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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">TcZgrjswfZ8NwhrxdnGC66p</article-id>
      <article-id pub-id-type="other">00022</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2007000200022</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(07)08800222</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2007000200022</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Relato de Caso</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Compress&#227;o extr&#237;nseca da art&#233;ria coron&#225;ria esquerda por dilata&#231;&#227;o aneurism&#225;tica do tronco pulmonar em adolescente: involu&#231;&#227;o ap&#243;s oclus&#227;o cir&#250;rgica de comunica&#231;&#227;o interatrial seio venoso e plastia redutora do tronco pulmonar</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Amaral</surname>
            <given-names>Fernando T. V.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Alves Jr.</surname>
            <given-names>Lafaiete</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Granzotti</surname>
            <given-names>Jo&#227;o A.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Manso</surname>
            <given-names>Paulo H.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Lima Filho</surname>
            <given-names>Moys&#233;s O.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Jurca</surname>
            <given-names>Mauro C.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Rodrigues</surname>
            <given-names>Alfredo J.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Vicente</surname>
            <given-names>Walter V. A.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
      </contrib-group>
      <aff id="aff01">
        <addr-line>
          <named-content content-type="city">Ribeir&#227;o Preto</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">Hospital das Cl&#237;nicas </institution>
        <country country="BR">Brazil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>02</month>
        <year>2007</year>
      </pub-date>
      <volume>88</volume>
      <issue>2</issue>
      <fpage>e40</fpage>
      <lpage>e43</lpage>
      <history>
        <date date-type="received">
          <day>18</day>
          <month>03</month>
          <year>2006</year>
        </date>
        <date date-type="rev-recd">
          <day>18</day>
          <month>03</month>
          <year>2006</year>
        </date>
        <date date-type="accepted">
          <day>18</day>
          <month>05</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 adolescente encaminhado com o diagn&#243;stico de hipertens&#227;o pulmonar. A investiga&#231;&#227;o n&#227;o invasiva detectou comunica&#231;&#227;o interatrial seio venoso com sinais de hipertens&#227;o pulmonar. No estudo hemodin&#226;mico o diagn&#243;stico foi confirmado, sendo tamb&#233;m notada compress&#227;o esquerda pelo tronco pulmonar. O paciente foi submetido &#224; oclus&#227;o cir&#250;rgica da comunica&#231;&#227;o interatrial e &#224; plastia redutora do tronco pulmonar. Dois anos ap&#243;s o procedimento, o paciente encontra-se bem, com sinais cl&#237;nicos e ecocardiogr&#225;ficos de hipertens&#227;o pulmonar discreta e sem evid&#234;ncias, tamb&#233;m pelo ecocardiograma, de obstru&#231;&#227;o do tronco da art&#233;ria coron&#225;ria esquerda.</p>
      </abstract>
      <kwd-group xml:lang="pt" kwd-group-type="author-generated">
        <kwd>Hipertens&#227;o pulmonar</kwd>
        <kwd>anomalias das art&#233;rias coron&#225;rias</kwd>
        <kwd>comunica&#231;&#227;o interatrial</kwd>
