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  <front>
    <journal-meta>
      <journal-id journal-id-type="nlm-ta">Arq Bras Cardiol</journal-id>
      <journal-id journal-id-type="publisher-id">abc</journal-id>
      <journal-title-group>
        <journal-title>Arquivos Brasileiros de Cardiologia</journal-title>
        <abbrev-journal-title abbrev-type="publisher">Arq. Bras. Cardiol.</abbrev-journal-title>
      </journal-title-group>
      <issn pub-type="ppub">0066-782X</issn>
      <issn pub-type="epub">1678-4170</issn>
      <publisher>
        <publisher-name>Sociedade Brasileira de Cardiologia - SBC</publisher-name>
      </publisher>
    </journal-meta>
    <article-meta>
      <article-id specific-use="scielo-v3" pub-id-type="publisher-id">TbBnRGFFYHjjpRv8Hn6mQzF</article-id>
      <article-id specific-use="scielo-v2" pub-id-type="publisher-id">S0066-782X2021000300036</article-id>
      <article-id pub-id-type="publisher-id">abc.20190485</article-id>
      <article-id pub-id-type="doi">10.36660/abc.20190485</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Relato de Caso</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Ecocardiografia Tridimensional Revela o Verdadeiro Inimigo em um Jovem de Sexo Masculino com Infarto do Mioc&#225;rdio com Supradesnivelamento do Segmento ST e Regurgita&#231;&#227;o Mitral Grave: &#8220;Pseudo-Fenda&#8221; Posterior e Prolapso da Valva Mitral</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <contrib-id contrib-id-type="orcid">0000-0002-5032-1940</contrib-id>
          <name>
            <surname>Mihaila</surname>
            <given-names>Sorina</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">
            <sup>1</sup>
          </xref>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Velcea</surname>
            <given-names>Andreea Elena</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">
            <sup>1</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Badano</surname>
            <given-names>Luigi Paolo</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">
            <sup>2</sup>
          </xref>
          <xref ref-type="aff" rid="aff3">
            <sup>3</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Dragos</surname>
            <given-names>Vinereanu</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">
            <sup>1</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Muraru</surname>
            <given-names>Denisa</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">
            <sup>2</sup>
          </xref>
          <xref ref-type="aff" rid="aff3">
            <sup>3</sup>
          </xref>
        </contrib>
        <aff id="aff1">
          <label>1</label>
          <institution content-type="orgname">University of Medicine and Pharmacy Carol Davila Bucharest</institution>
          <institution content-type="orgdiv1">Emergency University Hospital Bucharest</institution>
          <addr-line>
            <named-content content-type="city">Bucharest</named-content>
          </addr-line>
          <country country="RO">Rom&#234;nia</country>
          <institution content-type="original">University of Medicine and Pharmacy Carol Davila Bucharest - Emergency University Hospital Bucharest, Bucharest - Rom&#234;nia</institution>
        </aff>
        <aff id="aff2">
          <label>2</label>
          <institution content-type="orgname">San Luca Hospital</institution>
          <institution content-type="orgdiv1">Department of cardiac, neural and metabolic sciences</institution>
          <institution content-type="orgdiv2">Istituto Auxologico Italiano, IRCCS</institution>
          <addr-line>
            <named-content content-type="city">Mil&#227;o</named-content>
          </addr-line>
          <country country="IT">It&#225;lia</country>
          <institution content-type="original">Istituto Auxologico Italiano, IRCCS, Department of cardiac, neural and metabolic sciences, San Luca Hospital, Mil&#227;o - It&#225;lia</institution>
        </aff>
        <aff id="aff3">
          <label>3</label>
          <institution content-type="orgname">University of Milano-Bicocca</institution>
          <institution content-type="orgdiv1">Department of Medicine and Surgery</institution>
          <addr-line>
            <named-content content-type="city">Mil&#227;o</named-content>
