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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Introdu&#231;&#227;o</span><p id="par0105" class="elsevierStylePara elsevierViewall">A miocardiopatia hipertr&#243;fica &#40;MCH&#41; &#233; uma patologia de transmiss&#227;o essencialmente autoss&#243;mica dominante&#44; express&#227;o cl&#237;nica vari&#225;vel e penetr&#226;ncia incompleta&#44; dependente da idade<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">1</span></a>&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">As manifesta&#231;&#245;es cl&#237;nicas desta patologia s&#227;o igualmente heterog&#233;neas&#44; desde a aus&#234;ncia de sintomas at&#233; &#224; apresenta&#231;&#227;o com sinais francos de insufici&#234;ncia card&#237;aca ou morte s&#250;bita<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">2</span></a>&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">O objetivo do rastreio familiar &#233; o de identificar a ocorr&#234;ncia ou o risco de desenvolvimento da doen&#231;a nos parentes em primeiro grau do caso &#237;ndex&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">As mais recentes normas de orienta&#231;&#227;o da <span class="elsevierStyleItalic">European Society of Cardiology</span> &#40;ESC&#41; e da <span class="elsevierStyleItalic">American College of Cardiology and American Heart Association</span> &#40;ACCF&#47;AHA&#41; recomendam a avalia&#231;&#227;o dos familiares em idade pedi&#225;trica a partir dos 10&#8208;12 anos<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">3&#44;4</span></a>&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Estima&#8208;se que em cerca de 50&#8208;60&#37; dos casos de MCH familiar seja poss&#237;vel identificar uma muta&#231;&#227;o nos genes que codificam as prote&#237;nas sarcom&#233;ricas<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">5</span></a>&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">No entanto&#44; em crian&#231;as com fen&#243;tipo negativo&#44; o valor progn&#243;stico da identifica&#231;&#227;o de muta&#231;&#245;es de genes sarcom&#233;ricos permanece incerto&#46;</p><p id="par0135" class="elsevierStylePara elsevierViewall">Avaliaram&#8208;se os resultados do rastreio pedi&#225;trico de MCH familiar num centro de referencia&#231;&#227;o terci&#225;ria de cardiologia pedi&#225;trica e o valor preditivo do estudo gen&#233;tico neste contexto&#46; Foi ainda aferida a penetr&#226;ncia fenot&#237;pica da doen&#231;a&#44; ao longo do tempo de seguimento destas crian&#231;as&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">M&#233;todos</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Popula&#231;&#227;o em estudo</span><p id="par0140" class="elsevierStylePara elsevierViewall">Todos os casos de MCH familiar foram seguidos num centro de referencia&#231;&#227;o terci&#225;ria de cardiologia pedi&#225;trica&#44; em consulta dedicada&#44; entre 2004&#8208;2013&#46; Foram inclu&#237;das crian&#231;as com idade inferior a 18 anos&#44; pertencentes a fam&#237;lias em que o caso &#237;ndex tinha estudo gen&#233;tico positivo para muta&#231;&#227;o de gene sarcom&#233;rico&#46;</p><p id="par0145" class="elsevierStylePara elsevierViewall">A referencia&#231;&#227;o dos descendentes em primeiro grau do caso &#237;ndex diagnosticado com MCH foi feita&#44; primariamente&#44; atrav&#233;s da consulta de cardiologia do mesmo centro&#46;</p><p id="par0150" class="elsevierStylePara elsevierViewall">Nos casos em que o caso &#237;ndex era uma crian&#231;a&#44; os seus irm&#227;os foram referenciados &#224; consulta de cardiologia pedi&#225;trica e os pais &#224; consulta de cardiologia&#46;</p><p id="par0155" class="elsevierStylePara elsevierViewall">Todos os doentes foram ainda referenciados &#224; consulta de gen&#233;tica m&#233;dica do mesmo centro&#44; para aconselhamento gen&#233;tico&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Avalia&#231;&#227;o cl&#237;nica e estudo gen&#233;tico</span><p id="par0160" class="elsevierStylePara elsevierViewall">A avalia&#231;&#227;o inicial desta popula&#231;&#227;o incluiu observa&#231;&#227;o cl&#237;nica&#44; eletrocardiograma &#40;ECG&#41; de 12 deriva&#231;&#245;es em repouso&#44; ecocardiograma transtor&#225;cico e teste gen&#233;tico para as oito muta&#231;&#245;es de genes sarcom&#233;ricos mais frequentes &#40;<span class="elsevierStyleItalic">MYH7</span>&#44; <span class="elsevierStyleItalic">MYL2</span>&#44; <span class="elsevierStyleItalic">MYL3</span>&#44; <span class="elsevierStyleItalic">MYBPC3</span>&#44; <span class="elsevierStyleItalic">TNNI3</span>&#44; <span class="elsevierStyleItalic">TNNT2</span>&#44; <span class="elsevierStyleItalic">TPM1</span> e <span class="elsevierStyleItalic">ACTC1</span>&#41;&#46;</p><p id="par0165" class="elsevierStylePara elsevierViewall">A pesquisa de muta&#231;&#245;es nos genes <span class="elsevierStyleItalic">MYH7</span>&#44; <span class="elsevierStyleItalic">MYL2</span>&#44; <span class="elsevierStyleItalic">MYL3</span>&#44; <span class="elsevierStyleItalic">MYBPC3</span>&#44; <span class="elsevierStyleItalic">TNNI3</span>&#44; <span class="elsevierStyleItalic">TNNT2</span>&#44; <span class="elsevierStyleItalic">TPM1</span> e <span class="elsevierStyleItalic">ACTC1</span> &#40;totalidade da regi&#227;o codificante&#44; incluindo transi&#231;&#245;es intr&#227;o&#8208;ex&#227;o&#41; foi efetuada atrav&#233;s da t&#233;cnica de <span class="elsevierStyleItalic">polymerase chain reaction</span> &#40;PCR&#41;&#44; com sequencia&#231;&#227;o direta &#40;combina&#231;&#227;o de sequencia&#231;&#227;o de nova gera&#231;&#227;o com <span class="elsevierStyleItalic">coverage</span> m&#237;nima de 30<span class="elsevierStyleHsp" style=""></span>X e sequencia&#231;&#227;o de Sanger&#41; dos produtos de PCR&#46; A metodologia utilizada tem uma sensibilidade anal&#237;tica de 99&#37; para a dete&#231;&#227;o de substitui&#231;&#245;es nucleot&#237;dicas e pequenas dele&#231;&#245;es e inser&#231;&#245;es&#46;</p><p id="par0170" class="elsevierStylePara elsevierViewall">Para classifica&#231;&#227;o das variantes de ADN quanto &#224; sua patogenicidade foram consultadas as bases de dados da Clinvar e da <span class="elsevierStyleItalic">Human Gene Mutation Database</span> &#40;HGMD&#41;&#46; Nas situa&#231;&#245;es em que a muta&#231;&#227;o n&#227;o tinha sido previamente descrita e para as variantes gen&#233;ticas de significado incerto&#44; foram utilizadas as ferramentas bioinform&#225;ticas de predi&#231;&#227;o de patogenicidade PolyPhen&#8208;2 e <span class="elsevierStyleItalic">Mutation Taster</span>&#44; para aferi&#231;&#227;o da sua relev&#226;ncia funcional&#46;</p><p id="par0175" class="elsevierStylePara elsevierViewall">As crian&#231;as com mais do que uma muta&#231;&#227;o foram definidas como apresentando um gen&#243;tipo complexo&#46;</p><p id="par0180" class="elsevierStylePara elsevierViewall">A avalia&#231;&#227;o ecocardiogr&#225;fica bidimensional&#44; em modo M e Doppler&#44; foi realizada de acordo com as recomenda&#231;&#245;es da <span class="elsevierStyleItalic">American Society of Echocardiography</span><a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">6</span></a>&#46;</p><p id="par0185" class="elsevierStylePara elsevierViewall">Foram avaliadas as dimens&#245;es das cavidades card&#237;acas&#44; septo interventricular e parede posterior do ventr&#237;culo esquerdo &#40;VE&#41;&#44; pesquisado SAM da v&#225;lvula mitral e gradiente na c&#226;mara de sa&#237;da do VE&#44; em repouso e durante a manobra de Valsava&#46; Foi definido como obstru&#231;&#227;o do trato de sa&#237;da do ventr&#237;culo esquerdo &#40;TSVE&#41; um gradiente em repouso<span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>30<span class="elsevierStyleHsp" style=""></span>mmHg<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46;</p><p id="par0190" class="elsevierStylePara elsevierViewall">Do ponto de vista ecocardiogr&#225;fico&#44; o diagn&#243;stico de MCH foi feito quando o valor m&#225;ximo de espessura da parede posterior do VE era superior a duas vezes o desvio padr&#227;o &#40;DP&#41; da m&#233;dia prevista&#44; relativamente &#224; superf&#237;cie corporal &#40;SC&#41;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46; A SC foi calculada de acordo com a f&#243;rmula de Haycock<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">7</span></a>&#46;</p><p id="par0195" class="elsevierStylePara elsevierViewall">O tra&#231;ado eletrocardiogr&#225;fico foi analisado quanto &#224; presen&#231;a de desvio do eixo do QRS&#44; invers&#227;o da onda T<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>1<span class="elsevierStyleHsp" style=""></span>mm&#44; infra desnivelamento do segmento ST<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>2<span class="elsevierStyleHsp" style=""></span>mm e onda S<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>onda R em V<span class="elsevierStyleInf">4</span>&#46; Foram calculados a soma da amplitude de QRS nas deriva&#231;&#245;es dos membros&#44; o produto da amplitude&#8208;dura&#231;&#227;o de QRS nas 12 deriva&#231;&#245;es e o QTc &#40;segundo a f&#243;rmula de Bazett&#41;<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">8</span></a>&#46;</p><p id="par0200" class="elsevierStylePara elsevierViewall">A estratifica&#231;&#227;o do risco de morte s&#250;bita foi realizada de acordo com o modelo descrito por &#214;stman&#8208;Smith et al&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">9</span></a>&#46; Os doentes foram pontuados nos oito par&#226;metros analisados de 1&#8208;3 &#40;<span class="elsevierStyleItalic">score</span> m&#225;ximo<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>14&#41;&#46; Um <span class="elsevierStyleItalic">score</span><span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>6 representa um risco elevado de morte s&#250;bita card&#237;aca&#46; Apesar deste modelo preditivo ter sido desenvolvido para adultos com MCH&#44; o mesmo modelo foi aplicado por estes autores a uma popula&#231;&#227;o pedi&#225;trica&#44; tendo