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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">ZzWGdGqvYsWCwnN7ChRwVRG</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2013000300017</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(13)10000300017</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20130071</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 2/2013: mulher de 23 anos com ventr&#237;culo direito dilatado, sem defeitos residuais, ap&#243;s corre&#231;&#227;o de tetralogia de fallot</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">Cl&#237;nica privada do Dr. Edmar Atik</institution>
        <country country="BR">Brasil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>03</month>
        <year>2013</year>
      </pub-date>
      <volume>100</volume>
      <issue>3</issue>
      <fpage>e30</fpage>
      <lpage>e31</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>15</day>
        <month>04</month>
        <year>2013</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>CORRELA&#199;&#195;O CL&#205;NICO-RADIOGR&#193;FICA</bold>
    </p>
    <p>
      <bold>Caso 2/2013 - mulher de 23 anos com ventr&#237;culo direito dilatado, sem defeitos residuais, ap&#243;s corre&#231;&#227;o de tetralogia de fallot</bold>
    </p>
    <p>
      <bold>Edmar Atik</bold>
    </p>
    <p>Cl&#237;nica privada do Dr. Edmar Atik, S&#227;o Paulo, SP - Brasil</p>
    <p>
      <xref ref-type="corresp" rid="end">Correspond&#234;ncia</xref>
    </p>
    <p><bold>Palavras-chave:</bold> Tetralogia de Fallot, ventr&#237;culo direito dilatado.</p>
    <p><bold>Dados cl&#237;nicos:</bold> Ap&#243;s corre&#231;&#227;o de tetralogia de Fallot de boa anatomia com ressec&#231;&#227;o da estenose pulmonar infundibular, com miectomia e amplia&#231;&#227;o da via de sa&#237;da do ventr&#237;culo direito com remendo de peric&#225;rdio, al&#233;m de valvotomia pulmonar sem abertura do anel pulmonar e fechamento da comunica&#231;&#227;o interventricular perimembranosa, com 12 meses de idade, verificou-se boa evolu&#231;&#227;o cl&#237;nica posterior. Atualmente tolera bem os exerc&#237;cios f&#237;sicos, mas desde os 15 anos de idade refere palpita&#231;&#245;es precordiais n&#227;o relacionadas a esfor&#231;os e taquicardias espor&#225;dicas, com frequ&#234;ncia card&#237;aca vari&#225;vel de 110-170 bpm. Eletrocardiograma din&#226;mico de 24 horas nesse per&#237;odo demonstrava extrass&#237;stoles ventriculares polim&#243;rficas de 33-1.623 epis&#243;dios, refrat&#225;rias &#224; medica&#231;&#227;o digit&#225;lica e betabloqueadora adren&#233;rgica. Manteve, desde a opera&#231;&#227;o, gradiente de at&#233; 17 mmHg na via de sa&#237;da do ventr&#237;culo direito, correlacionado com ausculta de sopro sist&#243;lico, discreto e persistente, na borda esternal esquerda alta, sem nenhum outro defeito card&#237;aco residual.</p>
    <p><bold>Exame f&#237;sico:</bold> Bom estado geral, eupneica, acian&#243;tica, pulsos normais. Peso: 46,5 kg. Altura: 157 cm. PA: 105/70 mmHg. FC: 54 bpm. A aorta n&#227;o era palpada na f&#250;rcula.</p>
    <p>No prec&#243;rdio, o <italic>ictus cordis</italic> n&#227;o era palpado e n&#227;o havia impuls&#245;es sist&#243;licas. As bulhas card&#237;acas eram normofon&#233;ticas e auscultava-se sopro sist&#243;lico discreto, de intensidade +, rude, na &#225;rea pulmonar. F&#237;gado n&#227;o palpado e pulm&#245;es limpos.</p>
    <p>
      <bold>Exames complementares</bold>
    </p>
    <p><bold>Eletrocardiograma</bold> mostrava ritmo sinusal e sinais de bloqueio completo do ramo direito, inalterados desde a corre&#231;&#227;o operat&#243;ria. AP: +10<sup>o</sup>. AQRS: +160<sup>o</sup>. AT: +60<sup>o</sup>. A dura&#231;&#227;o do QRS era de 0,14&#8221;, PR = 0,14&#8221; e QTc = 0,40&#8221;.</p>
    <p><bold>Radiografia de t&#243;rax</bold> mostrou &#225;rea card&#237;aca discretamente aumentada &#224; custa das cavidades direitas e trama vascular pulmonar normal. Manteve, desde a corre&#231;&#227;o cir&#250;rgica, aumento similar e persistente da &#225;rea card&#237;aca, com &#237;ndice cardiotor&#225;cico de cerca de 0,55, como se demonstra na sequ&#234;ncia radiogr&#225;fica, 2, 4 e 21 anos ap&#243;s a opera&#231;&#227;o (<xref ref-type="fig" rid="a17fig01">Figura 1</xref>).</p>