      </kwd-group>
      <pub-date publication-format="electronic" date-type="pub">
        <day>20</day>
        <month>03</month>
        <year>2007</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>RELATO DE CASO</bold>
    </p>
    <p> Compress&#227;o extr&#237;nseca da art&#233;ria coron&#225;ria esquerda por dilata&#231;&#227;o aneurism&#225;tica do tronco pulmonar em adolescente. Involu&#231;&#227;o ap&#243;s oclus&#227;o cir&#250;rgica de comunica&#231;&#227;o interatrial seio venoso e plastia redutora do tronco pulmonar</p>
    <p>
      <bold>Fernando T. V. Amaral; Lafaiete Alves Jr.; Jo&#227;o A. Granzotti; Paulo H. Manso; Moys&#233;s O. Lima Filho; Mauro C. Jurca; Alfredo J. Rodrigues; Walter V. A. Vicente</bold>
    </p>
    <p> Hospital das Cl&#237;nicas da Faculdade de Medicina de Ribeir&#227;o Preto - Ribeir&#227;o Preto, 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 adolescente encaminhado com o diagn&#243;stico de hipertens&#227;o pulmonar. A investiga&#231;&#227;o n&#227;o invasiva detectou comunica&#231;&#227;o interatrial seio venoso com sinais de hipertens&#227;o pulmonar. No estudo hemodin&#226;mico o diagn&#243;stico foi confirmado, sendo tamb&#233;m notada compress&#227;o esquerda pelo tronco pulmonar. O paciente foi submetido &#224; oclus&#227;o cir&#250;rgica da comunica&#231;&#227;o interatrial e &#224; plastia redutora do tronco pulmonar. Dois anos ap&#243;s o procedimento, o paciente encontra-se bem, com sinais cl&#237;nicos e ecocardiogr&#225;ficos de hipertens&#227;o pulmonar discreta e sem evid&#234;ncias, tamb&#233;m pelo ecocardiograma, de obstru&#231;&#227;o do tronco da art&#233;ria coron&#225;ria esquerda.</p>
    <p><bold>Palavras-chave:</bold> Hipertens&#227;o pulmonar, anomalias das art&#233;rias coron&#225;rias, comunica&#231;&#227;o interatrial / cirurgia.</p>
    <p>
      <bold>Introdu&#231;&#227;o</bold>
    </p>
    <p>A compress&#227;o extr&#237;nseca da art&#233;ria coron&#225;ria esquerda (ACE) pelo tronco da art&#233;ria pulmonar (TAP) &#233; de ocorr&#234;ncia rara, estando habitualmente associada &#224; presen&#231;a de comunica&#231;&#227;o interatrial (CIA) e/ou hipertens&#227;o pulmonar<sup>1-5</sup> ou, mais raramente, &#224; persist&#234;ncia do canal arterial<sup>6</sup>, tetralogia de Fallot<sup>7</sup> e defeito do septo atrioventricular<sup>8</sup>. A grande maioria dos casos publicados &#233; de pacientes adultos que podem apresentar dor precordial e isquemia mioc&#225;rdica, necessitando revasculariza&#231;&#227;o simultaneamente &#224; corre&#231;&#227;o do defeito causador da dilata&#231;&#227;o do TAP<sup>1,4,6</sup>. Relatamos a seguir o caso de um adolescente portador de CIA seio venoso com compress&#227;o proximal da ACE que evoluiu bem ap&#243;s oclus&#227;o do defeito e plastia redutora do TAP.</p>
    <p>
      <bold>Relato do Caso</bold>
    </p>
    <p>Adolescente de 16 anos, com queixa de dispn&#233;ia de esfor&#231;o e dor precordial at&#237;pica e ocasional, foi encaminhado ao Hospital das Cl&#237;nicas da Faculdade de Medicina de Ribeir&#227;o Preto (HCFMRP) com diagn&#243;stico provis&#243;rio de hipertens&#227;o pulmonar prim&#225;ria sem defeito estrutural no ecocardiograma. No exame f&#237;sico o paciente estava em bom estado geral, os pulsos eram normais e notava-se sopro sist&#243;lico ++/6+ no foco pulmonar, sem fr&#234;mito. A segunda bulha estava fixamente desdobrada e o componente pulmonar era hiperfon&#233;tico. O eletrocardiograma (ECG) mostrava sinais de sobrecarga ventricular direita importante, e na radiografia simples do t&#243;rax havia hiperfluxo