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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">kJKxffyjkPJXBzqDMBcjLPb</article-id>
      <article-id specific-use="scielo-v2" pub-id-type="publisher-id">S0066-782X2024000201001</article-id>
      <article-id pub-id-type="other">01001</article-id>
      <article-id pub-id-type="doi">10.36660/abc.20230222</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Imagem</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Fibroelastoma, uma Doen&#231;a <italic>Incidentaloma</italic>? &#8211; Imagem de Casos de Fibroelastomas como Achados Incidentais em Quatro Pacientes, Quatro Valvas Diferentes</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <contrib-id contrib-id-type="orcid">0000-0002-7787-0878</contrib-id>
          <name>
            <surname>Lopes</surname>
            <given-names>Joana Lima</given-names>
          </name>
          <role>Concep&#231;&#227;o e desenho da pesquisa</role>
          <role>Obten&#231;&#227;o de dados</role>
          <role>An&#225;lise e interpreta&#231;&#227;o dos dados e Reda&#231;&#227;o do manuscrito</role>
          <xref ref-type="aff" rid="aff1">
            <sup>1</sup>
          </xref>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Freitas</surname>
            <given-names>Antonio</given-names>
          </name>
          <role>Obten&#231;&#227;o de dados</role>
          <role>Revis&#227;o cr&#237;tica do manuscrito quanto ao conte&#250;do intelectual importante</role>
          <xref ref-type="aff" rid="aff1">
            <sup>1</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Augusto</surname>
            <given-names>Jo&#227;o Bicho</given-names>
          </name>
          <role>Concep&#231;&#227;o e desenho da pesquisa</role>
          <role>Revis&#227;o cr&#237;tica do manuscrito quanto ao conte&#250;do intelectual importante</role>
          <xref ref-type="aff" rid="aff1">
            <sup>1</sup>
          </xref>
        </contrib>
        <aff id="aff1">
          <label>1</label>
          <institution content-type="orgname">Hospital Prof. Doutor Fernando Fonseca</institution>
          <addr-line>
            <named-content content-type="city">Amadora</named-content>
          </addr-line>
          <country country="PT">Portugal</country>
          <institution content-type="original">Hospital Prof. Doutor Fernando Fonseca, Amadora &#8211; Portugal</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c1"><bold>Correspond&#234;ncia: Joana Lima Lopes</bold> &#8226; Hospital Prof Doutor Fernando Fonseca, EPE - IC19, <postal-code>2720-276</postal-code> Amadora &#8211; Portugal E-mail: <email>joana.l.lopes@hff.min-saude.pt</email></corresp>
        <fn fn-type="conflict">
          <p>
            <bold>Potencial conflito de interesse</bold>
          </p>
          <p>N&#227;o h&#225; conflito com o presente artigo</p>
        </fn>
      </author-notes>
      <pub-date publication-format="electronic" date-type="pub">
        <day>23</day>
        <month>02</month>
        <year>2024</year>
      </pub-date>
      <pub-date publication-format="electronic" date-type="collection">
        <year>2024</year>
      </pub-date>
      <volume>121</volume>
      <issue>2</issue>
      <elocation-id>e20230222</elocation-id>
      <history>
        <date date-type="received">
          <day>30</day>
          <month>03</month>
          <year>2023</year>
        </date>
        <date date-type="rev-recd">
          <day>14</day>
          <month>10</month>
          <year>2023</year>
        </date>
        <date date-type="accepted">
          <day>14</day>
          <month>11</month>
          <year>2023</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xml:lang="en" xlink:href="https://creativecommons.org/licenses/by/4.0/">
          <license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution License</license-p>
        </license>
      </permissions>
      <abstract>
        <title>Resumo</title>
