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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">RPLFBJm7gBnr3hmDx9pFb8B</article-id>
      <article-id pub-id-type="other">00016</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2011001200016</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(11)09700316</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2011001200016</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Correla&#231;&#227;o Cl&#237;nico-Radiogr&#225;fica</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Caso 5/2011 - Lactente de 17 meses de idade, com tetralogia de fallot em franca insufici&#234;ncia card&#237;aca por domin&#226;ncia hemodin&#226;mica da comunica&#231;&#227;o interventricular</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Atik</surname>
            <given-names>Edmar</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">SP</named-content>
        </addr-line>
        <institution content-type="orgname">Hospital S&#237;rio-Liban&#234;s</institution>
        <country country="BR">Brasil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>09</month>
        <year>2011</year>
      </pub-date>
      <volume>97</volume>
      <issue>3</issue>
      <fpage>e48</fpage>
      <lpage>e49</lpage>
      <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>
      <pub-date publication-format="electronic" date-type="pub">
        <day>21</day>
        <month>10</month>
        <year>2011</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>CORRELA&#199;&#195;O CL&#205;NICO-RADIOGR&#193;FICA</bold>
    </p>
    <p>
      <bold>Caso 5/2011 - Lactente de 17 meses de idade, com tetralogia de fallot em franca insufici&#234;ncia card&#237;aca por domin&#226;ncia hemodin&#226;mica da comunica&#231;&#227;o interventricular</bold>
    </p>
    <p>
      <bold>Edmar Atik</bold>
    </p>
    <p>Hospital S&#237;rio-Liban&#234;s, S&#227;o Paulo, SP, Brasil</p>
    <p>
      <xref ref-type="corresp" rid="back">Correspond&#234;ncia</xref>
    </p>
    <p><bold>Palavras-chave: </bold> Cardiopatias cong&#234;nitas, tetralogia de fallot, insufici&#234;ncia card&#237;aca, comunica&#231;&#227;o interventricular/complica&#231;&#245;es</p>
    <p>
      <bold>Aspectos cl&#237;nicos</bold>
    </p>
    <p>Desde o nascimento, observa-se dispneia persistente, agita&#231;&#227;o psicomotora e baixo ganho ponderal. Nesse per&#237;odo, n&#227;o surgiu cianose, mesmo com o choro. Ao exame f&#237;sico, havia sinais caracter&#237;sticos da s&#237;ndrome de Down e taquidispneia moderada, estando acian&#243;tico e com pulsos normais. O paciente apresentou peso de 8.200 g. e altura de 74 cm. O prec&#243;rdio mostrava abaulamento pronunciado, com impuls&#245;es sist&#243;licas moderadas. O <italic>ictus cordis</italic> era difuso no 4&#186; e 5&#186; espa&#231;os intercostais. Havia sopro sist&#243;lico no 3&#186; e 4&#186; espa&#231;os intercostais na borda esternal e com irradia&#231;&#227;o para a borda esternal alta. A segunda bulha era hiperfon&#233;tica e desdobrada. O f&#237;gado era palpado a dois cent&#237;metros da reborda costal direita.</p>
    <p>
      <bold>Exames complementares</bold>
    </p>
    <p><bold>Eletrocardiograma</bold> exibia sinais de sobrecarga biventricular, sem hemibloqueio anterior esquerdo. O eixo de QRS estava desviado para a direita a +110&#186;, e as ondas RS eram amplas de V2 a V5 e polif&#225;sicas em V1 (<xref ref-type="fig" rid="f1">Figura 1</xref>).</p>
    <fig id="f1">
      <graphic xlink:href="1678-4170-abc-97-03-0e48-00016-gf01.jpg"/></fig>
    <p><bold>Radiografia de t&#243;rax</bold> mostrava acentuado aumento da &#225;rea card&#237;aca e da trama vascular pulmonar. O arco ventricular esquerdo era arredondado e longo, continuando-se com o arco m&#233;dio (<xref ref-type="fig" rid="f2">Figura 2</xref>).</p>
    <fig id="f2">
      <graphic xlink:href="1678-4170-abc-97-03-0e48-00016-gf02.jpg"/></fig>
    <p><bold>Ecocardiograma</bold> mostrava comunica&#231;&#227;o interventricular (CIV) perimembranosa de 12 mm, dextroposi&#231;&#227;o da aorta de 50,0%, desvio anterior do septo infundibular com estenose discreta com 30 mmHg de gradiente. O <italic>shunt</italic> era bidirecional pela comunica&#231;&#227;o interventricular.</p>
    <p>
      <bold>Diagn&#243;stico</bold>
    </p>
    <p>Tetralogia de Fallot em franca insufici&#234;ncia card&#237;aca por domin&#226;ncia hemodin&#226;mica da comunica&#231;&#227;o interventricular.</p>