obtido semelhante valor preditivo &#40;estudo n&#227;o publicado&#44; apresentado no <span class="elsevierStyleItalic">46th Annual Meeting of the Association for European Paediatric and Congenital Cardiology</span>&#44; Istambul&#44; Turquia&#44; 23&#8208;26 de maio 2012&#41;&#46;</p><p id="par0205" class="elsevierStylePara elsevierViewall">Foram ainda analisados os seguintes fatores de risco para morte s&#250;bita card&#237;aca<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">10</span></a>&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">&#40;I&#41;</span><p id="par0210" class="elsevierStylePara elsevierViewall">hist&#243;ria familiar de morte s&#250;bita card&#237;aca&#58; morte n&#227;o&#8208;traum&#225;tica e prematura &#40;idade<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>40 anos&#41;&#59; morte ocorrida uma hora ap&#243;s in&#237;cio dos sintomas na aus&#234;ncia de sintomatologia pr&#233;via&#44; incluindo morte noturna inesperada e n&#227;o presenciada ou equivalentes&#44; como epis&#243;dio de ressuscita&#231;&#227;o cardiorrespirat&#243;ria ou choque apropriado de cardio&#8208;desfibrilhador implant&#225;vel &#40;CDI&#41;&#59;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">&#40;II&#41;</span><p id="par0215" class="elsevierStylePara elsevierViewall">s&#237;ncope inexplicada de etiologia n&#227;o neurocardiog&#233;nica&#59;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">&#40;III&#41;</span><p id="par0220" class="elsevierStylePara elsevierViewall">taquicardia ventricular n&#227;o mantida&#58; um ou mais per&#237;odos de<span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>3 extras&#237;stoles ventriculares consecutivas&#44; a uma frequ&#234;ncia<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>120<span class="elsevierStyleHsp" style=""></span>bpm com dura&#231;&#227;o<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>30 segundos&#44; em prova de esfor&#231;o ou em <span class="elsevierStyleItalic">Holter</span> de 24 horas&#59;</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">&#40;IV&#41;</span><p id="par0225" class="elsevierStylePara elsevierViewall">hipertrofia ventricular esquerda extrema&#58; espessura m&#225;xima da parede do VE<span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>30<span class="elsevierStyleHsp" style=""></span>mm ou <span class="elsevierStyleItalic">Z&#8208;score</span><span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>6<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46;</p></li></ul></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Plano de seguimento</span><p id="par0230" class="elsevierStylePara elsevierViewall">Os doentes com gen&#243;tipo e fen&#243;tipo positivos foram definidos como familiares afetados e seguidos em consulta espec&#237;fica de cardiologia pedi&#225;trica&#44; com uma periodicidade semestral&#46;</p><p id="par0235" class="elsevierStylePara elsevierViewall">Foram definidos como familiares em risco de desenvolvimento de MCH&#44; portadores de muta&#231;&#227;o de gene sarcom&#233;rico&#44; variantes gen&#233;ticas de significado incerto ou muta&#231;&#245;es n&#227;o anteriormente descritas como associadas &#224; ocorr&#234;ncia de MCH&#44; na aus&#234;ncia de manifesta&#231;&#245;es fenot&#237;picas de doen&#231;a&#46;</p><p id="par0240" class="elsevierStylePara elsevierViewall">Num dos casos&#44; o estudo gen&#233;tico encontra&#8208;se pendente e foi considerado existir risco de desenvolvimento de doen&#231;a&#46; As crian&#231;as nestas condi&#231;&#245;es foram seguidas com periodicidade anual&#46;</p><p id="par0245" class="elsevierStylePara elsevierViewall">As crian&#231;as com fen&#243;tipo negativo e aus&#234;ncia de muta&#231;&#227;o foram consideradas como n&#227;o tendo risco de desenvolvimento de MCH e dispensadas de seguimento&#46;</p><p id="par0250" class="elsevierStylePara elsevierViewall">As consultas de seguimento inclu&#237;ram avalia&#231;&#227;o cl&#237;nica&#44; realiza&#231;&#227;o de ECG de 12 deriva&#231;&#245;es e ecocardiograma transtor&#225;cico&#46; Foi requisitada monitoriza&#231;&#227;o Holter de 24 horas sempre que clinicamente relevante&#46;</p><p id="par0255" class="elsevierStylePara elsevierViewall">Todos os doentes com idade superior a sete anos realizaram prova de esfor&#231;o convencional&#46; Dois dos doentes foram adicionalmente submetidos a ecocardiograma de esfor&#231;o&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">An&#225;lise estat&#237;stica</span><p id="par0260" class="elsevierStylePara elsevierViewall">As vari&#225;veis cont&#237;nuas s&#227;o apresentadas como m&#233;dias e desvio padr&#227;o quando obedecendo a distribui&#231;&#227;o normal e como medianas&#44; m&#237;nimo e m&#225;ximo quando n&#227;o obedecendo a distribui&#231;&#227;o normal&#46; As vari&#225;veis categ&#243;ricas s&#227;o descritas de acordo com a sua frequ&#234;ncia e percentagem&#46;</p></span></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Resultados</span><p id="par0265" class="elsevierStylePara elsevierViewall">Foram inclu&#237;das no nosso estudo de MCH familiar 20 crian&#231;as pertencentes a 10 fam&#237;lias &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figuras 1&#8211;3</a>&#41;&#46; Tr&#234;s das crian&#231;as constitu&#237;ram&#8208;se como o caso &#237;ndex e as restantes eram parentes em primeiro grau de um doente com MCH &#40;80&#37; sexo masculino&#59; idade mediana<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>10 anos&#59; min<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>1 m&#234;s&#59; m&#225;x<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>16 anos&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0270" class="elsevierStylePara elsevierViewall">O motivo da referencia&#231;&#227;o dos tr&#234;s casos &#237;ndex &#224; consulta de cardiologia pedi&#225;trica foi&#44; respetivamente&#58; presen&#231;a de altera&#231;&#245;es no ECG&#44; toracalgia com o esfor&#231;o e sopro card&#237;aco&#46;</p><p id="par0275" class="elsevierStylePara elsevierViewall">Nas fam&#237;lias <span class="elsevierStyleSmallCaps">I</span>&#44; <span class="elsevierStyleSmallCaps">IV</span> e <span class="elsevierStyleSmallCaps">V</span>&#44; registava&#8208;se hist&#243;ria familiar de morte s&#250;bita card&#237;aca&#46;</p><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Achados cl&#237;nicos &#224; inclus&#227;o &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Tabela 1</a>&#41;</span><p id="par0280" class="elsevierStylePara elsevierViewall">&#192;&#44; data da primeira consulta&#44; a maioria das crian&#231;as &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>16&#59; 80&#37;&#41; estava assintom&#225;tica&#46; Em duas das crian&#231;as havia refer&#234;ncia a um epis&#243;dio de s&#237;ncope inexplicada e outras duas referiam toracalgia com o esfor&#231;o&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0285" class="elsevierStylePara elsevierViewall">Sete &#40;50&#37;&#41; dos 14 portadores de muta&#231;&#227;o apresentavam fen&#243;tipo positivo na primeira avalia&#231;&#227;o&#46; A fun&#231;&#227;o sist&#243;lica do VE encontrava&#8208;se conservada em todos eles&#46;</p><p id="par0290" class="elsevierStylePara elsevierViewall">Nenhum dos doentes apresentava obstru&#231;&#227;o sist&#233;mica significativa em repouso&#46;</p><p id="par0295" class="elsevierStylePara elsevierViewall">Num dos doentes elicitou&#8208;se obstru&#231;&#227;o intraventricular esquerda significativa com o esfor&#231;o&#44; tendo sido medicado com bloqueador&#8208;beta e aconselhado a restri&#231;&#227;o da atividade f&#237;sica&#46;</p><p id="par0300" class="elsevierStylePara elsevierViewall">Oito crian&#231;as &#40;40&#37;&#41; eram portadoras de muta&#231;&#227;o de um gene sarcom&#233;rico&#44; embora sem manifesta&#231;&#227;o fenot&#237;pica de doen&#231;a &#224; apresenta&#231;&#227;o&#46; Foram consideradas em risco de desenvolvimento de MCH&#46;</p><p id="par0305" class="elsevierStylePara elsevierViewall">Cinco dos familiares avaliados &#40;25&#37;&#41; apresentavam fen&#243;tipo e gen&#243;tipo negativos&#44; pelo que se considerou n&#227;o haver risco de desenvolvimento de MCH&#44; tendo sido dispensados de seguimento&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Estudo gen&#233;tico &#40;<a class="elsevierStyleCrossRef" href="#tbl0010">Tabela 2</a>&#41;</span><p id="par0310" class="elsevierStylePara elsevierViewall">&#192;&#44; data da primeira consulta&#44; 14 crian&#231;as &#40;70&#37;&#41; eram portadoras de muta&#231;&#227;o de um ou mais genes sarcom&#233;ricos &#40;80&#37; sexo masculino&#59; idade mediana<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>8 anos&#59; min<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>1 m&#234;s&#59; m&#225;x<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>16 anos&#41;&#46;</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0315" class="elsevierStylePara elsevierViewall">Verificaram&#8208;se muta&#231;&#245;es dos genes <span class="elsevierStyleItalic">MYBPC3</span> &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>14&#59; 78&#37;&#41;&#44; <span class="elsevierStyleItalic">MYH7</span> &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>2&#59; 11&#37;&#41;&#44; <span class="elsevierStyleItalic">TNNT2</span> &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>1&#59; 5&#44;5&#37;&#41; e <span class="elsevierStyleItalic">MYL3</span> &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>1&#59; 5&#44;5&#37;&#41;&#46;</p><p id="par0320" class="elsevierStylePara elsevierViewall">Um dos doentes &#40;caso 8&#44; fam&#237;lia <span class="elsevierStyleSmallCaps">IV</span>&#41; apresentava em heterozigotia duas muta&#231;&#245;es no gene <span class="elsevierStyleItalic">MYBPC3</span> &#40;ex&#245;es 19 e 32&#41;&#44; uma delas previamente descrita em doentes com MCH &#40;p&#46;Gly1206Asp&#41; e a outra uma variante gen&#233;tica de significado incerto &#40;p&#46;Asp610Asn&#41;&#46; No entanto&#44; esta muta&#231;&#227;o foi predita como provavelmente patog&#233;nica pelos m&#233;todos