<!DOCTYPE article PUBLIC "-//NLM//DTD JATS (Z39.96) Journal Publishing DTD v1.1 20151215//EN" "https://jats.nlm.nih.gov/publishing/1.1/JATS-journalpublishing1.dtd">
<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" article-type="case-report" dtd-version="1.1" specific-use="sps-1.8" 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">TDzgxHsgLpfS39t5Cr8mbnc</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2018001300115</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20180106</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Image</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Challenging Evaluation of Aortic Regurgitation: More Than a Quadricuspid Valve</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Pestana</surname>
            <given-names>Gon&#231;alo</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Sousa</surname>
            <given-names>Carla</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Pinho</surname>
            <given-names>Teresa</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Maia</surname>
            <given-names>Sara</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Maciel</surname>
            <given-names>M. J&#250;lia</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
      </contrib-group>
      <aff id="aff1">
        <institution content-type="orgname">Centro Hospitalar de S&#227;o Jo&#227;o</institution>
        <institution content-type="orgdiv1">Servi&#231;o de Cardiologia</institution>
        <addr-line>
          <city>Porto</city>
        </addr-line>
        <country country="PT">Portugal</country>
        <institution content-type="original">Servi&#231;o de Cardiologia, Centro Hospitalar de S&#227;o Jo&#227;o, Porto - Portugal</institution>
      </aff>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Gon&#231;alo Pestana</bold>, Centro Hospitalar de S&#227;o Jo&#227;o - Servi&#231;o de Cardiologia, Alameda Prof. Hern&#226;ni Monteiro, 4200-319 Porto, Portugal. E-mail: <email>gnpestana@gmail.com</email>
				</corresp>
        <fn fn-type="con">
          <p>
            <bold>Author contributions</bold>
          </p>
          <p>Acquisition of data: Pestana G, Sousa C, Pinho T, Maia S; Analysis and interpretation of the data: Pestana G, Sousa C, Maia S; Writing of the manuscript: Pestana G; Critical revision of the manuscript for intellectual content: Pestana G, Sousa C, Pinho T, Maciel MJ.</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>7</month>
        <year>2018</year>
      </pub-date>
      <volume>111</volume>
      <issue>1</issue>
      <fpage>115</fpage>
      <lpage>116</lpage>
      <history>
        <date date-type="received">
          <day>24</day>
          <month>10</month>
          <year>2017</year>
        </date>
        <date date-type="rev-recd">
          <day>26</day>
          <month>02</month>
          <year>2018</year>
        </date>
        <date date-type="accepted">
          <day>26</day>
          <month>02</month>
          <year>2018</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, 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>Aortic Valve Insufficiency</kwd>
        <kwd>Echocardiography, Transesophageal</kwd>
        <kwd>Echocardiography, Three-Dimensional</kwd>
        <kwd>Pulmonary Disease, Chronic Obstructive</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <p>A 61-year-old female patient with chronic obstructive pulmonary disease, and no other comorbidities, was referred for cardiological assessment due to aggravated exertional dyspnea (New York Heart Association - NYHA class III) and atypical chest pain. Physical examination disclosed only a diastolic murmur in the second intercostal space in the right sternal border.</p>
    <p>The transthoracic echocardiogram (TTE) showed aortic regurgitation (<xref ref-type="fig" rid="f1">Figure 1A</xref>), with non-dilated cardiac chambers and preserved biventricular function. The acoustic window limited the evaluation of the valvular lesion severity and its importance in the context of the complaints, although the continuous Doppler spectrum suggested significant regurgitation (<xref ref-type="fig" rid="f1">Figure 1B</xref>). The evaluation was further impaired by a systolic flow acceleration in the Left Ventricular Outflow Tract (LVOT), with no significant gradient and an undetermined cause. The aortic root had normal size, but it was not possible to carry out an adequate morphological and functional characterization of the valve.</p>
