<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1688-1249</journal-id>
<journal-title><![CDATA[Archivos de Pediatría del Uruguay]]></journal-title>
<abbrev-journal-title><![CDATA[Arch. Pediatr. Urug.]]></abbrev-journal-title>
<issn>1688-1249</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Uruguaya de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1688-12492020000400237</article-id>
<article-id pub-id-type="doi">10.31134/ap.91.4.6</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Miocardiopatía arritmogénica del ventrículo derecho en pediatría]]></article-title>
<article-title xml:lang="en"><![CDATA[Arrhythmogenic right ventricular cardiomyopathy in pediatrics]]></article-title>
<article-title xml:lang="pt"><![CDATA[Cardiomiopatia arritmogênica do ventrículo direito em pacientes pediátricos]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pérez]]></surname>
<given-names><![CDATA[Joaquín]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Maurente]]></surname>
<given-names><![CDATA[Lucía]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pérez]]></surname>
<given-names><![CDATA[Bettina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Koziol]]></surname>
<given-names><![CDATA[Silvia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ferreira]]></surname>
<given-names><![CDATA[Carina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Arana]]></surname>
<given-names><![CDATA[Maite]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Santoro]]></surname>
<given-names><![CDATA[Anabella]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Abdala]]></surname>
<given-names><![CDATA[Diego]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,UDELAR Facultad de Medicina Pediatría]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica &#8220;B&#8221;]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,UDELAR Facultad de Medicina ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica &#8220;C&#8221;]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,UDELAR Facultad de Medicina ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica &#8220;C&#8221;]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af7">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica &#8220;C&#8221;]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af8">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica &#8220;B&#8221;]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>00</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>00</month>
<year>2020</year>
</pub-date>
<volume>91</volume>
<numero>4</numero>
<fpage>237</fpage>
<lpage>242</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S1688-12492020000400237&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S1688-12492020000400237&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S1688-12492020000400237&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen:  Introducción:  la clasificación clásica de las miocardiopatías incluye la hipertrófica, dilatada, restrictiva y no compactada. La miocardiopatía arritmogénica del ventrículo derecho (MAVD) es una variedad hereditaria, infiltrativa, con sustitución progresiva de miocardio ventricular por tejido fibroadiposo que afecta principalmente al ventrículo derecho. Si bien los síntomas son infrecuentes en la edad pediátrica, estos pacientes presentan más episodios de muerte súbita (MS) que los adultos.  Caso clínico: paciente de 12 años, de sexo masculino, portador de miocardiopatía que se manifiesta por arritmia ventricular a los siete años de vida (extrasístoles y taquicardia ventricular no sostenida). La paraclínica realizada fue compatible con MAVD, por lo que se decide realizar un estudio electrofisiológico con ablación. El procedimiento logró una leve mejoría transitoria. Sin embargo no se logró eliminar la arritmia, por lo que se implantó un desfibrilador automático. Éste no ha presentado descargas en dos años de evolución.  Discusión:  para el diagnóstico de nuestro paciente se utilizaron los criterios establecidos por la Task Force 2010. El diagnóstico imagenológico está basado en la demostración de alteraciones morfológicas (aneurismas) o alteraciones funcionales (aquinesia, disquinesia o asincronía de la pared), que traducen la sustitución fibroadiposa del miocardio. La presentación de arritmias de origen en el ventrículo derecho de difícil manejo farmacológico determinaron la ablación y luego la colocación de un desfibrilador.  Conclusiones: se trata de una patología infrecuente pero de muy mal pronóstico por la severidad de las arritmias que se desencadenan en la evolución. El tratamiento de mayor efectividad es la colocación de un cardiodesfibrilador.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Summary:  Introduction: the classical cardiomyopathy phenotypic classification includes hypertrophic, dilated, restrictive and non-compacted types. Arrythmogenic Right Ventricle Cardiomyopathy (ARVC) is a hereditary, infiltrative variety that causes the progressive replacement of ventricular myocardium with fibroadipose tissue that mainly affects the right ventricle.1-2-3 Although the onset of symptoms is uncommon in children, they seem to show more sudden death episodes than adults.  Clinical case: 12 year-old male, with arrhythmogenic right ventricle cardiomyopathy (ARVC), showing ventricular arrhythmia at 7 years of age, (extrasystoles and unsustained ventricular tachycardia). Further studies confirmed electrical abnormality, so we performed an electrophysiological study with ablation. The procedure resulted in a slight temporary improvement, however, but the arrhythmia was not completely eliminated. This maintained the risk of sudden death, so we implanted a portable cardiodesfibrilador, which did not record discharges since then.  Discussion . for this patient&#8217;s diagnosis we used the criteria set by Task Force 2010.4 The imaging diagnosis was based on the demonstration of morphological (aneurysms) or functional alterations; akinesia, dyskinesia or asynchrony of the wall, which show myocardium fibrofatty substitution. 3 Recurring right ventricle arrhythmias, despite intense pharmacological treatment, led to a radiofrequency myocardium ablation and later to the placement of an implantable cardiodesfibrillator.  Conclusions:  it is a rare pathology with extremely poor prognosis due to the severe and uncontrollable arrhythmias triggered by the altered myocardium tissue. The most effective treatment is the placement of a cardiodesfibrillator.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo:  Introdução: a classificação fenotípica clássica da cardiomiopatia inclui os tipos hipertrófico, dilatado, restritivo e não compactado. A cardiomiopatia arritmogênica do ventrículo direito (CAVD) é uma variedade hereditária infiltrativa que causa a substituição progressiva do miocárdio ventricular por tecido fibro-gorduroso que afeta principalmente o ventrículo direito. Embora o início dos sintomas seja incomum em crianças, elas parecem apresentar mais episódios de morte súbita do que adultos.  Caso clínico: menino de 12 anos, com cardiomiopatia arritmogênica de ventrículo direito (CAVD), apresentou arritmia ventricular aos 7 anos de idade (extrassístoles e taquicardia ventricular não sustentada). Novos estudos confirmaram anormalidade elétrica, por isso realizamos um estudo eletrofisiológico com ablação. O procedimento resultou numa ligeira melhora temporária, porém, a arritmia não foi completamente eliminada. Isso manteve o risco de morte súbita, por isso implantamos um cardiodesfibrilador portátil, que desde então não tem registrado descargas.  Discussão: para o diagnóstico do paciente, utilizamos os critérios estabelecidos pela Força-Tarefa 2010. O diagnóstico imagenológico foi baseado na demonstração de alterações morfológicas (aneurismas) ou funcionais; acinesia, discinesia ou assincronia da parede, que mostraram substituição fibrogordurosa do miocárdio. As arritmias de ventrículo direito recorrentes, apesar do intenso tratamento farmacológico, levaram à ablação do miocárdio por radiofrequência e, posteriormente, à colocação dum cardiodesfibrilador implantável.  Conclusões:  a CAVD é uma patologia rara de prognóstico extremamente ruim devido às arritmias graves e incontroláveis desencadeadas pelo tecido miocárdico alterado. O tratamento mais eficaz é a colocação de cardiodesfibrilador.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Displasia ventricular derecha arritmogénica]]></kwd>
<kwd lng="es"><![CDATA[Pediatría]]></kwd>
<kwd lng="en"><![CDATA[Arrhythmogenic right ventricular dysplasia]]></kwd>
<kwd lng="en"><![CDATA[Pediatrics]]></kwd>
<kwd lng="pt"><![CDATA[Displasia arritmogênica ventricular direita]]></kwd>
<kwd lng="pt"><![CDATA[Pediatria]]></kwd>
</kwd-group>
</article-meta>
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