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<article xmlns:xlink="http://www.w3.org/1999/xlink" specific-use="sps-1.9" dtd-version="1.1" xml:lang="pt" article-type="case-report">
  <front>
    <journal-meta>
      <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-loc>S&#227;o Paulo, SP, Brazil</publisher-loc>
      </publisher>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v3">6n6j6Y9DcFyW4ZzzCSDCKrN</article-id>
      <article-id pub-id-type="other">00009</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2005001500009</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(05)08500209</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2005001500009</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Relato de Caso</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Tratamento cir&#250;rgico sem circula&#231;&#227;o extracorp&#243;rea de arteriopatia relacionada com arterite de Takayasu envolvendo aorta e vasos da base</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Milani</surname>
            <given-names>Rodrigo</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Brofman</surname>
            <given-names>Paulo</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Sandri</surname>
            <given-names>Tayse</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Varela</surname>
            <given-names>Alexandre</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Souza</surname>
            <given-names>Jos&#233; Augusto</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Emed</surname>
            <given-names>Luiz Gustavo</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Silveira</surname>
            <given-names>Stefan da</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Dantas</surname>
            <given-names>Marcelo</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Guimar&#227;es</surname>
            <given-names>Maximiliano</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Pontarolli</surname>
            <given-names>Rafael</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Maia</surname>
            <given-names>Francisco</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
      </contrib-group>
      <aff id="aff01">
        <addr-line>
          <named-content content-type="city">Curitiba</named-content>
          <named-content content-type="state">PR</named-content>
        </addr-line>
        <institution content-type="orgname">PUC</institution>
        <institution content-type="orgdiv1">Santa Casa de Miseric&#243;rdia </institution>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>08</month>
        <year>2005</year>
      </pub-date>
      <volume>85</volume>
      <issue>2</issue>
      <fpage>124</fpage>
      <lpage>127</lpage>
      <history>
        <date date-type="accepted">
          <day>23</day>
          <month>11</month>
          <year>2004</year>
        </date>
        <date date-type="received">
          <day>30</day>
          <month>03</month>
          <year>2004</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/4.0/" xml:lang="en">
          <license-p>This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.</license-p>
        </license>
      </permissions>
      <abstract>
        <p>Relatamos o caso de duas pacientes portadoras de arterite de Takayasu, encaminhadas ao nosso servi&#231;o onde as les&#245;es acometiam a aorta tor&#225;cica descendente e os vasos da base e, em uma delas, les&#227;o obstrutiva cr&#237;tica do &#243;stio da coron&#225;ria esquerda. Ambas as pacientes foram operadas sem circula&#231;&#227;o extracorp&#243;rea, com hepariniza&#231;&#227;o plena e aux&#237;lio de autotransfus&#227;o.</p>
      </abstract>
      <pub-date publication-format="electronic" date-type="pub">
        <day>29</day>
        <month>09</month>
        <year>2005</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>RELATO DE CASO</bold>
    </p>
    <p>Tratamento cir&#250;rgico sem circula&#231;&#227;o extracorp&#243;rea de arteriopatia relacionada com arterite de Takayasu envolvendo aorta e vasos da base</p>
    <p>
      <bold>Surgical treatment without extracorporeal circulation of artery disease related to Takayasu's arteritis involving the aorta and great vessels</bold>
    </p>
    <p>
      <bold>Rodrigo Milani; Paulo Brofman; Tayse Sandri; Alexandre Varela; Jos&#233; Augusto Souza; Luiz Gustavo Emed; Stefan da Silveira; Marcelo Dantas; Maximiliano Guimar&#227;es; Rafael Pontarolli; Francisco Maia</bold>
    </p>
    <p>Santa Casa de Miseric&#243;rdia - PUC - Curitiba, PR</p>
    <p>
      <xref ref-type="corresp" rid="back10">Endere&#231;o para correspond&#234;ncia</xref>
    </p>
    <p>
      <bold>RESUMO</bold>
