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  <front>
    <journal-meta>
      <journal-id journal-id-type="nlm-ta">Arq Bras Cardiol</journal-id>
      <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>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v3">pkjSTCnkNWLt3K5MCszr4nx</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2016000400342</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20160051</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Clinicoradiological Session</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Case 3/2016 - 36-Year-Old Man with Anomalous Origin of the Right Coronary Artery in the Left Sinus of Valsalva and Interarterial Course</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="aff1"/>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Kalil</surname>
            <given-names>Roberto</given-names>
            <suffix>Filho</suffix>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Jatene</surname>
            <given-names>Marcelo</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <aff id="aff1">
          <institution content-type="orgname">Hospital S&#237;rio Liban&#234;s de S&#227;o Paulo</institution>
          <addr-line>
            <named-content content-type="city">S&#227;o Paulo</named-content>
            <named-content content-type="state">SP</named-content>
          </addr-line>
          <country country="BR">Brazil</country>
          <institution content-type="original">Hospital S&#237;rio Liban&#234;s de S&#227;o Paulo, S&#227;o Paulo, SP - Brazil</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Edmar Atik</bold>, Office. Rua Dona Adma Jafet, 74 conj.73, Bela Vista. Postal Code 01308-050, S&#227;o Paulo, SP - Brazil. E-mail: <email>edmaratik@uol.com.br</email>
				</corresp>
      </author-notes>
      <pub-date pub-type="epub-ppub">
        <month>04</month>
        <year>2016</year>
      </pub-date>
      <volume>106</volume>
      <issue>4</issue>
      <fpage>342</fpage>
      <lpage>344</lpage>
      <history>
        <date date-type="received">
          <day>14</day>
          <month>07</month>
          <year>2015</year>
        </date>
        <date date-type="rev-recd">
          <day>24</day>
          <month>11</month>
          <year>2015</year>
        </date>
        <date date-type="accepted">
          <day>24</day>
          <month>11</month>
          <year>2015</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/4.0" xml:lang="en">
          <license-p>This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
        </license>
      </permissions>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Cardiac Surgical Procedures</kwd>
        <kwd>Coronary Vessel Anomalies / surgery</kwd>
        <kwd>Heart Defects, Congenital</kwd>
        <kwd>Sinus of Valsalva</kwd>
        <kwd>Cardiac Catheterization</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <p><bold>Clinical data:</bold> Three months ago, after physical effort, the patient had four episodes of paleness, excessive sweating and fatigue, relieved when lying down in supine position. In addition, the patient had pain of 2-hour duration in the left hemithorax at normal activity level a few days ago.</p>
    <p>Physical examination showed good general condition, not pale, anicteric, eupneic, normal pulse, and no palpable pulse in the suprasternal notch was detected. Body weight was 85 kg, height 170 cm, BP 120/70 mmHg, HR 75 bpm. No abnormalities in the precordium, normophonetic heart sounds, and no heart murmurs. No changes in the lungs or abdomen were detected.</p>
    <sec>
      <title>Complementary tests</title>
      <p><bold>Electrocardiogram</bold> showed sinus rhythm, no signs of atrial or ventricular overload and no changes in the ventricular repolarization. AP = +50&#186;, AQRS = +40&#186;, AT = +60&#186;</p>
      <p><bold>Chest radiography</bold> showed normal heart area and pulmonary vascular bed (<xref ref-type="fig" rid="f1">Figure 1</xref>).</p>
      <p>