PolyPhen&#8208;2 e <span class="elsevierStyleItalic">Mutation Taster</span>&#46;</p><p id="par0325" class="elsevierStylePara elsevierViewall">Nos irm&#227;os 12 e 13 &#40;fam&#237;lia <span class="elsevierStyleSmallCaps">VII</span>&#41; estavam presentes duas muta&#231;&#245;es no gene <span class="elsevierStyleItalic">MYBPC3</span> &#40;ex&#227;o 6 e 8&#41;&#44; uma delas previamente descrita em doentes com MCH &#40;p&#46;Glu258Lys&#41; e a outra &#40;p&#46;Gly279Ala&#41; predita como provavelmente benigna pelos m&#233;todos PolyPhen&#8208;2 e <span class="elsevierStyleItalic">Mutation Taster</span>&#46;</p><p id="par0330" class="elsevierStylePara elsevierViewall">No caso 19 &#40;fam&#237;lia <span class="elsevierStyleSmallCaps">X</span>&#41; foram encontradas em heterozigotia a muta&#231;&#227;o p&#46;Ala149Asp no ex&#227;o 4 do gene <span class="elsevierStyleItalic">MYBPC3</span> e a muta&#231;&#227;o p&#46;Glu49STOP no ex&#227;o 2 do gene <span class="elsevierStyleItalic">MYL3</span>&#46; A muta&#231;&#227;o p&#46;Ala149Asp n&#227;o foi previamente descrita em doentes com MCH e foi predita como provavelmente benigna pelos m&#233;todos PolyPhen&#8208;2 e <span class="elsevierStyleItalic">Mutation Taster</span>&#46; A muta&#231;&#227;o p&#46;Glu49STOP n&#227;o foi previamente descrita em doentes com MCH&#44; mas dada a consequ&#234;ncia para a sequ&#234;ncia proteica assumiu&#8208;se que pudesse constituir a causa gen&#233;tica da patologia observada&#46;</p><p id="par0335" class="elsevierStylePara elsevierViewall">Na fam&#237;lia <span class="elsevierStyleSmallCaps">IX</span>&#44; os casos 17 e 18 apresentavam estudo gen&#233;tico positivo para s&#237;ndrome de LEOPARD&#44; com identifica&#231;&#227;o de muta&#231;&#227;o patog&#233;nica no ex&#227;o12 do gene <span class="elsevierStyleItalic">PTPN11</span> &#40;c&#46;1403C<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>T&#59; p&#46;Thr468Met&#41;&#46; Foi&#8208;lhes identificada em heterozigotia uma muta&#231;&#227;o no ex&#227;o 17 do gene <span class="elsevierStyleItalic">MYBPC3</span> &#40;p&#46;Gly507Arg&#41;&#44; uma variante gen&#233;tica de significado incerto&#44; predita como provavelmente patog&#233;nica pelos m&#233;todos PolyPhen&#8208;2 e <span class="elsevierStyleItalic">Mutation Taster</span>&#46;</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Estratifica&#231;&#227;o do risco de morte s&#250;bita &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Tabela 3</a>&#41;</span><p id="par0340" class="elsevierStylePara elsevierViewall">Foi realizada an&#225;lise do risco de morte s&#250;bita nos doentes com fen&#243;tipo e gen&#243;tipo positivos&#46; Tr&#234;s dos sete doentes n&#227;o apresentavam qualquer fator de risco&#46;</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0345" class="elsevierStylePara elsevierViewall">Num dos doentes verificava&#8208;se um gen&#243;tipo complexo&#44; isto &#233;&#44; coexist&#234;ncia de mais do que uma muta&#231;&#227;o de um gene sarcom&#233;rico&#46;</p><p id="par0350" class="elsevierStylePara elsevierViewall">Quatro dos doentes apresentavam um <span class="elsevierStyleItalic">score</span> de risco eletrocardiogr&#225;fico<span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>6 &#40;<a class="elsevierStyleCrossRef" href="#tbl0020">Tabela 4</a>&#41;&#46; Dois dos tr&#234;s doentes com <span class="elsevierStyleItalic">score</span> de risco eletrocardiogr&#225;fico<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>6 n&#227;o apresentavam outros fatores de risco para morte s&#250;bita&#46;</p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Achados cl&#237;nicos no seguimento &#40;<a class="elsevierStyleCrossRef" href="#tbl0025">Tabela 5</a>&#41;</span><p id="par0355" class="elsevierStylePara elsevierViewall">O tempo m&#233;dio de seguimento destes doentes foi de 3&#44;5<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>0&#44;8 anos &#40;min<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>6 meses&#59; m&#225;x<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>9&#44;5 anos&#41;&#46;</p><elsevierMultimedia ident="tbl0025"></elsevierMultimedia><p id="par0360" class="elsevierStylePara elsevierViewall">Durante este per&#237;odo&#44; duas das crian&#231;as fen&#243;tipo negativo portadoras de muta&#231;&#227;o &#40;gene <span class="elsevierStyleItalic">MYBPC3</span>&#41; desenvolveram MCH&#44; aos 10 e 15 anos de idade &#40;28&#37; de taxa de penetr&#226;ncia&#41;&#46;</p><p id="par0365" class="elsevierStylePara elsevierViewall">Todos os doentes diagnosticados com MCH &#224; primeira avalia&#231;&#227;o mantinham o diagn&#243;stico &#224; data da &#250;ltima consulta&#46;</p><p id="par0370" class="elsevierStylePara elsevierViewall">N&#227;o se verificaram &#243;bitos durante o per&#237;odo de seguimento&#46; Um dos doentes foi submetido a implanta&#231;&#227;o de cardioversor&#8208;desfibrilhador como preven&#231;&#227;o prim&#225;ria&#44; ap&#243;s um epis&#243;dio de taquicardia ventricular n&#227;o mantida registado em Holter de 24 horas&#46;</p><p id="par0375" class="elsevierStylePara elsevierViewall">Este doente apresentava tr&#234;s fatores de risco cl&#225;ssicos &#40;s&#237;ncope&#44; hipertrofia ventricular esquerda extrema e hist&#243;ria de morte s&#250;bita de tia paterna com o diagn&#243;stico de MCH&#41; e um <span class="elsevierStyleItalic">score</span> de risco eletrocardiogr&#225;fico<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>12 &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Tabela 3</a>&#41;&#46;</p></span></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Discuss&#227;o</span><p id="par0380" class="elsevierStylePara elsevierViewall">A MCH tem uma incid&#234;ncia anual estimada de 0&#44;3&#8208;0&#44;5 por cada 100<span class="elsevierStyleHsp" style=""></span>000 crian&#231;as<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">3&#44;11</span></a>&#46;</p><p id="par0385" class="elsevierStylePara elsevierViewall">Na popula&#231;&#227;o adulta a MCH familiar resulta&#44; em 60&#37; dos casos&#44; de muta&#231;&#245;es nos genes das prote&#237;nas sarcom&#233;ricas<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46; Em pediatria&#44; no entanto&#44; esta patologia tem uma etiologia mais complexa e heterog&#233;nea&#46;</p><p id="par0390" class="elsevierStylePara elsevierViewall">No <span class="elsevierStyleItalic">Pediatric Cardiomyopathy Registry</span>&#44; numa popula&#231;&#227;o de 855 doentes com MCH&#44; a etiologia era conhecida em apenas 25&#44;8&#37;&#58; em 9&#37; dos casos registava&#8208;se a associa&#231;&#227;o a s&#237;ndromes malformativos&#44; em 8&#44;7&#37; a doen&#231;as do metabolismo e em 7&#44;5&#37; a doen&#231;as neuromusculares<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">12</span></a>&#46; Estima&#8208;se que em cerca de um ter&#231;o dos casos de MCH pedi&#225;trica a etiologia seja familiar&#44; resultante de muta&#231;&#245;es dos genes que codificam as prote&#237;nas sarcom&#233;ricas<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">9</span></a>&#46;</p><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Estudo gen&#233;tico na cardiomiopatia hipertr&#243;fica familiar</span><p id="par0395" class="elsevierStylePara elsevierViewall">Foram j&#225; descritas mais de 1400 muta&#231;&#245;es nos 11 genes que codificam as prote&#237;nas dos miofilamentos sarcom&#233;ricos e do disco&#8208;Z<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">5</span></a>&#46;</p><p id="par0400" class="elsevierStylePara elsevierViewall">Em cerca de 50&#8208;60&#37; dos casos de MCH familiar &#233; poss&#237;vel identificar uma muta&#231;&#227;o sarcom&#233;rica<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">5</span></a>&#46; Quando uma muta&#231;&#227;o patog&#233;nica &#233; identificada numa fam&#237;lia&#44; o diagn&#243;stico gen&#233;tico &#233; uma medida de rastreio familiar custo&#8208;efetiva e eficaz&#46;</p><p id="par0405" class="elsevierStylePara elsevierViewall">O objetivo deste &#250;ltimo &#233; o de identificar a ocorr&#234;ncia ou o risco de desenvolvimento da doen&#231;a nos familiares em primeiro grau do caso &#237;ndex&#44; para in&#237;cio precoce de terap&#234;utica e estratifica&#231;&#227;o do risco de morte s&#250;bita card&#237;aca&#46;</p><p id="par0410" class="elsevierStylePara elsevierViewall">As mais recentes normas de orienta&#231;&#227;o da ESC e da ACCF&#47;AHA recomendam a avalia&#231;&#227;o dos familiares em idade pedi&#225;trica a partir dos 10&#8208;12 anos<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">3&#44;4</span></a>&#46; No entanto&#44; em fam&#237;lias com patologia de apresenta&#231;&#227;o precoce poder&#225; ser apropriada a avalia&#231;&#227;o cl&#237;nica e gen&#233;tica anterior a esta idade<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46;</p><p id="par0415" class="elsevierStylePara elsevierViewall">Atualmente&#44; o valor do teste gen&#233;tico assenta sobretudo na decis&#227;o de manter ou dispensar do seguimento cl&#237;nico e ecocardiogr&#225;fico os familiares do caso &#237;ndex<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">3&#44;4</span></a>&#46;</p><p id="par0420" class="elsevierStylePara elsevierViewall">Na nossa amostra&#44; cinco &#40;25&#37;&#41; das crian&#231;as avaliadas apresentavam fen&#243;tipo e gen&#243;tipo negativos&#44; e foram dispensadas de seguimento regular&#46; Esta informa&#231;&#227;o tranquiliza os familiares n&#227;o afetados quanto &#224; possibilidade de desenvolvimento de doen&#231;a e liberta os servi&#231;os de consultas desnecess&#225;rias&#46;</p><p id="par0425" class="elsevierStylePara elsevierViewall">Por outro lado&#44; as crian&#231;as com gen&#243;tipo positivo foram divididas em dois grupos&#58; aquelas com fen&#243;tipo positivo &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>7&#59; 35&#37;&#41; &#8211; familiares afetados &#8211; e aquelas com fen&#243;tipo negativo &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>8&#59; 40&#37;&#41; &#8211; familiares em risco&#46;</p><p