    </p>
    <p>Relatamos o caso de duas pacientes portadoras de arterite de Takayasu, encaminhadas ao nosso servi&#231;o onde as les&#245;es acometiam a aorta tor&#225;cica descendente e os vasos da base e, em uma delas, les&#227;o obstrutiva cr&#237;tica do &#243;stio da coron&#225;ria esquerda. Ambas as pacientes foram operadas sem circula&#231;&#227;o extracorp&#243;rea, com hepariniza&#231;&#227;o plena e aux&#237;lio de autotransfus&#227;o.</p>
    <p>
      <bold>ABSTRACT</bold>
    </p>
    <p>We report the cases of 2 female patients with Takayasu's arteritis referred to our service with lesions affecting the descending thoracic aorta and great vessels. One of the patients had a critical obstructive lesion in the left coronary ostium. Both patients underwent surgery without extracorporeal circulation, with full heparinization and autotransfusion.</p>
    <p>A arterite de Takayasu &#233; uma vasculite cr&#244;nica de etiologia desconhecida, com o primeiro relato de caso datando de 1908<sup>1</sup>. Mulheres s&#227;o afetadas em 80 a 90% dos casos, comprometendo pacientes com idades entre 10 e 40 anos<sup>2,3</sup>. A arterite de Takayasu, primariamente, afeta a aorta e seus ramos prim&#225;rios<sup>4</sup>. A inflama&#231;&#227;o pode estar restrita &#224; aorta tor&#225;cica e/ou abdominal, al&#233;m de seus ramos, sendo que em alguns casos as manifesta&#231;&#245;es s&#227;o relacionadas ao comprometimento dos &#243;stios coronarianos. Na progress&#227;o da doen&#231;a, &#233; comum o aparecimento de les&#245;es em todos os vasos da base, variando desde les&#245;es localizadas at&#233; estenoses segmentares extensas. A aorta abdominal e art&#233;rias pulmonares s&#227;o envolvidas em cerca de 50% dos pacientes. O grau de atividade da doen&#231;a varia com o tempo, com aparentes exacerba&#231;&#245;es e redu&#231;&#245;es ou remiss&#245;es na intensidade do processo inflamat&#243;rio.</p>
    <p>A indica&#231;&#227;o para tratamento cir&#250;rgico da doen&#231;a &#233; relacionada, freq&#252;entemente, a graves complica&#231;&#245;es circulat&#243;rias, incluindo hipertens&#227;o intrat&#225;vel e insufici&#234;ncia card&#237;aca descompensada, al&#233;m de sinais de isquemia mioc&#225;rdica nos casos de les&#227;o envolvendo &#243;stio coronariano.</p>
    <p>A t&#233;cnica operat&#243;ria empregada varia de caso a caso devido ao grande n&#250;mero de vasos afetados e &#224; extens&#227;o do comprometimento das les&#245;es, fazendo com que a equipe envolvida na corre&#231;&#227;o necessite do emprego de todo arsenal cir&#250;rgico desenvolvido para as opera&#231;&#245;es card&#237;acas.</p>
    <p>
      <bold>Relato dos Casos</bold>
    </p>
    <p>Caso 1 - Mulher de 32 anos, de cor branca, deu entrada em nosso servi&#231;o por quadro de hipertens&#227;o arterial de dif&#237;cil controle, cefal&#233;ia e claudica&#231;&#227;o de membro inferior esquerdo, relatando que, aproximadamente, h&#225; oito meses come&#231;ou a ter quadro de cefal&#233;ia intensa, quando foi encaminhada ao cardiologista que detectou presen&#231;a de hipertens&#227;o grave, iniciando tratamento cl&#237;nico, com leve melhora da sintomatologia. Quarenta e cinco dias antes da interna&#231;&#227;o iniciou com dor em membros inferiores, especialmente &#224; esquerda, desencadeada pela atividade f&#237;sica e na evolu&#231;&#227;o com dor ao repouso. Ao exame f&#237;sico apresentava, na interna&#231;&#227;o, hipertens&#227;o arterial e diminui&#231;&#227;o importante dos pulsos em membros inferiores. Ecocardiograma transtor&#225;cico evidenciou hipertrofia mioc&#225;rdica importante. A tomografia toracoabdominal mostrava estenose segmentar extensa da aorta descendente, com ponto cr&#237;tico, 95%, ao n&#237;vel da transi&#231;&#227;o toracoabdominal (<xref ref-type="fig" rid="fig1">fig. 1</xref>). Submetida a angiografia de aorta ascendente, arco a&#243;rtico, aorta descendente e abdominal, confirmaram-se os achados da tomografia, al&#233;m de se detectar les&#227;o obstrutiva cr&#237;tica de tronco cel&#237;aco e les&#227;o segmentar extensa de car&#243;tida esquerda. N&#227;o foi evidenciado comprometimento de art&#233;ria pulmonar ou seus ramos.</p>
    <fig id="fig1">
      <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf01.jpg"/></fig>
    <p>As provas de atividade inflamat&#243;ria encontravam-se dentro dos limites da normalidade. Apenas o VHS apresentava-se levemente alterado. Afastada a possibilidade de epis&#243;dio ativo da doen&#231;a, foi indicada cirurgia, com objetivo de se restabelecer o fluxo entre a aorta descendente e abdominal e corrigir a les&#227;o na car&#243;tida esquerda.</p>