    <p content-type="asset">
      <fig id="a17fig01">
        <graphic xlink:href="1678-4170-abc-100-03-0e30-30071-gf01.jpg"/></fig>
    </p>
    <p><bold>Ecocardiograma </bold>mostrou cavidades card&#237;acas direitas dilatadas com AD = 44, VD = 50, AE = 35, VE = 41, Ao = 30 mm, fra&#231;&#227;o de eje&#231;&#227;o de VD = 63% (m&#233;todo Sympson), fra&#231;&#227;o de eje&#231;&#227;o de VE = 66%, gradiente de press&#227;o VD-TP = 15 mmHg e insufici&#234;ncia tric&#250;spide discreta. Com 15 anos, a rela&#231;&#227;o entre VD = 28 e VE = 40 era de 0,7, similar &#224; encontrada desde a opera&#231;&#227;o, mas h&#225; dois anos passou a ser de 1,2. </p>
    <p><bold>Resson&#226;ncia nuclear magn&#233;tica</bold> (<xref ref-type="fig" rid="a17fig02">Figura 2</xref>) salientou tamb&#233;m aumento das cavidades direitas com preserva&#231;&#227;o da fun&#231;&#227;o ventricular. Volume diast&#243;lico final de VD = 98 ml/m<sup>2</sup>, VE = 89 ml/m<sup>2</sup>, FEVD = 43% e FEVE = 72%.</p>
    <p content-type="asset">
      <fig id="a17fig02">
        <graphic xlink:href="1678-4170-abc-100-03-0e30-30071-gf02.jpg"/></fig>
    </p>
    <p><bold>Diagn&#243;stico cl&#237;nico: </bold>Tetralogia de Fallot de boa anatomia, mostrando sinais de dilata&#231;&#227;o do ventr&#237;culo direito persistente em evolu&#231;&#227;o tardia ap&#243;s a corre&#231;&#227;o operat&#243;ria, mesmo sem defeitos residuais significativos.</p>
    <p><bold>Racioc&#237;nio cl&#237;nico: </bold>Os elementos cl&#237;nicos evolutivos eram compat&#237;veis com o diagn&#243;stico de estenose pulmonar valvar residual discreta. Aus&#234;ncia de sintomas, boa toler&#226;ncia f&#237;sica e sem sinais de insufici&#234;ncia card&#237;aca s&#227;o compat&#237;veis ainda com a situa&#231;&#227;o din&#226;mica favor&#225;vel. A dilata&#231;&#227;o do ventr&#237;culo direito, observada desde a opera&#231;&#227;o, com acentua&#231;&#227;o h&#225; dois anos, n&#227;o se associa a aspecto anat&#244;mico patog&#234;nico residual. Esse elemento tem sido o respons&#225;vel pelas arritmias. </p>
    <p><bold>Diagn&#243;stico diferencial:</bold> A dilata&#231;&#227;o do ventr&#237;culo direito persistente, ap&#243;s a corre&#231;&#227;o operat&#243;ria de tetralogia de Fallot, se relaciona habitualmente &#224; insufici&#234;ncia valvar pulmonar decorrente da dilata&#231;&#227;o do anel pulmonar. Pode decorrer tamb&#233;m da valvotomia pulmonar, mesmo sem amplia&#231;&#227;o pr&#233;via do anel pulmonar. Nenhuma dessas situa&#231;&#245;es ocorreu nesse caso.</p>
    <p><bold>Conduta:</bold> Em face da boa evolu&#231;&#227;o e preserva&#231;&#227;o da fun&#231;&#227;o ventricular, adotou-se conduta cl&#237;nica expectante, com orienta&#231;&#227;o para atividade f&#237;sica recreativa e medica&#231;&#227;o vasodilatadora. </p>
    <p><bold>Coment&#225;rios: </bold>Pacientes com tetralogia de Fallot de boa anatomia, nos quais a amplia&#231;&#227;o do anel pulmonar se torna desnecess&#225;ria por ocasi&#227;o da corre&#231;&#227;o operat&#243;ria, apresentam-se com boa evolu&#231;&#227;o, dada a aus&#234;ncia de defeitos residuais que possam interferir desfavoravelmente. Dessa maneira, a evolu&#231;&#227;o p&#243;s-operat&#243;ria se assemelha &#224; de pessoas normais. No entanto, nota-se por vezes que, mesmo sem amplia&#231;&#227;o pr&#233;via do anel pulmonar, pode ocorrer dilata&#231;&#227;o posterior do ventr&#237;culo direito como um todo, tornando-se obrigat&#243;rio o questionamento de que a simples dilata&#231;&#227;o da via de sa&#237;da do ventr&#237;culo direito possa ser a causa do problema evolutivo, consequ&#234;ncia prov&#225;vel da deformidade da estrutura de todo o ventr&#237;culo direito. </p>
