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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">Py6X8t8gMGq9z6vwzwLTLmP</article-id>
      <article-id pub-id-type="other">00010</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2007000600010</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(07)08800610</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2007000600010</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 3/2007: lactente de tr&#234;s meses com criss-cross heart, discord&#226;ncia atrioventricular e dupla via de sa&#237;da de ventr&#237;culo direito, sem estenose pulmonar</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">USP</institution>
        <institution content-type="orgdiv1">FM </institution>
        <institution content-type="orgdiv2">Hospital das Cl&#237;nicas</institution>
        <country country="BR">Brasil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>06</month>
        <year>2007</year>
      </pub-date>
      <volume>88</volume>
      <issue>6</issue>
      <fpage>693</fpage>
      <lpage>695</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>23</day>
        <month>07</month>
        <year>2007</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>CORRELA&#199;&#195;O CL&#205;NICO-RADIOGR&#193;FICA</bold>
    </p>
    <p>Caso 3/2007 &#150; Lactente de tr&#234;s meses com <italic><bold>criss-cross heart</bold></italic>, discord&#226;ncia atrioventricular e dupla via de sa&#237;da de ventr&#237;culo direito, sem estenose pulmonar </p>
    <p>
      <bold>Edmar Atik</bold>
    </p>
    <p>Instituto do Cora&#231;&#227;o do Hospital das Cl&#237;nicas - FMUSP - S&#227;o Paulo, SP - Brasil</p>
    <p>
      <xref ref-type="corresp" rid="corr1">Correspond&#234;ncia</xref>
    </p>
    <p><bold>Palavras-chave:</bold> Cardiopatias cong&#234;nitas, lactente, cora&#231;&#227;o entrecruzado, dupla via de sa&#237;da do ventr&#237;culo direito.</p>
    <p>
      <bold>Dados cl&#237;nicos</bold>
    </p>
    <p>Lactente de tr&#234;s meses de idade, do sexo masculino e de cor branca, apresentava cansa&#231;o &#224;s mamadas desde o nascimento, com acentua&#231;&#227;o nos &#250;ltimos 60 dias, acompanhado de inquietude e de dificuldade em ganhar peso, com aumento de apenas 240g no &#250;ltimo m&#234;s. Tosse seca ocorria por vezes. Ao exame f&#237;sico estava dispn&#233;ico ++, acian&#243;tico, com pulsos normais e peso de 4.440 g. A aorta n&#227;o foi palpada. No prec&#243;rdio, havia impuls&#245;es discretas na borda esternal esquerda, o <italic>ictus cordis </italic>situava-se no 4&#186; espa&#231;o intercostal esquerdo, e o m&#250;sculo-valvar +/++, limitado por duas polpas digitais. As bulhas eram acentuadas, mais intensas na &#225;rea tric&#250;spide em rela&#231;&#227;o aos outros focos. A 3&#170; bulha era presente na &#225;rea mitral. Auscultava-se sopro sist&#243;lico +, eje&#231;&#227;o, rude, no 3&#186; e 4&#186; espa&#231;os intercostais esquerdos, irradiado ao bordo direito. O f&#237;gado foi palpado a 4 cm do rebordo costal direito e do ap&#234;ndice xif&#243;ide. </p>
    <p>O eletrocardiograma demonstrou ritmo sinusal com sinais de sobrecarga ventricular indeterminada em face de ondas S predominantes desde V3. Havia poss&#237;vel bloqueio da divis&#227;o &#226;ntero-superior do ramo esquerdo com eixo de QRS desviado para cima e &#224; direita a -120&#186;. S&#194;P: +40&#186;, S&#194;QRS: -120&#186;, S&#194;T: +80&#186;. </p>
    <p>
      <bold>Imagem radiogr&#225;fica</bold>
    </p>
    <p>Imagem demonstrando &#225;rea card&#237;aca aumentada, &#224;s custas do arco ventricular esquerdo longo e do &#225;trio direito, configurando morfologia ovalada. O arco m&#233;dio &#233; retificado e a trama vascular pulmonar est&#225; aumentada (<xref ref-type="fig" rid="a10fig01">fig. 1</xref>). </p>
    <p content-type="asset">
      <fig id="a10fig01">
        <graphic xlink:href="1678-4170-abc-88-06-0693-00010-gf01.jpg"/></fig>
    </p>
    <p>
      <bold>Impress&#227;o diagn&#243;stica</bold>
    </p>
    <p>Esta imagem sugere a de cardiopatia cong&#234;nita com hiperfluxo pulmonar tipo transposi&#231;&#227;o das grandes art&#233;rias em face da morfologia ovalada. </p>
    <p>
      <bold>Diagn&#243;stico diferencial</bold>
    </p>
    <p>Outras cardiopatias com hiperfluxo pulmonar com essa morfologia e que apresentem aumento do &#225;trio direito e do ventr&#237;culo esquerdo devem ser lembradas, como dupla via de sa&#237;da de ventr&#237;culo direito, atresia tric&#250;spide sem estenose pulmonar, atresia mitral sem estenose pulmonar, tronco arterial comum e ventr&#237;culo &#250;nico. </p>
    <p>
      <bold>Confirma&#231;&#227;o diagn&#243;stica </bold>
    </p>