        <fig id="f1">
          <label>Figure 1</label>
          <caption>
            <title>Chest radiograph shows normal heart area and pulmonary vascular bed. The elongated, dilated right upper arch may result from the modest increase of ascending aorta.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-106-04-0342-60051-gf01.jpg"/></fig>
      </p>
      <p><bold>Echocardiography</bold> showed no contractile or morphological changes, normal-sized cardiac chambers and normal ventricular function (71%).</p>
      <p><bold>Coronary Computed Tomography Angiography</bold> revealed anomalous origin of the right coronary artery from the left Valsalva sinus, with interarterial course between aorta and pulmonary trunk, with distinct narrowing of its proximal third and coronary ostium. The interarterial course of the right coronary artery was estimated at 10 mm (<xref ref-type="fig" rid="f2">Figure 2</xref>).</p>
      <p>
        <fig id="f2">
          <label>Figure 2</label>
          <caption>
            <title>Similar projections of coronary arteries by computed tomography angiography (A, B) and cardiac catheterization (C, D) highlight the origin of the right coronary artery in the left sinus of Valsalva, the interarterial course and sharp narrowing in its beginning. AO: aorta; RC: right coronary artery; LC: left coronary artery; RV: right ventricle; LV: left ventricle; PT: pulmonary trunk.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-106-04-0342-60051-gf02.jpg"/></fig>
      </p>
      <p><bold>Cardiac catheterization</bold> revealed the same aspect of the dominant right coronary artery, with anomalous origin from the left sinus of Valsalva and proximal course with sharp angulation and no obstructions (<xref ref-type="fig" rid="f2">Figure 2</xref>). Left anterior descending artery and circumflex artery originate from the bifurcated left coronary artery; long diagonal and septal branches; absence of collateral circulation or arterial obstruction.</p>
      <p><bold>TcMIBI stress myocardial scintigraphy</bold> did not show ischemia, but indicated pain in the left sternal border, of low intensity and minutes of duration. Images obtained after isotonic exercise and at rest showed normal perfusion of the left ventricular walls.</p>
      <p>Functional images combined with ECG (GSPECT) indicate normal motion and thickness of the left ventricular wall, and normal ejection fraction of the left ventricle (65%).</p>
      <p><bold>Clinical Diagnosis:</bold> Anomalous origin of the right coronary artery from the left Valsalva sinus, with interarterial course between the ascending aorta and pulmonary trunk, with signs and symptoms of arterial obstruction, but no evidence of myocardial ischemia.</p>
      <p><bold>Clinical reasoning:</bold> symptoms of low cardiac output combined with chest pain (even non-specific) suggest, a priori, aortic valve or coronary arterial malformation. The former was rejected due to the absence of heart murmurs. The abnormal origin of right coronary artery in the left sinus of Valsalva and its compression in the interarterial course were demonstrated by tomography.</p>
      <p><bold>Differential diagnosis:</bold> the same clinical picture may be seen in other coronary abnormalities, including the anomalous origin of the left coronary artery from the pulmonary trunk, but with large collateral circulation from the right coronary, enabling the development until adult age, in addition to the left coronary artery originating from the right sinus of Valsalva, with significant interarterial compression.</p>
      <p><bold>Management:</bold> The indication of surgery was evident in light of the clinical presentation of low cardiac output, chest pain (even non-specific), and unfavorable anatomy of the right coronary artery with interarterial compression, due to the risk of sudden death and/or ventricular arrhythmias, although no evidence of ischemia was detected by myocardial scintigraphy.</p>
