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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">5Crr5JPXF5rS7NdFxvnGBgn</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2012001300017</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(12)09900417</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2012001300017</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Relato de Caso</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Gest&#227;o pr&#233;-natal e resultados da taquicardia juncional ect&#243;pica acelerado e hidropisia</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Garc&#237;a-D&#237;az</surname>
            <given-names>Lutgardo</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Coserria</surname>
            <given-names>F&#233;lix</given-names>
          </name>
          <xref ref-type="aff" rid="aff02"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Costa</surname>
            <given-names>Susana</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Anti&#241;olo</surname>
            <given-names>Guillermo</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
          <xref ref-type="aff" rid="aff03"/>
        </contrib>
      </contrib-group>
      <aff id="aff01">
        <addr-line>
          <named-content content-type="city">Sevilla</named-content>
          <named-content content-type="state"/>
        </addr-line>
        <institution content-type="orgname">Universidad de Sevilla</institution>
        <institution content-type="orgdiv1">CSIC </institution>
        <institution content-type="orgdiv2">Hospital Universitario Virgen del Roc&#237;o</institution>
        <country country="ES">Spain</country>
      </aff>
      <aff id="aff03">
        <addr-line>
          <named-content content-type="city">Sevilla</named-content>
          <named-content content-type="state"/>
        </addr-line>
        <institution content-type="orgname">Centro de Investigaci&#243;n Biom&#233;dica en Red de Enfermedades Raras</institution>
        <country country="ES">Spain</country>
      </aff>
      <aff id="aff02">
        <addr-line>
          <named-content content-type="city">Sevilla</named-content>
        </addr-line>
        <institution content-type="orgname">Hospital Universitario Virgen del Roc&#237;o</institution>
        <institution content-type="orgdiv1">Hospital Infantil </institution>
        <institution content-type="orgdiv2">Secci&#243;n de Cardiolog&#237;a Infantil</institution>
        <country country="ES">Spain</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>10</month>
        <year>2012</year>
      </pub-date>
      <volume>99</volume>
      <issue>4</issue>
      <fpage>e145</fpage>
      <lpage>e148</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>30</day>
        <month>10</month>
        <year>2012</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>RELATO DE CASO</bold>
    </p>
    <p>
      <bold>Gest&#227;o pr&#233;-natal e resultados da taquicardia juncional ect&#243;pica acelerado e hidropisia</bold>
    </p>
    <p>
      <bold>Lutgardo Garc&#237;a-D&#237;az<sup>I</sup>; F&#233;lix Coserria<sup>II</sup>; Susana Costa<sup>I</sup>; Guillermo Anti&#241;olo<sup>I,III</sup></bold>
    </p>
    <p><sup>I</sup>Unidad de Gesti&#243;n Cl&#237;nica de Gen&#233;tica, Reproducci&#243;n y Medicina Fetal. Instituto de Biomedicina de Sevilla (IBIS) - Hospital Universitario Virgen del Roc&#237;o/CSIC/Universidad de Sevilla - Sevilla - Spain </p>
    <p><sup>II</sup>Unidad de Gesti&#243;n Cl&#237;nica de Pediatr&#237;a - Secci&#243;n de Cardiolog&#237;a Infantil - Hospital Infantil - Hospital Universitario Virgen del Roc&#237;o - Sevilla - Spain </p>
