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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">CP3FyLWny4gjVfK6hWvtykc</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2016004200276</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20160130</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Clinicoradiological Session</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Case 5/2016 - Native Coarctation of the Aortic Arch, Relieved By Percutaneous Treatment in an Adult</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>Arrieta</surname>
            <given-names>Raul</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <aff id="aff1">
          <institution content-type="orgname">Cl&#237;nica privada Dr. Edmar Atik</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">Cl&#237;nica privada Dr. Edmar Atik, 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>09</month>
        <year>2016</year>
      </pub-date>
      <volume>107</volume>
      <issue>3</issue>
      <fpage>276</fpage>
      <lpage>278</lpage>
      <history>
        <date date-type="received">
          <day>14</day>
          <month>07</month>
          <year>2015</year>
        </date>
        <date date-type="rev-recd">
          <day>16</day>
          <month>07</month>
          <year>2015</year>
        </date>
        <date date-type="accepted">
          <day>08</day>
          <month>09</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>Aortic Disease</kwd>
        <kwd>Aortic Disease/complications</kwd>
        <kwd>Cardiac Hypertrophy</kwd>
        <kwd>Angioplasty</kwd>
        <kwd>Hypertension</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <p><bold>Clinical data:</bold> the patient had a good clinical course after repair of severe coarctation of the aortic isthmus with end-to-end technique, closure of the interventricular communication at 18 days of life, and relief of moderate subaortic stenosis at 3 years of age. Currently, the patient can tolerate well routine exercise, with no symptoms. At last evaluation, blood pressure in the right arm was 140/70 mmHg and the systolic pressure in the left arm and lower limbs was 90 mmHg, suggesting aortic arch obstruction. Previous evaluations have shown a pressure gradient of 15 mmHg between the upper limbs.</p>
    <p>Physical examination: good general health, normal breathing, acyanotic, unequal pulse between the right upper arm and lower extremities. Weight: 65 Kg, height: 165 cm, right upper arm arterial pressure: 140/70 mmHg, left upper arm arterial pressure: 115/80 mmHg, right lower limb arterial pressure: 105/80 mmHg, and heart rate: 82 bpm. The aorta was palpable in the supra-sternal notch, with fremitus and systolic murmur (grade 2).</p>
    <p>In the precordium, the apex beat was not palpable and no impulse was detected during systole. Heart sounds were normal, and a harsh, grade 2 systolic murmur was heard in the aortic area and left sternal border with fremitus. The liver was not palpable.</p>
    <sec>
      <title>Complementary tests</title>
      <p><bold>Electrocardiogram</bold> showed sinus rhythm, signs of complete right bundle branch block wit QRS duration of 0.14'', and block of the anterior superior division of the left bundle branch (unchanged since neonatal aortic coarctation repair and interventricular communication). P axis: +20&#186;, QRS axis: +250&#186;, T axis: +35&#186;.</p>
      <p><bold>Chest radiograph</bold> showed normal heart area and myocardial hypertrophy, and normal pulmonary vasculature. </p>
      <p><bold>Echocardiogram</bold> showed normal-sized cardiac chambers, mild myocardial hypertrophy (septum = 14 mm and posterior wall = 11 mm), pressure gradient across the aortic arch = 61 mmHg, and bicuspid aortic valve. Ascending aorta = 29 mm, LA = 32 mm, RV = 26 mm, LV = 48 mm. Septum and left ventricle wall thickness = 10 mm three years ago.</p>
      <p><bold>Tomography of thoracic aorta</bold> showed aortic arch obstruction with diameters in the ascending aorta (19 mm), in the arch after left carotid artery (14 mm), in the isthmus after the left subclavian artery at the level where aortic coarctation repair had been performed (24 mm) (<xref ref-type="fig" rid="f1">Figure 1</xref>).</p>
      <p>
        <fig id="f1">