    <p>
      <fig id="f1">
        <label>Figure 1</label>
        <caption>
          <title>Aortic regurgitation jet visualized by transthoracic echocardiogram with color Doppler (A) and its respective spectrum on continuous Doppler wave (B); large jet visualized by transesophageal echocardiogram, with a 6 mm vena contracta (C), originating from the central coaptation defect of the quadricuspid aortic valve, with a regurgitant orifice of 0.35 cm<sup>2</sup> in three-dimensional planimetry (D); Almost circumferential thickening of the left ventricular outflow tract readily identified in the three-dimensional image in systole (E), confirming the presence of a subaortic membrane with evaluation of orthogonal planes (F).</title>
        </caption>
        <graphic xlink:href="1678-4170-abc-111-01-0115-80106-gf01.jpg"/></fig>
    </p>
    <p>The Transesophageal Echocardiogram (TEE) disclosed a quadricuspid aortic valve with central coaptation defect of 0.35 cm<sup>2</sup> through three-dimensional planimetry causing severe aortic regurgitation (<xref ref-type="fig" rid="f1">Figures 1C</xref> and <xref ref-type="fig" rid="f1">1D</xref>). The three-dimensional evaluation also showed a practically circumferential thickening in the LVOT, corresponding to a non-obstructive subaortic membrane, resulting in the observed flow acceleration (<xref ref-type="fig" rid="f1">Figures 1E</xref> and <xref ref-type="fig" rid="f1">1F</xref>).</p>
    <p>Medical therapy was optimized, and the patient was referred to valve replacement surgery.</p>
    <p>This case highlights the incremental role of TEE, complemented by three-dimensional imaging, in the thorough assessment of valvular disease, crucial for the correct therapeutic management. This association between aortic quadricuspid valve and the subaortic membrane is a rare finding, described in only one previous report in the literature.<xref ref-type="bibr" rid="B1">1</xref>
		</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>
    <ref-list>
      <title>Reference</title>
      <ref id="B1">
        <label>1</label>
        <mixed-citation>1 Zacharaki AA, Patrianakos AP, Parthenakis FI, Vardas PE. Quadricuspid aortic valve associated with non-obstructive sub-aortic membrane: a case report and review of the literature. Hellenic J Cardiol. 2009;50(6):544-7.</mixed-citation>
        <element-citation publication-type="journal">
          <person-group person-group-type="author">
            <name>
              <surname>Zacharaki</surname>
              <given-names>AA</given-names>
            </name>
            <name>
              <surname>Patrianakos</surname>
              <given-names>AP</given-names>
            </name>
            <name>
              <surname>Parthenakis</surname>
              <given-names>FI</given-names>
            </name>
            <name>
              <surname>Vardas</surname>
              <given-names>PE</given-names>
            </name>
          </person-group>
          <article-title>Quadricuspid aortic valve associated with non-obstructive sub-aortic membrane: a case report and review of the literature</article-title>
          <source>Hellenic J Cardiol</source>
          <year>2009</year>
          <volume>50</volume>
          <issue>6</issue>
          <fpage>544</fpage>
          <lpage>547</lpage>
        </element-citation>
      </ref>
    </ref-list>
  </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>Avalia&#231;&#227;o Desafiadora de Regurgita&#231;&#227;o A&#243;rtica: Mais que Uma V&#225;lvula Quadric&#250;spide</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Pestana</surname>
            <given-names>Gon&#231;alo</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Sousa</surname>
            <given-names>Carla</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Pinho</surname>
            <given-names>Teresa</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Maia</surname>
            <given-names>Sara</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Maciel</surname>
            <given-names>M. J&#250;lia</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
      </contrib-group>
      <aff id="aff2">
        <institution content-type="original">Servi&#231;o de Cardiologia, Centro Hospitalar de S&#227;o Jo&#227;o, Porto - Portugal</institution>
      </aff>
      <author-notes>
