www.uptodate.com/">http&#58;&#47;&#47;www&#46;uptodate&#46;com&#47;</a>&#44; accessed October 1&#44; 2013&#46;"
        "tabla" => array:3 [
          "leyenda" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">ARVC&#58; arrhythmogenic right ventricular cardiomyopathy&#59; BSA&#58; body surface area&#59; MRI&#58; magnetic resonance imaging&#59; PLAX&#58; parasternal long-axis view&#59; PSAX&#58; parasternal short-axis view&#59; RV&#58; right ventricular&#59; RVOT&#58; right ventricular outflow tract&#59; SAECG&#58; signal-averaged ECG&#46;</p>"
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">I&#46; Global and&#47;or regional dysfunction and structural alterations</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By 2D echocardiography&#58;- Regional RV akinesia&#44; dyskinesia or aneurysm- and one of the following &#40;end diastole&#41;&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; PLAX RVOT &#8805;32 mm &#40;corrected for body size &#91;PLAX&#47;BSA&#93; &#8805;19 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; PSAX RVOT &#8805;36 mm &#40;corrected for body size &#91;PSAX&#47;BSA&#93; &#8805;21 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or fractional area change &#8804;33&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By MRI&#58;- Regional RV akinesia&#44; dyskinesia or dyssynchronous RV contraction- and one of the following&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; Ratio of RV end-diastolic volume to BSA &#8805;110 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;male&#41; or &#8805;100 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;female&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or RV ejection fraction &#8804;40&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By RV angiography&#58;- Regional RV akinesia&#44; dyskinesia or aneurysm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By 2D echocardiography&#58;- Regional RV akinesia or dyskinesia- and one of the following &#40;end diastole&#41;&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; PLAX RVOT &#8805;29 to &#60;32 mm &#40;corrected for body size &#91;PLAX&#47;BSA&#93; &#8805;16 to &#60;19 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; PSAX RVOT &#8805;32 to &#60;36 mm &#40;corrected for body size &#91;PSAX&#47;BSA&#93; &#8805;18 to &#60;21 mm&#47;m<span class="elsevierStyleSup">2</span>&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or fractional area change &#62;33&#37; to &#8804;40&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">By MRI&#58;- Regional RV akinesia or dyskinesia or dyssynchronous RV contraction- and one of the following&#58;<span class="elsevierStyleHsp" style=""></span>&#8211; Ratio of end-diastolic volume to BSA &#8805;100 to &#60;110 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;male&#41; or &#8805;90 to &#60;100 ml&#47;m<span class="elsevierStyleSup">2</span> &#40;female&#41;<span class="elsevierStyleHsp" style=""></span>&#8211; or fractional area change &#62;40&#37; to &#8804;45&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">II&#46; Tissue characterization of ventricular wall</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Residual myocytes &#60;60&#37; by morphometric analysis &#40;or &#60;50&#37; if estimated&#41;&#44; with fibrous replacement of the RV free wall myocardium in &#8805;1 sample&#44; with or without fatty replacement of tissue on endomyocardial biopsy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Residual myocytes 60&#37; to 75&#37; by morphometric analysis &#40;or 50&#37; to 65&#37; if estimated&#41;&#44; with fibrous replacement of the RV free wall myocardium in &#8805;1 sample&#44; with or without fatty replacement of tissue on endomyocardial biopsy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">III&#46; Ventricular repolarization abnormalities</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Inverted T waves in right precordial leads &#40;V<span class="elsevierStyleInf">1</span>&#44; V<span class="elsevierStyleInf">2</span> and V<span class="elsevierStyleInf">3</span>&#41; or beyond in individuals &#62;14 years of age &#40;in the absence of complete right bundle branch block with QRS &#8805;120 ms&#41;&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Inverted T waves in leads V<span class="elsevierStyleInf">1</span> and V<span class="elsevierStyleInf">2</span> in individuals &#62;14 years of age &#40;in the absence of complete right bundle branch block with QRS &#8805;120 ms&#41; or in V<span class="elsevierStyleInf">4</span>&#44; V<span class="elsevierStyleInf">5</span> or V<span class="elsevierStyleInf">6</span>- Inverted T waves in leads V<span class="elsevierStyleInf">1</span>&#44; V<span class="elsevierStyleInf">2</span>&#44; V<span class="elsevierStyleInf">3</span> and V<span class="elsevierStyleInf">4</span> in individuals &#62;14 years of age in the presence of complete right bundle branch block with QRS &#8805;120 ms&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">IV&#46; Depolarization&#47;conduction abnormalities</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Epsilon