          </addr-line>
          <country country="IT">It&#225;lia</country>
          <institution content-type="original">University of Milano-Bicocca, Department of Medicine and Surgery, Mil&#227;o - It&#225;lia</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c1"><bold>Correspond&#234;ncia: Sorina Mihaila Baldea</bold> &#8226; Splaiul Independentei no 169, sector 5, 10th floor, Bucharest, Romania - Cardiology and Cardiovascular Surgery Department University Hospital of Bucharest, Romania. CEP: <postal-code>050098</postal-code>. Email: <email>sorinamihaila1981@gmail.com</email> </corresp>
        <fn fn-type="con">
          <p>
            <bold>Contribui&#231;&#227;o dos autores</bold>
          </p>
          <p>Concep&#231;&#227;o e desenho da pesquisa: Baldea SM, Vinereanu D; Obten&#231;&#227;o de dados e Reda&#231;&#227;o do manuscrito: Baldea SM, Velcea AE; An&#225;lise e interpreta&#231;&#227;o dos dados: Baldea SM, Badano LP, Muraru D; Revis&#227;o cr&#237;tica do manuscrito quanto ao conte&#250;do intelectual importante: Baldea SM, Velcea AE, Badano LP, Vinereanu D, Muraru D.</p>
        </fn>
        <fn fn-type="conflict">
          <p>
            <bold>Potencial conflito de interesses</bold>
          </p>
          <p>Declaro n&#227;o haver conflito de interesses pertinentes.</p>
        </fn>
      </author-notes>
      <pub-date date-type="pub" publication-format="electronic">
        <day>05</day>
        <month>02</month>
        <year>2021</year>
      </pub-date>
      <pub-date date-type="collection" publication-format="electronic">
        <month>02</month>
        <year>2021</year>
      </pub-date>
      <volume>116</volume>
      <issue>2 suppl 1</issue>
      <fpage>36</fpage>
      <lpage>38</lpage>
      <history>
        <date date-type="received">
          <day>23</day>
          <month>07</month>
          <year>2019</year>
        </date>
        <date date-type="rev-recd">
          <day>11</day>
          <month>06</month>
          <year>2020</year>
        </date>
        <date date-type="accepted">
          <day>05</day>
          <month>08</month>
          <year>2020</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="https://creativecommons.org/licenses/by/4.0/" xml:lang="en">
          <license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution License</license-p>
        </license>
      </permissions>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Valva Mitral/anormalidades</kwd>
        <kwd>Infarto do Mioc&#225;rdio</kwd>
        <kwd>Ecocardiografia Tridimensional/m&#233;todos</kwd>
        <kwd>Diagn&#243;stico por Imagem</kwd>
        <kwd>Adulto Jovem</kwd>
      </kwd-group>
      <counts>
        <fig-count count="4"/>
        <table-count count="0"/>
        <equation-count count="0"/>
        <ref-count count="6"/>
        <page-count count="3"/>
      </counts>
    </article-meta>
  </front>
  <body>
    <sec sec-type="intro">
      <title>Introdu&#231;&#227;o</title>
      <p>A ecocardiografia tridimensional (E3D) desempenha um papel cada vez mais importante no diagn&#243;stico das valvopatias, na avalia&#231;&#227;o da morfologia valvar de maneira anat&#244;mica e no estabelecimento da reparabilidade da valva, sem ter as limita&#231;&#245;es da ecocardiografia bidimensional (E2D) convencional.<xref ref-type="bibr" rid="B1"><sup>1</sup></xref> </p>
      <p>N&#243;s relatamos o caso de um paciente jovem que apresentou infarto agudo do mioc&#225;rdio anterior com supradesnivelamento do segmento ST e regurgita&#231;&#227;o mitral (RM) grave, ap&#243;s interven&#231;&#227;o coron&#225;ria percut&#226;nea (ICP) prim&#225;ria bem-sucedida da art&#233;ria descendente anterior esquerda, cuja ecocardiografia transesof&#225;gica tridimensional (ETE 3D) revelou uma causa inesperada da RM, a saber, patologia complexa da valva mitral (VM) constituindo prolapso dos escalopes P2-3, <italic>flail</italic> de corda e pseudo-fenda do folheto posterior separando o segmento P1 do segmento P2.</p>
    </sec>
    <sec sec-type="cases">
      <title>Relado de Caso</title>