    <p><sup>III</sup>Centro de Investigaci&#243;n Biom&#233;dica en Red de Enfermedades Raras (CIBERER) - Sevilla - Spain</p>
    <p>
      <xref ref-type="corresp" rid="end">Correspond&#234;ncia</xref>
    </p>
    <p><bold>Palavras-chave:</bold> Pr&#233;-Natal / utiliza&#231;&#227;o; taquicardia ect&#243;pica de jun&#231;&#227;o; frequ&#234;ncia card&#237;aca; hidropisia fetal.</p>
    <p>
      <bold>Introdu&#231;&#227;o</bold>
    </p>
    <p>Arritmias fetais s&#227;o relatadas em cerca de 1-2% de todas as gesta&#231;&#245;es e &#233; uma raz&#227;o relativamente comum para o encaminhamento para centros de medicina fetal<sup>1-3</sup>. A taquicardia fetal &#233; uma condi&#231;&#227;o grave na qual o feto est&#225; em risco de insufici&#234;ncia card&#237;aca congestiva e subsequente desenvolvimento de hidropisia<sup>1-4</sup>. Esta situa&#231;&#227;o est&#225; associada com morbimortalidade significativa<sup>1-4</sup>. Em geral, o diagn&#243;stico e a avalia&#231;&#227;o dessas arritmias podem ser feitos em forma precisa usando ultrassom 2-D de alta resolu&#231;&#227;o em modo M e estudo com Doppler da rela&#231;&#227;o entre as contra&#231;&#245;es atriais e ventriculares<sup>5</sup>. A taquicardia juncional ect&#243;pica (TJE) &#233; uma forma rara de taquiarritmia fetal que normalmente ocorre no &#226;mbito da cirurgia de cardiopatia cong&#234;nita<sup>6</sup>. Al&#233;m disso, uma variedade de cong&#234;nita da TJE n&#227;o relacionada com a cirurgia tamb&#233;m tem sido descrita. Enquanto a TJE p&#243;s-cir&#250;rgica tem uma mortalidade de at&#233; 14%, a TJE cong&#234;nita tem uma mortalidade de at&#233; 34%<sup>6</sup>. A forma cong&#234;nita da TJE tem sido raramente relatada durante o per&#237;odo pr&#233;-natal<sup>7,8</sup>. N&#243;s descrevemos um caso em que o diagn&#243;stico foi suspeitado no &#250;tero de uma paciente encaminhada ao nosso departamento por causa da presen&#231;a de ascite fetal importante. O diagn&#243;stico foi baseado na evid&#234;ncia de epis&#243;dios de taquiarritmia moderada sem rela&#231;&#227;o AV 1:1 e aus&#234;ncia intermitente da onda de contra&#231;&#227;o atrial, consistente com dissocia&#231;&#227;o atrioventricular (AV), juntamente com onda diast&#243;lica reversa do ducto venoso, indicando insufici&#234;ncia card&#237;aca leve. O diagn&#243;stico de TJE foi confirmado ap&#243;s o nascimento.</p>
    <p>
      <bold>Relato do Caso</bold>
    </p>
    <p>Uma mulher de 32 anos de idade, gr&#225;vida 1, foi encaminhada ao nosso Departamento com 23 semanas de idade gestacional por causa da presen&#231;a de ascite fetal importante. No exame de ultrassom (Voluson e8, GE), n&#227;o foram observadas anomalias no cora&#231;&#227;o nem outras anomalias estruturais, o crescimento fetal era adequado para a idade gestacional, e polihidr&#226;mnios bem como ascite foram observados, indicando hidropisia fetal. Epis&#243;dios de taquicardia ventricular sem rela&#231;&#227;o AV 1:1 e dissocia&#231;&#227;o atrioventricular (AV) foram observados (<xref ref-type="fig" rid="a17fig01">Figura 1</xref>). Al&#233;m disso, tamb&#233;m foi observada uma onda diast&#243;lica reversa do ducto venoso. O diagn&#243;stico fetal foi de dissocia&#231;&#227;o AV com TJE ou taquicardia ventricular. A TJE fetal foi suspeitada com base em epis&#243;dios de taquicardia ventricular raramente excedendo 190 bpm sem rela&#231;&#227;o AV 1:1 e dissocia&#231;&#227;o atrioventricular (AV), juntamente com onda diast&#243;lica reversa do ducto venoso e hidropisia, indicando falha card&#237;aca. Assim, o feto foi diagnosticado como tendo taquiarritmia, consistente com a TJE, e hidropisia. A