pulmonar, dilata&#231;&#227;o do ramo direito da art&#233;ria pulmonar e TAP muito dilatado (<xref ref-type="fig" rid="fig01">fig.1A</xref>). A tomografia computadorizada do t&#243;rax (<xref ref-type="fig" rid="fig01">fig.1B</xref>), assim como a angiorresson&#226;ncia magn&#233;tica (<xref ref-type="fig" rid="fig01">fig.1C</xref>) deixaram evidentes a importante dilata&#231;&#227;o dos ramos e tronco pulmonar e a relativa diminui&#231;&#227;o do calibre da aorta. O ecocardiograma transesof&#225;gico evidenciou CIA seio venoso de 10 mm com drenagem venosa an&#244;mala da veia pulmonar superior direita no &#225;trio direito. O estudo hemodin&#226;mico confirmou os achados n&#227;o invasivos e revelou importante compress&#227;o do tronco da ACE pelo TAP (<xref ref-type="fig" rid="fig01">fig.1D</xref>). A press&#227;o na aorta era de 126/93 mmHg, enquanto na art&#233;ria pulmonar era de 110/70 mmHg, caindo para 73/52 mmHg com inala&#231;&#227;o de O<sub>2</sub>. A resist&#234;ncia vascular sist&#234;mica (RVS) era de 13 UWood, pulmonar (RVP) 6,8 UWood, e a rela&#231;&#227;o de fluxo pulmonar/sist&#234;mico era de 2,9. O paciente foi operado em 28/1/04 e, ap&#243;s abertura do peric&#225;rdio, chamava a aten&#231;&#227;o o di&#226;metro do TAP tr&#234;s vezes maior que o da aorta. A CIA foi oclu&#237;da com selo de peric&#225;rdio bovino, a veia pulmonar an&#244;mala foi direcionada para o &#225;trio esquerdo e foi feita plastia redutora do TAP. A evolu&#231;&#227;o imediata foi favor&#225;vel e o paciente teve alta uma semana ap&#243;s a interna&#231;&#227;o em uso de captopril. Seis meses ap&#243;s, o paciente estava bem, e no estudo hemodin&#226;mico havia CIA residual m&#237;nima com rela&#231;&#227;o de fluxo pulmonar/sist&#234;mico de 1,05, RVP: 7 UWood, RVS: 13,7 UWood, press&#227;o na art&#233;ria pulmonar: 75/52 mmHg e press&#227;o na aorta: 110/83 mmHg, sendo tamb&#233;m notada diminui&#231;&#227;o da compress&#227;o extr&#237;nseca da ACE (<xref ref-type="fig" rid="fig02">fig. 2A</xref>). Com 21 meses ap&#243;s a cirurgia o paciente estava assintom&#225;tico, em uso de 50 mg/dia de captopril e na ausculta card&#237;aca havia "click" pulmonar com discreta hiperfonese da segunda bulha. No eletrocardiograma notou-se regress&#227;o importante da sobrecarga ventricular direita e na radiografia do t&#243;rax havia discreta diminui&#231;&#227;o do TAP (<xref ref-type="fig" rid="fig02">fig. 2B</xref>). O ecocardiograma transtor&#225;cico mostrou press&#227;o sist&#243;lica da art&#233;ria pulmonar de 55 mm/Hg, septo interatrial &#237;ntegro, hipertrofia do ventr&#237;culo direito, aus&#234;ncia de compress&#227;o da ACE (<xref ref-type="fig" rid="fig02">fig. 2C</xref>) e TAP ainda dilatado (<xref ref-type="fig" rid="fig02">fig. 2D</xref>). O ecocardiograma de estresse com dobutamina foi negativo para isquemia mioc&#225;rdica.</p>
    <fig id="fig01">
      <graphic xlink:href="1678-4170-abc-88-02-0e40-00022-gf01.jpg"/></fig>
    <fig id="fig02">
      <graphic xlink:href="1678-4170-abc-88-02-0e40-00022-gf02.jpg"/></fig>
    <p>
      <bold>Discuss&#227;o</bold>
    </p>
    <p>Descrita pela primeira vez h&#225; quase meio s&#233;culo<sup>9</sup>, a compress&#227;o extr&#237;nseca da ACE pelo TAP &#233; entidade pouco freq&#252;ente. Estudos com casu&#237;stica importante s&#227;o raros; por&#233;m, h&#225; informa&#231;&#245;es na literatura da ocorr&#234;ncia de dois pacientes (1,8%) entre 48 casos de CIA estudados por Kothari e cols.<sup>10</sup>. Outro estudo importante foi realizado em 1989 por Mitsudo e cols.<sup>1</sup> e tem servido como marco para discuss&#227;o do tema. Estudando 38 pacientes com idade de 15 a 62 anos, esse autor encontrou sete (18%) casos com compress&#227;o extr&#237;nseca da ACE. Nos casos em que havia hipertens&#227;o pulmonar associada, esse n&#250;mero chegava a 44%.</p>