    <p><bold>Conduta:</bold> &#192; cirurgia, em circula&#231;&#227;o extracorp&#243;rea de 85 minutos, pelo &#225;trio direito, grande comunica&#231;&#227;o interventricular suba&#243;rtica foi visibilizada, fechada com retalho de peric&#225;rdio. A abertura do tronco pulmonar identificou valva pulmonar bivalvulada, com textura fina e com boa abertura, em anel pulmonar pequeno de 12 mm, o qual foi preservado. Ap&#243;s abertura da via de sa&#237;da de ventr&#237;culo direito, foi realizada miectomia infundibular al&#233;m da amplia&#231;&#227;o da regi&#227;o com peric&#225;rdio bovino e da regi&#227;o do tronco pulmonar.</p>
    <p>O p&#243;s-operat&#243;rio foi complicado por pneumot&#243;rax espont&#226;neo, baixo d&#233;bito card&#237;aco consequente, aspira&#231;&#227;o br&#244;nquica e pneumonia, que requereram tratamento prolongado por 14 dias at&#233; a alta hospitalar.</p>
    <p><bold>Considera&#231;&#245;es:</bold> Nos primeiros meses de vida, a tetralogia de Fallot se exterioriza por sopro card&#237;aco que se assemelha ao da comunica&#231;&#227;o interventricular, baixo na borda esternal esquerda, decorrente do fluxo de sangue da esquerda para a direita e, por isso, a cianose n&#227;o se mostra neste per&#237;odo. Com a anterioriza&#231;&#227;o maior do septo infundibular, e o consequente agravamento da obstru&#231;&#227;o da via de sa&#237;da do ventr&#237;culo direito, manifesta-se o fluxo de sangue da direita para a esquerda com aparecimento de cianose e de crises hipox&#234;micas, que surgem geralmente entre tr&#234;s a seis meses de idade. Em raros casos, a comunica&#231;&#227;o interventricular pode predominar sobre a estenose infundibular, o que origina quadro de hiperfluxo pulmonar, sobrecarga de volume das cavidades card&#237;acas com consequente insufici&#234;ncia card&#237;aca, como ocorreu no caso apresentado.</p>
    <p>Dessa maneira, a exterioriza&#231;&#227;o cl&#237;nica de congest&#227;o venocapilar pulmonar em cardiopatias que se comportam como comunica&#231;&#227;o interventricular grande com insufici&#234;ncia card&#237;aca pode tamb&#233;m se apresentar na tetralogia de Fallot, em presen&#231;a de n&#237;tido predom&#237;nio da comunica&#231;&#227;o interventricular sobre a estenose pulmonar infundibular, apesar ainda da presen&#231;a de dextroposi&#231;&#227;o da aorta e da hipertrofia de ventr&#237;culo direito. Ressalta-se claramente que no quadro cl&#225;ssico da tetralogia de Fallot, a exterioriza&#231;&#227;o cl&#237;nica dominante sem d&#250;vida decorre da predomin&#226;ncia da estenose pulmonar infundibular.</p>
    <p>
      <fn id="back" fn-type="corresp">
        <p>Correspond&#234;ncia:</p>
        <p> Edmar Atik </p>
        <p> Rua Dona Adma Jafet, 74 conj. 73 - Bela Vista </p>
        <p> 01308-050 - S&#227;o Paulo, SP - Brasil </p>
        <p> E-mail: </p>
        <p><email xlink:href="eatik@cardiol.br">eatik@cardiol.br</email>, </p>
        <p>
          <email xlink:href="conatik@incor.usp.br">conatik@incor.usp.br</email>
        </p>
      </fn>
    </p>
    <p>Artigo recebido em 29/07/10; revisado recebido em 13/01/11, aceito em 13/01/11.</p>
  </body>
  <sub-article article-type="translation" id="TRen" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Clinical-Radiographic Correlation</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title xml:lang="en">Case 5/2011 - Infant of 17 months of age with tetralogy of fallot in open heart failure due to hemodynamic dominance of the ventricular septal defect</article-title>
      </title-group>
    </front-stub>
    <body>
      <p>
        <bold>CLINICO RADIOLOGICAL SESSION</bold>
      </p>
      <p>
        <bold>Case 5/2011 - Infant of 17 months of age with tetralogy of fallot in open heart failure due to hemodynamic dominance of the ventricular septal defect</bold>
      </p>
      <p>
        <bold>Edmar Atik</bold>
      </p>
      <p>Hospital S&#237;rio-Liban&#234;s, S&#227;o Paulo, SP, Brazil</p>
      <p>
        <xref ref-type="corresp" rid="back-body2">Mailing Address</xref>
      </p>
      <p><bold>Keywords:</bold> Heart defects congenital; tetralogy of fallot; heart failure; heart septal defects, ventricular/complications.</p>
      <p>
        <bold>Clinical Aspects</bold>
      </p>