gestante foi hospitalizada para uma tentativa de cardiovers&#227;o com digoxina. Depois do ECG e eletr&#243;litos maternos normais, o tratamento foi iniciado com digoxina oral 0,25 mg a cada 6 horas, para um total de 2 mg, seguido por uma dose di&#225;ria de 0,25 mg a cada 8 horas. Uma vez que a digoxina esteve dentro da faixa terap&#234;utica (0,8-2 ng/ml), a paciente recebeu alta e a terapia de monitoramento materno-fetal foi realizada em forma ambulatorial. O tratamento de controle foi baseado em digoxinemia e ECG maternos, bem como ultrassonografia fetal duas vezes por semana. O n&#237;vel de digoxina em sangue manteve-se dentro da faixa terap&#234;utica durante o tratamento, o volume de l&#237;quido amni&#243;tico retornou ao valor normal, e a ascite fetal foi reduzida e manteve-se est&#225;vel, apesar da onda diast&#243;lica reversa do ducto venoso indicar insufici&#234;ncia card&#237;aca leve. Apesar da resposta, na 28&#170; semana de gesta&#231;&#227;o, decidimos administrar inje&#231;&#245;es de betametasona para promover a matura&#231;&#227;o pulmonar, uma vez que a continua&#231;&#227;o da gravidez n&#227;o estava garantida devido ao progn&#243;stico geral desfavor&#225;vel e a dificuldade da gest&#227;o da TJE ou taquicardia ventricular. Na 34&#170; semana de gest&#227;o, uma cesariana foi realizada devido ao aparecimento de taquicardia fetal s&#250;bita e permanente, apesar da terapia com digoxina. Nasceu uma crian&#231;a de sexo masculino, pesando 2.500 g, com &#237;ndice de Apgar 10/10/10, e o neonato foi internado para estudo no Departamento de Neonatologia. Na admiss&#227;o, o ECG neonatal mostrou taquicardia regular com QRS estreito, dissocia&#231;&#227;o AV, e o diagn&#243;stico de TJE foi estabelecido (<xref ref-type="fig" rid="a17fig02">Figura 2</xref>). O tratamento com adenosina foi iniciado para descartar completamente um flutter atrial. Na medida em que o diagn&#243;stico de TJE foi confirmado, utilizamos apenas propranolol, e tamb&#233;m amiodarona associada ao propranolol, mas com pobres resultados. Atualmente, o rec&#233;m-nascido tem 9 meses de idade, apresenta um ECG com caracter&#237;stica semelhante, e est&#225; sendo tratado com propranolol e flecainida. Ele apresenta uma fun&#231;&#227;o ventricular muito boa (FE 72%) pelo ecocardiograma e um Holter com uma frequ&#234;ncia m&#233;dia de 138 bpm.</p>
    <p content-type="asset">
      <fig id="a17fig01">
        <graphic xlink:href="1678-4170-abc-99-04-e145-00017-gf01.jpg"/></fig>
    </p>
    <p content-type="asset">
      <fig id="a17fig02">
        <graphic xlink:href="1678-4170-abc-99-04-e145-00017-gf02.jpg"/></fig>
    </p>
    <p>
      <bold>Discuss&#227;o</bold>
    </p>
    <p>As arritmias card&#237;acas s&#227;o detectadas em aproximadamente 1% de todos os fetos<sup>1,2</sup>. A maioria destes dist&#250;rbios do ritmo &#233; resultado de extrass&#237;stoles e de pouco significado cl&#237;nico<sup>1,2</sup>. A incid&#234;ncia de taquicardia fetal &#233; de 1:10.000 a 1:25.000. As taquiarritmias fetais podem resultar no desenvolvimento de insufici&#234;ncia card&#237;aca congestiva e, portanto, a interven&#231;&#227;o pr&#233;-natal pode ser necess&#225;ria<sup>1,2</sup>. A taquicardia juncional ect&#243;pica &#233; geralmente vista em crian&#231;as durante o per&#237;odo p&#243;s-operat&#243;rio imediato de cirurgia card&#237;aca cong&#234;nita. A forma cong&#234;nita &#233; menos frequente e tem sido raramente relatada no per&#237;odo pr&#233;-natal<sup>7,8</sup>. Acredita-se que a TJE cong&#234;nita seja causada por um foco ect&#243;pico autom&#225;tico na jun&#231;&#227;o AV<sup>3</sup>. Em geral o diagn&#243;stico diferencial pr&#233;-natal entre TJE e taquicardia ventricular, em ecocardiograma fetal &#233; um desafio. No nosso caso, o diagn&#243;stico da TJE fetal foi suspeitada com base em epis&#243;dios de taquicardia ventricular raramente excedendo 190 bpm sem rela&#231;&#227;o AV 1:1 e dissocia&#231;&#227;o atrioventricular (AV), juntamente com onda diast&#243;lica reversa do ducto venoso e hidropisia, indicando falha card&#237;aca. </p>