    <p>O acesso utilizado foi a toracolaparotomia mediana, com a incis&#227;o estendendo-se desde a f&#250;rcula esternal at&#233; a s&#237;nfise p&#250;bica. Ap&#243;s a exposi&#231;&#227;o da aorta abdominal abaixo da art&#233;ria mesent&#233;rica inferior, ao n&#237;vel da bifurca&#231;&#227;o das art&#233;rias il&#237;acas, a paciente recebeu uma dose de 4 mg/kg de heparina. Com uma press&#227;o sist&#243;lica estabilizada em 100 mmHg, foi aplicado pin&#231;amento parcial da aorta ascendente acima da origem dos &#243;stios coronarianos e, a seguir, realizada uma aortotomia em forma de elipse na face lateral da aorta e um tubo de dacron de 22 mm de di&#226;metro foi anastomosado com sutura continua de prolene 4-0. Este tubo, ap&#243;s ter sua por&#231;&#227;o proximal conectada a aorta ascendente (<xref ref-type="fig" rid="fig2">fig. 2</xref>), foi trazido para o abd&#244;me passando ao lado do &#225;trio direito, em cima da veia cava inferior, por uma abertura realizada no diafragma, ao lado do f&#237;gado e por baixo do estomago, atingindo retroperit&#244;nio onde foi anastomosado &#224; aorta abdominal pr&#243;ximo &#224; sua bifurca&#231;&#227;o com sutura continua de prolene 4-0 (<xref ref-type="fig" rid="fig3">fig. 3</xref>). Em seguida, a art&#233;ria car&#243;tida esquerda foi exposta em seu ter&#231;o m&#233;dio, antes de sua bifurca&#231;&#227;o, e um tubo de dacron com 6 mm de di&#226;metro foi anastomosado (<xref ref-type="fig" rid="fig4">fig. 4</xref>). A por&#231;&#227;o proximal deste tubo foi levada, atrav&#233;s da regi&#227;o cervical, passando por baixo da musculatura, at&#233; o tubo maior, saindo da aorta ascendente. Neste ponto foi realizada a anastomose entre os dois tubos. Ao t&#233;rmino desta anastomose, a heparina foi revertida em 75% de sua dose inicial. Todo este procedimento foi realizado sem aux&#237;lio de circula&#231;&#227;o extracorp&#243;rea. N&#227;o houveram intercorr&#234;ncias p&#243;s-operat&#243;rias, com a paciente recebendo alta da unidade de terapia intensiva no 2&#176; p&#243;s-operat&#243;rio e alta hospitalar no 7&#176; dia de p&#243;s-operat&#243;rio.</p>
    <fig id="fig2">
      <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf02.jpg"/></fig>
    <fig id="fig3">
      <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf03.jpg"/></fig>
    <fig id="fig4">
      <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf04.jpg"/></fig>
    <p>Caso 2 - Mulher de 25 anos, de origem asi&#225;tica, relatava dor precordial aos esfor&#231;os, de in&#237;cio h&#225; aproximadamente 120 dias, irradiada para membro superior esquerdo, acompanhada de sudorese, com importante piora do quadro 15 dias antes da interna&#231;&#227;o, evoluindo com dor ao repouso. Aos 17 anos de idade foi submetida &#224; opera&#231;&#227;o para revasculariza&#231;&#227;o do mioc&#225;rdio, realizada ponte com art&#233;ria tor&#225;cica interna esquerda para ramo descendente anterior e ponte com art&#233;ria tor&#225;cica interna direita para ramo marginal da art&#233;ria circunflexa. Na admiss&#227;o hospitalar, eletrocardiograma apresentava isquemia extensa de toda parede anterior e o teste de esfor&#231;o, 30 dias antes da interna&#231;&#227;o, era positivo para isquemia. O ecocardiograma transtor&#225;cico mostrou hipocontratilidade moderada da parede &#226;ntero-septal, com fun&#231;&#227;o ventricular preservada. Tomografia de t&#243;rax e abd&#244;me apresentava les&#227;o segmentar extensa na aorta descendente com gradiente de press&#227;o entre a aorta ascendente e aorta abdominal de 80 mmHg. Realizado em seguida cateterismo card&#237;aco e angiografia de aorta ascendente, arco, descendente e abdominal. A coronariografia mostrava les&#227;o obstrutiva cr&#237;tica do &#243;stio da coron&#225;ria esquerda, com oclus&#227;o total de ambas as art&#233;rias tor&#225;cicas. Na angiografia, evidenciou-se oclus&#227;o total da car&#243;tida esquerda, com enchimento por circula&#231;&#227;o colateral atrav&#233;s das art&#233;rias vertebrais e oclus&#227;o total da art&#233;ria subcl&#225;via esquerda. A angiografia confirmou ainda, o achado tomogr&#225;fico, com presen&#231;a de les&#227;o segmentar extensa na aorta descendente. As provas de atividade inflamat&#243;ria encontravam-se normais, excluindo-se assim evento agudo da doen&#231;a. Com base nos achados, foi indicada nova opera&#231;&#227;o, com objetivo de corrigir as les&#245;es coronarianas e melhorar o fluxo sang&#252;&#237;neo entre a aorta ascendente e abdominal.</p>