    <p>Os elementos cl&#237;nicos levam ao diagn&#243;stico de cardiopatia com hiperfluxo pulmonar com comunica&#231;&#227;o interventricular associada, em decorr&#234;ncia de sopro sist&#243;lico baixo na borda esternal esquerda. A orienta&#231;&#227;o do eixo el&#233;trico de QRS para a direita e para cima (poss&#237;vel hemibloqueio anterior esquerdo) e com sobrecarga ventricular indeterminada suscita d&#250;vidas e acrescenta elementos &#224; suspeita da exist&#234;ncia de cardiopatia mais complexa. O ecocardiograma (<xref ref-type="fig" rid="a10fig02">fig. 2</xref>) demonstrou <italic>criss-cross heart </italic>com discord&#226;ncia atrioventricular e dupla via de sa&#237;da de ventr&#237;culo direito, saindo a aorta &#224; direita e a art&#233;ria pulmonar &#224; esquerda com duplo infund&#237;bulo, sem estenose pulmonar. A comunica&#231;&#227;o interatrial era restritiva. A comunica&#231;&#227;o interventricular de via de entrada n&#227;o era relacionada aos vasos arteriais, permitindo que a valva tric&#250;spide se relacionasse ao ventr&#237;culo esquerdo e a valva mitral, ao ventr&#237;culo direito. O ventr&#237;culo direito se posicionava &#224; direita e superiormente, era hipertr&#243;fico e com cavidade de tamanho normal. O ventr&#237;culo esquerdo estava em posi&#231;&#227;o inferior e &#224; esquerda e era dilatado. A hipertens&#227;o pulmonar era acentuada, com press&#227;o m&#233;dia calculada de 68 mmHg. </p>
    <p content-type="asset">
      <fig id="a10fig02">
        <graphic xlink:href="1678-4170-abc-88-06-0693-00010-gf02.jpg"/></fig>
    </p>
    <p>
      <bold>Conduta</bold>
    </p>
    <p>Em face da insufici&#234;ncia card&#237;aca e da dificuldade em ganhar peso em cardiopatia de dif&#237;cil corre&#231;&#227;o cir&#250;rgica, foi realizada bandagem pulmonar, com boa evolu&#231;&#227;o. Houve controle da insufici&#234;ncia card&#237;aca, ap&#243;s alguns meses. Na evolu&#231;&#227;o posterior, tornou-se discretamente cian&#243;tico apenas com 10 anos de idade e em presen&#231;a de sopro sist&#243;lico ++, no 2&#186; e no 1&#186; espa&#231;os intercostais esquerdos e na f&#250;rcula. O eletrocardiograma manteve, nesse per&#237;odo, as mesmas caracter&#237;sticas e o ecocardiograma demonstrou bandagem pulmonar efetiva, com 94 mmHg de gradiente de press&#227;o. Radiografia de t&#243;rax na evolu&#231;&#227;o demonstrou arco arredondado &#224; esquerda, com ponta elevada e ainda preservando discreto aumento da trama vascular pulmonar. N&#227;o se considerou, ainda, a opera&#231;&#227;o tipo Fontan diante da evolu&#231;&#227;o favor&#225;vel, mesmo 10 anos ap&#243;s a bandagem pulmonar. </p>
    <p>
      <fn id="corr1" fn-type="corresp">
        <label>
          <bold>Correspond&#234;ncia:</bold>
        </label>
        <p> Edmar Atik </p>
        <p>InCor - Av. Dr. En&#233;as Carvalho de Aguiar, 44 </p>
        <p> 05403-000 - S&#227;o Paulo, SP - Brasil </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="conatik@incor.usp.br">conatik@incor.usp.br</email>
        </p>
      </fn>
    </p>
  </body>
  <sub-article article-type="translation" id="TRen" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Clinicoradiological Session</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title xml:lang="en">Case 3/2007: a three-month-old male infant with criss-cross heart, atrioventricular discordance, and double-outlet right ventricle, without pulmonary stenosis</article-title>
      </title-group>
    </front-stub>
    <body>
      <p>
        <bold>CLINICORADIOLOGIAL SESSION</bold>
      </p>
      <p>Case 3/2007 &#150; A three-month-old male infant with criss-cross heart, atrioventricular discordance, and double-outlet right ventricle, without pulmonary stenosis </p>
      <p>
        <bold>Edmar Atik</bold>
      </p>
      <p>Instituto do Cora&#231;&#227;o do Hospital das Cl&#237;nicas - FMUSP - S&#227;o Paulo, SP - Brazil </p>
      <p>
        <xref ref-type="corresp" rid="corr1-body2">Mailing address</xref>
      </p>
      <p><bold>Key words: </bold>Heart defects, congenital; infant; double outlet right ventricle; crisscross heart. </p>
      <p>
        <bold>Clinical data</bold>
      </p>
      <p>A three-month-old white male infant with history of fatigue while breast-feeding since birth, which had gotten worse over the last 60 days; restlessness; difficulty in gaining weight (he had put on only 240 g in the previous month); and, sometimes, dry cough. On physical examination he had dyspnea + +, normal pulses, and weighed 4440g. No cyanosis was observed. The aorta was not palpable. Chest examination showed mild impulses along the left sternal border and a valve and muscular apical impulse +/++ located at the 4<sup>th </sup>left intercostal space limited by the breadth of two fingers. Heart sounds were loud and most intense in the tricuspid area. The third sound was heard in the mitral area. A harsh systolic ejection murmur+ was heard in the 3<sup>rd</sup>, and 4<sup>th </sup>left intercostal spaces, irradiating to the right sternal border. The liver was palpable 4 cm from the right costal margin and xiphoid process. </p>