    <p>O tratamento ideal de taquiarritmias fetais permanece indeterminado. A seguran&#231;a da m&#227;e &#233; de grande preocupa&#231;&#227;o na gest&#227;o de taquicardia fetal, uma vez que a administra&#231;&#227;o de drogas antiarr&#237;tmicas para o tratamento intrauterino pode causar pr&#243;-arritmia e amea&#231;ar a m&#227;e. A digoxina tem sido amplamente aceita como pilar da terapia, por causa de sua seguran&#231;a e administra&#231;&#227;o conveniente<sup>1,2,5</sup>. Flecainida, sotalol e amiodarona tamb&#233;m t&#234;m sido utilizadas no tratamento de taquiarritmias fetais<sup>5,9,10</sup>. No entanto, preocupam os efeitos secund&#225;rios mais s&#233;rios sobre a m&#227;e e o feto<sup>5</sup>. </p>
    <p>O resultado na TJE cong&#234;nita mostra que &#233; uma taquiarritmia maligna, que causa cardiomiopatia induzida por taquicardia e morte s&#250;bita<sup>6</sup>. Devido &#224; efici&#234;ncia limitada da maioria dos f&#225;rmacos antiarr&#237;tmicos na TJE, a gest&#227;o intrauterina desses casos &#233; dif&#237;cil, e dever&#225; ter como objetivo manter o d&#233;bito card&#237;aco adequado para permitir o prolongamento da gravidez at&#233; que a matura&#231;&#227;o pulmonar seja alcan&#231;ada<sup>7,8</sup>. Al&#233;m disso, a presen&#231;a de hidropisia no nosso caso sugeria um progn&#243;stico desfavor&#225;vel, apesar do fato de que, antes da terapia com digoxina, a taquiarritmia foi epis&#243;dica e s frequ&#234;ncia ventricular raramente excedeu 190 batimentos/min.</p>
    <p>No caso aqui relatado, a terapia com digoxina foi suficiente para controlar a frequ&#234;ncia card&#237;aca fetal, reduzir a ascite fetal e manter o l&#237;quido amni&#243;tico dentro da normalidade, apesar da onda diast&#243;lica reversa do ducto venoso, indicando insufici&#234;ncia card&#237;aca leve. Finalmente, &#224;s 34 semanas de gesta&#231;&#227;o o feto passou a n&#227;o responder &#224; terapia com digoxina e decidimos interromper a gravidez para evitar uma maior deteriora&#231;&#227;o card&#237;aca fetal.</p>
    <p>Em conclus&#227;o, relatamos um caso de TJE fetal complicada, com terapia fetal transplacent&#225;ria com digoxina. A gest&#227;o perinatal da arritmia fetal &#233; importante para melhorar o resultado do feto afetado. Um diagn&#243;stico pr&#233;-natal preciso &#233; crucial para a sele&#231;&#227;o dos tratamentos pr&#233;-natal e p&#243;s-natal adequados. A TJE fetal &#233; uma condi&#231;&#227;o rara e o diagn&#243;stico pr&#233;-natal continua sendo um desafio, no entanto o diagn&#243;stico diferencial de taquiarritmia fetal deve incluir esse dist&#250;rbio.</p>
    <p>
      <bold>Potencial Conflito de Interesses</bold>
    </p>
    <p>Declaro n&#227;o haver conflito de interesses pertinentes.</p>
    <p>
      <bold>Fontes de Financiamento</bold>
    </p>
    <p>O presente estudo n&#227;o teve fontes de financiamento externas.</p>
    <p>
      <bold>Vincula&#231;&#227;o Acad&#234;mica</bold>
    </p>
    <p>N&#227;o h&#225; vincula&#231;&#227;o deste estudo a programas de p&#243;s-gradua&#231;&#227;o.</p>
    <p>
      <fn id="end" fn-type="corresp">
        <label>
          <bold> Correspond&#234;ncia:</bold>
        </label>
        <p> Guillermo Anti&#241;olo </p>