    <p>A maioria dos casos descritos mostra associa&#231;&#227;o freq&#252;ente com CIA<sup>1,2,4,5,11</sup>; por&#233;m, outras entidades como persist&#234;ncia do canal arterial<sup>6</sup>, tetralogia de Fallot<sup>7</sup> e defeito do septo atrioventricular<sup>8</sup> podem estar envolvidas. Pacientes portadores de hipertens&#227;o pulmonar prim&#225;ria com dilata&#231;&#227;o importante do TAP podem tamb&#233;m apresentar o mesmo quadro<sup>3,12</sup>. Essa grave complica&#231;&#227;o pode ser suspeitada pela presen&#231;a de dor precordial em pacientes com esse tipo de cardiopatia que apresentem dilata&#231;&#227;o importante do TAP na radiografia de t&#243;rax. O ECG pode mostrar altera&#231;&#245;es isqu&#234;micas<sup>2,3,6</sup> e a obstru&#231;&#227;o coronariana pode ser visualizada pelo ecocardiograma e pela resson&#226;ncia magn&#233;tica nuclear. Entretanto, a confirma&#231;&#227;o diagn&#243;stica e avalia&#231;&#227;o da gravidade da compress&#227;o &#233; geralmente feita com maior seguran&#231;a pela coronariografia.</p>
    <p>O caso aqui apresentado tem algumas caracter&#237;sticas que, a nosso ver, justificam esse relato. Apesar do tema j&#225; ter sido discutido em expressiva casu&#237;stica nacional<sup>4</sup>, nosso paciente parece ser o primeiro publicado em nossos peri&#243;dicos. Al&#233;m disso, a ocorr&#234;ncia dessa complica&#231;&#227;o em adolescente, como aqui descrito, parece ser bastante rara, havendo, ao que sabemos, somente um relato nessa faixa et&#225;ria<sup>11</sup>.</p>
    <p>Um outro aspecto interessante, tamb&#233;m evidenciado em nosso caso, &#233; a conduta adotada. Alguns autores acreditam que h&#225; necessidade de revasculariza&#231;&#227;o nos casos com obstru&#231;&#227;o importante<sup>1</sup>. Outros sugerem que somente a redu&#231;&#227;o cir&#250;rgica do TAP pode eliminar a compress&#227;o da ACE, principalmente se a hipertens&#227;o pulmonar for considerada revers&#237;vel<sup>2</sup>. Nosso paciente tinha sinais importantes de hipertens&#227;o pulmonar grave com boa resposta ao O<sub>2</sub> e notamos, quase dois anos ap&#243;s a cirurgia, involu&#231;&#227;o consider&#225;vel da obstru&#231;&#227;o pelo ecocardiograma.</p>
    <p>Concluindo, podemos dizer que a compress&#227;o extr&#237;nseca da ACE pelo TAP &#233; bastante rara, podendo, entretanto, estar subdiagnosticada, como j&#225; sugerido<sup>4</sup>. Em virtude da possibilidade de involu&#231;&#227;o da obstru&#231;&#227;o, como aqui mostrado, muitos casos de CIA operados sem visualiza&#231;&#227;o das coron&#225;rias podem n&#227;o ter sido adequadamente documentados. Pacientes portadores de cardiopatias que levem a um aumento do TAP (com ou sem hipertens&#227;o pulmonar) devem ser investigados cuidadosamente, em especial se a queixa de dor precordial estiver presente. Em virtude da raridade dessa complica&#231;&#227;o, impossibilitando estudo terap&#234;utico randomizado, acreditamos ser dif&#237;cil definir a necessidade ou n&#227;o de revasculariza&#231;&#227;o rotineira nos casos com obstru&#231;&#227;o importante. Entretanto, baseado no caso aqui apresentado e nas poucas informa&#231;&#245;es da literatura, parece-nos que em pacientes jovens, sem les&#227;o em outras art&#233;rias e com hipertens&#227;o pulmonar revers&#237;vel, a conduta mais apropriada seja a corre&#231;&#227;o do defeito card&#237;aco associada &#224; plastia redutora do TAP.</p>