      <p>During the heart surgery, dissection of the right coronary artery originating from next to the left coronary ostium in the left sinus of Valsalva with an interarterial course between the aorta and pulmonary trunk was performed. The right coronary artery exhibited non-obstructive plaques in its proximal third, where it was cut and anastomosed to the anterior wall of the aorta with continuous suture (7-0 Prolene).</p>
      <p><bold>Comments:</bold> Anomalous origin of the right or left coronary artery from the sinus of Valsalva is a rare, congenital abnormality, and most of patients are asymptomatic. However, despite this apparently favorable condition, identified during routine or symptom assessment, the indication of surgery should be considered in order to prevent sudden death. This is a literature consensus, and few authors adopt a different viewpoint due to a conservative attitude. This approach may be used in the absence of symptoms or myocardial ischemia. Right coronary artery anomalies are ten times more common than in the left coronary artery. Physical effort is the trigger of ischemic symptoms, often fatal and without notice, be it for the interarterial obstruction, be it for the coronary artery arising from the aorta at an acute angle. The necessity of surgery is based on symptoms, regardless of the negative result for ischemia. The currently most accepted surgical techniques recommend reimplantation of the coronary artery in the sinus of Valsalva. Both coronary artery bypass surgery and the internal thoracic artery bypass have not been the approaches of choice due to the possibility of future obstructions, similarly to the placement of stents, which would continue to be exposed to the action of the arteries.<xref ref-type="bibr" rid="B1">1</xref><sup>-</sup><xref ref-type="bibr" rid="B3">3</xref>
			</p>
    </sec>
  </body>
  <back>
    <ref-list>
      <title>References</title>
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        <label>1</label>
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              <surname>Bataille</surname>
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        <mixed-citation>3 Cho SH, Joo HC, Yoo KJ, Youn YN. Anomalous origin of right coronary artery from left coronary sinus: surgical management and clinical result. Thorac Cardiovasc Surg. 2015;63(5):360-6.</mixed-citation>
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            </name>
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  </back>
  <sub-article article-type="translation" id="s1" xml:lang="pt">
    <front-stub>
      <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 3/2016 - Homem de 36 anos, com Origem An&#244;mala da Art&#233;ria Coron&#225;ria Direita no Seio de Valsalva Esquerdo e Trajeto Interarterial</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="aff2">1</xref>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Filho</surname>
            <given-names>Roberto Kalil</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">1</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Jatene</surname>
            <given-names>Marcelo</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">1</xref>
        </contrib>
        <aff id="aff2">
          <institution content-type="original">Hospital S&#237;rio Liban&#234;s de S&#227;o Paulo, S&#227;o Paulo, SP - Brasil</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c2"><bold>Correspond&#234;ncia: Edmar Atik</bold>, Consult&#243;rio privado. Rua Dona Adma Jafet, 74 conj.73, Bela Vista. CEP 01308-050, S&#227;o Paulo, SP - Brasil. E-mail: <email>edmaratik@uol.com.br</email>
				</corresp>
      </author-notes>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Procedimentos Cir&#250;rgicos Card&#237;acos</kwd>
        <kwd>Anomalias dos Vasos Coron&#225;rios / cirurgia</kwd>
        <kwd>Cardiopatias Cong&#234;nitas</kwd>
        <kwd>Seio A&#243;rtico</kwd>