    <p>A via de acesso, como na primeira paciente, foi a esternotomia associada &#224; laparotomia mediana, estendendo-se da f&#250;rcula esternal at&#233; a s&#237;nfise p&#250;bica. Devido ao processo inflamat&#243;rio, o cora&#231;&#227;o encontrava-se extremamente aderido e a libera&#231;&#227;o das ader&#234;ncias bastante trabalhosa. Ao contr&#225;rio do primeiro caso onde a aorta ascendente encontrava-se livre de processo inflamat&#243;rio, este caso apresentava um espessamento severo das paredes deste segmento, dificultando a confec&#231;&#227;o da anastomose. Igualmente, como na primeira paciente, foi administrada dose de 4 mg/kg de heparina e feito pin&#231;amento parcial da aorta ascendente, com aortotomia em forma de elipse na face lateral e anastomose de tubo de dacron com 20 mm de di&#226;metro com sutura continua de prolene 4-0. A parte distal do tubo foi levada ao abd&#244;me como j&#225; descrito no primeiro caso, sendo a anastomose feita junto &#224; bifurca&#231;&#227;o com sutura continua de prolene 4-0. Terminada a corre&#231;&#227;o da aorta, a paciente foi colocada em posi&#231;&#227;o de Trendelemburg e a mesa operat&#243;ria girada para o lado direito. Nesta posi&#231;&#227;o e com aux&#237;lio de estabilizador de tecidos por suc&#231;&#227;o (<italic>Octopuss &#150; Medtronic</italic>), procedeu-se a revasculariza&#231;&#227;o do mioc&#225;rdio com ponte de veia safena para ramo marginal da art&#233;ria circunflexa e ponte de safena para ramo descendente anterior. Devido a grande espessura da aorta as anastomoses proximais foram feitas no tubo de dacron em sua origem na aorta ascendente. N&#227;o houve intercorr&#234;ncias p&#243;s&#150;operat&#243;rias permanecendo a paciente dois dias na UTI e mais oito dias de interna&#231;&#227;o hospitalar. O eletrocardiograma na alta apresentava melhora da isquemia em rela&#231;&#227;o ao pr&#233;-operat&#243;rio. Como a primeira paciente, esta opera&#231;&#227;o tamb&#233;m foi realizada sem aux&#237;lio de circula&#231;&#227;o extracorp&#243;rea. Ambas as pacientes foram anticoaguladas antes da alta hospitalar.</p>
    <p>
      <bold>Discuss&#227;o</bold>
    </p>
    <p>Nas doen&#231;as inflamat&#243;rias da aorta, o <italic>bypass</italic> a&#243;rtico com emprego de pr&#243;tese tubular, &#233; normalmente o m&#233;todo selecionado para tratamento de estenoses segmentares, evitando-se assim a manipula&#231;&#227;o dos segmentos mais doentes da aorta<sup>5</sup>. O acometimento dos vasos da base &#233; relativamente comum e, apesar da revasculariza&#231;&#227;o desses vasos ser o ideal, nem sempre &#233; poss&#237;vel devido &#224; extens&#227;o da les&#227;o envolvendo &#224;s vezes segmento intracraniano da car&#243;tida e fibrose extensa desses vasos. As les&#245;es coronarianas, quando localizadas, podem ser tratadas atrav&#233;s de angioplastia<sup>6</sup> e na maioria dos casos por meio de revasculariza&#231;&#227;o cir&#250;rgica do mioc&#225;rdio<sup>7</sup>. Quando a les&#227;o coronariana &#233; limitada aos &#243;stios, alguns autores defendem a corre&#231;&#227;o atrav&#233;s de ostioplastia<sup>8</sup>.</p>
    <p>No caso 1, a doen&#231;a limitava-se a aorta descendente, art&#233;ria car&#243;tida esquerda com uma les&#227;o localizada e tronco cel&#237;aco. A realiza&#231;&#227;o do <italic>bypass</italic> a&#243;rtico, trabalhando-se em &#225;rea livre de inflama&#231;&#227;o, mostrou-se uma opera&#231;&#227;o relativamente simples, devendo-se ter alguns cuidados t&#233;cnicos na transi&#231;&#227;o toracoabdominal atrav&#233;s do diafragma, evitando-se a forma&#231;&#227;o de h&#233;rnias bem como a eros&#227;o de &#243;rg&#227;os abdominais pela presen&#231;a do tubo. A presen&#231;a de uma art&#233;ria mesent&#233;rica inferior livre de doen&#231;a garante boa circula&#231;&#227;o colateral para regi&#227;o do tronco cel&#237;aco, exigindo muita aten&#231;&#227;o devido a sua proximidade com a bifurca&#231;&#227;o a&#243;rtica na confec&#231;&#227;o da anastomose abdominal. Este tipo de opera&#231;&#227;o mostrou-se bastante eficiente em aliviar os sintomas da paciente, hipertens&#227;o e claudica&#231;&#227;o de membros inferiores. A les&#227;o de car&#243;tida, limitada a um segmento proximal, tamb&#233;m pode ser tratada atrav&#233;s de implante de pr&#243;tese, com bons resultados em curto prazo.</p>