      <p>Um paciente do sexo masculino, 38 anos de idade, sem fatores de risco cardiovascular conhecidos, apresentou quadro in&#237;cio agudo de dor tor&#225;cica constritiva. O exame card&#237;aco revelou ritmo regular, sopro sist&#243;lico apical e press&#227;o arterial normal. O eletrocardiograma de 12 deriva&#231;&#245;es em repouso de emerg&#234;ncia mostrou eleva&#231;&#227;o do segmento ST nas deriva&#231;&#245;es V<sub>1-6</sub> e taquicardia ventricular n&#227;o sustentada recorrente. A angiografia coron&#225;ria de emerg&#234;ncia mostrou oclus&#227;o tromb&#243;tica aguda da art&#233;ria descendente anterior esquerda proximal, estenose n&#227;o cr&#237;tica da art&#233;ria coron&#225;ria direita e estenose de 90% da art&#233;ria circunflexa esquerda. Foi realizada ICP prim&#225;ria com implante de stent na art&#233;ria descendente anterior esquerda, com bons resultados procedurais.</p>
      <p>A E2D transtor&#225;cica p&#243;s-procedimento mostrou ventr&#237;culo esquerdo (VE) n&#227;o dilatado, anormalidades do movimento da parede septal e discreta disfun&#231;&#227;o sist&#243;lica do VE (fra&#231;&#227;o de eje&#231;&#227;o do VE = 50%), bem como dilata&#231;&#227;o moderada do &#225;trio esquerdo (AE) e RM grave com um jato exc&#234;ntrico, dirigido anteriormente para o AE (regurgita&#231;&#227;o holossist&#243;lica; &#225;rea efetiva do orif&#237;cio regurgitante = 0,4 cm<sup>2</sup>; volume regurgitante = 55 ml/m<sup>2</sup>). Um discreto prolapso do folheto posterior da VM tamb&#233;m foi detectado pela ecocardiografia transtor&#225;cica bidimensional (ETT 2D). No entanto, nem a anormalidade do movimento da parede septal nem o prolapso da VM, visto por ETT 2D, explicaram completamente a gravidade da RM. Neste contexto, os mecanismos e a gravidade da RM foram mais explorados por meio da ecocardiografia transesof&#225;gica, incluindo avalia&#231;&#227;o por E3D. A avalia&#231;&#227;o da VM por ETE 3D da &#8220;vis&#227;o cir&#250;rgica&#8221; mostrou prolapso dos segmentos P<sub>2-3</sub> (<xref ref-type="fig" rid="f1">Figura 1</xref>, Painel A), ruptura de corda fixada no folheto posterior da VM e uma indenta&#231;&#227;o profunda na VM posterior (<xref ref-type="fig" rid="f1">Figura 1</xref>, Painel B), levando a um jato regurgitante exc&#234;ntrico no AE at&#233; as veias pulmonares. Com a finalidade de determinar a reparabilidade da VM, foi realizado o exame com avalia&#231;&#227;o da VM por ETE 3D da vis&#227;o ventricular (<xref ref-type="fig" rid="f1">Figura 1</xref>, Painel C), onde foi detectado uma pseudo-fenda do folheto posterior, com o escalope P<sub>1</sub> separados dos segmentos de prolapso P<sub>2-3</sub>. A ETE 2D em cores mostrou um jato &#8220;dividido&#8221; de RM (<xref ref-type="fig" rid="f2">Figura 2</xref>, Painel A), enquanto a ETE 3D em cores mostrou um jato exc&#234;ntrico de RM, com origem ampla, direcionado anteriormente (<xref ref-type="fig" rid="f2">Figura 2</xref>, Pain&#233;is B e C), explicando melhor o mecanismo da RM.</p>
      <fig id="f1">
        <label>Figura 1</label>
        <caption>
          <title>Avalia&#231;&#227;o morfol&#243;gica tridimensional da valva mitral (abordagem transesof&#225;gica). Painel A) &#8220;Vis&#227;o cir&#250;rgica&#8221; da valva mitral fechada, do lado do &#225;trio esquerdo, que mostra o prolapso complexo dos escalopes P<sub>2-3</sub>. O folheto anterior da valva mitral tem morfologia normal. Painel B) Abertura da valva mitral revela que o segmento P<sub>1</sub> est&#225; separado dos segmentos P<sub>2-3</sub>, levantando a suspeita de uma pseudo-fenda. Painel C) Valva mitral visualizada do lado do ventr&#237;culo esquerdo. A pseudo-fenda do folheto posterior da valva mitral, entre os segmentos P<sub>1</sub> e P<sub>2-3</sub>, pode ser identificada. AE: &#225;trio esquerdo, Ao: aorta, RM: regurgita&#231;&#227;o mitral, VA: valva a&#243;rtica, VE: ventr&#237;culo esquerdo, VMA: valva mitral anterior, VSVE: via de sa&#237;da do ventr&#237;culo esquerdo.</title>
        </caption>
        <graphic xlink:href="1678-4170-abc-116-2suppl1-0036-90485-gf01.tif"/></fig>
      <fig id="f2">
        <label>Figura 2</label>
        <caption>
          <title>Avalia&#231;&#227;o bi- e tridimensional em cores da regurgita&#231;&#227;o mitral (abordagem transesof&#225;gica). Painel A) A valva mitral, visualizada a 85 graus, mostra os dois componentes da regurgita&#231;&#227;o mitral, causada pelo prolapso e pela pseudo-fenda. Painel B) A vis&#227;o de eixo longo dos escalopes A2/P2 mostra o jato da regurgita&#231;&#227;o mitral causado pelo prolapso de P2, oposto ao escalope. Painel C) A &#8220;vis&#227;o cir&#250;rgica&#8221; da valva mitral mostra a origem ampla do jato da regurgita&#231;&#227;o mitral, visto do &#225;trio esquerdo, que &#233; direcionado anteriormente (s&#227;o exibidos apenas os fluxos retr&#243;grados). Ao: aorta; RM: regurgita&#231;&#227;o mitral, VA: valva a&#243;rtica, VMA: valva mitral anterior, VMP: valva mitral posterior.</title>