        <kwd>Cateterismo Card&#237;aco</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>Dados cl&#237;nicos: H&#225; 3 meses, ap&#243;s esfor&#231;o f&#237;sico, surgiram palidez, sudorese excessiva e cansa&#231;o extremo, em quatro vezes intercaladas , com melhora ap&#243;s ado&#231;&#227;o de dec&#250;bito dorsal. Ademais desses quadros, sentiu h&#225; alguns dias, dor no hemit&#243;rax esquerdo com dura&#231;&#227;o de duas horas no trabalho habitual.</p>
      <p>Exame F&#237;sico mostrou bom estado geral, eupneico, corado, pulsos normais. Peso 85 Kg, altura 170 cm, PA = 120/70 mm Hg, FC = 75 bpm. A aorta n&#227;o era palpada na f&#250;rcula. Sem deformidades no prec&#243;rdio, bulhas card&#237;acas normofon&#233;ticas, sem sopros card&#237;acos. Pulm&#245;es e Abdome sem altera&#231;&#245;es.</p>
      <sec>
        <title>Exames Complementares</title>
        <p>Eletrocardiograma mostrava ritmo sinusal, sem sobrecargas cavit&#225;rias e sem altera&#231;&#245;es da repolariza&#231;&#227;o ventricular. AP = +50&#186;, AQRS = +40&#186;, AT = +60&#186;.</p>
        <p>Radiografia de T&#243;rax mostrou &#225;rea card&#237;aca e trama vascular pulmonar normais (<xref ref-type="fig" rid="f3">Figura1</xref>).</p>
        <p>
          <fig id="f3">
            <label>Figura 1</label>
            <caption>
              <title>Radiografia de t&#243;rax mostra &#225;rea card&#237;aca e trama vascular pulmonar normais. O arco superior direito, alongado e dilatado, pode decorrer de aumento discreto da aorta ascendente.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-106-04-0342-60051-gf01-pt.jpg"/></fig>
        </p>
        <p>Ecocardiograma n&#227;o mostrou altera&#231;&#245;es contr&#225;cteis ou anormalidades morfol&#243;gicas. Cavidades de tamanho normal e fun&#231;&#227;o ventricular normal (71%).</p>
        <p>Tomografia de art&#233;rias coron&#225;rias revelou origem an&#244;mala da art&#233;ria coron&#225;ria direita a partir do seio de Valsalva esquerdo, com trajeto entre a aorta e o tronco pulmonar e n&#237;tido estreitamento no ter&#231;o inicial e no &#243;stio coron&#225;rio. Foi estimada a extens&#227;o de 10 mm da coron&#225;ria direita em trajeto interarterial (<xref ref-type="fig" rid="f4">Figura 2</xref>).</p>
        <p>
          <fig id="f4">
            <label>Figura 2</label>
            <caption>
              <title>Art&#233;rias coron&#225;rias em proje&#231;&#245;es similares pela angiotomografia (A, B) e cateterismo card&#237;aco (C, D) , salientam a origem da art&#233;ria coron&#225;ria direita do seio de Valsalva esquerdo e o trajeto interarterial desta art&#233;ria com n&#237;tido estreitamento em seu in&#237;cio. Abreviaturas: Ao: aorta, CD: art&#233;ria coron&#225;ria direita, CE: art&#233;ria coron&#225;ria esquerda, VD: ventr&#237;culo direito, VE: ventr&#237;culo esquerdo, TP: tronco pulmonar.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-106-04-0342-60051-gf02-pt.jpg"/></fig>
        </p>
        <p>Cateterismo card&#237;aco mostrou mesmo aspecto, com art&#233;ria coron&#225;ria direita dominante com origem an&#244;mala no seio de Valsalva esquerdo e trajeto proximal com angula&#231;&#227;o extrema, sem obstru&#231;&#245;es (<xref ref-type="fig" rid="f4">Figura 2</xref>). Tronco de coron&#225;ria esquerda bifurcado origina art&#233;ria descendente anterior e art&#233;ria circunflexa bem desenvolvidas; ramos diagonais e septais longos; aus&#234;ncia de circula&#231;&#227;o colateral ou obstru&#231;&#245;es arteriais.</p>
        <p>Cintilografia mioc&#225;rdica com MIBI e esfor&#231;o f&#237;sico n&#227;o revelou isquemia, mas houve refer&#234;ncia de dor na borda esternal esquerda, de fraca intensidade, e dura&#231;&#227;o de minutos. As imagens obtidas ap&#243;s exerc&#237;cio isot&#244;nico e em repouso mostraram perfus&#227;o normal das paredes do ventr&#237;culo esquerdo.</p>
        <p>As imagens funcionais adquiridas e sincronizadas com o ECG (GSPECT) evidenciam movimenta&#231;&#227;o e espessamento normais das paredes do ventr&#237;culo esquerdo. Fra&#231;&#227;o de eje&#231;&#227;o normal do ventr&#237;culo esquerdo (65%).</p>