id="par0430" class="elsevierStylePara elsevierViewall">Para classifica&#231;&#227;o das variantes de ADN quanto &#224; sua patogenicidade&#44; foram consultadas as bases de dados da Clinvar e da HGMD&#46;</p><p id="par0435" class="elsevierStylePara elsevierViewall">Em oito dos casos&#44; as muta&#231;&#245;es encontradas tinham sido previamente descritas como patog&#233;nicas&#46;</p><p id="par0440" class="elsevierStylePara elsevierViewall">Nas situa&#231;&#245;es em que a muta&#231;&#227;o n&#227;o tinha sido previamente descrita &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>3&#41; e para as variantes gen&#233;ticas de significado incerto &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>7&#41;&#44; foram utilizados os m&#233;todos PolyPhen&#8208;2 e <span class="elsevierStyleItalic">Mutation Taster</span>&#44; para aferi&#231;&#227;o da sua relev&#226;ncia funcional&#46;</p><p id="par0445" class="elsevierStylePara elsevierViewall">Opt&#225;mos por incluir neste estudo os doentes pertencentes &#224; fam&#237;lia <span class="elsevierStyleSmallCaps">IX</span> que&#44; apesar de terem o diagn&#243;stico gen&#233;tico de s&#237;ndrome de LEOPARD e esta ser por si s&#243; explicativa do fen&#243;tipo de MCH&#44; apresentavam paralelamente em heterozigotia uma muta&#231;&#227;o no ex&#227;o 17 do gene <span class="elsevierStyleItalic">MYBPC3</span> &#40;p&#46;Gly507Arg&#41;<span class="elsevierStyleItalic">&#46;</span> Esta constitui&#8208;se como uma variante gen&#233;tica de significado incerto&#44; predita como provavelmente patog&#233;nica pelos m&#233;todos PolyPhen&#8208;2 e <span class="elsevierStyleItalic">Mutation Taster</span>&#46; Neste contexto&#44; a patogenicidade da muta&#231;&#227;o sarcom&#233;rica encontrada permanece por esclarecer&#46;</p><p id="par0450" class="elsevierStylePara elsevierViewall">Foi nossa pol&#237;tica manter em seguimento os doentes com variantes gen&#233;ticas de significado incerto e aqueles apresentando muta&#231;&#245;es n&#227;o anteriormente descritas como estando associadas &#224; ocorr&#234;ncia de MCH&#44; mesmo na aus&#234;ncia de manifesta&#231;&#245;es fenot&#237;picas de doen&#231;a &#40;&#171;familiares em risco&#187;&#41;&#46;</p><p id="par0455" class="elsevierStylePara elsevierViewall">No entanto&#44; para al&#233;m da avalia&#231;&#227;o sequencial&#44; o manuseio cl&#237;nico dos portadores de muta&#231;&#227;o de genes sarcom&#233;ricos sem fen&#243;tipo de doen&#231;a n&#227;o est&#225; estabelecido<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46;</p><p id="par0460" class="elsevierStylePara elsevierViewall">De acordo com as mais recentes recomenda&#231;&#245;es internacionais&#44; as crian&#231;as gen&#243;tipo positivo&#47;fen&#243;tipo negativo devem ser avaliadas cada 12&#8208;18 meses&#44; enquanto os adultos apenas a cada 2&#8208;5 anos<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46;</p><p id="par0465" class="elsevierStylePara elsevierViewall">Em doentes portadores de muta&#231;&#227;o de um gene sarcom&#233;rico mas sem express&#227;o fenot&#237;pica de doen&#231;a&#44; o risco de eventos card&#237;acos adversos &#233; baixo&#44; tendo sido reportada num estudo recente uma taxa de morte s&#250;bita card&#237;aca de 0&#44;13&#37; por pessoa&#8208;ano nesta popula&#231;&#227;o<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">13</span></a>&#46;</p></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Morte s&#250;bita e estratifica&#231;&#227;o do risco de morte s&#250;bita card&#237;aca</span><p id="par0470" class="elsevierStylePara elsevierViewall">Estima&#8208;se que o risco anual de morte s&#250;bita card&#237;aca em doentes com MCH ronde 1&#37;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">13</span></a>&#46; No entanto&#44; um estudo dinamarqu&#234;s&#44; realizado entre 2000&#8208;2006&#44; revelou um risco de morte s&#250;bita card&#237;aca em contexto de MCH<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>0&#44;1&#37; por pessoa&#8208;ano&#44; em doentes com idades compreendidas entre 1&#8208;35 anos<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">14</span></a>&#46;</p><p id="par0475" class="elsevierStylePara elsevierViewall">As mais recentes recomenda&#231;&#245;es da ESC postulam como fatores de risco <span class="elsevierStyleItalic">major</span> para a ocorr&#234;ncia de morte s&#250;bita em crian&#231;as com MCH a hipertrofia ventricular esquerda extrema &#40;definida como uma espessura ventricular esquerda m&#225;xima<span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>30<span class="elsevierStyleHsp" style=""></span>mm ou <span class="elsevierStyleItalic">Z&#8208;score</span><span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>6&#41;&#44; ocorr&#234;ncia de s&#237;ncope inexplicada&#44; taquicardia ventricular n&#227;o mantida e hist&#243;ria familiar de morte s&#250;bita card&#237;aca<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">3</span></a>&#46;</p><p id="par0480" class="elsevierStylePara elsevierViewall">Na nossa amostra&#44; optou&#8208;se pela estratifica&#231;&#227;o do risco de morte s&#250;bita apenas nos familiares afetados&#44; tal como preconizado nas atuais recomenda&#231;&#245;es<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">4</span></a>&#46;</p><p id="par0485" class="elsevierStylePara elsevierViewall">Foram considerados&#44; a par dos fatores de risco cl&#225;ssicos&#44; outros fatores de risco descritos na literatura&#58; presen&#231;a de obstru&#231;&#227;o no TSVE<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">9</span></a> e gen&#243;tipo complexo &#40;coexist&#234;ncia de mais do que uma muta&#231;&#227;o de um gene sarcom&#233;rico&#41;<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">3&#44;15</span></a>&#46;</p><p id="par0490" class="elsevierStylePara elsevierViewall">Foi ainda aplicado o <span class="elsevierStyleItalic">score</span> de risco eletrocardiogr&#225;fico descrito por &#214;stman&#8208;Smith et al&#46;&#44; que num estudo desenvolvido em adultos com MCH se associou significativamente &#224; ocorr&#234;ncia morte s&#250;bita na popula&#231;&#227;o estudada&#44; com elevadas sensibilidade &#40;85&#37;&#41; e especificidade &#40;100&#37;&#41;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">9</span></a>&#46;</p><p id="par0495" class="elsevierStylePara elsevierViewall">Este modelo preditivo avalia a presen&#231;a de desvio do eixo QRS&#44; invers&#227;o patol&#243;gica das ondas T&#44; depress&#227;o do segmento ST&#44; S dominante em V4&#44; soma da amplitude de QRS nas deriva&#231;&#245;es dos membros&#44; produto amplitude&#8208;dura&#231;&#227;o de QRS nas 12 deriva&#231;&#245;es e QTc&#46;</p><p id="par0505" class="elsevierStylePara elsevierViewall">&#201; interessante notar que&#44; na nossa amostra&#44; o &#250;nico doente com complica&#231;&#245;es &#40;implanta&#231;&#227;o de CDI&#41; apresentava&#44; al&#233;m de tr&#234;s fatores de risco cl&#225;ssicos&#44; o <span class="elsevierStyleItalic">score</span> de risco eletrocardiogr&#225;fico mais elevado da popula&#231;&#227;o em estudo&#46;</p></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Express&#227;o fenot&#237;pica da miocardiopatia hipertr&#243;fica</span><p id="par0510" class="elsevierStylePara elsevierViewall">A express&#227;o cl&#237;nica da MCH &#233; determinada por uma complexa intera&#231;&#227;o de fatores gen&#233;ticos&#44; epigen&#233;ticos e ambientais&#46; Tal como outras patologias de transmiss&#227;o autoss&#243;mica dominante&#44; demonstra marcada variabilidade fenot&#237;pica&#44; mesmo no seio das fam&#237;lias afetadas&#46;</p><p id="par0515" class="elsevierStylePara elsevierViewall">Foram propostas como manifesta&#231;&#245;es pr&#233;&#8208;fenot&#237;picas detet&#225;veis em portadores de muta&#231;&#245;es sarcom&#233;ricas a presen&#231;a de criptas mioc&#225;rdicas&#44; o alongamento dos folhetos mitrais&#44; a disfun&#231;&#227;o diast&#243;lica&#44; o aumento da deposi&#231;&#227;o de colag&#233;nio e a presen&#231;a de fibrose mioc&#225;rdica<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">3&#44;4</span></a>&#46;</p><p id="par0520" class="elsevierStylePara elsevierViewall">A heterogeneidade cl&#237;nica desta patologia &#233; tamb&#233;m longitudinal&#58; a penetr&#226;ncia da express&#227;o fenot&#237;pica aumenta com a idade&#44; mantendo&#8208;se no entanto inferior a 100&#37;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">5</span></a>&#46;</p><p id="par0525" class="elsevierStylePara elsevierViewall">Um estudo recente descreveu uma incid&#234;ncia de MCH manifesta em portadores de muta&#231;&#245;es sarcom&#233;ricas com idade inferior a 40 anos<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>0&#44;10&#37; por pessoa&#8208;ano<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">13</span></a>&#46;</p><p id="par0530" class="elsevierStylePara elsevierViewall">Diversos estudos pedi&#225;tricos demonstraram uma penetr&#226;ncia da express&#227;o fen&#243;tipica que varia entre 6&#8208;31&#37; em crian&#231;as portadoras de muta&#231;&#245;es de genes sarcom&#233;ricos&#44; ap&#243;s um per&#237;odo de seguimento m&#225;ximo de 12 anos<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">2&#44;16</span></a>&#46;</p><p id="par0535" class="elsevierStylePara elsevierViewall">Na nossa amostra&#44; duas das crian&#231;as fen&#243;tipo negativo portadoras de muta&#231;&#227;o desenvolveram o fen&#243;tipo de MCH ap&#243;s 3&#44;5<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>0&#44;8 anos de seguimento&#44; aos dez e 15 anos&#46; A taxa de penetr&#226;ncia resultante &#40;28&#37;&#41; enquadra&#8208;se nas anteriormente descritas na literatura&#46;</p><p id="par0540" class="elsevierStylePara elsevierViewall">Este facto sublinha a import&#226;ncia do seguimento longitudinal dos doentes portadores de muta&#231;&#245;es dos genes sarcom&#233;ricos&#44; e perspetiva o desenvolvimento futuro de terap&#234;uticas modeladoras da express&#227;o da doen&#231;a&#46;</p></span><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0145">Limita&#231;&#245;es