    <p>
      <fn id="end" fn-type="corresp">
        <label>
          <bold>Correspond&#234;ncia:</bold>
        </label>
        <p> Fernando T. V. Amaral </p>
        <p> Rua Jo&#227;o Pandovan, 195 </p>
        <p> 14024-030 - Ribeir&#227;o Preto, SP </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="ftamaral@cardiol.br">ftamaral@cardiol.br</email>
        </p>
      </fn>
    </p>
    <p> Artigo recebido em 18/03/06; revisado recebido em 18/03/06; aceito em 18/05/06.</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">Extrinsic compression of left main coronary artery from aneurysmal dilation of pulmonary trunk in a adolescent: involution after surgery occlusion of sinus venosus atrial septal defect and pulmonary trunk plasty for reduction</article-title>
      </title-group>
      <abstract xml:lang="en">
        <p>We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence - also on echocardiogram - of left coronary artery trunk obstruction.</p>
      </abstract>
      <kwd-group xml:lang="en">
        <kwd>Pulmonary hypertension</kwd>
        <kwd>coronary artery anomalies</kwd>
        <kwd>atrial septal defect</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>
        <bold>CASE REPORT</bold>
      </p>
      <p> Extrinsic compression of left main coronary artery from aneurysmal dilation of pulmonary trunk in an adolescent. Involution after surgery occlusion of sinus venosus atrial septal defect and pulmonary trunk plasty for reduction </p>
      <p>
        <bold>Fernando T. V. Amaral, Lafaiete Alves Jr.; Jo&#227;o A. Granzotti; Paulo H. Manso; Moys&#233;s O. Lima Filho; Mauro C. Jurca; Alfredo J. Rodrigues; Walter V. A. Vicente</bold>
      </p>
      <p> Hospital das Cl&#237;nicas da Faculdade de Medicina de Ribeir&#227;o Preto - Ribeir&#227;o Preto, SP - Brazil</p>
      <p>
        <xref ref-type="corresp" rid="end-body2">Mailing Address</xref>
      </p>
      <p>
        <bold>ABSTRACT</bold>
      </p>
      <p>We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence &#150; also on echocardiogram &#150; of left coronary artery trunk obstruction.</p>
      <p><bold>Key words:</bold> Pulmonary hypertension, coronary artery anomalies, atrial septal defect / surgery.</p>
      <p>
        <bold>Introduction</bold>
      </p>
      <p>Extrinsic compression of left coronary artery (LCA) by pulmonary artery trunk (PAT) is rare, and usually associated to IAC and/or pulmonary hypertension<sup>1-5</sup>. Even more rarerely it is associated to persistence of arterial canal<sup>6</sup>, tetralogy of Fallot (TOF)<sup>7</sup>, and atrioventricular septal defect<sup>8</sup>. Most studies that have been published report on adult patients that may present precordial pain and myocardial ischemia, requiring revascularization simultaneously to the correction of defect that causes PAT dilation<sup>1,4,6</sup>. We report the case of an adolescent with sinus venosus atrial septal defect (ASD), with proximal compression of LCA, with uneventful evolution after the occlusion of defect and PAT reduction plasty. </p>
      <p>
        <bold>Case Report</bold>
      </p>
