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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" article-type="case-report" dtd-version="1.0" specific-use="sps-1.6" xml:lang="en">
  <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 pub-id-type="publisher-id" specific-use="scielo-v3">ZnqGRDNzCQJnYLqkBRf8gPJ</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2017000700090</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20170077</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Image</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Angiosarcoma Arising from the Main Pulmonary Artery Mimicking Pulmonary Thromboembolism</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Ferreira</surname>
            <given-names>Joana Sofia Silva Moura</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Moreira</surname>
            <given-names>N&#225;dia</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Ferreira</surname>
            <given-names>Maria Jo&#227;o</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Antunes</surname>
            <given-names>Manuel</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <aff id="aff1">
          <institution content-type="orgname">Centro Hospitalar e Universit&#225;rio de Coimbra</institution>
          <country country="PT">Portugal</country>
          <institution content-type="original">Centro Hospitalar e Universit&#225;rio de Coimbra, Portugal</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Joana Sofia Silva Moura Ferreira</bold>, Rua do Padr&#227;o, 479, Vila Maior, Coimbra - Portugal. E-mail: <email>joanasofia.moura@gmail.com</email>, <email>joanasofia_moura@hotmail.com</email>
				</corresp>
        <fn fn-type="con">
          <p>
            <bold>Author contributions</bold>
          </p>
          <p>Conception and design of the research: Ferreira JSSM; Writing of the manuscript: Ferreira JSSM, Moreira N; Supervision: Ferreira MJ, Antunes M.</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>
      <pub-date pub-type="epub-ppub">
        <month>07</month>
        <year>2017</year>
      </pub-date>
      <volume>109</volume>
      <issue>1</issue>
      <fpage>90</fpage>
      <lpage>91</lpage>
      <history>
        <date date-type="received">
          <day>17</day>
          <month>10</month>
          <year>2016</year>
        </date>
        <date date-type="rev-recd">
          <day>27</day>
          <month>10</month>
          <year>2016</year>
        </date>
        <date date-type="accepted">
          <day>27</day>
          <month>10</month>
          <year>2016</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="http://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, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
        </license>
      </permissions>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Echocardiography</kwd>
        <kwd>Pulmonary Embolism</kwd>
        <kwd>Thoracic Surgery</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <p>A 79-year-old female with no relevant past medical history was admitted in our emergency department for dyspnea on minimal exertion and chest discomfort over 2 weeks. Blood gas analysis showed severe hypoxemia and hypocapnia. Troponin was slightly positive. Despite a negative D-dimer assay, contrast-enhanced chest CT was performed to exclude pulmonary embolism. It showed a large filling defect centered in the pulmonary valve plane (Panels A and B). Bedside transthoracic echocardiogram showed a large echodense mass, apparently mobile, extending across the right ventricle outflow tract, pulmonary valve, and the main pulmonary artery, with dilatation of the right sided chambers and transtricuspid peak gradient of 70 mmHg (Panels C and D). Lower-limb venous compression ultrasound was negative for deep vein thrombosis. The patient remained stable, but required high oxygen inspiration fraction to maintain saturation above 90%. As pulmonary embolism was deemed unlikely given the clinical findings, the patient underwent cardiac surgery. Surgery revealed a pearly mass in the main pulmonary artery obliterating almost the entire lumen and with upstream extension to the pulmonary valve and right ventricle outflow tract (Panel E). The tumor was excised as much as possible and the pulmonary valve was replaced by a homograft. Pathological examination was compatible with angiosarcoma.</p>
    <p>Pulmonary artery angiosarcoma is exceedingly rare and carries a very poor prognosis. It can be clinical and radiologically indistinguishable from acute or chronic pulmonary artery thromboembolism. Our clinical suspicion was heightened by a negative D-dimer assay and venous ultrasound and the apparent infiltration of pulmonary arterial walls on CT.</p>
    <p>
      <fig id="f1">
        <label>Figure 1</label>