        <p>Os fibroelastomas s&#227;o o segundo tumor card&#237;aco benigno mais comum. S&#227;o estruturas pequenas, avasculares, com uma dimens&#227;o m&#233;dia de 9mm, podendo atingir at&#233; 70mm, habitualmente aderentes &#224; superf&#237;cie das v&#225;lvulas card&#237;acas (v&#225;lvulas a&#243;rtica e mitral s&#227;o as mais comumente afetadas, seguidas das v&#225;lvulas tric&#250;spide e pulmonar). A etiologia n&#227;o &#233; clara, sendo a hip&#243;tese de forma&#231;&#227;o de microtrombos nas margens de coapta&#231;&#227;o das v&#225;lvulas a mais aceite. Na ecocardiografia apresentam aspeto pediculado, m&#243;vel, com superf&#237;cie filamentosa, tipicamente com uma apar&#234;ncia pontilhada nas margens e ecolucente. Do ponto de vista cl&#237;nico, podem estar associados a fen&#243;menos emb&#243;licos, no entanto, na maioria dos casos o diagn&#243;stico &#233; incidental. Apresentamos de seguida quatro casos de diagn&#243;stico incidental de fibroelastomas nas quatro v&#225;lvulas card&#237;acas, diagnosticados por ecocardiograma transtor&#225;cico (ETT) (<xref ref-type="fig" rid="f1">V&#237;deo 1</xref>; <xref ref-type="fig" rid="f2">Figura 1</xref>).</p>
        <p>
          <fig id="f1">
            <label>V&#237;deo 1</label>
            <caption>
              <title>Da esquerda para a direita, de cima para baixo: fibroelastomas no folheto anterior da v&#225;lvula tric&#250;spide, folheto anterior da v&#225;lvula mitral, c&#250;spide esquerda da v&#225;lvula pulmonar e c&#250;spide esquerda da v&#225;lvula a&#243;rtica, cada um correspondendo a um doente diferente. Em: <ext-link ext-link-type="uri" xlink:href="http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4">http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4</ext-link></title>
            </caption>
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          </fig>
          <fig id="f2">
            <label>Figura 1</label>
            <caption>
              <title>Da esquerda para a direita, de cima para baixo: fibroelastomas no folheto anterior da v&#225;lvula tric&#250;spide, folheto anterior da v&#225;lvula mitral, c&#250;spide esquerda da v&#225;lvula pulmonar e c&#250;spide esquerda da v&#225;lvula a&#243;rtica, cada um correspondendo a um doente diferente.</title>
            </caption>
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          </fig>
        </p>
      </abstract>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Fibroelastoma</kwd>
        <kwd>Achados Incidentais</kwd>
        <kwd>Neoplasias Card&#237;acas/cirurgia</kwd>
        <kwd>Valvas Card&#237;acas</kwd>
        <kwd>Ecocardiografia Transesofagiana/m&#233;todos</kwd>
        <kwd>Emboliza&#231;&#227;o</kwd>
      </kwd-group>
      <counts>
        <fig-count count="4"/>
        <table-count count="0"/>
        <equation-count count="0"/>
        <ref-count count="6"/>
      </counts>
    </article-meta>
  </front>
  <body>
    <sec sec-type="discussion">
      <title>Descri&#231;&#227;o e Discuss&#227;o</title>
      <p>Apresentamos quatro casos de fibroelastomas diagnosticados por ecocardiografia transtor&#225;cica, cada um numa v&#225;lvula card&#237;aca distinta.<sup><xref ref-type="bibr" rid="B1">1</xref>,<xref ref-type="bibr" rid="B2">2</xref></sup> Em todos os casos, o achado ecocardiogr&#225;fico foi incidental, em contraste com o t&#237;pico diagn&#243;stico que surge na sequ&#234;ncia da investiga&#231;&#227;o de um fen&#244;meno emb&#243;lico.<sup><xref ref-type="bibr" rid="B3">3</xref>,<xref ref-type="bibr" rid="B4">4</xref></sup> Uma caracteriza&#231;&#227;o mais detalhada das estruturas foi obtida com o ecocardiograma transesof&#225;gico (ETE), confirmando a sua forma, bem como o ped&#237;culo que os unia &#224; v&#225;lvula card&#237;aca, a elevada mobilidade e apar&#234;ncia pontilhada, esta &#250;ltima correspondendo &#224;s proje&#231;&#245;es papilares encontradas na superf&#237;cie. Nesta fase, o diagn&#243;stico diferencial torna-se mandat&#243;rio, nomeadamente com vegeta&#231;&#227;o (recente ou antiga/calcificada) ou com trombo. Todavia, existiam pistas ecocardiogr&#225;ficas decisivas que apontavam para a hip&#243;tese de se tratar de fibroelastomas: (1) a forma oval ou arredondada dos fibroelastomas, (2) a sua apar&#234;ncia bem delimitada e homog&#234;nea (<italic>vs</italic> a apar&#234;ncia heterog&#233;nea das vegeta&#231;&#245;es ou dos trombos), e (3) o aspeto pontilhado ao longo do per&#237;metro (que n&#227;o ocorre nas vegeta&#231;&#245;es nem nos trombos). Adicionalmente, do ponto de vista cl&#237;nico, a aus&#234;ncia de febre, par&#226;metros inflamat&#243;rios ou qualquer sinal ou sintoma sugestivo de quadro infecioso, tornam improv&#225;vel a hip&#243;tese diagn&#243;stica de endocardite infeciosa / vegeta&#231;&#245;es no ecocardiograma.</p>