        </caption>
        <graphic xlink:href="1678-4170-abc-116-2suppl1-0036-90485-gf02.tif"/></fig>
      <p>Foram exclu&#237;das potenciais causas adquiridas desses achados morfol&#243;gicos, tais como trauma pr&#233;vio da VM, cirurgia ou endocardite infecciosa e o diagn&#243;stico final foi de RM grave devido ao prolapso complexo da VM dos segmentos P<sub>2-3</sub> e ruptura da corda fixada no folheto posterior da VM, associada a uma pseudo-fenda do folheto posterior entre os segmentos P<sub>1</sub> e P<sub>2</sub>. O paciente foi encaminhado para parecer cir&#250;rgico, devido ao aumento do AE (mostrando evolu&#231;&#227;o prolongada da RM) e novo aparecimento de sintomas ap&#243;s o evento agudo (dispneia de exerc&#237;cio). Foram realizados com sucesso o reparo da VM, incluindo ressec&#231;&#227;o do prolapso, sutura da pseudo-fenda da VM e anuloplastia mitral, associados ao enxerto da art&#233;ria circunflexa esquerda. No seguimento de tr&#234;s anos, o paciente n&#227;o apresentou recidiva da RM.</p>
    </sec>
    <sec sec-type="discussion">
      <title>Discuss&#227;o</title>
      <p>Nosso caso cl&#237;nico mostra a utilidade da E3D para o diagn&#243;stico e avalia&#231;&#227;o morfol&#243;gica de les&#245;es complexas da VM, principalmente quando a etiologia &#233; incerta, bem como o seu papel no planejamento de procedimentos cir&#250;rgicos. A suspeita inicial da etiologia da RM era isqu&#234;mica; entretanto, o curto per&#237;odo de isquemia (menos de 2 horas at&#233; a revasculariza&#231;&#227;o), as pequenas anormalidades no movimento da parede do VE e a boa fun&#231;&#227;o sist&#243;lica do VE tornaram essa causa improv&#225;vel. A avalia&#231;&#227;o detalhada da E2D revelou prolapso leve da VM posterior, que tamb&#233;m foi insuficiente para explicar a gravidade da RM. Por outro lado, a ETE 3D revelou o prolapso complexo da VM dos segmentos, a ruptura de corda e a pseudo-fenda do folheto posterior da VM separando o escalope P<sub>1</sub> do P<sub>2</sub>.</p>
      <p>Hipotetiza-se que as fendas s&#227;o resultado da express&#227;o incompleta de um defeito do coxim endoc&#225;rdico, na maioria das vezes envolvendo a parte central do folheto anterior da VM.<xref ref-type="bibr" rid="B2"><sup>2</sup></xref><sup>,</sup><xref ref-type="bibr" rid="B3"><sup>3</sup></xref> Fendas verdadeiras que afetam a VM posterior s&#227;o extremamente raras,<xref ref-type="bibr" rid="B2"><sup>2</sup></xref> no entanto, as pseudo-fendas s&#227;o uma classe separada de anomalias morfol&#243;gicas do folheto posterior da VM. As pseudo-fendas s&#227;o indenta&#231;&#245;es profundas que compartilham a localiza&#231;&#227;o das fendas normais entre os escalopes da VM posterior, mas com mais de 50% da profundidade dos escalopes adjacentes.<xref ref-type="bibr" rid="B4"><sup>4</sup></xref> Esta anomalia est&#225; frequentemente associada &#224; rota&#231;&#227;o anti-hor&#225;ria dos m&#250;sculos papilares, m&#250;sculo papilar acess&#243;rio ou folheto da VM acess&#243;rio, e prolapso da v&#225;lvula mitral.<xref ref-type="bibr" rid="B5"><sup>5</sup></xref> Nosso paciente apresentou RM como consequ&#234;ncia de prolapso complexo da VM com ruptura de corda, associado &#224; pseudo-fenda. As altas press&#245;es diast&#243;licas finais do VE no contexto do evento isqu&#234;mico e a disfun&#231;&#227;o sist&#243;lica do VE provavelmente pioraram a gravidade da RM, pois o paciente negava dispneia antes da interna&#231;&#227;o hospitalar. Al&#233;m disso, permanece a d&#250;vida se a ruptura da corda ocorreu antes ou se estava relacionada ao evento isqu&#234;mico.</p>
      <p>No entanto, apesar de n&#227;o ser totalmente respons&#225;vel pela RM, a presen&#231;a da pseudo-fenda tem influ&#234;ncia adicional na decis&#227;o cir&#250;rgica quanto &#224; reparabilidade da VM. Mantovani et al.,<xref ref-type="bibr" rid="B6"><sup>6</sup></xref> mostraram que 35% dos pacientes com prolapso da VM apresentavam pseudo-fendas, n&#227;o vistas pela E2D e reveladas apenas pela E3D. A presen&#231;a de pseudo-fendas n&#227;o resolvidas em pacientes com prolapso da VM foi associada a pior progn&#243;stico ap&#243;s o reparo da VM e maior recorr&#234;ncia da RM no seguimento. Neste contexto, foi realizado o reparo da VM em nosso paciente, incluindo a sutura da pseudo-fenda da VM.</p>