      <p>The ECG showed sinus rhythm and signs of ventricular over load that could not be determined due to the predominance of S-waves from V3. Left anterior fascicular block may be present, with the QRS axis deviated superiorly and to the right at - 120&#186;. P-axis:+40&#186; , QRS-axis:-120&#186; , T-axis:+80&#186;. </p>
      <p>
        <bold>Radiographic examination</bold>
      </p>
      <p>Radiographic findings included an enlarged cardiac silhouette due to the long ventricular arch and right atrium, assuming an oval-shaped configuration (egg-on-side); a straight mid-arch; and increased pulmonary vascularity (<xref ref-type="fig" rid="en_a10fig01-body2">Figure 1</xref>). </p>
      <p content-type="asset">
        <fig id="en_a10fig01-body2">
          <graphic xlink:href="1678-4170-abc-88-06-0693-00010-gf01-en.jpg"/></fig>
      </p>
      <p>
        <bold>Diagnostic impression</bold>
      </p>
      <p>This oval-shaped configuration is consistent with TGA-type congenital heart disease with increased pulmonary blood flow. </p>
      <p>
        <bold>Differential diagnosis</bold>
      </p>
      <p>Other heart diseases associated with increased pulmonary blood flow and exhibiting this morphology, as well as right atrium and left ventricular enlargement, must be considered, such as double-outlet right ventricle, tricuspid atresia without pulmonary stenosis, mitral atresia without pulmonary stenosis, common arterial trunk, and single ventricle. </p>
      <p>
        <bold>Diagnostic confirmation</bold>
      </p>
      <p>Clinical findings point to the diagnosis of heart disease with increased pulmonary blood flow and associated ventricular septal defect, given the low-systolic murmur audible along the left sternal border. The QRS axis rightward and superior shift (suggesting left anterior fascicular block) and the undetermined ventricular overload raise doubts and add further suspicion of a more complex heart disease. The echocardiogram (<xref ref-type="fig" rid="en_a10fig02-body2">Figure 2</xref>) showed a criss-cross heart with atrio ventricular discordance and double-outlet right ventricle, arising from the right aorta and pulmonary artery to the left with double infundibulum, with no pulmonary stenosis. It was a restrictive ventricular septal defect. The inlet ventricular septal defect was not artery-related and thus allowed the tricuspid valve to relate to the left ventricle and mitral valve to the right ventricle. The right ventricle, with normal cavity size and lying in a superior and rightward position, was found to be hypertrofic. The left ventricle was dilated and lying in a inferior, leftward position. Marked pulmonary hypertension (mean PAP 68 mm Hg) was present. </p>
      <p content-type="asset">
        <fig id="en_a10fig02-body2">
          <graphic xlink:href="1678-4170-abc-88-06-0693-00010-gf02-en.jpg"/></fig>
      </p>
      <p>
        <bold>Management</bold>
      </p>
      <p>Considering the heart failure and the patient's difficulty in gaining weight, in the setting of a challenging heart condition to be treated surgically, a successful pulmonary artery banding was performed. After some months, the heart failure was controlled. Only when he was ten years old did he become mildly cyanotic, and a systolic murmur ++ was heard in the 1<sup>st </sup>and 2<sup>nd </sup>left intercostal spaces and in the suprasternal notch. During this period, the electrocardiogram findings remained unchanged, and the echocardiogram showed pulmonary banding with a 94-mm Hg pressure gradient. A follow-up chestx-rayr evealed a left ward bowing ventricular arch with an elevated cardiacapex; pulmonary vascularity remained slightly increased. As the patient was progressing favorably, even 10 years after pulmonary banding, a Fontan-type operation was not considered. </p>
      <p>
        <fn id="corr1-body2" fn-type="corresp">
          <label>
            <bold>Mailing address:</bold>
          </label>
          <p> Edmar Atik</p>
          <p> InCor - Av. Dr. En&#233;as de Carvalho Aguiar, 44</p>
          <p> 05403-000 - S&#227;o Paulo, SP - Brazil</p>
          <p> E-mail: </p>
          <p>
            <email xlink:href="conatik@incor.usp.br">conatik@incor.usp.br</email>
          </p>
        </fn>
      </p>
    </body>
  </sub-article>
</article>