    <p>Na literatura, menciona-se que os fatores determinantes de maior dilata&#231;&#227;o ventricular direita, ap&#243;s a opera&#231;&#227;o corretiva da tetralogia de Fallot, se relacionam a um per&#237;odo mais prolongado desde o reparo operat&#243;rio, &#224; maior dura&#231;&#227;o do QRS e regurgita&#231;&#227;o valvar pulmonar. A piora da fun&#231;&#227;o ventricular, por seu lado, se relaciona &#224; movimenta&#231;&#227;o anormal da via de sa&#237;da ventricular, ao per&#237;odo longo ap&#243;s a opera&#231;&#227;o e tamb&#233;m &#224; maior dura&#231;&#227;o do QRS. No caso em discuss&#227;o notamos como causa persistente da dilata&#231;&#227;o ventricular direita a amplia&#231;&#227;o pr&#233;via da via de sa&#237;da do ventr&#237;culo que pode, ao longo do tempo, ter alterado a estrutura dessa cavidade. Esse elemento, assim, passa a constituir outra preocupa&#231;&#227;o cir&#250;rgica por ocasi&#227;o da corre&#231;&#227;o da tetralogia de Fallot, mesmo entre aqueles com boa anatomia.</p>
    <p>
      <fn id="end" fn-type="corresp">
        <p>Correspond&#234;ncia:</p>
        <p>Edmar Atik </p>
        <p> Rua Dona Adma Jafet, 74 cj 73 </p>
        <p> CEP 01308-050, S&#227;o Paulo, SP - Brasi </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 2/2013: 23-year-old woman with dilated right ventricle, with no residual defects after repair of tetralogy of fallot</article-title>
      </title-group>
    </front-stub>
    <body>
      <p>
        <bold>CLINICORADIOLOGICAL SESSION</bold>
      </p>
      <p>
        <bold>Case 2/2013 - 23-year-old woman with dilated right ventricle, with no residual defects after repair of tetralogy of fallot</bold>
      </p>
      <p>
        <bold>Edmar Atik</bold>
      </p>
      <p>Cl&#237;nica privada do Dr. Edmar Atik, S&#227;o Paulo, SP - Brasil</p>
      <p>
        <xref ref-type="corresp" rid="end-body2">Mailing Address</xref>
      </p>
      <p><bold>Keywords:</bold> Tetralogy of Fallot, dilated right ventricle.</p>
      <p><bold>Clinical data:</bold> After repair of tetralogy of Fallot with good anatomical features through resection of infundibular pulmonary stenosis, with a myectomy and expansion of the right ventricular outflow tract with pericardial patch, and pulmonary valvotomy without annulus opening and closing of perimembranous ventricular septal defect at 12 months of age, there was a good subsequent clinical outcome. The patient currently tolerates physical exercise, but since the age of 15 reports precordial palpitations and sporadic tachycardia unrelated to exertion, with variable heart rate of 110-170 bpm. A 24-hour ambulatory electrocardiogram during this period showed polymorphic ventricular extrasystoles of 33-1623 episodes, refractory to adrenergic beta-blockers and digitalis medication. The patient has maintained, since the surgery, a gradient of up to 17 mmHg in the right ventricular outflow tract, correlated with slight and persistent systolic murmur auscultation in the left upper sternal border, without any other residual heart defect.</p>
      <p><bold>Physical</bold><bold>Examination:</bold> Good general status, eupneic, acyanotic, normal pulses. Weight: 46.5 kg. Height: 157 cm. BP: 105/70 mmHg. HR: 54 bpm. The aorta was not palpable in the suprasternal notch. The <italic>ictus cordis</italic> was not palpable in the precordium and there were no systolic impulses. Heart sounds were normal and slight systolic murmur was present at auscultation, of + intensity, in the pulmonary area. The liver was not palpable and lungs were clear.</p>
      <p>
        <bold>Complementary Examinations</bold>
      </p>
      <p><bold>Electrocardiogram</bold> showed sinus rhythm and signs of right bundle branch block, unchanged since the surgical repair. AP: +10&#186;. AQRS: +160&#186;. AT: +60&#186;. QRS duration was 0.14", PR = 0.14" and QTc = 0.40".</p>
      <p><bold>Chest X-ray </bold>showed slightly enlarged cardiac silhouette at the expense of the right chambers and normal pulmonary vascular network. The patient has maintained, since the surgical repair, similar and persistent enlargement in the cardiac area, with a cardiothoracic index of approximately 0.55, as shown in the sequence of radiographic images taken 2, 4 and 21 years after the surgery (<xref ref-type="fig" rid="en_a17fig01-body2">Figure 1)</xref>.</p>