        <caption>
          <title>Angiosarcoma.</title>
        </caption>
        <graphic xlink:href="1678-4170-abc-109-01-0090-70077-gf01.jpg"/></fig>
    </p>
  </body>
  <back>
    <fn-group>
      <fn fn-type="supported-by">
        <p>
          <bold>Sources of Funding</bold>
        </p>
        <p>There were no external funding sources for this study.</p>
      </fn>
      <fn fn-type="other">
        <p>
          <bold>Study Association</bold>
        </p>
        <p>This study is not associated with any thesis or dissertation work.</p>
      </fn>
    </fn-group>
  </back>
  <sub-article article-type="translation" id="s1" xml:lang="pt">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Imagem</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Angiossarcoma no Tronco Pulmonar Imitando Tromboembolismo Pulmonar</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Ferreira</surname>
            <given-names>Joana Sofia Silva Moura</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Moreira</surname>
            <given-names>N&#225;dia</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Ferreira</surname>
            <given-names>Maria Jo&#227;o</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Antunes</surname>
            <given-names>Manuel</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <aff id="aff2">
          <institution content-type="original">Centro Hospitalar e Universit&#225;rio de Coimbra, Portugal</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c2"><bold>Correspond&#234;ncia: Joana Sofia Silva Moura Ferreira</bold>, Rua do Padr&#227;o, 479, Vila Maior, Coimbra - Portugal. E-mail: <email>joanasofia.moura@gmail.com</email>, <email>joanasofia_moura@hotmail.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: Ferreira JSSM; Reda&#231;&#227;o do manuscrito: Ferreira JSSM, Moreira N; Supervis&#227;o: Ferreira MJ, Antunes M.</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>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Ecocardiografia</kwd>
        <kwd>Embolia Pulmonar</kwd>
        <kwd>Cirurgia Tor&#225;cica</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>Uma mulher de 79 anos de idade, sem hist&#243;rico m&#233;dico passado relevante, foi internada em nosso departamento de emerg&#234;ncia devido a dispneia com esfor&#231;o m&#237;nimo e desconforto tor&#225;cico que perdurava h&#225; 2 semanas. A an&#225;lise da gasometria sangu&#237;nea mostrou hipoxemia e hipocapnia graves. A troponina estava ligeiramente positiva. Apesar do teste de d&#237;mero-D ter sido negativo, foi realizada TC com contraste para excluir embolia pulmonar. Ela mostrou um grande defeito de preenchimento centrado no plano da v&#225;lvula pulmonar (Pain&#233;is A e B). O ecocardiograma transtor&#225;cico &#224; beira do leito mostrou uma grande massa ecodensa, aparentemente m&#243;vel, estendendo-se atrav&#233;s da via de sa&#237;da do ventr&#237;culo direito, v&#225;lvula pulmonar e tronco pulmonar, com dilata&#231;&#227;o das c&#226;maras do lado direito e gradiente transtric&#250;spide de pico de 70 mmHg (Pain&#233;is C e D). O ultrassom de compress&#227;o venosa dos membros inferiores foi negativo para trombose venosa profunda. A paciente manteve-se est&#225;vel mas necessitou de fra&#231;&#227;o de inspira&#231;&#227;o de oxig&#234;nio elevada para manter a satura&#231;&#227;o acima de 90%. Como a embolia pulmonar foi considerada improv&#225;vel, devido aos achados cl&#237;nicos, a paciente foi submetida &#224; cirurgia card&#237;aca. A cirurgia revelou uma massa perolada na art&#233;ria pulmonar principal obliterando quase todo o l&#250;men e com extens&#227;o a montante para a v&#225;lvula pulmonar e via de sa&#237;da do ventr&#237;culo direito (Painel E). O tumor foi excisado tanto quanto poss&#237;vel e a v&#225;lvula pulmonar foi substitu&#237;da por um homoenxerto. O exame patol&#243;gico foi compat&#237;vel com angiossarcoma.</p>
      <p>O angiossarcoma da art&#233;ria pulmonar &#233; extremamente raro e tem um progn&#243;stico muito ruim. Pode ser cl&#237;nico e radiologicamente indistingu&#237;vel do tromboembolismo agudo ou cr&#244;nico da art&#233;ria pulmonar. Nossa suspeita cl&#237;nica foi aumentada pelo teste negativo de d&#237;mero D e ultrassom venoso, e a aparente infiltra&#231;&#227;o de paredes arteriais pulmonares na TC.</p>
      <p>
        <fig id="f2">
          <label>Figura 1</label>
          <caption>
            <title>Angiosarcoma.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-109-01-0090-70077-gf01-pt.jpg"/></fig>
      </p>
    </body>
    <back>
      <fn-group>
        <fn fn-type="supported-by">
          <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">
          <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-group>
    </back>
  </sub-article>
</article>