do estudo</span><p id="par0545" class="elsevierStylePara elsevierViewall">As limita&#231;&#245;es deste estudo s&#227;o inerentes ao seu desenho retrospetivo e ao reduzido tamanho da amostra&#46;</p><p id="par0550" class="elsevierStylePara elsevierViewall">&#201; importante ressaltar que a taxa de convers&#227;o fenot&#237;pica depende provavelmente de uma multiplicidade de fatores&#44; entre os quais o sexo&#44; a ra&#231;a e o gen&#243;tipo&#46; Na nossa amostra est&#227;o representadas apenas crian&#231;as de ra&#231;a caucasiana e com muta&#231;&#245;es em quatro dos genes sarcom&#233;ricos &#40;<span class="elsevierStyleItalic">MYBPC3</span>&#44; <span class="elsevierStyleItalic">MYH7</span>&#44; <span class="elsevierStyleItalic">TNNT2 e MYL3</span>&#41;&#46;</p><p id="par0555" class="elsevierStylePara elsevierViewall">O reduzido n&#250;mero de complica&#231;&#245;es ocorridas na nossa amostra n&#227;o nos permitiu estabelecer associa&#231;&#245;es entre estas e os fatores de risco identificados&#46;</p></span></span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0150">Conclus&#227;o</span><p id="par0560" class="elsevierStylePara elsevierViewall">Quando uma muta&#231;&#227;o patog&#233;nica &#233; identificada numa fam&#237;lia&#44; o teste gen&#233;tico &#233; uma medida eficaz de rastreio familiar&#46; Este &#250;ltimo permite aferir quais os familiares em risco de desenvolvimento de doen&#231;a e aqueles que podem ser dispensados do seguimento&#46;</p><p id="par0565" class="elsevierStylePara elsevierViewall">Na nossa amostra&#44; a penetr&#226;ncia de MCH em crian&#231;as com gen&#243;tipo positivo e fen&#243;tipo normal &#224; apresenta&#231;&#227;o foi de 28&#37; ap&#243;s 3&#44;5 anos de seguimento&#46; Este facto sublinha a import&#226;ncia da avalia&#231;&#227;o longitudinal dos portadores de muta&#231;&#227;o de genes sarcom&#233;ricos&#44; independentemente da presen&#231;a de fen&#243;tipo patol&#243;gico&#46;</p></span><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0155">Responsabilidades &#233;ticas</span><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0160">Prote&#231;&#227;o de pessoas e animais</span><p id="par0570" class="elsevierStylePara elsevierViewall">Os autores declaram que para esta investiga&#231;&#227;o n&#227;o se realizaram experi&#234;ncias em seres humanos e&#47;ou animais&#46;</p></span><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0165">Confidencialidade dos dados</span><p id="par0575" class="elsevierStylePara elsevierViewall">Os autores declaram ter seguido os protocolos do seu centro de trabalho acerca da publica&#231;&#227;o de dados de pacientes&#46;</p></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0170">Direito &#224; privacidade e consentimento escrito</span><p id="par0580" class="elsevierStylePara elsevierViewall">Os autores declaram que n&#227;o aparecem dados que permitam a identifica&#231;&#227;o de pacientes neste artigo&#46;</p></span></span><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0175">Conflito de interesses</span><p id="par0585" class="elsevierStylePara elsevierViewall">Os autores declaram n&#227;o haver conflito de interesses&#46;</p></span></span>"
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              "titulo" => "Achados cl&#237;nicos &#224; inclus&#227;o &#40;Tabela 1&#41;"
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              "titulo" => "Morte s&#250;bita e estratifica&#231;&#227;o do risco de morte s&#250;bita card&#237;aca"
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              "titulo" => "Express&#227;o fenot&#237;pica da miocardiopatia hipertr&#243;fica"
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              "titulo" => "Limita&#231;&#245;es do estudo"
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              "titulo" => "Prote&#231;&#227;o de pessoas e animais"
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              "titulo" => "Direito &#224; privacidade e consentimento escrito"
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    "fechaRecibido" => "2016-01-23"
    "fechaAceptado" => "2016-09-26"
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            0 => "Crian&#231;as"
            1 => "Miocardiopatia hipertr&#243;fica familiar"
            2 => "Diagn&#243;stico gen&#233;tico"
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            0 => "Children"
            1 => "Familial hypertrophic cardiomyopathy"
            2 => "Genetic testing"
            3 => "Penetrance"
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    "resumen" => array:2 [
      "pt" => array:3 [
        "titulo" => "Resumo"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introdu&#231;&#227;o</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A miocardiopatia hipertr&#243;fica &#40;MCH&#41; &#233; uma patologia com transmiss&#227;o essencialmente autoss&#243;mica dominante&#44; express&#227;o cl&#237;nica vari&#225;vel e penetr&#226;ncia incompleta&#46; O rastreio familiar tem por objetivo identificar a ocorr&#234;ncia ou o risco de desenvolvimento da doen&#231;a nos parentes em primeiro grau do caso &#237;ndex&#46; As normas de orienta&#231;&#227;o da ESC e da ACCF&#47;AHA recomendam a avalia&#231;&#227;o dos familiares em idade pedi&#225;trica a partir dos 10&#8208;12 anos&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Objetivos</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Avaliaram&#8208;se os resultados de um programa de rastreio pedi&#225;trico de MCH familiar e o valor preditivo do estudo gen&#233;tico nexte contexto&#46; Foi ainda aferida a penetr&#226;ncia fenot&#237;pica ao longo do tempo de seguimento destas crian&#231;as&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">M&#233;todos e resultados</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Foram inclu&#237;das 20 crian&#231;as pertencentes a dez fam&#237;lias &#40;2004&#8208;2013&#41;&#46; Tr&#234;s das crian&#231;as constitu&#237;ram&#8208;se como o caso &#237;ndex&#44; sendo as restantes parentes em primeiro grau de um doente com MCH &#40;80&#37; sexo masculino&#59; idade mediana<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>10 anos&#41;&#46; Catorze crian&#231;as eram portadoras de muta&#231;&#227;o de um gene sarcom&#233;rico &#40;70&#37;&#59; idade mediana<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>8 anos&#41;&#46; Sete &#40;50&#37;&#41; dos 14 portadores de muta&#231;&#227;o apresentavam fen&#243;tipo positivo na primeira avalia&#231;&#227;o&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Foram definidos como &#171;familiares em risco&#187; aqueles com teste gen&#233;tico positivo&#44; mas com fen&#243;tipo normal &#224; apresenta&#231;&#227;o&#46; Ap&#243;s 3&#44;5<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>0&#44;8 anos de seguimento&#44; duas das crian&#231;as fen&#243;tipo negativo portadoras de muta&#231;&#227;o &#40;gene <span class="elsevierStyleItalic">MYBPC3</span>&#41; desenvolveram MCH&#44; aos dez e 15 anos de idade &#40;28&#37; de taxa de penetr&#226;ncia&#41;&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclus&#245;es</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A penetr&#226;ncia de MCH em crian&#231;as com fen&#243;tipo normal &#224; apresenta&#231;&#227;o foi de 28&#37; ap&#243;s 3&#44;5 anos de seguimento&#46; Tal sublinha a import&#226;ncia da avalia&#231;&#227;o longitudinal dos portadores de muta&#231;&#227;o de genes sarcom&#233;ricos&#44; independentemente da presen&#231;a de fen&#243;tipo patol&#243;gico&#46;</p></span>"
        "secciones" => array:4 [
          0 => array:2 [
            "identificador" => "abst0005"
            "titulo" => "Introdu&#231;&#227;o"
          ]
          1 => array:2 [
            "identificador" => "abst0010"
            "titulo" => "Objetivos"
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          2 => array:2 [
            "identificador" => "abst0015"
            "titulo" => "M&#233;todos e resultados"
          ]
          3 => array:2 [
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            "titulo" => "Conclus&#245;es"
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      "en" => array:3 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Introduction</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Hypertrophic cardiomyopathy &#40;HCM&#41; is most often of autosomal dominant inheritance with incomplete penetrance and variable expression&#46; The main purpose of family screening is to identify relatives with unrecognized HCM and to monitor those at risk for disease&#44; in order to minimize complications and to assess risk of sudden cardiac death&#46; The ESC and ACCF&#47;AHA guidelines on the diagnosis and management of HCM recommend the screening of child relatives from the age of 10&#8208;12 years&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Objectives</span><p id="spar0120" class="elsevierStyleSimplePara elsevierViewall">We studied the outcome of clinical screening and genetic testing of child probands and relatives &#40;&#60;18 years of age&#41; from families with HCM and assessed the age&#8208;related penetrance of HCM during the follow&#8208;up of these young relatives&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Methods and Results</span><p id="spar0125" class="elsevierStyleSimplePara elsevierViewall">Twenty patients from ten families were included between 2004 and 2013&#44; consisting of three probands and 17 first&#8208;degree relatives &#40;80&#37; male&#59; median age 10 years&#41;&#46; Fourteen child relatives were mutation carriers &#40;70&#37;&#59; median age eight years&#41;&#46; Seven &#40;50&#37;&#41; of the 14 mutation carriers were diagnosed with HCM at initial assessment&#46; At&#8208;risk child relatives were defined as those with a positive mutation but a negative phenotype at enrollment&#46;</p><p id="spar0130" class="elsevierStyleSimplePara elsevierViewall">After 3&#46;5&#177;0&#46;8 years of follow&#8208;up&#44; two of the phenotype&#8208;negative mutation carriers developed HCM at 10 and 15 years of age &#40;28&#37; penetrance rate&#41;&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusions</span><p id="spar0135" class="elsevierStyleSimplePara elsevierViewall">The penetrance of HCM in phenotype&#8208;negative child relatives was 28&#37; after 3&#46;5 years of follow&#8208;up&#46; This underlines the need for long&#8208;term monitoring of mutation carriers irrespective of the presence of a positive phenotype&#46;</p></span>"