      <p>Since birth, the patient has had persistent dyspnea, psychomotor agitation and poor weight gain. Over this period, no cyanosis has appeared, even with the crying. On physical examination, there were characteristic signs of Down syndrome and moderate tachydyspnea; the patient has been acyanotic and with normal pulse. The patient had a weight of 8,200 g. and height of 74 cm. The precordium showed pronounced bulging with moderate systolic impulses. Apical impulse was diffuse in the fourth and fifth intercostal spaces. There was systolic murmur in the third and fourth intercostal spaces at the left sternal edge irradiating to the upper sternal edge. The second sound was accentuated and split. The liver was palpable two centimeters at the right coastal edge.</p>
      <p>
        <bold>Complementary tests</bold>
      </p>
      <p><bold>Electrocardiogram</bold> revealed signs of severe biventricular overload with no left anterior hemiblock. The QRS axis was deviated to the right at +110&#186;, and the RS waves were large from V2 to V5 and polyphasic in V1 (<xref ref-type="fig" rid="f1-body2">Figure 1</xref>).</p>
      <fig id="f1-body2">
        <graphic xlink:href="1678-4170-abc-97-03-0e48-00016-gf01-en.jpg"/></fig>
      <p><bold>Chest radiography</bold> revealed marked increase in heart size and pulmonary vasculature. The left ventricular arch was rounded and long, continuing with the medial arch (<xref ref-type="fig" rid="f2-body2">Figure 2</xref>).</p>
      <fig id="f2-body2">
        <graphic xlink:href="1678-4170-abc-97-03-0e48-00016-gf02-en.jpg"/></fig>
      <p><bold>Echocardiogram</bold> showed 12 mm perimembranous ventricular septal defect (VSD), aortic dextroposition of 50.0%, anterior deviation of the infundibular septum with mild stenosis with 30 mmHg gradient. The shunt was bidirectional for ventricular septal defect.</p>
      <p>
        <bold>Diagnosis</bold>
      </p>
      <p>Tetralogy of Fallot in open heart failure due to hemodynamic dominance of the ventricular septal defect.</p>
      <p><bold>Conduct:</bold> In surgery, extracorporeal circulation of 85 minutes, through the right atrium, a large subaortic ventricular septal defect was found, which was closed with pericardium patch. The opening of the pulmonary trunk identified a bivalve pulmonary valve, with fine texture and good opening in a small pulmonary annulus of 12 mm, which was preserved. After opening the right ventricle outflow tract, infundibular myectomy was performed in addition to enlarging the region with bovine pericardium and the pulmonary region.</p>
      <p>The postoperative course was complicated by spontaneous pneumothorax, consequent low cardiac output, bronchial aspiration and pneumonia, which required prolonged treatment for 14 days until discharge.</p>
      <p><bold>Considerations:</bold> In the first months of life, tetralogy of Fallot is externalized by heart murmur that resembles that of the ventricular septal defect, low in the left sternal border, resulting in blood flow from left to right and therefore no cyanosis is found in this period. With a larger anterior infundibular septum, and the consequent worsening of obstruction of right ventricular outflow tract, blood flows from right to left with the appearance of cyanosis and hypoxemia crises, which usually appear from three to six months of age. In rare cases, ventricular septal defect may predominate over the infundibular stenosis, which leads to increased pulmonary flow, volume overload of the heart chambers and consequent cardiac failure, as was the case presented.</p>
      <p>Thus, the clinical manifestation of pulmonary venous congestion in heart defects that behave as large ventricular septal defect with heart failure may also be present in tetralogy of Fallot in the presence of a clear prevalence of ventricular septal defects on pulmonary infundibular stenosis, yet despite the presence of dextroposition of aorta and right ventricular hypertrophy. It is noteworthy that in the classic case of tetralogy of Fallot, the dominant clinical manifestation undoubtedly stems from the prevalence of infundibular pulmonary stenosis.</p>
      <p>
        <fn id="back-body2" fn-type="corresp">
          <p>Mailing Address:</p>
          <p> Edmar Atik</p>
          <p> Rua Dona Adma Jafet, 74 conj. 73 - Bela Vista</p>
          <p> 01308-050 - S&#227;o Paulo, SP - Brazil</p>
          <p> E-mail: </p>
          <p><email xlink:href="eatik@cardiol.br">eatik@cardiol.br</email>, </p>
          <p>
            <email xlink:href="conatik@incor.usp.br">conatik@incor.usp.br</email>
          </p>
        </fn>
      </p>
      <p>Manuscript received July 29, 2010; revised manuscript received January 13, 2011; accepted on January 13, 2011.</p>
    </body>
  </sub-article>
</article>