    </sec>
    <sec sec-type="conclusions">
      <title>Conclus&#245;es</title>
      <p>A ETE 3D &#233; uma t&#233;cnica &#250;til e vi&#225;vel para o diagn&#243;stico correto em pacientes com doen&#231;a complexa da VM, especialmente quando a etiologia &#233; incerta, bem como para a determina&#231;&#227;o da a reparabilidade da valva. Embora as pseudo-fendas da VM raramente levem &#224; regurgita&#231;&#227;o, elas est&#227;o associadas a piores desfechos p&#243;s-operat&#243;rios; portanto, precisam ser suturadas durante o reparo da VM.</p>
    </sec>
  </body>
  <back>
    <fn-group>
      <fn fn-type="supported-by" id="fn1">
        <p>
          <bold>Fontes de financiamento</bold>
        </p>
        <p>O presente estudo n&#227;o teve fontes de financiamento externas.</p>
      </fn>
      <fn fn-type="other" id="fn2">
        <p>
          <bold>Vincula&#231;&#227;o Acad&#234;mica</bold>
        </p>
        <p>N&#227;o h&#225; vincula&#231;&#227;o deste estudo a programas de p&#243;s-gradua&#231;&#227;o.</p>
      </fn>
      <fn fn-type="other" id="fn3">
        <p>
          <bold>Aprova&#231;&#227;o &#233;tica e consentimento informado</bold>
        </p>
        <p>Este estudo foi aprovado pelo Comit&#234; de &#201;tica do Emergency University Hospital Bucharest sob o n&#250;mero de protocolo 15/2.07. Todos os procedimentos envolvidos nesse estudo est&#227;o de acordo com a Declara&#231;&#227;o de Helsinki de 1975, atualizada em 2013. O consentimento informado foi obtido de todos os participantes inclu&#237;dos no estudo</p>
      </fn>
    </fn-group>
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      <title>Refer&#234;ncias</title>
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      <ref id="B3">
        <label>3</label>
        <element-citation publication-type="journal">
          <person-group person-group-type="author">
            <name>
              <surname>Miglioranza</surname>
              <given-names>MH</given-names>
            </name>
            <name>
              <surname>Muraru</surname>
              <given-names>D</given-names>
            </name>
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  <sub-article article-type="translation" id="S01" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Case Report</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Three-dimensional Echocardiography Reveals the True Enemy in a Young Male with ST-Elevation Myocardial Infarction and Severe Mitral Regurgitation: Posterior Mitral Valve &#8220;Pseudo-Cleft&#8221; and Prolapse</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <contrib-id contrib-id-type="orcid">0000-0002-5032-1940</contrib-id>
          <name>
            <surname>Mihaila</surname>
            <given-names>Sorina</given-names>
          </name>
          <xref ref-type="aff" rid="aff4">
            <sup>1</sup>
          </xref>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Velcea</surname>
            <given-names>Andreea Elena</given-names>
          </name>
          <xref ref-type="aff" rid="aff4">
            <sup>1</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Badano</surname>
            <given-names>Luigi Paolo</given-names>
          </name>
          <xref ref-type="aff" rid="aff5">
            <sup>2</sup>
          </xref>
          <xref ref-type="aff" rid="aff6">
            <sup>3</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Dragos</surname>
            <given-names>Vinereanu</given-names>
          </name>
          <xref ref-type="aff" rid="aff4">
            <sup>1</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Muraru</surname>
            <given-names>Denisa</given-names>
          </name>
          <xref ref-type="aff" rid="aff5">
            <sup>2</sup>
          </xref>
          <xref ref-type="aff" rid="aff6">
            <sup>3</sup>
          </xref>
        </contrib>
        <aff id="aff4">
          <label>1</label>
          <addr-line>
            <named-content content-type="city">Bucharest</named-content>