      <p>A 16-year-old adolescent reporting complaints of stress dyspnea and atypical, occasional precordial pain was referred to the Clinics Hospital, at Ribeir&#227;o Preto Medical School, with an initial diagnosis of primary pulmonary hypertension, and with no structural defect shown on echocardiogram. The pacient was in good condition at physical exam, pulse was normal, and ++/6+ systolic murmur at pulmonary focus, with no thrill. Heart sound 2 was steadily unfolded, with a hyperphonetic pulmonary component. ECG showed signs of significant right ventricular overburden. Thoracic X-Ray showed pulmonary hyperflow, right branch of pulmonary artery dilation, and quite dilated PAT (<xref ref-type="fig" rid="fig01-body2">Fig. 1A</xref>). Thoracic CAT scan (<xref ref-type="fig" rid="fig01-body2">Fig. 1B</xref>), as well as magnetic angioresonance (<xref ref-type="fig" rid="fig01-body2">Fig. 1C</xref>) pointed out the significant dilation of pulmonary branches and trunk, and relative aortic caliber decrease. Transesophageal echocardiogram showed a 10 mm sinus venosus IAC, with anomalous venous drainage of right upper pulmonary vein in right atrium. The hemodynamic study confirmed non-invasive findings and showed significant compression of LCA trunk by PAT (<xref ref-type="fig" rid="fig01-body2">Fig. 1D</xref>). Aortic pressure was 126/93 mmHg, while pulmonary artery pressure was 110/70 mmHg, decreasing to 73/52 mmHg with O<sub>2</sub> inhalation. Systemic vascular resistance (SVR) was 13 UWood, pulmonary vascular resistance (PVR) was 6.8 UWood. Pulmonary/systemic flow ratio was 2.9. The patient was submitted to surgery on January 28, 2004. After pericardium opening, PAT diameter called the attention since it was three times larger than aortic diameter. The ASD was occluded with bovine pericardial strip, the anomalous pulmonary vein was directed into the left atrium, with reduction plasty of PAT. Immediate evolution was favorable and the patient was discharged on captopril one week after hospital admission. Six months later the patient was in good condition, and the hemodynamic study showed slight residual ASD, with pulmonary/systemic flow ratio at 1.05, PVR: 7 UWood, SVR: 13.7 UWood, pulmonary artery pressure: 75/52 mmHg, and aortic pressure: 110/83 mmHg. Extrinsic compression of LCA was also observed to have been reduced (<xref ref-type="fig" rid="fig02-body2">Fig. 2A</xref>). Twenty-one months after the surgery the patient was asymptomatic, on 50 mg/day of captopril. Cardiac auscultation revealed a pulmonary click with S2 mildly hyperphonetic. Significant regression of right ventricular overburden was shown on the ECG; discreet reduction of PAT was shown on the X-Ray (<xref ref-type="fig" rid="fig02-body2">Fig. 2B</xref>). Transthoracic echocardiogram showed pulmonary artery systolic pressure at 55 mm/Hg, intact interatrial septum, right ventricle hypertrophy, no compression of LCA (<xref ref-type="fig" rid="fig02-body2">Fig. 2C</xref>), and PAT still dilated (<xref ref-type="fig" rid="fig02-body2">Fig. 2D</xref>). Stress echocardiogram with dobutamine was negative for myocardial ischemia.</p>
      <fig id="fig01-body2">
        <graphic xlink:href="1678-4170-abc-88-02-0e40-00022-gf01-en.jpg"/></fig>
      <fig id="fig02-body2">
        <graphic xlink:href="1678-4170-abc-88-02-0e40-00022-gf02-en.jpg"/></fig>
      <p>
        <bold>Discussion</bold>
      </p>
      <p>Described for the first time almost half a century ago<sup>9</sup>, extrinsic compression of LCA by PAT is not common. Comprehensive studies are rare, although information has been available in literature on the occurrence of 2 patients (1.8%) out of 48 IAC cases studied by Kothari et al<sup>10</sup>. Another relevant study was conducted in 1989 by Mitsudo<sup>1</sup>, and has stood as a landmark for topic discussion. While studying 38 patients in the 15-62-year-old range, the author found 7 cases (18%) of extrinsic compression of LCA. The figures reached 44% for cases with pulmonary hypertension associated.</p>