      <p content-type="asset">
        <fig id="en_a17fig01-body2">
          <graphic xlink:href="1678-4170-abc-100-03-0e30-30071-gf01-en.jpg"/></fig>
      </p>
      <p><bold>Echocardiogram </bold>showed dilated right heart chambers with RA = 44, RV = 50, LA = 35, LV = 41, Ao = 30 mm, RV ejection fraction = 63% (Simpson's method), LV ejection fraction = 66%, RV/PT pressure gradient = 15 mmHg and mild tricuspid regurgitation. At 15 years, the ratio between RV = 28 and LV = 40 was 0.7, similar to that found since the surgery, but two years ago it increased to 1.2.</p>
      <p><bold>Magnetic Nuclear Resonance </bold>(<xref ref-type="fig" rid="en_a17fig02-body2">Figure 2</xref>) also showed increased right chambers with preserved ventricular function. RV end-diastolic volume = 98 ml/m<sup>2</sup>, LV = 89 ml/m<sup>2</sup>, RVEF = 43% and LVEF = 72%.</p>
      <p content-type="asset">
        <fig id="en_a17fig02-body2">
          <graphic xlink:href="1678-4170-abc-100-03-0e30-30071-gf02-en.jpg"/></fig>
      </p>
      <p><bold>Clinical Diagnosis:</bold> Tetralogy of Fallot with good anatomical features, showing signs of persistent right ventricular dilation at late outcome after surgical repair, even without significant residual defects. </p>
      <p><bold>Clinical Rationale:</bold> Clinical findings were compatible with the diagnosis of mild residual pulmonary stenosis. No symptoms, good tolerance to physical exertion and no signs of heart failure are also compatible with the favorable dynamic situation. The right ventricular dilation observed since the surgery, which increased two years ago, is not associated with any residual pathogenic anatomical aspect. This element has been responsible for the arrhythmias. </p>
      <p><bold>Differential Diagnosis:</bold> The persistent right ventricular dilation after surgical repair of tetralogy of Fallot is usually related to the pulmonary valve failure due to pulmonary annulus dilation. It may also derive from pulmonary valvotomy, even without prior expansion of the pulmonary annulus. None of these situations occurred in this case. </p>
      <p><bold>Conduct:</bold> Considering the good evolution and preservation of ventricular function, the expectant conduct was adopted, instructing the patient regarding recreational physical activity and prescribing vasodilator medication.</p>
      <p><bold>Comments:</bold> Patients with tetralogy of Fallot and good anatomical features, in which the expansion of the pulmonary annulus becomes unnecessary during surgical repair, have a good outcome, given the absence of residual defects that may be unfavorable. Thus, the postoperative course is similar to that of normal individuals. However, it is observed that sometimes, even without prior expansion of the pulmonary annulus, subsequent right ventricular dilation as a whole may occur, making it compulsory to question whether the simple dilation of the right ventricle outflow tract can be the cause of the evolutionary problem, probably a result of the deformity of the entire right ventricular structure.</p>
      <p>In the literature, it is mentioned that the determinants of bigger right ventricular dilation after surgical repair of tetralogy of Fallot is related to a longer period since the repair surgery, the longer QRS duration and pulmonary valve regurgitation. The worsening in ventricular function, in turn, is related to the abnormal movement in the ventricular outflow tract, during the long period after the surgery and also the longer QRS duration. In our case, we observed as the cause of persistent right ventricular dilation the prior expansion of the right ventricular outflow tract, which may have, over time, altered the structure of the cavity. Thus, this element becomes another surgical concern during the repair of the tetralogy of Fallot, even among those with good anatomical features.</p>
      <p>
        <fn id="end-body2" fn-type="corresp">
          <p>Mailing Address:</p>
          <p>Rua Dona Adma Jafet, 74 cj 73</p>
          <p> Postal Code 01308-050, S&#195;&#163;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>