    <p>No caso 2, o comprometimento dos vasos da base era mais importante, levando &#224; oclus&#227;o total da car&#243;tida e subcl&#225;via esquerdas, com seus territ&#243;rios irrigados por meio de circula&#231;&#227;o colateral, que se mostrava eficiente. Os vasos ocluidos apresentavam-se com importante fibrose sendo dif&#237;cil a sua revasculariza&#231;&#227;o. Al&#233;m disto, a paciente j&#225; havia sido operada devido ao acometimento de &#243;stio da coron&#225;ria esquerda 7 anos antes, demonstrando o car&#225;ter evolutivo da doen&#231;a. Um dos prov&#225;veis fatores respons&#225;vel pela oclus&#227;o da art&#233;ria tor&#225;cica interna esquerda pode ter sido a obstru&#231;&#227;o total da art&#233;ria subcl&#225;via esquerda, levando a uma interroga&#231;&#227;o quanto &#224; utiliza&#231;&#227;o deste tipo de enxerto neste subgrupo especial de pacientes. Uma alternativa &#224; revasculariza&#231;&#227;o tradicional &#233; a ostioplastia, procedimento que apresenta bons resultados em pacientes sem acometimento pela doen&#231;a da aorta ascendente<sup>9</sup>, o que n&#227;o ocorria neste caso, onde este segmento da aorta era extremamente espesso, impossibilitando sua realiza&#231;&#227;o e optando-se pela opera&#231;&#227;o tradicional para revasculariza&#231;&#227;o do mioc&#225;rdio.</p>
    <p>Ambas as pacientes evolu&#237;ram de modo bastante satisfat&#243;rio, demonstrando grande benef&#237;cio para este grupo de pacientes, a realiza&#231;&#227;o de opera&#231;&#245;es complexas sem aux&#237;lio da circula&#231;&#227;o extracorp&#243;rea. Como a quantidade de material prot&#233;tico utilizada nos dois casos foi grande, optou-se por anticoagula&#231;&#227;o plena e devido ao car&#225;ter evolutivo da doen&#231;a, o uso de corticoterapia em doses baixas a fim de prevenir novos surtos da doen&#231;a. Nos casos de exacerba&#231;&#227;o, a pulsoterapia se faz necess&#225;ria.</p>
    <p>Em conclus&#227;o, doen&#231;as inflamat&#243;rias da aorta, como a arterite de Takayasu, apresentam uma s&#233;rie de varia&#231;&#245;es do ponto de vista anat&#244;mico quanto &#224; presen&#231;a de estenoses, sendo as opera&#231;&#245;es reservadas para os casos onde o acometimento de art&#233;rias importantes ocorreu sem que houvesse a presen&#231;a de uma circula&#231;&#227;o colateral satisfat&#243;ria, ocasionando sintomas de isquemia. Assim, o planejamento cir&#250;rgico deve levar em conta o objetivo da opera&#231;&#227;o neste tipo de doen&#231;a, que &#233; a melhora dos sintomas, como nos casos relatados, onde no caso 1 melhorou-se a circula&#231;&#227;o na aorta toracoabdominal, diminuindo assim a isquemia de membros inferiores e simultaneamente tentando-se corrigir a hipertens&#227;o e, no caso 2, onde al&#233;m destes dois sintomas corrigiu-se a isquemia mioc&#225;rdica.</p>
    <p>
      <fn id="back10" fn-type="corresp">
        <label>
          <bold>Endere&#231;o para correspond&#234;ncia</bold>
        </label>
        <p> Rodrigo Mussi Milani </p>
        <p> Rua Sergio Pereira da Silva, 74 - Casa 2 </p>
        <p> 82100-210 - Curitiba, PR </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="grmilani@cardiol.br">grmilani@cardiol.br</email>
        </p>
      </fn>
    </p>
    <p>Enviado em 30/03/2004 </p>
    <p> Aceito em 23/11/2004</p>
  </body>
  <back>
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  </back>
  <sub-article article-type="translation" id="TRen" 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 xml:lang="en">Surgical treatment without extracorporeal circulation of artery disease related to Takayasu's arteritis involving the aorta and great vessels</article-title>
      </title-group>
      <abstract xml:lang="en">
        <p>We report the cases of 2 female patients with Takayasu's arteritis referred to our service with lesions affecting the descending thoracic aorta and great vessels. One of the patients had a critical obstructive lesion in the left coronary ostium. Both patients underwent surgery without extracorporeal circulation, with full heparinization and autotransfusion.</p>
      </abstract>
    </front-stub>
    <body>
      <p>
        <bold>CASE REPORT</bold>
      </p>
      <p>Surgical treatment without extracorporeal circulation of artery disease related to Takayasu's arteritis involving the aorta and great vessels</p>
      <p>
        <bold>Rodrigo Milani; Paulo Brofman; Tayse Sandri; Alexandre Varela; Jos&#233; Augusto Souza; Luiz Gustavo Emed; Stefan da Silveira; Marcelo Dantas; Maximiliano Guimar&#227;es; Rafael Pontarolli; Francisco Maia </bold>
      </p>
      <p>Santa Casa de Miseric&#243;rdia - PUC - Curitiba, PR - Brazil</p>
      <p>
        <xref ref-type="corresp" rid="back10-body2">Correspondence</xref>
      </p>
      <p>
        <bold>ABSTRACT</bold>
      </p>
      <p>We report the cases of 2 female patients with Takayasu's arteritis referred to our service with lesions affecting the descending thoracic aorta and great vessels. One of the patients had a critical obstructive lesion in the left coronary ostium. Both patients underwent surgery without extracorporeal circulation, with full heparinization and autotransfusion.</p>