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                "termino" => "MCH"
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              8 => array:2 [
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              14 => array:2 [
                "termino" => "PCR"
                "descripcion" => "<p id="par0060" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Polymerase chain reaction</span></p>"
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                "termino" => "SNP"
                "descripcion" => "<p id="par0070" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Single nucleotide polymorphism</span></p>"
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                "descripcion" => "<p id="par0075" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Single nucleotide polymorphism database</span></p>"
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Toracalgia com esfor&#231;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">II</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Irm&#227;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">II</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pr&#243;prio&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Altera&#231;&#245;es do ECG&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Caso &#237;ndex&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">II</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Irm&#227;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">10&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar sem risco de doen&#231;a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">III</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">III</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">IV</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Tia paterna&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">11&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar sem risco de doen&#231;a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">IV</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Tia paterna&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">S&#237;ncope&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">V</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">10&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">V</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">11&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">VI</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">13&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">S&#237;ncope&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">VII</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pr&#243;prio&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Toracalgia com esfor&#231;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Toracalgia com esfor&#231;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Caso &#237;ndex&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">13&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">VII</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Irm&#227;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">10&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">14&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">VIII</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">14&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar sem risco de doen&#231;a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">VIII</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar sem risco de doen&#231;a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">16&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">IX</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Irm&#227;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#44;08&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">S&#237;ndrome LEOPARD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#63;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">IX</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Irm&#227;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">S&#237;ndrome LEOPARD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">Familiar em risco</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">IX</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pr&#243;prio&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#44;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Sopro&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">S&#237;ndrome LEOPARD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Caso &#237;ndex&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">X</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">16&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">20&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleBold">X</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pai&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">11&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">HF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar sem risco de doen&#231;a&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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          "leyenda" => "<p id="spar0090" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleItalic">MYBPC3</span>&#58; <span class="elsevierStyleItalic">myosin binding protein</span> C&#59; <span class="elsevierStyleItalic">MYH7&#58; myosin heavy chain</span>&#59; <span class="elsevierStyleItalic">MYL3</span>&#58; <span class="elsevierStyleItalic">myosin light chain</span> 3&#59; <span class="elsevierStyleItalic">TNNT2</span>&#58; <span class="elsevierStyleItalic">cardiac troponin</span><span class="elsevierStyleSmallCaps">T</span>&#46;</p>"
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                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Fam&#237;lia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Gene mutado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Altera&#231;&#227;o cDNA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Altera&#231;&#227;o prote&#237;na&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " colspan="2" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">Significado cl&#237;nico</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">I&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 27 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;2864&#95;2865delCT&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Pro955ArgfsX95&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CD982813</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">II&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 18 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;1684G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Ala562Thr&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Variante gen&#233;tica de significado incerto</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Possibly Damaging</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Disease Causing</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">II&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 18 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;1684G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Ala562Thr&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Variante gen&#233;tica de significado incerto</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Possibly Damaging</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Disease Causing</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">II&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">III&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 22 do gene <span class="elsevierStyleItalic">MYH7</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;2539&#95;2541delAAG&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Lys847del&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CD046025<br>HGMD CM0910620</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">III&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 22 do gene <span class="elsevierStyleItalic">MYH7</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;2539&#95;2541delAAG&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Lys847del&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CD046025<br>HGMD CM0910620</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IV&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IV&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 19 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;1828G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A e&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Asp610Asn&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Variante gen&#233;tica de significado incerto</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Probably Damaging</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Disease Causing</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 32 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;3617G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Gly1206Asp&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CM057198</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">V&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 4 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;458C<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Pro153His&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Muta&#231;&#227;o n&#227;o descrita</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Probably Damaging</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Disease