      <p>Most cases described reveal a frequent association with IAC<sup>1,2,4,5,11</sup>; however, other conditions &#150; such as persistence of arterial canal<sup>6</sup>, tetralogy of Fallot (TOF)<sup>7</sup>, and atrioventricular septal defect<sup>8</sup> &#150; may be involved. Primary pulmonary hypertension patients with significant PAT dilation may also report the same conditions<sup>3,12</sup>. Precordial pain may act as suspicion of such serious complication in patients with this type of cardiopathy and with significant PAT dilation on thoracic X-Ray. The ECG may reveal ischemic changes<sup>2, 3,6</sup>, and coronary obstruction may be visualized on echocardiogram and on nuclear magnetic resonance. However, diagnostic confirmation and the assessment of compression severity is usually more reliable through coronariography.</p>
      <p>The case presented includes some characteristics which we understand justify this report. Although the topic has been discussed based on fairly extensive studies in this country<sup>4</sup>, our patient seems to be the first case to be published in our journals. Additionally, the occurrence of such complication in an adolescent &#150; as described &#150; seems to be quite rare. To our knowledge, there is only one report in that age range<sup>11</sup>.</p>
      <p>Another interesting aspect &#150; also made evident in our case &#150; is the conduct adopted. Some authors believe in the need of revascularization for cases with significant obstruction<sup>1</sup>. Others suggest that surgical reduction of PAT only may eliminate compression of the LCA, particularly if pulmonary hypertension is considered reversible<sup>2</sup>. Our patient had significant signs of severe pulmonary hypertension, with good response to O<sub>2</sub>. Almost 2 years after surgery, considerable involution of the obstruction could be seen on the echocardiogram. </p>
      <p>To conclude, we could say that extrinsic compression of LCA by PAT is quite rare. As suggested, it may, therefore, be underdiagnosed<sup>4</sup>. Due to the possibility of obstruction involution, as demonstrated, many IAC cases that have been operated on without visualization of the coronaries may not have been properly documented. Cardioapathy patients that develop PAT increase (with or without pulmonary hypertension), must be carefully investigated, particularly if associated to precordial pain complaint. Due to the rare degree of the condition - which hinders any randomized therapy study &#150; the authors find it hard to define whether routine revascularization for cases with significant obstruction is required or not. However, basead on the case reported here, and on the small amount of information available in the literature, it does seem that for young patients with no injury in other arteries and with reversible pulmonary hypertension, the most approppriate conduct is correction of cardiac defect associated to PAT reduction plasty.</p>
      <p>
        <bold>References</bold>
      </p>
      <p>
        <fn id="end-body2" fn-type="corresp">
          <label>
            <bold>Mailing Address:</bold>
          </label>
          <p> Fernando T. V. Amaral</p>
          <p> Rua Jo&#227;o Pandovan, 195 </p>
          <p> 14024-030 - Ribeir&#227;o Preto, SP - Brazil</p>
          <p> E-mail: </p>
          <p>
            <email xlink:href="ftamaral@cardiol.br">ftamaral@cardiol.br</email>
          </p>
        </fn>
      </p>
      <p> Manuscript received March 03, 2005; revised manuscript received May 18, 2006; accepted on March 18, 2006.</p>
    </body>
  </sub-article>
</article>