        <p>Diagn&#243;stico cl&#237;nico: Origem an&#244;mala da art&#233;ria coron&#225;ria direita do seio de Valsalva esquerdo com trajeto interarterial entre a aorta ascendente e o tronco pulmonar, com sintomas e sinais de obstru&#231;&#227;o arterial, mas sem evid&#234;ncias de isquemia mioc&#225;rdica.</p>
        <p>Racioc&#237;nio cl&#237;nico: sintomas de baixo d&#233;bito card&#237;aco, complementados por dor tor&#225;cica, mesmo inespec&#237;fica, orientam, <italic>a priori</italic>, &#224; malforma&#231;&#227;o valvar a&#243;rtica ou arterial coron&#225;ria. A primeira foi descartada pela aus&#234;ncia de sopros card&#237;acos. A origem an&#244;mala da art&#233;ria coron&#225;ria direita no seio de Valsalva esquerdo e sua compress&#227;o no trajeto interarterial foram evidenciadas pela tomografia.</p>
        <p>Diagn&#243;stico diferencial: o mesmo quadro pode ser evolutivamente observado em outras anomalias coron&#225;rias, como a origem an&#244;mala da art&#233;ria coron&#225;ria esquerda a partir do tronco pulmonar, mas com circula&#231;&#227;o colateral abundante proveniente da coron&#225;ria direita, que permite evolu&#231;&#227;o at&#233; a idade adulta, al&#233;m da origem da art&#233;ria coron&#225;ria esquerda do seio de Valsalva direito, cuja compress&#227;o interarterial pode se mostrar mais n&#237;tida.</p>
        <p>Conduta: A indica&#231;&#227;o operat&#243;ria pareceu n&#237;tida em face da manifesta&#231;&#227;o cl&#237;nica de baixo d&#233;bito, dor tor&#225;cica, mesmo que inespec&#237;fica, e anatomia desfavor&#225;vel da art&#233;ria coron&#225;ria direita com compress&#227;o interarterial, dado o n&#237;tido risco de morte s&#250;bita e/ou de arritmias ventriculares, apesar da cintilografia mioc&#225;rdica n&#227;o ter revelado isquemia.</p>
        <p>Na opera&#231;&#227;o card&#237;aca, foi dissecada a coron&#225;ria direita que se originava pr&#243;ximo ao &#243;stio da coron&#225;ria esquerda no seio de Valsalva esquerdo e apresentava um trajeto interarterial entre a aorta e o tronco pulmonar. A coron&#225;ria direita mostrava placas n&#227;o obstrutivas no seu ter&#231;o proximal, onde foi seccionada e translocada para a parede anterior da aorta em anastomose com sutura cont&#237;nua (prolene 7.0).</p>
        <p>Coment&#225;rios: A origem an&#244;mala da art&#233;ria coron&#225;ria direita ou esquerda, a partir do seio de Valsalva inverso, constitui-se em uma anomalia rara cong&#234;nita, e a maioria dos pacientes mostra-se assintom&#225;tica. No entanto, apesar dessa condi&#231;&#227;o aparentemente favor&#225;vel, identificada em avalia&#231;&#227;o de rotina ou de sintomas, a indica&#231;&#227;o operat&#243;ria deve ser considerada a fim de prevenir a morte s&#250;bita. Esse &#233; um consenso na literatura, e poucos autores posicionam-se diferentemente por atitudes conservadoras. Esta conduta pode ser adotada na eventualidade da aus&#234;ncia de sintomas e sem isquemia mioc&#225;rdica demonstrada. A anomalia da art&#233;ria coron&#225;ria direita &#233; dez vezes mais comum que a anomalia da coron&#225;ria esquerda. O esfor&#231;o f&#237;sico &#233; o respons&#225;vel pelo desencadeamento dos sintomas isqu&#234;micos, por vezes fatais e sem aviso pr&#233;vio, quer por obstru&#231;&#227;o interarterial, como pela origem coronariana na aorta em &#226;ngulo agudo. A necessidade de cirurgia &#233; vinculada a sintomas, independente de resultado negativo de isquemia. As t&#233;cnicas operat&#243;rias mais aceitas hoje orientam ao reimplante coron&#225;rio no devido seio de Valsalva. A ponte de safena e a ponte da art&#233;ria tor&#225;cica interna t&#234;m sido preteridas , dada a poss&#237;veis obstru&#231;&#245;es futuras, assim como a coloca&#231;&#227;o de <italic>stents</italic> dilatadores que continuariam sofrendo externamente a a&#231;&#227;o das art&#233;rias.<xref ref-type="bibr" rid="B1">1</xref><sup>-</sup><xref ref-type="bibr" rid="B3">3</xref>
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