        <p> UGC de Gen&#233;tica, Reproducci&#243;n y Medicina Fetal. Hospital Universitario Virgen del Rocio </p>
        <p> Avenida Manuel Siurot, s/n </p>
        <p> CEP 41013 - Sevilla, Spain </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="guillermo.antinolo.sspa@juntadeandalucia.es">guillermo.antinolo.sspa@juntadeandalucia.es</email>
        </p>
      </fn>
    </p>
    <p>Artigo recebido em 12/11/11; revisado em 12/11/11; aceito em 09/04/12.</p>
  </body>
  <back>
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  <sub-article article-type="translation" id="TRen" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Case Report</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title xml:lang="en">Prenatal management and outcome of junctional ectopic tachycardia and hydrops</article-title>
      </title-group>
    </front-stub>
    <body>
      <p>
        <bold>CASE REPORT</bold>
      </p>
      <p>
        <fn id="enda-body2">
          <label>
            <bold>Prenatal management and outcome of junctional ectopic tachycardia and hydrops</bold>
          </label>
          <p>
            <bold>Lutgardo Garc&#237;a-D&#237;az<sup>I</sup>; F&#233;lix Coserria<sup>II</sup>; Susana Costa<sup>I</sup>; Guillermo Anti&#241;olo<sup>I,III</sup></bold>
          </p>
        </fn>
      </p>
      <p><sup>I</sup>Unidad de Gesti&#243;n Cl&#237;nica de Gen&#233;tica, Reproducci&#243;n y Medicina Fetal. Instituto de Biomedicina de Sevilla (IBIS) - Hospital Universitario Virgen del Roc&#237;o/CSIC/Universidad de Sevilla - Sevilla - Spain</p>
      <p><sup>II</sup>Unidad de Gesti&#243;n Cl&#237;nica de Pediatr&#237;a - Secci&#243;n de Cardiolog&#237;a Infantil - Hospital Infantil - Hospital Universitario Virgen del Roc&#237;o - Sevilla - Spain</p>
      <p><sup>III</sup>Centro de Investigaci&#243;n Biom&#233;dica en Red de Enfermedades Raras (CIBERER) - Sevilla - Spain</p>
      <p>Mailing Address</p>
      <p><bold>Keywords: </bold>Prenatal care / utilization; tachycardia; ectopic junctional; heart rate; hydrops fetalis.</p>
      <p>
        <bold>Introduction</bold>
      </p>
      <p>Fetal dysrhythmias are reported in approximately 1-2% of all pregnancies and are a relatively common reason for referral to fetal medicine centers<sup>1-3</sup>. Fetal tachycardia is a serious condition in which the fetus is at risk of congestive heart failure and the subsequent development of hydrops<sup>1-4</sup>. This situation is associated with significant morbidity and mortality<sup>1-4</sup>. In general, the diagnosis and assessment of these dysrhythmias can be accurately made using high-resolution 2-D ultrasound using M mode and Doppler assessment of the relationship between the atrial and ventricular contractions<sup>5</sup>. Junctional ectopic tachycardia (JET) is a rare form of fetal tachyarrhythmia, which usually occurs in the setting of surgery for congenital heart disease<sup>6</sup>. In addition, a congenital variety of JET not related to surgery has also been described. While post-surgical JET has a mortality up to 14%, congenital JET has a mortality up to 34%<sup>6</sup>. The congenital form of JET has rarely been reported during the prenatal period<sup>7,8</sup>. We describe a case in which the diagnosis was suspected in utero in a patient referred to our Department because of the presence of important fetal ascitis. The diagnosis was based on the evidence of moderate tachyarrhythmia episodes without 1:1 AV relationship and intermittent absence of atrial contraction wave consistent with atrioventricular (AV) dissociation, together with ductus venosus reverse diastolic wave indicating mild cardiac failure. JET diagnosis was confirmed after birth.</p>