      <p>Nos quatro casos apresentados, a localiza&#231;&#227;o, forma (em todos os casos redonda/oval, regular e com aspeto homog&#233;neo), tamanho (3 a 8mm) e caracteriza&#231;&#227;o detalhada fornecida pelo ETE permitiram o diagn&#243;stico presuntivo como fibroelastomas.<sup><xref ref-type="bibr" rid="B1">1</xref></sup> N&#227;o se verificaram diferen&#231;as significativas entre fibroelastomas do cora&#231;&#227;o direito <italic>vs</italic> cora&#231;&#227;o esquerdo. Segundo a literatura, tais diferen&#231;as nunca foram documentadas. A tomografia computorizada card&#237;aca e a resson&#226;ncia magn&#233;tica card&#237;aca s&#227;o modalidades de imagem igualmente &#250;teis, particularmente para avalia&#231;&#227;o de v&#225;lvulas do cora&#231;&#227;o direito, onde a caracteriza&#231;&#227;o por ETT ou ETE pode ser dif&#237;cil. Ainda assim, por limita&#231;&#245;es relacionadas com a resolu&#231;&#227;o temporal, os fibroelastomas de menores dimens&#245;es podem permanecer indetect&#225;veis.</p>
      <p>Atualmente, n&#227;o existem diretrizes dirigidas ao tratamento dos fibroelastomas. Segundo a literatura mais recente, &#233; aceite que os fibroelastomas sintom&#225;ticos devem ser removidos cirurgicamente.<sup><xref ref-type="bibr" rid="B5">5</xref></sup> J&#225; em rela&#231;&#227;o aos assintom&#225;ticos, as opini&#245;es divergem e baseiam-se na mobilidade da massa e, consequentemente, no seu risco emb&#243;lico, posi&#231;&#227;o, necessidade de cirurgia card&#237;aca por outro motivo adicional e comorbilidades do doente.<sup><xref ref-type="bibr" rid="B6">6</xref></sup> O tamanho e mobilidade da massa constituem os preditores independentes mais importantes de eventos emb&#243;licos, independentemente da localiza&#231;&#227;o no cora&#231;&#227;o esquerdo ou direito. Os quatro casos foram discutidos em <italic>Heart Team</italic> e a remo&#231;&#227;o cir&#250;rgica foi considerada o melhor curso de a&#231;&#227;o. A an&#225;lise histol&#243;gica que se seguiu confirmou o diagn&#243;stico de fibroelastomas em todos os casos.</p>
      <p>Nos quatro casos, a cirurgia card&#237;aca decorreu sem complica&#231;&#245;es. N&#227;o se registaram eventos emb&#243;licos nos pacientes ap&#243;s 2 anos de follow-up. Do ponto de vista ecocardiogr&#225;fico, a morfologia das v&#225;lvulas card&#237;acas manteve-se preservada, sem insufici&#234;ncia ou estenose, e n&#227;o houve recorr&#234;ncia da massa.</p>
      <p>Os fibroelastomas s&#227;o tumores benignos relativamente comuns, que podem afetar diferentes partes do cora&#231;&#227;o, com especial &#234;nfase nas quatro v&#225;lvulas card&#237;acas. Afetam o funcionamento da v&#225;lvula e s&#227;o uma fonte de emboliza&#231;&#227;o. N&#227;o existem, atualmente, diretrizes para o tratamento dos fibroelastomas. Nos pacientes sintom&#225;ticos, a remo&#231;&#227;o cir&#250;rgica &#233; aconselhada. Nos assintom&#225;ticos, uma decis&#227;o partilhada deve tomar lugar, baseada nas caracter&#237;sticas da massa, riso emb&#243;lico e risco cir&#250;rgico do doente.</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 artigo n&#227;o cont&#233;m estudos com humanos ou animais realizados por nenhum dos autores.</p>
      </fn>
    </fn-group>
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    </ref-list>
  </back>
  <sub-article article-type="translation" id="S01" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Image</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Fibroelastoma, an Incidentaloma Disease? &#8211; Image Cases of Fibroelastomas as Incidental Findings in Four Patients, Four Different Valves</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <contrib-id contrib-id-type="orcid">0000-0002-7787-0878</contrib-id>
          <name>
            <surname>Lopes</surname>
            <given-names>Joana Lima</given-names>