Causing</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">10&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">V&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 27 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;2827C<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>T&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Arg943STOP&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CM032959</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">11&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VI&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 11 do gene <span class="elsevierStyleItalic">TNNT2</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;458&#95;489del3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Glu163del&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CD9518665</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VII&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 6 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;772G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Glu258Lys&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CM981322</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 8 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;836G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>C&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Gly279Ala&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Variante gen&#233;tica de significado incerto<br>HGMD CM031257</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Benign</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Polymorphism</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">13&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VII&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 6 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;772G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Glu258Lys&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Muta&#231;&#227;o patog&#233;nica<br>HGMD CM981322&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 8 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;836G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>C&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Gly279Ala&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Variante gen&#233;tica de significado incerto<br>HGMD CM031257</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Benign</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Polymorphism</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">14&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VIII&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VIII&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">16&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IX&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Aguarda&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Aguarda&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Aguarda&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IX&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 17 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;1519G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Gly507Arg&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Variante gen&#233;tica de significado incerto<br>HGMD CM032598</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Probably Damaging</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Disease Causing</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IX&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 17 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;1519G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Gly507Arg&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Variante gen&#233;tica de significado incerto<br>HGMD CM032598</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Probably Damaging</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Disease Causing</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">X&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 4 do gene <span class="elsevierStyleItalic">MYBPC3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;446C<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Ala149Asp&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " rowspan="2" align="left" valign="top">Muta&#231;&#227;o n&#227;o descrita</td><td class="td" title="table-entry  " align="left" valign="top">PolyPhen&#8208;2&#58; <span class="elsevierStyleItalic">Benign</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Polymorphism</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Ex&#227;o 2 do gene <span class="elsevierStyleItalic">MYL3</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">c&#46;145G<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>T&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">p&#46;Glu49STOP&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Muta&#231;&#227;o n&#227;o descrita&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Mutation Taster&#58; Disease causing</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">20&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">X&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab1365214.png"
              ]
            ]
          ]
        ]
        "descripcion" => array:1 [
          "pt" => "<p id="spar0085" class="elsevierStyleSimplePara elsevierViewall">Muta&#231;&#245;es de genes sarcom&#233;ricos identificadas nos doentes em estudo</p>"
        ]
      ]
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        "identificador" => "tbl0015"
        "etiqueta" => "Tabela 3"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "detalles" => array:1 [
          0 => array:3 [
            "identificador" => "at3"
            "detalle" => "Tabela "
            "rol" => "short"
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        ]
        "tabla" => array:3 [
          "leyenda" => "<p id="spar0100" class="elsevierStyleSimplePara elsevierViewall">AF&#58; antecedentes familiares&#59; ECG&#58; eletrocardiograma&#59; SAM&#58; <span class="elsevierStyleItalic">Systolic anterior movement</span>&#59; SIVd&#58; dimens&#227;o diast&#243;lica do septo interventricular&#59; TSVE&#58; trato de sa&#237;da do ventr&#237;culo esquerdo&#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=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Fam&#237;lia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Fator de risco &#171;cl&#225;ssico&#187;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Gen&#243;tipo&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Obstru&#231;&#227;o TSVE &#40;&#916;<span class="elsevierStyleHsp" style=""></span>m&#225;x TSVE&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black"><span class="elsevierStyleItalic">Score</span> de risco ECG&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">I&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">AF morte s&#250;bita&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">II&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">SIVd<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>30<span class="elsevierStyleHsp" style=""></span>mm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IV&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">S&#237;ncope&#59; AF morte s&#250;bita&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Gen&#243;tipo complexo<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">&#42;</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">11&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VI&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">S&#237;ncope&#59; SIVd<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>30<span class="elsevierStyleHsp" style=""></span>mm&#59; AF morte s&#250;bita&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VII&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">SAM&#44; &#916;<span class="elsevierStyleHsp" style=""></span>intraventricular<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>100<span class="elsevierStyleHsp" style=""></span>mmHg &#40;esfor&#231;o&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IX&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">X&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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          "notaPie" => array:1 [
            0 => array:3 [
              "identificador" => "tblfn0005"
              "etiqueta" => "&#42;"
              "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Gen&#243;tipo complexo &#8211; doentes portadores de mais do que uma muta&#231;&#227;o do mesmo gene&#46;</p>"
            ]
          ]
        ]
        "descripcion" => array:1 [
          "pt" => "<p id="spar0095" class="elsevierStyleSimplePara elsevierViewall">Estratifica&#231;&#227;o do risco de morte s&#250;bita dos doentes em seguimento</p>"
        ]
      ]
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        "identificador" => "tbl0020"
        "etiqueta" => "Tabela 4"
        "tipo" => "MULTIMEDIATABLA"
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        "mostrarDisplay" => false
        "detalles" => array:1 [
          0 => array:3 [
            "identificador" => "at4"
            "detalle" => "Tabela "
            "rol" => "short"
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        ]
        "tabla" => array:2 [