          <label>Figure 1</label>
          <caption>
            <title>Chest tomography (A) and aortic angiography (B) clearly show the coarctation of the aortic arch after the left carotid artery, featuring coarctation of this region in progression.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-107-03-0276-60130-gf01.jpg"/></fig>
      </p>
      <p><bold>Ambulatory Blood Pressure Monitoring</bold> showed normal blood pressure in the left arm and blood pressure levels higher than 135/85 mmHg in the right arm in 85% of the time.</p>
      <p><bold>Clinical diagnosis:</bold> Progressive native coarctation of the aortic arch, and previously repaired coarctation of the aortic isthmus.</p>
      <p><bold>Clinical reasoning:</bold> the clinical course was compatible with coarctation of the aortic arch due to the pressure gradient between the upper limbs. The absence of symptoms and the physical tolerance denoted a good dynamic behavior. The absence of pressure gradient between the left upper limb and lower limbs predicted the absence of recoarctation of aortic isthmus. These facts were confirmed by echocardiographic and chest computed tomography images (<xref ref-type="fig" rid="f1">Figure 1</xref>).</p>
      <p><bold>Differential diagnosis:</bold> other diseases accompanied by different levels of aortic obstruction should be considered, such as Kawasaki disease and Takayasu's arteritis, although they are associated with inflammation and occur earlier in life.</p>
      <p><bold>Medical management:</bold> given the severity and progression of aortic arch obstruction, systolic artery hypertension and myocardial hypertrophy, surgical correction of the obstruction has been decided on. Placement of a plastic tube between ascending and descending aorta was ruled out and percutaneous treatment was performed. A CP 8Z stent (45 mm) was implanted with a dilation balloon (size 18) from the brachiocephalic artery to the beginning of the descending aorta, with inflation of the stent at the ostium of the left carotid artery (<xref ref-type="fig" rid="f2">Figure 2</xref>). Immediately after stent implantation, the pressure gradient of 20 mmHg decreased and the equalization of pressure was established (96 mmHg). On the next day, blood pressures were 110/70 mmHg in the right arm and 120/80 mmHg in the right dorsalis pedis artery. Heart murmur remained unchanged.</p>
      <p>
        <fig id="f2">
          <label>Figure 2</label>
          <caption>
            <title>Angioplasty of aortic arch with CP 8Z stent implanted from the brachiocephalic artery to the isthmus. Angiographic image of aortic obstruction after the left carotid artery (A), stent placed during angioplasty (B), stent and angiography of aortic arch (C), and inflated stent in the beginning of the aortic arch.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-107-03-0276-60130-gf02.jpg"/></fig>
      </p>
      <p><bold>Comments:</bold> the progressive aortic coarctation after surgical repair at the aortic isthmus is found in 10-20% of cases with long term follow-up. For this reason, aortic coarctation repair performed at early ages has been extended to the aortic arch, using a technique known as "extended end-to-end anastomosis" of the aorta. The diagnosis of coarctation of the aortic arch is easily established in the late stages of the disease provided that blood pressure is systematically measured in all four limbs, as a medical requirement. Narrowing of the aortic arch becomes more evident in the late phase, with no previous parameters for earlier diagnosis of the condition. Therefore, in suspected cases, the extended arch repair for coarctation to becomes the method of choice.</p>
      <p>The percutaneous treatment for aortic coarctation, particularly in the isthmus region, has been routinely performed in adult age. Treatment of aortic arch, however, is less frequently performed, despite the favorable clinical course described here and in the literature.</p>
    </sec>
  </body>
  <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 5/2016 - Coarta&#231;&#227;o Nativa na Cro&#231;a da Aorta, Aliviada por Tratamento Percut&#226;neo em Adulto</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"/>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Arrieta</surname>