wave in the right precordial leads &#40;V<span class="elsevierStyleInf">1</span> to V<span class="elsevierStyleInf">3</span>&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Late potentials by SAECG in &#8805;1 of 3 parameters in the absence of a QRS duration &#8805;110 ms on the standard 12-lead ECG<span class="elsevierStyleHsp" style=""></span>&#8211; Filtered QRS duration &#40;fQRS&#41; &#8805;114 ms<span class="elsevierStyleHsp" style=""></span>&#8211; Duration of terminal QRS &#60;40 &#956;V &#40;low-amplitude signal duration&#41; &#8805;38 ms<span class="elsevierStyleHsp" style=""></span>&#8211; Root-mean-square voltage of terminal 40 ms &#8804;20 &#956;V<span class="elsevierStyleHsp" style=""></span>&#8211; Terminal activation duration of QRS &#8805;55 ms measured from the nadir of the S wave to the end of the QRS&#44; including R&#8242;&#44; in V<span class="elsevierStyleInf">1</span>&#44; V<span class="elsevierStyleInf">2</span>&#44; V<span class="elsevierStyleInf">3</span>&#44; in the absence of complete right bundle branch block&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">V&#46; Arrhythmias</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Nonsustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis &#40;negative or indeterminate QRS in leads II&#44; III&#44; and aVF and positive in lead aVL&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- Nonsustained or sustained ventricular tachycardia of RV outflow configuration&#44; left bundle-branch block morphology with inferior axis &#40;positive QRS in leads II&#44; III&#44; and aVF and negative in lead aVL&#41; or of unknown axis- &#62;500 ventricular extrasystoles per 24 hours &#40;Holter&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">VI&#46; Family history</span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- ARVC confirmed in a first-degree relative who meets Task Force criteria- ARVC confirmed pathologically at autopsy or surgery in a first-degree relative- Identification of a pathogenic mutation<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> categorized as associated or probably associated with ARVC in the patient under evaluation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">- History of ARVC in a first-degree relative in whom it is not possible or practical to determine whether the family member meets Task Force criteria- Premature sudden death &#40;&#60;35 years of age&#41; due to suspected ARVC in a first-degree relative- ARVC confirmed pathologically or by current Task Force criteria in second-degree relative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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              "identificador" => "tblfn0005"
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              "nota" => "<p class="elsevierStyleNotepara" id="npar0005">A pathogenic mutation is a DNA alteration associated with ARVC that alters or is expected to alter the encoded protein&#44; is unobserved or rare in a large non-ARVC control population&#44; and either alters or is predicted to alter the structure or function of the protein or has demonstrated linkage to the disease phenotype in a conclusive pedigree&#46;</p>"
            ]
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        "descripcion" => array:1 [
          "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">2010 Revised Task Force diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy&#46;</p>"
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      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0005"
          "bibliografiaReferencia" => array:10 [
            0 => array:3 [
              "identificador" => "bib0005"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "ACC&#47;AHA&#47;ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death&#58; a report of the American College of Cardiology&#47;American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines &#40;Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death&#41;"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "D&#46;P&#46; Zipes"
                            1 => "A&#46;J&#46; Camm"
                            2 => "M&#46; Borggrefe"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jacc.2006.07.010"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Am Coll Cardiol"
                        "fecha" => "2006"
                        "volumen" => "48"
                        "paginaInicial" => "e247"
                        "paginaFinal" => "e346"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16949478"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0010"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Arrhythmogenic right ventricular cardiomyopathy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "C&#46; Gemayel"
                            1 => "A&#46; Pelliccia"
                            2 => "P&#46;D&#46; Thompson"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
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Revista Portuguesa de Cardiologia (English edition)
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