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  <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">H87JRN7x4hfj3DPnwKkwngm</article-id>
      <article-id pub-id-type="other">00021</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2007000500021</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(07)08800521</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2007000500021</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Relato de Caso</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Revasculariza&#231;&#227;o do mioc&#225;rdio em paciente com situs inversus totalis</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Pego-Fernandes</surname>
            <given-names>Paulo Manuel</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Serro-Azul</surname>
            <given-names>Jo&#227;o Batista de</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Matheus</surname>
            <given-names>Fernando</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Maehara</surname>
            <given-names>Bruno Shoiti</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
      </contrib-group>
      <aff id="aff01">
        <addr-line>
          <named-content content-type="city">S&#227;o Paulo</named-content>
          <named-content content-type="state">S&#227;o Paulo</named-content>
        </addr-line>
        <institution content-type="orgname">Universidade de S&#227;o Paulo</institution>
        <institution content-type="orgdiv1">FM </institution>
        <institution content-type="orgdiv2">Hospital das Cl&#237;nicas</institution>
        <country country="BR">Brazil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>05</month>
        <year>2007</year>
      </pub-date>
      <volume>88</volume>
      <issue>5</issue>
      <fpage>e103</fpage>
      <lpage>e106</lpage>
      <history>
        <date date-type="accepted">
          <day>17</day>
          <month>01</month>
          <year>2007</year>
        </date>
        <date date-type="received">
          <day>20</day>
          <month>04</month>
          <year>2006</year>
        </date>
        <date date-type="rev-recd">
          <day>08</day>
          <month>11</month>
          <year>2006</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 um paciente com situs inversus totalis associado a coronariopatia obstrutiva em art&#233;rias descendente anterior e posterior, coron&#225;ria direita, primeiro ramo diagonal e ramo marginal esquerdo, quadro condizente com a interven&#231;&#227;o de revasculariza&#231;&#227;o do mioc&#225;rdio. Esse procedimento n&#227;o &#233; freq&#252;ente na literatura m&#233;dica, sendo encontrado apenas um relato na literatura brasileira. A revasculariza&#231;&#227;o do mioc&#225;rdio foi realizada com a art&#233;ria mam&#225;ria interna direita para a art&#233;ria descendente anterior, e uma ponte de safena para coron&#225;ria direita, marginal esquerda, primeiro ramo diagonal e descendente posterior. A cirurgia foi realizada com o aux&#237;lio de circula&#231;&#227;o extracorp&#243;rea.</p>
      </abstract>
      <kwd-group xml:lang="pt" kwd-group-type="author-generated">
        <kwd>Dextrocardia</kwd>
        <kwd>cirurgia card&#237;aca</kwd>
        <kwd>revasculariza&#231;&#227;o mioc&#225;rdica</kwd>
        <kwd>situs inversus</kwd>
      </kwd-group>
      <pub-date publication-format="electronic" date-type="pub">
        <day>15</day>
        <month>06</month>
        <year>2007</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>RELATO DE CASO</bold>
    </p>
    <p>
      <bold>Revasculariza&#231;&#227;o do mioc&#225;rdio em paciente com <italic>situs inversus totalis</italic></bold>
    </p>
    <p>
      <bold>Paulo Manuel Pego-Fernandes; Jo&#227;o Batista de Serro-Azul; Fernando Matheus; Bruno Shoiti Maehara</bold>
    </p>
    <p>Instituto do Cora&#231;&#227;o do Hospital das Cl&#237;nicas &#150; FMUSP &#150; S&#227;o Paulo, SP</p>
    <p>
      <xref ref-type="corresp" rid="end">Correspond&#234;ncia</xref>