      <p>
        <bold>Case Report</bold>
      </p>
      <p>A 32-year-old woman, gravid 1, was referred to our Department at 23 weeks' gestational age because of the presence of important fetal ascitis. On ultrasound examination (Voluson e8, GE), no heart or other structural anomalies were noted, fetal growth was adequate for gestational age, and polyhydramnios as well as ascitis were observed, indicating fetal hydrops. Ventricular tachycardia episodes without 1:1 AV relationship and atrioventricular (AV) dissociation were observed (<xref ref-type="fig" rid="en_a17fig01-body2">figure 1</xref>). In addition, a ductus venosus reverse diastolic wave was also noted. Fetal diagnosis was AV dissociation with either JET or ventricular tachycardia. Fetal JET was suspected on the basis of ventricular tachycardia episodes rarely exceeding 190 bpm without 1:1 AV relationship and atrioventricular (AV) dissociation, together with ductus venosus reverse diastolic wave and hydrops indicating cardiac failure. Thus, the fetus was diagnosed as having tachyarrhythmia, consistent with JET, and hydrops. The pregnant woman was admitted for an attempt of cardioversion with digoxin. After normal maternal ECG and electrolytes, treatment was started with oral digoxin 0.25 mg at 6 hourly intervals for a total of 2 mg, followed by a daily dose 0.25 mg at 8 hourly intervals. Once digoxin was within the therapeutic range (0.8-2 ng/ml), the patient was discharged and fetomaternal therapy monitoring was performed in an outpatient basis. Treatment control was based in maternal digoxinemia and ECG, as well as fetal ultrasonography twice a week. Blood digoxin level kept at therapeutic range during treatment, amniotic fluid volume returned to normal range, and fetal ascitis get reduced and remained stable, despite ductus venosus reverse diastolic wave indicating mild cardiac failure. Despite that response, at 28 weeks gestation we decided to give betametasone injections to promote lung maturation as prolongation of pregnancy was not guaranteed due to the general unfavorable prognosis and difficult management of JET or VT. At 34 weeks'gestational age, a cesarean section was performed due to the appearance of sudden and permanent fetal tachycardia despite digoxin therapy. A male weighing 2500 g with Apgar score 10/10/10 was born and the newborn was admitted for study at the Neonatology Department. At admission, neonatal ECG showed a regular narrow QRS tachycardia with AV dissociation, and the diagnose of JET was established (<xref ref-type="fig" rid="en_a17fig02-body2">Figure 2</xref>). Treatment with adenosine was started to completely rule out an atrial flutter. As far as JET diagnosis was confirmed, we used propranolol only, and amiodarone associated to propranolol as well, but with poor outcome. Currently, the newborn is 9 month of age, presents a similar characteristic EKG and he is being treated with propranolol and flecainide. He shows a very good ventricular function (EF 72%) by echocardiography and an EKG holter recording with an average frequency 138 bpm.</p>
      <p content-type="asset">
        <fig id="en_a17fig01-body2">
          <graphic xlink:href="1678-4170-abc-99-04-e145-00017-gf01-en.jpg"/></fig>
      </p>
      <p content-type="asset">
        <fig id="en_a17fig02-body2">
          <graphic xlink:href="1678-4170-abc-99-04-e145-00017-gf02-en.jpg"/></fig>
      </p>
      <p>
        <bold>Discussion</bold>
      </p>