          </addr-line>
          <country country="RO">Romania</country>
          <institution content-type="original">University of Medicine and Pharmacy Carol Davila Bucharest - Emergency University Hospital Bucharest, Bucharest - Romania</institution>
        </aff>
        <aff id="aff5">
          <label>2</label>
          <addr-line>
            <named-content content-type="city">Milan</named-content>
          </addr-line>
          <country country="IT">Italy</country>
          <institution content-type="original">Istituto Auxologico Italiano, IRCCS, Department of cardiac, neural and metabolic sciences, San Luca Hospital, Milan - Italy</institution>
        </aff>
        <aff id="aff6">
          <label>3</label>
          <addr-line>
            <named-content content-type="city">Milan</named-content>
          </addr-line>
          <country country="IT">Italy</country>
          <institution content-type="original">University of Milano-Bicocca, Department of Medicine and Surgery, Milan - Italy</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c2"><bold>Mailing Address: Sorina Mihaila Baldea</bold> &#8226; Splaiul Independentei no 169, sector 5, 10th floor, Bucharest, Romania - Cardiology and Cardiovascular Surgery Department University Hospital of Bucharest, Romania. CEP <postal-code>050098</postal-code>. Email: <email>sorinamihaila1981@gmail.com</email> </corresp>
        <fn fn-type="con">
          <p>
            <bold>Author contributions</bold>
          </p>
          <p>Conception and design of the research: Baldea SM, Vinereanu D; Data acquisition and Writing of the manuscript: Baldea SM, Velcea AE; Analysis and interpretation of the data: Baldea SM, Badano LP; Critical revision of the manuscript for intellectual content: Baldea SM, Velcea AE, Badano LP, Vinereanu D.</p>
        </fn>
        <fn fn-type="conflict">
          <p>
            <bold>Potential Conflict of Interest</bold>
          </p>
          <p>No potential conflict of interest relevant to this article was reported.</p>
        </fn>
      </author-notes>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Mitral Valve/abnormalities</kwd>
        <kwd>Myocardial Infarction</kwd>
        <kwd>Echocardiography, Three Dimensional/methods</kwd>
        <kwd>Diagnosis Imaging</kwd>
        <kwd>Young Adult</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <sec sec-type="intro">
        <title>Introduction</title>
        <p>Three-dimensional echocardiography (3DE) plays an increasingly important role in the diagnosis of valvular heart disease, in the assessment of valvular morphology in an anatomical manner, and in establishing valve repairability, beyond the limitations of conventional two-dimensional echocardiography (2DE).<xref ref-type="bibr" rid="B1"><sup>1</sup></xref> </p>
        <p>We report the case of a young patient presenting with acute anterior ST-segment elevation myocardial infarction and severe mitral regurgitation (MR) after successful primary percutaneous coronary intervention (PCI) of the left anterior descending artery, whose three-dimensional transesophageal echocardiography (3D TEE) revealed an unexpected cause of the MR, namely, complex mitral valve (MV) pathology consisting of prolapse of the P<sub>2-3</sub> scallops, flail chordae, and pseudo-cleft of the posterior leaflet separating the P<sub>1</sub> from the P<sub>2</sub> segment.</p>
      </sec>
      <sec sec-type="cases">
        <title>Case Report</title>
        <p>A 38-year-old male patient, without any known cardiovascular risk factors, presented with acute onset of constrictive thoracic pain. Cardiac examination revealed regular rhythm, apical systolic murmur, and normal blood pressure. Emergency 12-lead resting electrocardiogram showed ST-segment elevation in the V<sub>1-6</sub> leads and recurrent non-sustained ventricular tachycardia. The emergency coronary angiogram showed acute thrombotic occlusion of the proximal left anterior descending artery, non-critical stenosis of the right coronary artery, and 90% stenosis of the left circumflex artery. Primary PCI with stenting of the left anterior descending artery was performed, with good procedural results.</p>