        <corresp id="c2"><bold>Correspond&#234;ncia: Gon&#231;alo Pestana</bold>, Centro Hospitalar de S&#227;o Jo&#227;o - Servi&#231;o de Cardiologia, Alameda Prof. Hern&#226;ni Monteiro, 4200-319 Porto, Portugal. E-mail: <email>gnpestana@gmail.com</email>
				</corresp>
        <fn fn-type="con">
          <p>
            <bold>Contribui&#231;&#227;o dos autores</bold>
          </p>
          <p>Obten&#231;&#227;o de dados: Pestana G, Sousa C, Pinho T, Maia S; An&#225;lise e interpreta&#231;&#227;o dos dados: Pestana G, Sousa C, Maia S; Reda&#231;&#227;o do manuscrito: Pestana G; Revis&#227;o cr&#237;tica do manuscrito quanto ao conte&#250;do intelectual importante: Pestana G, Sousa C, Pinho T, Maciel MJ.</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>Insufici&#234;ncia da Valva A&#243;rtica</kwd>
        <kwd>Ecocardiografia Transesofagiana</kwd>
        <kwd>Ecocardiografia Tridimensional</kwd>
        <kwd>Doen&#231;a Pulmonar Obstrutiva Cr&#244;nica</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>Paciente do sexo feminino de 61 anos com doen&#231;a pulmonar obstrutiva cr&#244;nica, sem outras comorbidades, referenciada para avalia&#231;&#227;o da Cardiologia por dispneia de esfor&#231;o em agravamento (classe III da <italic>New York Heart Association</italic> &#8722; NYHA) e dor tor&#225;cica at&#237;pica. O exame f&#237;sico revelou apenas um sopro diast&#243;lico no segundo espa&#231;o intercostal no rebordo esternal direito.</p>
      <p>O ecocardiograma transtor&#225;cico mostrou insufici&#234;ncia a&#243;rtica (<xref ref-type="fig" rid="f2">Figura 1A</xref>), com c&#226;maras card&#237;acas n&#227;o dilatadas e fun&#231;&#227;o biventricular conservada. A janela ac&#250;stica limitou a avalia&#231;&#227;o da gravidade da les&#227;o valvular e sua valoriza&#231;&#227;o no contexto das queixas, embora o espectro em Doppler cont&#237;nuo sugerisse regurgita&#231;&#227;o importante (<xref ref-type="fig" rid="f2">Figura 1B</xref>). A avalia&#231;&#227;o era ainda dificultada por uma acelera&#231;&#227;o de fluxo sist&#243;lico na C&#226;mara de Sa&#237;da do Ventr&#237;culo Esquerdo (CSVE), sem gradiente significativo, de causa n&#227;o esclarecida. A raiz a&#243;rtica tinha dimens&#245;es normais, mas n&#227;o foi poss&#237;vel uma adequada caracteriza&#231;&#227;o morfol&#243;gica e funcional da v&#225;lvula.</p>
      <p>
        <fig id="f2">
          <label>Figura 1</label>
          <caption>
            <title>Jato de regurgita&#231;&#227;o a&#243;rtica visualizado por Doppler colorido em ecocardiografia transtor&#225;cica (A) e respectivo espectro em Doppler cont&#237;nuo (B); jato largo visualizado por ecocardiografia transesof&#225;gica, com vena contracta de 6 mm (C) com origem em defeito de coapta&#231;&#227;o central de v&#225;lvula a&#243;rtica quadric&#250;spide com orif&#237;cio regurgitante de 0,35 cm<sup>2</sup> em planimetria tridimensional (D); espessamento quase circunferencial na c&#226;mara de sa&#237;da do ventr&#237;culo esquerdo prontamente identificado na imagem tridimensional em s&#237;stole (E), confirmando-se a presen&#231;a de membrana suba&#243;rtica na avalia&#231;&#227;o dos planos ortogonais (F).</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-01-0115-80106-gf01-pt.jpg"/></fig>
      </p>
      <p>O Ecocardiograma Transesof&#225;gico (ETE) revelou v&#225;lvula a&#243;rtica quadric&#250;spide com defeito de coapta&#231;&#227;o central de 0,35 cm<sup>2</sup> por planimetria tridimensional causando insufici&#234;ncia a&#243;rtica grave (<xref ref-type="fig" rid="f2">Figuras 1C</xref> e <xref ref-type="fig" rid="f2">1D</xref>). A avalia&#231;&#227;o tridimensional mostrou tamb&#233;m um espessamento praticamente circunferencial na CSVE, correspondendo a uma membrana suba&#243;rtica n&#227;o obstrutiva, condicionando a acelera&#231;&#227;o de fluxo observada (<xref ref-type="fig" rid="f2">Figuras 1E</xref> e <xref ref-type="fig" rid="f2">1F</xref>).</p>
      <p>A terap&#234;utica m&#233;dica foi otimizada, e a paciente foi orientada para cirurgia de substitui&#231;&#227;o valvular.</p>
      <p>Este caso destaca o papel incremental do ETE, complementado com imagem tridimensional, na avalia&#231;&#227;o exaustiva da doen&#231;a valvular, determinante na correta atua&#231;&#227;o terap&#234;utica. Esta associa&#231;&#227;o entre quadricuspidia a&#243;rtica e membrana suba&#243;rtica &#233; um achado raro, descrito em apenas um relato pr&#233;vio na literatura.<xref ref-type="bibr" rid="B1">1</xref>
			</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>
