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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">6LtwHmVwMjpQPkYR3MnfbNQ</article-id>
      <article-id pub-id-type="other">00014</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2008001600014</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(08)09100414</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2008001600014</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 5/2008: lactente de oito meses, do sexo masculino, com s&#237;ndrome de down, comunica&#231;&#227;o interventricular e hipertens&#227;o arterial 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">Hospital S&#237;rio-Liban&#234;s de S&#227;o Paulo</institution>
        <country country="BR">Brasil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>10</month>
        <year>2008</year>
      </pub-date>
      <volume>91</volume>
      <issue>4</issue>
      <fpage>289</fpage>
      <lpage>290</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>11</day>
        <month>11</month>
        <year>2008</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>CORRELA&#199;&#195;O CL&#205;NICO-RADIOGR&#193;FICA</bold>
    </p>
    <p>
      <bold>Caso 5/2008 - Lactente de oito meses, do sexo masculino, com s&#237;ndrome de down, comunica&#231;&#227;o interventricular e hipertens&#227;o arterial pulmonar </bold>
    </p>
    <p>
      <bold>Edmar Atik</bold>
    </p>
    <p>Hospital S&#237;rio-Liban&#234;s de S&#227;o Paulo, 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, defeitos do septo interatrial, hipertens&#227;o pulmonar.Dados cl&#237;nicos</p>
    <p>
      <bold>Dados cl&#237;nicos</bold>
    </p>
    <p>Cansa&#231;o acentuado era notado desde o nascimento, sem modifica&#231;&#245;es significantes ao longo do tempo, al&#233;m de baixo ganho ponderal. O uso de medica&#231;&#227;o, representada por digoxina, furosemida, captopril e cloreto de pot&#225;ssio, n&#227;o modificou essa evolu&#231;&#227;o cl&#237;nica.</p>
    <p>
      <bold>Exame f&#237;sico</bold>
    </p>
    <p>Dispn&#233;ico +++, acian&#243;tico, corado e pulsos normalmente palpados nos quatro membros. Peso: 5.330 g. PA: 90/60 mmHg. FC: 126 bpm. A aorta n&#227;o era palpada na f&#250;rcula.</p>
    <p>No prec&#243;rdio havia abaulamento e impuls&#245;es discretas e o <italic>ictus cordis</italic> n&#227;o foi palpado. As bulhas card&#237;acas eram hiperfon&#233;ticas, sendo a segunda bulha at&#233; clangorosa na &#225;rea pulmonar. N&#227;o havia sido auscultado sopro card&#237;aco, em v&#225;rias consultas. Por vezes auscultava-se sopro sist&#243;lico, + de intensidade, limitado aos 3&#186; e 4&#186; espa&#231;os intercostais esquerdos.</p>
    <p>Os pulm&#245;es eram limpos e o f&#237;gado foi palpado a 4 cm do rebordo costal direito.</p>
    <p>O eletrocardiograma (<xref ref-type="fig" rid="f1">fig.1</xref>) mostrava ritmo sinusal e sinais de sobrecarga das cavidades ventriculares, com complexo RS de V2 a V5 e Rs em V1. &#202;QRS:+180&#186;, &#202;P: +30&#186; e &#202;T:+50&#186;.</p>
    <p content-type="asset">
      <fig id="f1">
        <graphic xlink:href="1678-4170-abc-91-04-0289-00014-gf01.jpg"/></fig>
    </p>
    <p>
      <bold>Imagem radiogr&#225;fica</bold>
    </p>
    <p>Mostra &#225;rea card&#237;aca e trama vascular pulmonar aumentadas, de grau moderado. Salientam-se os contornos atriais e ventriculares, estando retificado o arco m&#233;dio (<xref ref-type="fig" rid="fig2">fig. 2</xref>).</p>
    <fig id="fig2">
      <graphic xlink:href="1678-4170-abc-91-04-0289-00014-gf02.jpg"/></fig>
    <p>
      <bold>Impress&#227;o diagn&#243;stica</bold>
    </p>
    <p>Essa imagem &#233; compat&#237;vel com o diagn&#243;stico de cardiopatia cong&#234;nita acianog&#234;nica com hiperfluxo pulmonar tipo comunica&#231;&#227;o interventricular.</p>
    <p>
      <bold>Diagn&#243;stico diferencial</bold>
    </p>
    <p>Outras cardiopatias tipo defeitos do septo atrioventricular podem ser invocadas, assim como f&#237;stulas arteriovenosas.</p>