      <p>Takayasu's arteritis is a chronic vasculitis of unknown etiology, whose first case report dates back to 1908<sup>1</sup>. Women account for 80 to 90% of the cases, and the ages of the affected patients range from 10 and 40 years<sup>2,3</sup>. Takayasu's arteritis affects initially the aorta and its primary<sup>4</sup>. The inflammation may be restricted to the thoracic or abdominal aorta, or both, and its, and, sometimes, the manifestations are related to impairment of the coronary ostia. As the disease progresses, all great vessels are commonly affected, and the impairment may range from local lesions to extensive segmentary stenoses. The abdominal aorta and pulmonary arteries are involved in approximately 50% of the patients. The degree of activity of the inflammatory process varies with time, with apparent exacerbations and reductions or remissions.</p>
      <p>The indication for surgical treatment is often related to severe circulatory complications, such as refractory hypertension and decompensated heart failure, in addition to signs of myocardial ischemia in cases with lesions involving the coronary ostia.</p>
      <p>The surgical technique varies from case to case, because the number of vessels affected and the extent of the impairment vary, which requires the use of all surgical resources developed for cardiac surgery.</p>
      <p>
        <bold>Case Report</bold>
      </p>
      <p>Case 1 - A 32-year-old white female, who sought our service due to arterial hypertension of difficult control, headache, and claudication of the left lower limb. The patient reported that she began to experience intense headache approximately 8 months before, being then referred to the cardiologist, who diagnosed severe hypertension. Clinical treatment was initiated, and the symptoms improved slightly. Forty-five days before admission, she reported pain in the lower limbs, mainly in the left side, triggered by physical activity, which evolved to pain at rest. On admission, the physical examination revealed arterial hypertension and a significant decrease in the pulses in the lower limbs. Transthoracic echocardiography showed a significant myocardial hypertrophy. Thoracoabdominal tomography showed extensive segmentary stenosis of the descending aorta, with a critical point (95%) at the level of the thoracoabdominal transition (<xref ref-type="fig" rid="fig1-body2">fig. 1</xref>). The patient underwent angiography of the ascending aorta, aortic arch, and descending and abdominal aorta. Not only were the tomographic findings confirmed, but a critical obstructive lesion was detected in the celiac trunk, as was an extensive segmentary lesion in the left carotid artery. Neither the pulmonary artery, nor its branches had any lesion.</p>
      <fig id="fig1-body2">
        <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf01-en.jpg"/></fig>
      <p>The tests of inflammatory activity were within the normal range. Only VHS was slightly altered. Once the possibility of a new active episode of the disease was ruled out, surgery was indicated with the objective to re-establish the flow between the descending aorta and the abdominal aorta and to correct the lesion in the left carotid artery.</p>
      <p>The access used was median thoracolaparotomy, and the incision extended from the manubrium of the sternum to the pubic symphysis. After exposure of the abdominal aorta below the inferior mesenteric artery at the level of the bifurcation of the iliac arteries, the patient received heparin at the dose of 4 mg/kg. With the systolic pressure stabilized at 100 mmHg, the ascending aorta was partially clamped above the origin of the coronary ostia. Then, aortotomy was performed in the form of an ellipse in the lateral face of the aorta, and a 22 mm diameter dacron tube was anastomosed with a continuous 4-0 Prolene suture. After connecting the proximal portion of the tube to the ascending aorta (<xref ref-type="fig" rid="fig2-body2">fig. 2</xref>), the tube was led to the abdomen, passing beside the right atrium, above the inferior vena cava, through an opening performed in the diaphragm, beside the liver and below the stomach, reaching the retroperitoneum, where it was anastomosed to the abdominal aorta close to its bifurcation with a continuous 4-0 Prolene suture (<xref ref-type="fig" rid="fig3-body2">fig. 3</xref>). Then, the left carotid artery was exposed in its middle third, before its bifurcation, and a 6 mm diameter Dacron tube was anastomosed (<xref ref-type="fig" rid="fig4-body2">fig. 4</xref>). The proximal portion of that tube was led through the cervical region, passing below the musculature, until it reached the greater tube emerging from the ascending aorta. At this point, the 2 tubes were anastomosed. At the end of this anastomosis, heparin was reverted in 75% of its initial dose. The entire procedure was performed without the aid of extracorporeal circulation. The postoperative period was uneventful. The patient was discharged from the intensive care unit on the second postoperative day and from the hospital on the seventh postoperative day.</p>