    </p>
    <p>
      <bold>RESUMO</bold>
    </p>
    <p>Relatamos o caso de um paciente com <italic>situs inversus totalis</italic> associado a coronariopatia obstrutiva em art&#233;rias descendente anterior e posterior, coron&#225;ria direita, primeiro ramo diagonal e ramo marginal esquerdo, quadro condizente com a interven&#231;&#227;o de revasculariza&#231;&#227;o do mioc&#225;rdio. Esse procedimento n&#227;o &#233; freq&#252;ente na literatura m&#233;dica, sendo encontrado apenas um relato na literatura brasileira. A revasculariza&#231;&#227;o do mioc&#225;rdio foi realizada com a art&#233;ria mam&#225;ria interna direita para a art&#233;ria descendente anterior, e uma ponte de safena para coron&#225;ria direita, marginal esquerda, primeiro ramo diagonal e descendente posterior. A cirurgia foi realizada com o aux&#237;lio de circula&#231;&#227;o extracorp&#243;rea.</p>
    <p><bold>Palavras-chave: </bold>Dextrocardia, cirurgia card&#237;aca, revasculariza&#231;&#227;o mioc&#225;rdica, <italic>situs inversus.</italic></p>
    <p>
      <bold>Introdu&#231;&#227;o</bold>
    </p>
    <p>A dextrocardia associada a <italic>situs inversus totalis</italic> &#233; uma condi&#231;&#227;o rara, com incid&#234;ncia de 1:10.000 na popula&#231;&#227;o em geral<sup>1,2</sup>. Ela &#233; caracterizada pelo posicionamento anormal do cora&#231;&#227;o (com seu &#225;pice posicionado no lado direito) e das v&#237;sceras. Entretanto, a maioria dos indiv&#237;duos afetados pode viver uma vida normal.</p>
    <p>Na dextrocardia, o tubo primitivo do cora&#231;&#227;o se dobra &#224; esquerda em uma imagem especular do la&#231;o bulboventricular normal. Tal condi&#231;&#227;o &#233; transmitida por genes autoss&#244;micos recessivos. </p>
    <p>A associa&#231;&#227;o entre <italic>situs inversus totalis</italic> e doen&#231;as ateroscler&#243;ticas coronarianas tem incid&#234;ncia semelhante &#224; da popula&#231;&#227;o em geral. Isso explica os poucos casos de revasculariza&#231;&#227;o do mioc&#225;rdio em pacientes com essa condi&#231;&#227;o relatados na literatura m&#233;dica<sup>3-6</sup>.</p>
    <p>
      <bold>Relato do Caso</bold>
    </p>
    <p>Paciente do sexo masculino, branco, 63 anos de idade, com <italic>situs inversus totalis,</italic> portador de diabete melito do tipo II, hipertens&#227;o arterial sist&#234;mica e insufici&#234;ncia card&#237;aca congestiva. Apresentava dispn&#233;ia aos grandes esfor&#231;os h&#225; quatro anos e, duas semanas antes da cirurgia, relatava dispn&#233;ia aos pequenos esfor&#231;os associada a dor precordial, compat&#237;vel com angina inst&#225;vel. Dados obtidos pela eletrocardiografia revelaram dextrocardia t&#237;pica com poss&#237;vel isquemia de parede anterior (<xref ref-type="fig" rid="fig01">fig. 1</xref>).</p>
    <fig id="fig01">
      <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf01.jpg"/></fig>
    <p>A cineangiocoronariografia revelava placas ateroscler&#243;ticas coronarianas, localizadas em descendentes anterior e posterior, primeiro ramo diagonal, ramo marginal esquerdo e coron&#225;ria direita (<xref ref-type="fig" rid="fig02">figs. 2 a 4</xref>).</p>
    <fig id="fig02">
      <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf02.jpg"/></fig>
    <p>
      <graphic xlink:href="1678-4170-abc-88-05-e103-00021-e01.jpg"/></p>
    <p>
      <graphic xlink:href="1678-4170-abc-88-05-e103-00021-e02.jpg"/></p>
    <p>A opera&#231;&#227;o foi realizada com o intuito de promover revasculariza&#231;&#227;o das &#225;reas isqu&#234;micas, com enxertos de veia safena magna e utiliza&#231;&#227;o de art&#233;ria mam&#225;ria interna direita.</p>