      <p>Cardiac arrhythmias are detected in approximately 1% of all fetuses<sup>1,2</sup> . Most of these rhythm disturbances are the result of extrasystoles and are of little clinical significance<sup>1,2</sup>. The incidence of fetal tachycardia is 1:10,000 to 1:25,000. Fetal tachyarrhythmias can result in the development of congestive heart failure and therefore prenatal intervention may be necessary<sup>1,2</sup>. Junctional ectopic tachycardia is usually seen in children during the immediate postoperative period of congenital heart surgery. The congenital form is less frequent and has rarely been reported prenatally<sup>7,8</sup>. Congenital JET is believed to be caused by an automatic ectopic focus in the AV junction<sup>3</sup>. In general, prenatal differential diagnosis between JET and VT on fetal echocardiogram is a challenge. In our case, the diagnosis of JET in utero was suspected on the basis of ventricular tachycardia episodes rarely exceeding 190 beats/min without 1:1 AV relationship and atrioventricular (AV) dissociation, together with ductus venosus reverse diastolic wave and ascitis indicating cardiac failure. </p>
      <p>The optimal treatment of fetal tachyarrhythmias remains undetermined. The safety of the mother is of great concern when managing fetal tachycardia, as administration of antiarrhythmic drugs for intrauterine treatment may cause pro-arrhythmia and threaten the mother. Digoxin has been widely accepted as the mainstay of therapy because of its safety and convenient administration<sup>1,2,5</sup>. Flecainide, sotalol and amiodarone has also been used in the treatment of fetal tachyarrhythmias<sup>5,9,10</sup>. However, more serious side effects on both mother and fetus have been a concern<sup>5</sup>. </p>
      <p>The outcome in congenital JET shows that is a malignant tachyarrhythmia causing tachycardia-induced cardiomyopathy and sudden death<sup>6</sup>. Because of the limited efficiency of most antiarrhythmic medications in JET, the intrauterine management of such cases is difficult and should aim at maintaining adequate cardiac output to allow prolongation of the pregnancy until lung maturation is achieved<sup>7,8</sup>. In addition, the presence of hydrops in our case suggested an unfavorable prognosis, despite the fact that, before digoxin therapy, tachyarrhythmia was episodic and ventricular rate rarely exceeded 190 beats/min.</p>
      <p>In the case reported here, digoxin therapy was enough to control fetal heart rate, reduce fetal ascitis and keep amniotic fluid within the normal range, despite ductus venosus reverse diastolic wave indicating mild cardiac failure. Finally, at 34 weeks' gestation the fetus became non-responder to digoxin therapy and we decided to terminate pregnancy to avoid further fetal cardiac deterioration.</p>
      <p>In conclusion, we report a case of complicated fetal JET that underwent transplacental fetal therapy with digoxin. Prenatal management of fetal arrhythmia is important to improve the outcome of affected fetus. Accurate prenatal diagnosis is crucial to the selection of the appropriate prenatal and postnatal treatments. Fetal JET is a rare condition and prenatal diagnosis remains a challenge, however differential diagnosis of fetal tachyarrhythmia should include this disorder.</p>
      <p>
        <bold>Potential Conflict of Interest</bold>
      </p>
      <p>No potential conflict of interest relevant to this article was reported.</p>
      <p>
        <bold>Sources of Funding</bold>
      </p>
      <p>There were no external funding sources for this study.</p>
      <p>
        <bold>Study Association</bold>
      </p>
      <p>This study is not associated with any post-graduation program.</p>
      <p>
        <bold>References</bold>
      </p>
    </body>
  </sub-article>
</article>