      <fig id="fig2-body2">
        <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf02-en.jpg"/></fig>
      <fig id="fig3-body2">
        <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf03-en.jpg"/></fig>
      <fig id="fig4-body2">
        <graphic xlink:href="1678-4170-abc-85-02-0124-00009-gf04-en.jpg"/></fig>
      <p>Case 2 - The patient was a 25-year-old female of Asian heritage, who complained of effort-related chest pain for approximately 120 days, which irradiated to the left upper limb, accompanied by sweating. She reported a significant worsening of the symptoms 15 days before admission, and evolution to pain at rest. She also reported having undergone surgical myocardial revascularization at the age of 17 years, in which a bypass of the left internal thoracic artery was performed to the anterior descending branch, and another bypass of the right internal thoracic artery was performed to the marginal branch of the circumflex artery. On hospital admission, the electrocardiogram showed extensive ischemia of the entire anterior wall. The exercise test, performed 30 days before admission, was positive for ischemia. The transthoracic echocardiogram showed moderate hypocontractility of the anteroseptal wall, with preserved ventricular function. Tomography of the thorax and abdomen showed an extensive segmentary lesion in the descending aorta with an 80-mmHg pressure gradient between the ascending aorta and the abdominal aorta. Then, cardiac catheterization and angiography of the ascending aorta, aortic arch, and descending and abdominal aorta were performed. Coronary angiography showed a critical obstructive lesion of the left coronary ostium with total occlusion of both thoracic arteries. The angiography showed total occlusion of the left carotid artery with filling through collateral circulation through the vertebral arteries, and total occlusion of the left subclavian artery. Angiography confirmed the tomographic finding, the presence of an extensive segmentary lesion in the descending aorta. As the tests of inflammatory activity were normal, an acute event was ruled out. Based on the findings, a new surgery was indicated, aiming at correcting the coronary lesions and improving the blood flow between the ascending aorta and the abdominal aorta.</p>
      <p>The access route, like that in the first patient, was sternotomy in association with median laparotomy, extending from the manubrium of the sternum to the pubic symphysis. Because of the inflammatory process, the heart had numerous adhesions, whose release was extremely difficult. Contrary to the first patient, whose ascending aorta had no inflammatory process, the walls of this patient's ascending aorta were extremely thickened, which made the anastomosis difficult. Similarly to the first patient, the second patient received 4 mg/kg of heparin, and her ascending aorta was partially clamped. Then, aortotomy was performed in the form of an ellipse in the lateral face of the aorta, and a 20 mm diameter Dacron tube was anastomosed with a continuous 4-0 Prolene suture. The distal part of the tube was led to the abdomen as reported in the first case, and the anastomosis was performed close to the bifurcation with a continuous 4-0 Prolene suture. When the correction of the aorta was finished, the patient was put in the Trendelemburg position, and the surgical table was rotated to the right side. In that position and with the aid of a suction tissue stabilizer (Octopuss &#150; Medtronic), myocardial revascularization was performed with saphenous vein bypass graft to the marginal branch of the circumflex artery and saphenous vein bypass graft to the anterior descending branch. Because the aorta was extremely thick, the proximal anastomoses were performed in the Dacron tube in its origin from the ascending aorta. The postoperative period was uneventful, and the patient stayed 2 days in the ICU and 8 more days at the hospital. On hospital discharge, the electrocardiogram showed an improvement in ischemia as compared with that in the preoperative period. As in the first patient, this surgery was performed without extracorporeal circulation. Both patients underwent anticoagulation before hospital discharge.</p>
      <p>
        <bold>Discussion</bold>
      </p>