    <p>Em 17 de fevereiro de 2004, o paciente foi submetido a interven&#231;&#227;o cir&#250;rgica. Ap&#243;s assepsia e anti-sepsia em dec&#250;bito dorsal e realiza&#231;&#227;o de toracotomia mediana, o paciente foi colocado em circula&#231;&#227;o extracorp&#243;rea, devidamente heparinizado e em hipotermia de 32&#176;C, na qual permaneceu por 96 minutos. Uma particularidade desse tipo de cirurgia &#233; que o cirurgi&#227;o deve se posicionar no lado esquerdo do paciente, o que facilita seu trabalho. A art&#233;ria mam&#225;ria interna direita foi anastomosada &#224; art&#233;ria descendente anterior. Al&#233;m disso, enxertos de veia safena magna foram utilizados para revascularizar a coron&#225;ria direita, o ramo marginal esquerdo, o primeiro ramo diagonal e o ramo descendente posterior.</p>
    <p>O p&#243;s-operat&#243;rio ocorreu sem maiores problemas, e o paciente apresentou melhora cl&#237;nica da insufici&#234;ncia card&#237;aca e da fun&#231;&#227;o do ventr&#237;culo esquerdo.</p>
    <p>Vinte dias ap&#243;s a cirurgia, o paciente foi reavaliado ambulatorialmente. N&#227;o foi observada dispn&#233;ia e o paciente apresentava bom estado geral, sem edema e com freq&#252;&#234;ncia card&#237;aca de 80 bpm. </p>
    <p>Ap&#243;s sete meses da realiza&#231;&#227;o da cirurgia, o paciente foi novamente avaliado, apresentando-se sem queixas, em bom estado geral, com press&#227;o arterial de 110x70 mmHg. A ecocardiografia revelou fun&#231;&#227;o card&#237;aca normal. Angiotomografia realizada 32 meses ap&#243;s a opera&#231;&#227;o revelou que todos os enxertos estavam p&#233;rvios e sem les&#245;es (<xref ref-type="fig" rid="fig05">figs. 5 a 7</xref>).</p>
    <fig id="fig05">
      <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf03.jpg"/></fig>
    <p>
      <graphic xlink:href="1678-4170-abc-88-05-e103-00021-e03.jpg"/></p>
    <p>
      <graphic xlink:href="1678-4170-abc-88-05-e103-00021-e04.jpg"/></p>
    <p>
      <bold>Discuss&#227;o</bold>
    </p>
    <p>A cirurgia de revasculariza&#231;&#227;o do mioc&#225;rdio em pacientes com dextrocardia, apesar de n&#227;o possuir vasta literatura a respeito, tem se mostrado um m&#233;todo eficiente e vi&#225;vel para a revers&#227;o de situa&#231;&#245;es isqu&#234;micas card&#237;acas. O presente relato confirma os benef&#237;cios trazidos por esse procedimento.</p>
    <p>Vale ressaltar que o &#250;nico relato de revasculariza&#231;&#227;o do mioc&#225;rdio em paciente com <italic>situs inversus</italic> na literatura internacional &#233; de autoria de brasileiros (Abensur e cols.<sup>5</sup>).</p>
    <p>O primeiro relato da literatura brasileira foi realizado em 2002<sup>7</sup>, mas apesar de n&#227;o ser o primeiro relato brasileiro, pode ser considerado o procedimento mais complexo at&#233; ent&#227;o realizado, tendo em vista do n&#250;mero de anastomoses realizadas.</p>
    <p>
      <fn id="end" fn-type="corresp">
        <label>
          <bold>Correspond&#234;ncia:</bold>
        </label>
        <p> Paulo Manuel Pego-Fernandes </p>
        <p> Av. Dr. En&#233;as Carvalho de Aguiar, 44 &#150; Bloco II &#150; 2o andar &#150; Cerqueira C&#233;sar </p>
        <p> 05403-000 &#150; S&#227;o Paulo, SP </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="paulopego@incor.usp.br">paulopego@incor.usp.br</email>
        </p>
      </fn>
    </p>
    <p>Artigo recebido em 20/04/06; revisado recebido em 08/11/06; aceito em 17/01/07.</p>
  </body>
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  <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">Myocardial revascularization in a patient with situs inversus totalis</article-title>
      </title-group>
      <abstract xml:lang="en">
        <p>We report the case of a patient with dextrocardia and situs inversus totalis associated with obstructive coronariopathy in the anterior and posterior descending arteries, right coronary artery, first diagonal branch and left marginal branch. The patient underwent coronary artery bypass grafting surgery. This surgery has been rarely reported in literature and we found only one similar case in the national medical literature. The myocardial revascularization was carried out with the right mammary artery for the anterior descending artery. The saphenous vein anastomosed the aorta to the right coronary artery, left marginal branch, fist diagonal branch and posterior descending artery. The surgery was performed with extracorporeal circulation.</p>