            <given-names>Raul</given-names>
          </name>
          <xref ref-type="aff" rid="aff2"/>
        </contrib>
        <aff id="aff2">
          <institution content-type="original">Cl&#237;nica privada Dr. Edmar Atik, 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. 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>Doen&#231;as da Art&#233;ria Aorta</kwd>
        <kwd>Coarta&#231;&#227;o A&#243;rtica/complica&#231;&#245;es</kwd>
        <kwd>Hipertrofia Card&#237;aca</kwd>
        <kwd>Angioplastia</kwd>
        <kwd>Hipertens&#227;o</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p><bold>Dados cl&#237;nicos:</bold> O paciente apresentou boa evolu&#231;&#227;o cl&#237;nica ap&#243;s corre&#231;&#227;o de acentuada coarta&#231;&#227;o da aorta na regi&#227;o &#237;stmica, t&#233;cnica t&#233;rmino-terminal, fechamento de comunica&#231;&#227;o interventricular com 18 dias de vida, al&#233;m do al&#237;vio de estenose suba&#243;rtica moderada com 3 anos. Atualmente, tolera bem os exerc&#237;cios rotineiros, sem sintomas. Na &#250;ltima avalia&#231;&#227;o, a press&#227;o arterial em membro superior direito era = 140x70 mmHg e no superior esquerdo e membros inferiores a press&#227;o sist&#243;lica era de 90 mmHg, que sugeria obstru&#231;&#227;o na cro&#231;a da aorta. Avalia&#231;&#245;es pr&#233;vias mostravam discreto gradiente de 15 mmHg entre membros superiores.</p>
      <p>Exame f&#237;sico: bom estado geral, eupneico, acian&#243;tico, pulsos contrastantes entre o membro superior direito e as demais extremidades. Peso: 66,5 Kg, altura: 165 cm, press&#227;o arterial do membro superior direito (PAMSD): 140/80 mmHg, press&#227;o arterial do membro superior esquerdo (PAMSE) = 115/80 mmHg e press&#227;o arterial do membro inferior direito (PAMID) = 105/80 mmHg, FC: 82 bpm. A aorta era nitidamente palpada na f&#250;rcula esternal com fr&#234;mito e sopro sist&#243;licos, ++ de intensidade.</p>
      <p>No prec&#243;rdio, <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, na ausculta, detectou-se sopro sist&#243;lico moderado (++ de intensidade), rude, na &#225;rea a&#243;rtica e borda esternal esquerda, acompanhado de fr&#234;mito. O f&#237;gado n&#227;o era palpado.</p>
      <sec>
        <title>Exames Complementares</title>
        <p><bold>Eletrocardiograma</bold> mostrava ritmo sinusal, sinais de bloqueio completo do ramo direito (dura&#231;&#227;o QRS: 0,14"), bloqueio da divis&#227;o &#226;ntero-superior do ramo esquerdo (inalterados desde a corre&#231;&#227;o neonatal da coarta&#231;&#227;o da aorta e da comunica&#231;&#227;o interventricular). AP: +20&#186;, AQRS: +250&#186;, AT: +35&#186;.</p>
        <p><bold>Radiografia de t&#243;rax</bold> mostra &#225;rea card&#237;aca normal e hipertrofia mioc&#225;rdica, e trama vascular pulmonar normal.</p>
        <p><bold>Ecocardiograma</bold> mostrou cavidades card&#237;acas com dimens&#245;es normais, discreta hipertrofia mioc&#225;rdica (septo = 14 mm e parede posterior = 11 mm), gradiente de press&#227;o na cro&#231;a da aorta = 61 mmHg, e valva a&#243;rtica bic&#250;spide. Aorta ascendente = 29 mm, AE = 32 mm, VD = 26 mm, VE = 48 mm. H&#225; 3 anos o septo e a parede de VE mediam 10 mm.</p>
        <p><bold>Tomografia computadorizada da aorta tor&#225;cica</bold> mostrou obstru&#231;&#227;o na cro&#231;a da aorta com di&#226;metros na aorta ascendente (19 mm), na cro&#231;a ap&#243;s a art&#233;ria car&#243;tida esquerda (14 mm), na regi&#227;o do istmo ap&#243;s a art&#233;ria subcl&#225;via esquerda na altura da corre&#231;&#227;o pr&#233;via da coarta&#231;&#227;o da aorta (24 mm) (<xref ref-type="fig" rid="f3">Figura 1</xref>).</p>
        <p>
          <fig id="f3">
            <label>Figura 1</label>
            <caption>
              <title>Tomografia do t&#243;rax em A e angiografia da aorta em B mostram claramente a obstru&#231;&#227;o da cro&#231;a da aorta, ap&#243;s a art&#233;ria car&#243;tida esquerda, caracterizando a coarcta&#231;&#227;o dessa regi&#227;o, em evolu&#231;&#227;o.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-107-03-0276-60130-gf01-pt.jpg"/></fig>
        </p>
        <p><bold>Monitoriza&#231;&#227;o Ambulatorial da Press&#227;o Arterial</bold> mostrou press&#245;es normais no membro superior esquerdo, e n&#237;vel superior a 135/85 mmHg no membro superior direito em 85% do per&#237;odo.</p>