      <p>In the inflammatory diseases of the aorta, aortic bypass with tubular prosthesis is usually the method selected for treating segmentary stenoses, avoiding, therefore, the manipulation of the most impaired segments of the aorta<sup>5</sup>. Involvement of the great vessels is relatively common, and, although revascularization of those vessels is the ideal procedure, it is not always possible, due to the extensive fibrosis of those vessels and the extent of the lesion, sometimes involving the intracranial segment of the carotid artery. The coronary lesions, when localized, may be treated by use of angioplasty<sup>6</sup>, and most cases may be treated by surgical myocardial revascularization<sup>7</sup>. When the coronary lesion is limited to the ostia, some authors recommend correction through ostioplasty<sup>8</sup>.</p>
      <p>In case 1, the disease was limited to the descending aorta, a localized lesion in the left carotid artery and the celiac trunk. Aortic bypass, performed in an inflammation-free area, proved to be a relatively simple surgery, and some technical aspects should be considered in the thoracoabdominal transition through the diaphragm, avoiding the formation of hernias and the erosion of abdominal organs by the presence of the tube. The presence of a disease-free inferior mesenteric artery guaranteed good collateral circulation to the region of the celiac trunk. Due to its proximity to the aortic bifurcation, much attention is required in performing the abdominal anastomosis. This type of operation proved to be very efficient in alleviating the patient's symptoms, hypertension and claudication. The carotid lesion, limited to a proximal segment, may also be treated through the implantation of a prosthesis, with good short-term results.</p>
      <p>In case 2, the impairment of the great vessels was more important, leading to total occlusion of the carotid and left subclavian arteries, whose territories were irrigated through collateral circulation, which was efficient. The occluded vessels had important fibrosis, and their revascularization was difficult. In addition, the patient had already undergone surgery due to impairment of the left coronary ostium 7 years before, which showed the evolutionary character of the disease. One of the factors responsible for the occlusion of the left internal thoracic artery may have been the total obstruction of the left subclavian artery, leading to an interrogation in regard to the use of that type of graft in that special subgroup of patients. An alternative to the traditional revascularization is ostioplasty, a procedure with good results in patients without impairment of the ascending aorta<sup>9</sup>. In this case, however, that segment of the aorta was extremely thick, hindering the performance of that procedure, in which case, traditional myocardial revascularization was chosen.</p>
      <p>Both patients evolved in a very satisfactory way, showing that complex surgeries without extracorporeal circulation may be greatly beneficial for that group of patients. Because the amount of prosthetic material used in both cases was large, full anticoagulation was required, and, due to the evolutionary character of the disease, corticoid therapy was used at low doses to prevent new episodes of the disease. In cases of exacerbation, recurrent corticoid therapy may be necessary.</p>
      <p>In conclusion, inflammatory diseases of the aorta, such as Takayasu's arteritis, have several anatomic variations in regard to the presence of stenoses, and surgery is reserved for the cases with an important impairment in the arteries without the presence of a satisfactory collateral circulation, causing symptoms of ischemia. Therefore, surgical planning should consider the improvement of symptoms, as in the cases reported. In case 1, circulation in the thoracoabdominal aorta was improved, with a reduction in the ischemia of the lower limbs, and a simultaneous attempt to correct hypertension occurred. In case 2, in addition to those 2 symptoms, myocardial ischemia was also corrected.</p>
      <p>
        <bold>References</bold>
      </p>
      <p>
        <fn id="back10-body2" fn-type="corresp">
          <label>
            <bold>Correspondence to</bold>
          </label>
          <p> Rodrigo Mussi Milani</p>
          <p> Rua Sergio Pereira da Silva, 74 - Casa 2</p>
          <p> 82100-210 - Curitiba, PR - Brazil</p>
          <p> E-mail: </p>
          <p>
            <email xlink:href="grmilani@cardiol.br">grmilani@cardiol.br</email>
          </p>
        </fn>
      </p>
      <p>Sent for publication: 03/30/2004</p>
      <p> Accepted for publication: 11/23/2004</p>
      <p>Servi&#231;o de Cirurgia Cardiovascular - Santa Casa de Miseric&#243;rdia - PUC/PR</p>
      <p> English version by Stela Maris Costalonga</p>
    </body>
  </sub-article>
</article>