      </abstract>
      <kwd-group xml:lang="en">
        <kwd>Dextrocardia</kwd>
        <kwd>cardiac surgery</kwd>
        <kwd>myocardial revascularization</kwd>
        <kwd>situs inversus</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>
        <bold>CASE REPORT</bold>
      </p>
      <p>
        <bold>Myocardial revascularization in a patient with <italic>situs inversus totalis</italic></bold>
      </p>
      <p>
        <bold>Paulo Manuel Pego-Fernandes; Jo&#227;o Batista de Serro-Azul; Fernando Matheus; Bruno Shoiti Maehara</bold>
      </p>
      <p>Instituto do Cora&#231;&#227;o da Faculdade de Medicina da Universidade de S&#227;o Paulo &#150; FMUSP &#150; S&#227;o Paulo, SP, Brazil</p>
      <p>
        <xref ref-type="corresp" rid="end-body2">Mailing address</xref>
      </p>
      <p>
        <bold>SUMMARY</bold>
      </p>
      <p>We report the case of a patient with dextrocardia and <italic>situs inversus totalis</italic> associated with obstructive coronariopathy in the anterior and posterior descending arteries, right coronary artery, first diagonal branch and left marginal branch. The patient underwent coronary artery bypass grafting surgery. This surgery has been rarely reported in literature and we found only one similar case in the national medical literature. The myocardial revascularization was carried out with the right mammary artery for the anterior descending artery. The saphenous vein anastomosed the aorta to the right coronary artery, left marginal branch, fist diagonal branch and posterior descending artery. The surgery was performed with extracorporeal circulation.</p>
      <p><bold>Key words: </bold>Dextrocardia; cardiac surgery; myocardial revascularization; <italic>situs inversus</italic>.</p>
      <p>
        <bold>Introduction</bold>
      </p>
      <p>Dextrocardia associated with <italic>situs inversus totalis</italic> is a rare condition, with an incidence of 1:10,000 in the general population<sup>1,2</sup>. It is characterized by the abnormal position of the heart (with the apex positioned on the right side) and viscera. However, most of the affected individuals can lead a normal life.</p>
      <p>In dextrocardia, the primitive heart tube folds to the left in a specular image of the normal bulboventricular loop. Such condition is transmitted by autosomal recessive genes. </p>
      <p>The association of <italic>situs inversus totalis</italic> and coronary atherosclerotic disease has the same incidence when compared to the general population. Thus, there are few reported cases in medical literature on myocardial revascularization in patients with such condition<sup>3-6</sup>.</p>
      <p>
        <bold>Case Report</bold>
      </p>
      <p>White Caucasian male patient, 63 years of age, with <italic>situs inversus totalis</italic>. The patient presents diabetes mellitus type II, systemic arterial hypertension (SAH) and congestive heart failure (CHF).</p>
      <p>He had presented dyspnea on exertion for 4 years and two weeks before the surgery he reported dyspnea on slight exertion associated to precordial pain, compatible with unstable angina. Data obtained from the electrocardiogram (ECG) showed typical dextrocardia with possible anterior wall ischemia (<xref ref-type="fig" rid="fig01-body2">Figure 1</xref>).</p>
      <fig id="fig01-body2">
        <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf01-en.jpg"/></fig>
      <p>The coronary angiography showed coronary atherosclerotic plaques, located in the anterior and posterior descending arteries, first diagonal branch, left marginal branch and right coronary artery (<xref ref-type="fig" rid="fig02-body2">Figures 2</xref>, <xref ref-type="fig" rid="fig03-body2">3</xref> and <xref ref-type="fig" rid="fig04-body2">4</xref>).</p>
      <fig id="fig02-body2">