        <p><bold>Diagn&#243;stico Cl&#237;nico:</bold> Coarta&#231;&#227;o na cro&#231;a da aorta nativa e evolutiva, aliada &#224; coarta&#231;&#227;o da aorta na regi&#227;o &#237;stmica corrigida previamente.</p>
        <p><bold>Racioc&#237;nio Cl&#237;nico:</bold> a evolu&#231;&#227;o cl&#237;nica era compat&#237;vel com o diagn&#243;stico de coarta&#231;&#227;o da aorta na cro&#231;a pelo gradiente de press&#227;o entre os membros superiores. Aus&#234;ncia de sintomas e boa toler&#226;ncia f&#237;sica denotavam condi&#231;&#227;o din&#226;mica favor&#225;vel. Aus&#234;ncia de gradiente entre os membros superior esquerdo e os inferiores previa aus&#234;ncia de recoarta&#231;&#227;o da regi&#227;o &#237;stmica. Esses elementos foram confirmados pelas imagens do ecocardiograma e tomografia tor&#225;cica (<xref ref-type="fig" rid="f3">Figura 1</xref>).</p>
        <p><bold>Diagn&#243;stico diferencial:</bold> outras doen&#231;as em que se manifesta obstru&#231;&#227;o da aorta em diferentes n&#237;veis devem ser consideradas, como doen&#231;a de Kawasaki e arterite de Takayasu. No entanto, elas s&#227;o acompanhadas de quadro inflamat&#243;rio e ocorrem em fase mais precoce da vida.</p>
        <p><bold>Conduta:</bold> Em face da gravidade e progress&#227;o da obstru&#231;&#227;o na cro&#231;a da aorta, hipertens&#227;o arterial sist&#243;lica e hipertrofia mioc&#225;rdica, estabeleceu-se a necessidade da corre&#231;&#227;o cir&#250;rgica da obstru&#231;&#227;o. A coloca&#231;&#227;o de tubo pl&#225;stico entre a aorta ascendente e descendente foi descartada, e realizou-se tratamento percut&#226;neo com stent CP 8Z com 45 mm de extens&#227;o, usando-se bal&#227;o dilatador n&#250;mero 18, desde o tronco braquiocef&#225;lico at&#233; o in&#237;cio da aorta descendente, com dilata&#231;&#227;o do stent no &#243;stio da art&#233;ria car&#243;tida esquerda (<xref ref-type="fig" rid="f4">Figura 2</xref>). Houve imediato desaparecimento do gradiente de press&#227;o de 20 mmHg com equaliza&#231;&#227;o da press&#227;o em 96 mmHg. As press&#245;es medidas no dia seguinte foram 110/70 mmHg no membro superior direito e 120/80 mmHg na art&#233;ria pediosa direita. O sopro card&#237;aco permaneceu inalterado.</p>
        <p>
          <fig id="f4">
            <label>Figura 2</label>
            <caption>
              <title>Imagens da angioplastia da cro&#231;a da aorta com posicionamento do stent CP 8Z desde o tronco braquiocef&#225;lico at&#233; o istmo. Imagem angiogr&#225;fica da obstru&#231;&#227;o da aorta ap&#243;s a art&#233;ria car&#243;tida esquerda (A), o stent posicionado (B), stent com a angiografia da cro&#231;a (C), e stent mais alargado no in&#237;cio da cro&#231;a (D).</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-107-03-0276-60130-gf02-pt.jpg"/></fig>
        </p>
        <p><bold>Coment&#225;rios:</bold> A coarta&#231;&#227;o progressiva na cro&#231;a da aorta ap&#243;s corre&#231;&#227;o na regi&#227;o &#237;stmica tem sido verificada em 10 a 20% dos casos de acompanhamento em longo prazo. Em vista desse fato, a corre&#231;&#227;o da coarcta&#231;&#227;o da aorta em idade precoce tem sido estendida at&#233; a cro&#231;a da aorta em t&#233;cnica conhecida como "anastomose t&#233;rmino-terminal estendida" da aorta. O diagn&#243;stico da coarta&#231;&#227;o na cro&#231;a da aorta &#233; facilmente estabelecido em evolu&#231;&#227;o mais tardia desde que haja a preocupa&#231;&#227;o m&#233;dica de medir sistematicamente a press&#227;o arterial nos quatro membros. O estreitamento da aorta na cro&#231;a &#233; mais n&#237;tido na evolu&#231;&#227;o tardia, sem par&#226;metros pr&#233;vios para estabelecer seu diagn&#243;stico mais precocemente. Por isso, em casos suspeitos, a corre&#231;&#227;o estendida at&#233; a cro&#231;a passa a ser a de escolha.</p>
        <p>O tratamento percut&#226;neo da coarcta&#231;&#227;o da aorta tem sido feito de rotina na idade adulta especialmente na regi&#227;o &#237;stmica. O tratamento &#233; realizado com menor frequ&#234;ncia na cro&#231;a da aorta, apesar da boa evolu&#231;&#227;o observada neste caso e na literatura.</p>
      </sec>
    </body>
  </sub-article>
</article>