    <p>
      <bold>Confirma&#231;&#227;o diagn&#243;stica</bold>
    </p>
    <p>Os elementos cl&#237;nicos foram decisivos para o diagn&#243;stico de comunica&#231;&#227;o interventricular com hipertens&#227;o pulmonar. O ecocardiograma (<xref ref-type="fig" rid="f1">fig. 1</xref>) confirmou a exist&#234;ncia desse defeito, em regi&#227;o perimembranosa, com 7 mm de di&#226;metro. Havia gradiente entre os dois ventr&#237;culos de 20 mmHg e a press&#227;o sist&#243;lica de ventr&#237;culo direito foi estimada em 70 mmHg, com <italic>shunt</italic> detectado da esquerda para a direita.</p>
    <p>
      <bold>Conduta</bold>
    </p>
    <p>&#195; opera&#231;&#227;o, comunica&#231;&#227;o interventricular de 8 mm de di&#226;metro foi fechada atrav&#233;s a valva tric&#250;spide. O ventr&#237;culo direito e o tronco pulmonar eram muito aumentados e a rela&#231;&#227;o de press&#227;o interventricular resultou em 0,5, ap&#243;s a corre&#231;&#227;o do defeito. A evolu&#231;&#227;o p&#243;s-operat&#243;ria foi favor&#225;vel e recebeu alta em boas condi&#231;&#245;es com medica&#231;&#227;o anticongestiva usual (furosemida e captopril).</p>
    <p>
      <bold>Considera&#231;&#245;es</bold>
    </p>
    <p>Nesse caso houve grande dificuldade e certo temor por ocasi&#227;o da indica&#231;&#227;o cir&#250;rgica em vista da aus&#234;ncia de sopro card&#237;aco. No entanto, o conjunto dos outros elementos foi decisivo para a conduta adotada, especialmente pela presen&#231;a de sinais de insufici&#234;ncia card&#237;aca (dispn&#233;ia, hepatomegalia, cardiomegalia), ademais da sobrecarga biventricular no ECG, do <italic>shunt</italic> da esquerda para a direita mostrado no ecocardiograma, mesmo na aus&#234;ncia de dados hemodin&#226;micos.</p>
    <p>Salienta-se ainda que a oportunidade da opera&#231;&#227;o desse paciente foi impr&#243;pria, dado que se sujeitou a riscos evolutivos em face da grande repercuss&#227;o do defeito desde o nascimento at&#233; oito meses de idade, ocasi&#227;o da corre&#231;&#227;o cir&#250;rgica. Por isso, j&#225; mostrava at&#233; sinais de hipertens&#227;o pulmonar (2<underline>&#170;</underline> bulha clangorosa e aus&#234;ncia de sopro card&#237;aco).</p>
    <p>
      <fn id="back" fn-type="corresp">
        <label>
          <bold>Correspond&#234;ncia:</bold>
        </label>
        <p> Edmar Atik </p>
        <p> InCor </p>
        <p> 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>Clinicalradiological Session</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title xml:lang="en">Case 5/2008: eight-month-old male infant, with down's syndrome, interventricular communication and pulmonary artery hypertension</article-title>
      </title-group>
    </front-stub>
    <body>
      <p>
        <bold>CLINICORADIOLOGICAL SESSION</bold>
      </p>
      <p>
        <bold>Case 5/2008 - Eight-month-old male infant, with down's syndrome, interventricular communication and pulmonary artery hypertension</bold>
      </p>
      <p>
        <bold>Edmar Atik</bold>
      </p>
      <p>Hospital S&#237;rio-Liban&#234;s de S&#227;o Paulo, S&#227;o Paulo, SP - Brazil</p>
      <p>
        <xref ref-type="corresp" rid="back-body2">Mailling address</xref>
      </p>
      <p><bold>Key Words:</bold> Heart defects, congenital; heart septal defects, atrial; hypertension, pulmonary.</p>
      <p>
        <bold>Clinical data</bold>
      </p>
      <p>Accentuated fatigue was noticed from birth, with no significant alterations throughout time, in addition to low weight gain. Medication use, represented by digoxin, furosemide, captopril and potassium chloride did not alter the clinical evolution.</p>
      <p>At physical examination, the patient was dyspneic +++, acyanotic, had normal skin color and pulses were normally palpated in the lower and upper limbs. Weight was 5,330 g, BP = 90/60 mmHg, HR=126 bpm. The aorta was not palpated at the suprasternal notch.</p>