        <p>Post-procedural transthoracic 2DE showed non-dilated left ventricle (LV), septal wall motion abnormalities, and mild LV systolic dysfunction (LV ejection fraction = 50%), as well as moderate dilation of the left atrium (LA) and severe MR with an eccentric jet, directed anteriorly into the LA (holo-systolic regurgitation, effective regurgitant orifice area = 0.4 cm<sup>2</sup>, regurgitant volume = 55 ml/m<sup>2</sup>). A mild prolapse of the posterior MV leaflet was also detected by two-dimensional transthoracic echocardiography (2D TTE). However, neither the septal wall motion abnormality nor the MV prolapse as seen by 2D TTE entirely explained the severity of the MR. In this context, the mechanisms and the severity of the MR were further explored using transesophageal echocardiography, including 3DE assessment. 3D TEE assessment of the MV from the &#8220;surgical view&#8221; showed prolapse of the P<sub>2-3</sub> segments (<xref ref-type="fig" rid="f3">Figure 1</xref>, Panel A), a ruptured chordae attached to the posterior MV leaflet, and a deep indentation of the posterior MV (<xref ref-type="fig" rid="f3">Figure 1</xref>, Panel B), leading to an eccentric regurgitant jet into the LA up to the pulmonary veins. To establish MV repairability, the exam was completed with 3DE assessment of the MV from the ventricular view (<xref ref-type="fig" rid="f3">Figure 1</xref>, Panel C), where a pseudo-cleft of the posterior leaflet was detected, with the P<sub>1</sub> scallop separated from the P<sub>2-3</sub> prolapsing segments. Color 2D TEE showed a &#8220;split&#8221; jet of MR (<xref ref-type="fig" rid="f4">Figure 2</xref>, Panel A), while color 3D TEE showed an eccentric MR jet, with wide origin, directed anteriorly (<xref ref-type="fig" rid="f4">Figure 2</xref>, Panel B and Panel C), further explaining the mechanism of the MR.</p>
        <fig id="f3">
          <label>Figure 1</label>
          <caption>
            <title>Three-dimensional morphological assessment of the mitral valve (transesophageal approach). Panel A. &#8220;Surgical view&#8221; of the closed mitral valve, seen from the left atrial side, which shows a complex prolapse of the P2-3 scallops. The anterior mitral valve leaflet shows normal morphology. Panel B. Opening of the mitral valve reveals that the P1 segment is separated from the P2-3 segments, raising the suspicion of a pseudo-cleft. Panel C. The mitral valve visualized from the left ventricular side. The pseudo-cleft of the posterior mitral valve leaflet, between the P1 and the P2-3 segments, can be identified. AMV: anterior mitral valve; Ao: aorta; AoV: aortic valve; LA: left atrium; LV: left ventricle; LVOT: left ventricular outflow tract.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-116-2suppl1-0036-90485-gf01-en.tif"/></fig>
        <fig id="f4">
          <label>Figure 2</label>
          <caption>
            <title>Two- and three-dimensional color assessment of the mitral regurgitation (transesophageal approach). Panel A. The mitral valve, visualized at 85 degrees, shows the two components of the mitral regurgitation, caused by the prolapse and the pseudo-cleft. Panel B. Long-axis view throughout the A2/P2 scallops shows the mitral regurgitation jet caused by the prolapse of P2, opposite the scallop. Panel C. The &#8220;surgical view&#8221; of the mitral valve shows the wide origin of the mitral regurgitation jet, seen from the left atrium, which is directed anteriorily (only retrograde flows are displayed). AMV: anterior mitral valve, Ao: aorta, AoV: aortic valve, MR: mitral regurgitation, PMV: posterior mitral valve.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-116-2suppl1-0036-90485-gf02-en.tif"/></fig>
        <p>Potential acquired causes of these morphological findings, such as previous MV trauma, surgery, or infective endocarditis were excluded, and the final diagnosis was severe MR due to complex MV prolapse of the P<sub>2-3</sub> segments and ruptured chordae attached to the posterior MV leaflet, associated with a pseudo-cleft of the posterior leaflet between the P<sub>1</sub> and P<sub>2</sub> segments. The patient was further referred for surgical opinion, due to enlargement of the LA (showing a prolonged evolution of the MR), and new onset of symptoms after the acute event (exercise dyspnea). MV repair, including prolapse resection, suture of the MV pseudo-cleft, and mitral annuloplasty were successfully performed, associated with grafting of the left circumflex artery. At three-year follow-up, the patient showed no recurrence of the MR.</p>