          </name>
          <role>Conception and design of the research</role>
          <role>Acquisition of data</role>
          <role>Analysis and interpretation of the data and Writing of the manuscript</role>
          <xref ref-type="aff" rid="aff2">
            <sup>1</sup>
          </xref>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Freitas</surname>
            <given-names>Antonio</given-names>
          </name>
          <role>Acquisition of data</role>
          <role>Critical revision of the manuscript for important intellectual content</role>
          <xref ref-type="aff" rid="aff2">
            <sup>1</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Augusto</surname>
            <given-names>Jo&#227;o Bicho</given-names>
          </name>
          <role>Conception and design of the research</role>
          <role>Critical revision of the manuscript for important intellectual content</role>
          <xref ref-type="aff" rid="aff2">
            <sup>1</sup>
          </xref>
        </contrib>
        <aff id="aff2">
          <label>1</label>
          <addr-line>
            <named-content content-type="city">Amadora</named-content>
          </addr-line>
          <country country="PT">Portugal</country>
          <institution content-type="original">Hospital Prof. Doutor Fernando Fonseca, Amadora &#8211; Portugal</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c2"><bold>Mailing Address: Joana Lima Lopes</bold> &#8226; Hospital Prof Doutor Fernando Fonseca, EPE - IC19, <postal-code>2720-276</postal-code> Amadora &#8211; Portugal E-mail: <email>joana.l.lopes@hff.min-saude.pt</email></corresp>
        <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>
      <abstract>
        <title>Abstract</title>
        <p>Fibroelastomas are the second most common benign cardiac tumor<sup><xref ref-type="bibr" rid="B1">1</xref></sup>. They are small avascular structures with a mean size of 9mm, ranging up to 70mm, usually attached to the heart valves&#8217; surface (aortic and mitral are the most affected, followed by tricuspid and pulmonary valves). Their etiology is unclear, but the hypothesis of coalescence of microthrombus at the coaptation margins of valves is the most widely accepted theory. On echocardiography, they are pedicled, mobile, with a filamentous surface, and usually have a speckled appearance with echolucencies and a stippled pattern near the edges. Clinically, they may be associated with embolic phenomena; however, in most cases, the diagnosis is incidental. We present a series of four clinical cases with an incidental diagnosis of fibroelastomas across the four cardiac valves as assessed by transthoracic echocardiography (<xref ref-type="fig" rid="f3">Video 1</xref>; <xref ref-type="fig" rid="f4">Figure 1</xref>).</p>
        <p>
          <fig id="f3">
            <label>Video 1</label>
            <caption>
              <title>From left to right and top to bottom: fibroelastomas of the anterior leaflet of the tricuspid valve, anterior leaflet of the mitral valve, left cusp of the pulmonary valve and left cuspid of the aortic valve, each corresponding to a different patient. Link: <ext-link ext-link-type="uri" xlink:href="http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4">http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4</ext-link></title>
            </caption>
            <graphic xlink:href="1678-4170-abc-121-02-e20230222-30222-gf01-en.tif"/></fig>
          <fig id="f4">
            <label>Figure 1</label>
            <caption>
              <title>From left to right and top to bottom: fibroelastomas of the anterior leaflet of the tricuspid valve, anterior leaflet of the mitral valve, left cusp of the pulmonary valve and left cuspid of the aortic valve, each corresponding to a different patient.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-121-02-e20230222-30222-gf02-en.tif"/></fig>
        </p>
      </abstract>
      <kwd-group xml:lang="en">
        <title>Keywords:</title>
        <kwd>Fibroelastoma</kwd>
        <kwd>Incidental Findings</kwd>
        <kwd>Heart Neoplasias/surgery</kwd>
        <kwd>Heart Valves</kwd>
        <kwd>Echocardiography, Transesophageal/methods</kwd>