      <p>The precordium showed concavity and slight impulsions and the <italic>ictus cordis</italic> was not palpated. The heart sounds were hyperphonetic, with the second sound being high-pitched in the pulmonary area. No heart murmur had been ausculted at several examinations. Sometimes, a systolic murmur was heard, with a +intensity, limited to the 3rd and 4th left intercostal spaces.</p>
      <p>The lungs were clean and the liver was palpated at 4 cm from the right costal border.</p>
      <p>The electrocardiogram (<xref ref-type="fig" rid="en_14f1-body2">Figure 1</xref>) showed a sinus rhythm and signs of biventricular overload, with an RS complex in V2 to V5 and Rs in V1. &#202;QRS:+180&#186;, &#202;P: +30&#186; and &#202;T:+50&#186;.</p>
      <p content-type="asset">
        <fig id="en_14f1-body2">
          <graphic xlink:href="1678-4170-abc-91-04-0289-00014-gf01-en.jpg"/></fig>
      </p>
      <p>
        <bold>Radiographic imaging</bold>
      </p>
      <p>The radiographic imaging showed moderately increased cardiac area and pulmonary vascular network. The atrial and ventricular contours were noticeable, with a rectified middle arch (<xref ref-type="fig" rid="fig2-body2">Figure 2</xref>).</p>
      <fig id="fig2-body2">
        <graphic xlink:href="1678-4170-abc-91-04-0289-00014-gf02-en.jpg"/></fig>
      <p>
        <bold>Diagnostic impression</bold>
      </p>
      <p>This image is compatible with a diagnosis of acyanogenic congenital cardiopathy with interventricular-communication type pulmonary hyperflow.</p>
      <p>
        <bold>Differential diagnosis</bold>
      </p>
      <p>Other cardiopathies with atrioventricular septum defect can be considered, as well as arteriovenous fistulae.</p>
      <p>
        <bold>Diagnostic confirmation</bold>
      </p>
      <p>The clinical elements were decisive to attain the diagnosis of interventricular communication with pulmonary hypertension. The echocardiogram (<xref ref-type="fig" rid="en_14f1-body2">Figure 1</xref>) confirmed the existence of a 7-mm diameter defect in a perimembranous region. There was a gradient between the two ventricles of 20 mmHg and the right ventricle systolic pressure was estimated at 70 mmHg, with a detected left-to-right shunt.</p>
      <p>
        <bold>Procedure</bold>
      </p>
      <p>At surgery, an 8-mm interventricular communication was closed through the tricuspid valve. The right ventricle and the pulmonary trunk were greatly augmented and the interventricular pressure ratio was 0.5 after the correction of the defect. The postoperative evolution was favorable and the patient was discharged in good clinical condition under the usual anticongestive medication (furosemide and captopril).</p>
      <p>
        <bold>Considerations</bold>
      </p>
      <p>This case presented great difficulty and raised some concerns at the surgical indication considering the absence of heart murmur. However, the set of all the other elements was decisive to define the adopted procedure, especially considering the presence of signs of heart failure (dyspnea, hepatomegaly, cardiomegaly), in addition to the biventricular overload at the ECG, the left-to-right shunt demonstrated at the echocardiogram, even in the absence of hemodynamic data.</p>
      <p>It is worth mentioning that the timing for the operation in this patient was inadequate, as he was exposed to evolution risks, considering the severe consequences of the defect from birth to eight months of age, when the surgical correction was carried out.</p>
      <p>As a result of that, the patient already showed signs of pulmonary hypertension (high-pitched second heart sound and absence of heart murmur).</p>
      <p>
        <fn id="back-body2" fn-type="corresp">
          <p>Mailling address:</p>
          <p> Edmar Atik</p>
          <p> InCor - Av. Dr. En&#233;as Carvalho de 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>