      </sec>
      <sec sec-type="discussion">
        <title>Discussion</title>
        <p>Our clinical case shows the usefulness of 3DE for the diagnosis and morphological assessment of complex MV lesions, especially when the etiology is uncertain, as well as its role in planning surgical procedures. Initial suspicion of the MR etiology was ischemic; however, the short period of ischemia (less than 2 hours until revascularization), minor LV wall motion abnormalities, and good LV systolic function rendered this cause improbable. The careful 2DE assessment revealed mild prolapse of the posterior MV, which was also insufficient to explain the severity of the MR. Conversely, 3D TEE revealed complex MV prolapse of the P<sub>2-3</sub> segments, a ruptured chordae, and pseudo-cleft of the posterior MV leaflet separating the P<sub>1</sub> from the P<sub>2</sub> scallop.</p>
        <p>Clefts are hypothesized to be a result of incomplete expression of an endocardial cushion defect, most often involving the middle part of the anterior MV leaflet.<xref ref-type="bibr" rid="B2"><sup>2</sup></xref><sup>,</sup><xref ref-type="bibr" rid="B3"><sup>3</sup></xref> True clefts affecting the posterior MV are extremely rare.<xref ref-type="bibr" rid="B2"><sup>2</sup></xref> However, pseudo-clefts are a separate class of morphologic anomalies of the posterior MV leaflet. Pseudo-clefts are deep indentations, sharing the localization of normal slits between the scallops of the posterior MV, but with over 50% the depth of adjacent scallops.<xref ref-type="bibr" rid="B4"><sup>4</sup></xref> This anomaly is frequently associated with counterclockwise rotation of the papillary muscles, accessory papillary muscle or MV leaflet, and MV prolapse.<xref ref-type="bibr" rid="B5"><sup>5</sup></xref> Our patient presented MR as a consequence of complex MV prolapse with a ruptured chordae, associated with the pseudo-cleft. High LV end-diastolic pressures in the context of the ischemic event and LV systolic dysfunction probably worsened the severity of the MR, as the patient denied any dyspnea prior to hospitalization. Moreover, the question remains whether the rupture of the chordae occurred prior or was related to the ischemic event.</p>
        <p>However, even though not entirely responsible for the MR, the presence of the pseudo-cleft has an additional influence on the surgical decision regarding the reparability of the MV. Mantovani et al.<xref ref-type="bibr" rid="B6"><sup>6</sup></xref> showed that 35% of patients with MV prolapse have pseudo-clefts, not seen by 2DE and revealed only by 3DE. The presence of unsolved pseudo-clefts in patients with MV prolapse was associated with poor prognosis after MV repair and a higher recurrence of the MR at follow-up. In this context, MV repair was performed in our patient, and it included the suture of the MV pseudo-cleft.</p>
      </sec>
      <sec sec-type="conclusions">
        <title>Conclusions</title>
        <p>3D TEE is a useful and feasible technique for correct diagnosis in patients with complex MV disease, especially when etiology is uncertain, and for determining valve repairability. Even though MV pseudo-clefts rarely lead to regurgitation, they are associated with worse postoperative outcomes; therefore, they need to be sutured during MV repair.</p>
      </sec>
    </body>
    <back>
      <fn-group>
        <fn fn-type="supported-by" id="fn4">
          <p>
            <bold>Sources of Funding</bold>
          </p>
          <p>There were no external funding sources for this study.</p>
        </fn>
        <fn fn-type="other" id="fn5">
          <p>
            <bold>Study Association</bold>
          </p>
          <p>This study is not associated with any thesis or dissertation work.</p>
        </fn>
        <fn fn-type="other" id="fn6">
          <p>
            <bold>Ethics approval and consent to participate</bold>
          </p>
          <p>This study was approved by the Ethics Committee of the Emergency University Hospital Bucharest under the protocol number 15/2.07. All the procedures in this study were in accordance with the 1975 Helsinki Declaration, updated in 2013. Informed consent was obtained from all participants included in the study.</p>
        </fn>
      </fn-group>
    </back>
  </sub-article>
</article>