          "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=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="" valign="top" scope="col" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="" valign="top" scope="col" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso 1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso 3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso 8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso 11&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso 12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso 18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso 19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Desvio eixo QRS&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1 ponto&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Invers&#227;o T nas deriva&#231;&#245;es membros<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">&#42;</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1 ponto&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Invers&#227;o T nas deriva&#231;&#245;es precordiais<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">&#42;</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2 pontos&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Depress&#227;o ST<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>2<span class="elsevierStyleHsp" style=""></span>mm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2 pontos&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">S dominante V4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2 pontos&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#62;<span class="elsevierStyleHsp" style=""></span>7&#44;7 <span class="elsevierStyleHsp" style=""></span>mV<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>1 ponto&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Soma da amplitude QRS deriva&#231;&#245;es dos membros&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#62;<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleHsp" style=""></span>mV<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>2 pontos&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#62;<span class="elsevierStyleHsp" style=""></span>12<span class="elsevierStyleHsp" style=""></span>mV<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>3 pontos&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Produto amplitude&#8208;dura&#231;&#227;o QRS nas 12 deriva&#231;&#245;es&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#62;<span class="elsevierStyleHsp" style=""></span>2&#44;2<span class="elsevierStyleHsp" style=""></span>mV&#46;s<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>1 ponto&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#62;<span class="elsevierStyleHsp" style=""></span>2&#44;5<span class="elsevierStyleHsp" style=""></span>mV&#46;s<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>2 pontos&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#62;<span class="elsevierStyleHsp" style=""></span>3<span class="elsevierStyleHsp" style=""></span>mV&#46;s<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>3 pontos&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">QTc<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>440<span class="elsevierStyleHsp" style=""></span>ms&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1 ponto&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Score</span> de risco&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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          "pt" => "<p id="spar0105" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleItalic">Score</span> de risco eletrocardiogr&#225;fico</p>"
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          "leyenda" => "<p id="spar0115" class="elsevierStyleSimplePara elsevierViewall">CDI&#58; cardiodisfibrilhador implant&#225;vel&#59; F&#58; feminino&#59; M&#58; masculino&#59; MCH&#58; miocardiopatia hipertr&#243;fica&#59; N&#58; fen&#243;tipo normal&#59; <span class="elsevierStyleItalic">NYHA</span>&#58; <span class="elsevierStyleItalic">New York Heart Association</span>&#46;</p>"
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                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Caso&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Fam&#237;lia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Idade &#40;anos&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Fen&#243;tipo&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Sintomas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Complica&#231;&#245;es&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Classifica&#231;&#227;o&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">I&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">16&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">II&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">10&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">II&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Caso &#237;ndex&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">III&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Palpita&#231;&#245;es&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar em risco&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">III&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IV&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">9&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar em risco&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">10&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">V&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar em risco&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">11&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VI&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CDI&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">VII&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">16&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IX&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar em risco&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IX&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">N&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar em risco&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IX&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Caso &#237;ndex&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">19&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MCH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Familiar afetado&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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        "descripcion" => array:1 [
          "pt" => "<p id="spar0110" class="elsevierStyleSimplePara elsevierViewall">Caracter&#237;sticas dos doentes &#224; data da &#250;ltima consulta</p>"
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                    0 => array:2 [
                      "titulo" => "Proposal for contemporary screening strategies in families with hypertrophic cardiomyopathy"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "B&#46;J&#46; Maron"
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                    0 => array:2 [
                      "doi" => "10.1016/j.jacc.2004.08.052"
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                        "tituloSerie" => "J Am Coll Cardiol&#46;"
                        "fecha" => "2004"
                        "volumen" => "44"
                        "paginaInicial" => "2125"
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                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15582308"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Penetrance of hypertrophic cardiomyopathy in children and adolescents&#58; A 12&#8208;year follow&#8208;up study of clinical screening and predictive genetic testing"
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                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "M&#46;K&#46; Jensen"
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                            2 => "M&#46; Christiansen"
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                    0 => array:2 [
                      "doi" => "10.1161/CIRCULATIONAHA.111.090514"
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                        "tituloSerie" => "Circulation&#46;"
                        "fecha" => "2013"
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                        "paginaInicial" => "48"
                        "paginaFinal" => "54"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23197161"
                            "web" => "Medline"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy&#58; the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology &#40;ESC&#41;"
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                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
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                    0 => array:2 [
                      "doi" => "10.1093/eurheartj/ehu284"
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                        "paginaFinal" => "2779"
                        "link" => array:1 [
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                0 => array:2 [
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                      "titulo" => "ACCF&#47;AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy&#58; a report of the American College of Cardiology Foundation&#47;American Heart Association Task Force on Practice Guidelines"
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                    0 => array:2 [
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                          0 => array:2 [
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                      "titulo" => "Guidelines and standards for performance of a pediatric echocardiogram&#58; a report from the Task Force of the Pediatric Council of the American Society of Echocardiography"
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                          "autores" => array:3 [
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                        "tituloSerie" => "J Pediatr&#46;"
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                        "paginaInicial" => "62"
                        "paginaFinal" => "66"
                        "link" => array:1 [
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                          "autores" => array:1 [
                            0 => "H&#46; Bazett"
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                          "autores" => array:3 [
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                        "link" => array:1 [
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