        <kwd>Embolization</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <sec sec-type="discussion">
        <title>Description and Discussion</title>
        <p>We present four fibroelastomas that were first diagnosed by transthoracic echocardiography, each located in a different cardiac valve.<sup><xref ref-type="bibr" rid="B1">1</xref>,<xref ref-type="bibr" rid="B2">2</xref></sup> In all cases, the echocardiographic finding was incidental as opposed to a diagnosis that is typically ascertained following an investigation of an embolic phenomenon.<sup><xref ref-type="bibr" rid="B3">3</xref>,<xref ref-type="bibr" rid="B4">4</xref></sup> Further characterization of the masses with transesophageal echocardiography (TEE) was obtained, confirming their shape along with a pedicle attaching them to a heart valve, high mobility, and speckled appearance, the latter corresponding to the papillary projections found on the surface. At this stage, differential diagnosis is mandatory, namely with vegetation (new or calcified/ancient) or thrombus. However, crucial echocardiographic clues that pointed towards fibroelastoma were (1) the round or oval shape of fibroelastomas, (2) the well-demarcated and homogenous appearance (vs. heterogenous appearance of vegetation or thrombus), and (3) the speckled appearance with stippling around the perimeter (which does not exist in vegetations or thrombi). Additionally, from a clinical point of view, the absence of fever, inflammatory parameters, or any sign or symptom suggestive of an infectious condition makes the diagnostic hypothesis of infectious endocarditis/vegetation on the echocardiogram unlikely.</p>
        <p>In the four cases presented, the location, shape (in all cases oval/round, relatively regular and homogenous appearance), size (3 to 8mm), and further detailed characterization by TEE allowed for a presumptive diagnosis of fibroelastoma.<sup><xref ref-type="bibr" rid="B1">1</xref></sup> No relevant differences were found between right heart vs left heart fibroelastomas. According to recent literature, such differences were never documented. Cardiac computed tomography and cardiac magnetic resonance could also be used, particularly for right heart valves where TTE or TEE can be rather difficult; however, owing to temporal resolution limitations, small fibroelastomas can be missed.</p>
        <p>There are no current guidelines for the treatment of fibroelastomas. According to current literature, symptomatic fibroelastomas should be surgically removed.<sup><xref ref-type="bibr" rid="B5">5</xref></sup> As for asymptomatic fibroelastomas, opinions diverge and are based upon the mass mobility/embolic risk, position, concomitant cardiac surgery for other reasons, and comorbidities of the patient.<sup><xref ref-type="bibr" rid="B6">6</xref></sup> The size and mobility of the tumors are the most important independent predictors of embolic events, regardless of left or right heart location. All four cases were discussed in the Heart Team, and surgical removal was considered the best course of action. Histological analysis confirmed the diagnosis of fibroelastoma in all cases.</p>
        <p>In all cases, cardiac surgery was performed without complications. No embolic events were ever registered after a two-year follow-up. On echocardiographic follow-up, the valves morphology remains normal, no valve insufficiency or stenosis were noted, and there was no recurrence of the cardiac mass.</p>
        <p>Fibroelastomas are (relatively) benign masses that can affect different parts of the heart and, crucially, all four cardiac valves. They can affect valvular function and serve as embolization material. There are no current guidelines for the treatment of fibroelastomas. In symptomatic patients, surgical removal is advised. In asymptomatic patients, a shared decision should take place based on the characteristics of the mass, embolic risk, and surgical risk of the patient.</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 article does not contain any studies with human participants or animals performed by any of the authors.</p>
        </fn>
      </fn-group>
    </back>
  </sub-article>
</article>
