<?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>2182-5173</journal-id>
<journal-title><![CDATA[Revista Portuguesa de Medicina Geral e Familiar]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Port Med Geral Fam]]></abbrev-journal-title>
<issn>2182-5173</issn>
<publisher>
<publisher-name><![CDATA[Associação Portuguesa de Medicina Geral e Familiar]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2182-51732021000200170</article-id>
<article-id pub-id-type="doi">10.32385/rpmgf.v37i2.12749</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[Síndroma de QT Longo: relato de caso de uma causa rara de síncope em idade pediátrica.]]></article-title>
<article-title xml:lang="en"><![CDATA[Long qt syndrome: a case report of a rare cause of syncope in pediatrics.]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rebelo]]></surname>
<given-names><![CDATA[Alicia Raquel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lopes]]></surname>
<given-names><![CDATA[Andreia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Magalhães]]></surname>
<given-names><![CDATA[Catarina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sarmento]]></surname>
<given-names><![CDATA[João]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Salgado]]></surname>
<given-names><![CDATA[Miguel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Senhora da Oliveira  ]]></institution>
<addr-line><![CDATA[Guimarães ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Senhora da Oliveira  ]]></institution>
<addr-line><![CDATA[Guimarães ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidade do Porto Faculdade de Medicina Centro Hospitalar Universitário São João]]></institution>
<addr-line><![CDATA[Porto ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>30</day>
<month>04</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>04</month>
<year>2021</year>
</pub-date>
<volume>37</volume>
<numero>2</numero>
<fpage>170</fpage>
<lpage>173</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2182-51732021000200170&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2182-51732021000200170&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2182-51732021000200170&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo  Introdução:  A síncope é um motivo frequente de preocupação em idade pediátrica. Apesar da sua etiologia ser maioritariamente benigna, é essencial excluir causas graves e potencialmente fatais que, embora raras, devem fazer parte do diagnóstico diferencial destas situações. Para isso, é necessário existir um elevado índice de suspeição.  Descrição de caso:  Adolescente de 12 anos, previamente saudável, observado no serviço de urgência (SU) por primeiro episódio de perda de consciência (PC) com cinco minutos de duração e hipertonia generalizada. Mãe com episódios de PC classificada como epilepsia, sem outros antecedentes familiares relevantes, nomeadamente sem história familiar de patologia cardíaca em idade jovem ou morte súbita. Exame objetivo e eletrocardiograma (ECG) sem alterações, referenciado à consulta de neuropediatria onde realizou eletroencefalograma normal. Apresentou novo episódio de PC 18 meses depois, com recuperação espontânea em segundos, recorrendo ao SU. Repetiu ECG - ritmo sinusal e intervalo QT corrigido de 511 ms, tendo-se diagnosticado síndroma de QT Longo. Foi referenciado a consulta de cardiologia pediátrica e genética, tendo sido identificada mutação genética e medicado com beta-bloqueador. Após revisão da história clínica materna foi pedida avaliação por cardiologia, com confirmação do mesmo diagnóstico na mãe e na irmã.  Comentário:  A síndroma de QT Longo caracteriza-se por um defeito da repolarização do miocárdio, associado a risco aumentado de morte súbita. As manifestações clínicas variam, desde ausência de sintomas até palpitações, síncope ou paragem cardíaca. Apesar da raridade, o diagnóstico é acessível através do ECG. No estudo de uma PC, a existência de um traçado de ECG prévio normal não invalida a sua repetição, uma vez que a alteração do intervalo QT pode ser inconstante. A existência de problemas cardíacos na família favorece a hipótese de síncope de causa cardíaca. Em caso de diagnóstico primário na criança deve ser efetuado um rastreio familiar de modo a despistar outros casos eventuais.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Introduction:  Syncope is a frequent motive of concern in pediatrics. Despite its mostly benign etiology, it is fundamental to exclude life-threatening causes.  Case report:  We describe a 12-year-old boy brought to the emergency department because of a first syncopal event that lasted for five minutes with generalized hypertonia. His mother had a history of syncopal episodes who were classified as being epileptic, and there were no other relevant findings on family history. Physical exam and electrocardiogram (ECG) showed no anomalies. He was referred to a neuropediatric consult, where he underwent an electroencephalogram with a normal result. Eighteen months later he experienced another syncopal event that lasted a few seconds. He then repeated an ECG that showed sinus rhythm and a QTc of 511ms, leading to a diagnosis of long QT syndrome. He was referred to both pediatric cardiology and genetics consults, with identification of a genetic mutation, and was started on beta-blocker therapy. After studying his family, both his mother and his sister were confirmed as having the same diagnosis.  Comments:  Long QT syndrome is defined as a disorder of myocardial repolarization, associated with an increased risk of sudden death. Children may range from being asymptomatic to experiencing palpitations, syncope, or even cardiac arrest. Despite its rarity, the diagnosis may be suspected on a regular ECG. The approach of a syncopal event in a child with a previous normal ECG does not exclude the diagnosis, as this electrocardiographic change can be intermittent. A positive family history supports the diagnosis. When the child is the first described case, his relatives should be evaluated.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Adolescente]]></kwd>
<kwd lng="pt"><![CDATA[Síncope]]></kwd>
<kwd lng="pt"><![CDATA[QT Longo]]></kwd>
<kwd lng="en"><![CDATA[Adolescent]]></kwd>
<kwd lng="en"><![CDATA[Syncope]]></kwd>
<kwd lng="en"><![CDATA[Long QT syndrome]]></kwd>
</kwd-group>
</article-meta>
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