        <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf02-en.jpg"/></fig>
      <fig id="fig03-body2">
        <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf03-en.jpg"/></fig>
      <fig id="fig04-body2">
        <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf04-en.jpg"/></fig>
      <p>The surgery was carried out in order to promote the revascularization of the ischemic areas, with magna saphenous vein grafting and utilization of the right internal mammary artery (RIMA).</p>
      <p>On February 17, 2004, the patient was submitted to the surgical intervention. After asepsis and antisepsis in the dorsal decubitus position and median thoracotomy, the patient was placed on extracorporeal circulation (ECC), adequately heparinized and in hypothermia at 32&#176;C, which was kept for 96 minutes. A characteristic of this type of surgery is that the surgeon must be positioned on the patient&#146;s left side, which facilitates the operation. The RIMA was anastomosed to the anterior descending artery. Additionally, magna saphenous vein grafting was used for the revascularization of the right coronary artery, left marginal branch, first diagonal branch and posterior descending branch. </p>
      <p>The postoperative period was uneventful, with the patient presenting clinical improvement of the heart failure and left ventricular function. </p>
      <p>Twenty days after the surgery, the patient was reassessed on an outpatient level and did not present dyspnea, with good general health status, no edema and HR of 80 bpm. </p>
      <p>Six months after the surgery, the patient was again monitored and did not have complaints, presented good health status with BP = 110x70mmHg. The echocardiogram showed normal cardiac function. An angiotomography performed 32 months after the surgery showed all pervious grafts and no lesions (<xref ref-type="fig" rid="fig05-body2">Figures 5</xref>, <xref ref-type="fig" rid="fig06-body2">6</xref> and <xref ref-type="fig" rid="fig07-body2">7</xref>).</p>
      <fig id="fig05-body2">
        <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf05-en.jpg"/></fig>
      <fig id="fig06-body2">
        <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf06-en.jpg"/></fig>
      <fig id="fig07-body2">
        <graphic xlink:href="1678-4170-abc-88-05-e103-00021-gf07-en.jpg"/></fig>
      <p>
        <bold>Discussion</bold>
      </p>
      <p>The myocardial revascularization surgery in patients with dextrocardia, despite the scarcity of literature on the subject, has shown to be an efficient and viable method for reversing ischemic heart conditions. The present report confirms the benefits attained through the procedure. </p>
      <p>It is worth mentioning that the only report on myocardial revascularization in a patient with <italic>situs inversus</italic> in the international medical literature written by Brazilian authors was that by Abensur et al<sup>5</sup>. </p>
      <p>The first report in the Brazilian literature was in 2002<sup>7</sup>, but despite the fact this is not the first Brazilian report, it can be considered the most complex procedure to date, considering the number of anastomoses performed.</p>
      <p>
        <bold>References</bold>
      </p>
      <p>
        <fn id="end-body2" fn-type="corresp">
          <label>
            <bold>Mailing address:</bold>
          </label>
          <p> Paulo Manuel Pego-Fernandes</p>
          <p> Av. En&#233;as de Carvalho Aguiar, 44 &#150; Bloco II &#150; 2&#170; andar &#150; Cerqueira C&#233;sar</p>
          <p> 05403-000 &#150; S&#227;o Paulo, SP &#150; Brazil</p>
          <p> E-mail: </p>
          <p>
            <email xlink:href="paulopego@incor.usp.br">paulopego@incor.usp.br</email>
          </p>
        </fn>
      </p>
      <p>Manuscript received April 20, 2006; revised manuscript received November 8, 2006; accepted January 17, 2007.</p>
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  </sub-article>
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