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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" article-type="editorial" dtd-version="1.1" specific-use="sps-1.8" xml:lang="en">
  <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">LkT8r3fZDVW7QqpzYzdHCnr</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2019000200136</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20190021</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Short Editorial</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Fontan-Kreutzer Conversion to Total Cavopulmonary Surgery due to Failing Univentricular Circulation. A Feasible Therapeutic Option?</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <contrib-id contrib-id-type="orcid">0000-0003-3367-5779</contrib-id>
          <name>
            <surname>Guimar&#227;es</surname>
            <given-names>Isabel Cristina Britto</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
          <xref ref-type="aff" rid="aff2">2</xref>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
      </contrib-group>
      <aff id="aff1">
        <label>1</label>
        <institution content-type="orgname">Universidade Federal da Bahia</institution>
        <institution content-type="orgdiv1">Faculdade de Medicina da Bahia</institution>
        <addr-line>
          <city>Salvador</city>
          <state>BA</state>
        </addr-line>
        <country country="BR">Brazil</country>
        <institution content-type="original">Universidade Federal da Bahia - Faculdade de Medicina da Bahia, Salvador, BA - Brazil</institution>
      </aff>
      <aff id="aff2">
        <label>2</label>
        <institution content-type="orgname">Universidade Federal da Bahia</institution>
        <institution content-type="orgdiv1">Hospital Ana Nery</institution>
        <addr-line>
          <city>Salvador</city>
          <state>BA</state>
        </addr-line>
        <country country="BR">Brazil</country>
        <institution content-type="original">Hospital Ana Nery - Universidade Federal da Bahia, Salvador, BA - Brazil</institution>
      </aff>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Isabel Cristina Britto Guimar&#227;es</bold>, Pra&#231;a Ramos de Queiros, s/n. Postal Code 40026-010, Largo do Terreiro de Jesus, Salvador, BA - Brazil. E-mail: <email>isabelcbguimaraes@gmail.com</email>
				</corresp>
      </author-notes>
      <pub-date pub-type="epub-ppub">
        <month>02</month>
        <year>2019</year>
      </pub-date>
      <volume>112</volume>
      <issue>2</issue>
      <fpage>136</fpage>
      <lpage>137</lpage>
      <permissions>
        <license license-type="open-access" xlink:href="https://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>
      <related-article ext-link-type="doi" id="r01" page="130-135" related-article-type="commentary-article" vol="112" xlink:href="10.5935/abc.20180256"/>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Heart Defects, Congenital</kwd>
        <kwd>Tricuspide Atresia/surgery</kwd>
        <kwd>Procedure Fontan-Kreutzer</kwd>
        <kwd>Heart Bypass, Right</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <p>Fontan surgery, a palliative operation for patients with &#8220;single-ventricle&#8221; heart physiology, has undergone several modifications since the first procedure was performed in 1971 for individuals with diagnosis of tricuspid atresia.<xref ref-type="bibr" rid="B1">1</xref>
		</p>
    <p>The Fontan-Kreutzer (FK) atrial-pulmonary anastomosis technique was widely used in the 1980s. Publications regarding the late follow-up of patients submitted to the technique before 1990 showed a higher frequency of complications such as heart failure, arrhythmias, thromboembolic events, protein-losing enteropathy, plastic bronchitis, sudden death and liver failure.<xref ref-type="bibr" rid="B2">2</xref><sup>,</sup><xref ref-type="bibr" rid="B3">3</xref>
		</p>
    <p>The study by Fernandes et al.,<xref ref-type="bibr" rid="B4">4</xref> published in this issue of the journal, aimed to analyze the results of the conversion of FK to Total Cavopulmonary Connection (TCPC) of patients with signs of failing univentricular circulation submitted to surgery in a single institution.</p>
    <p>Of the 420 patients submitted to Fontan surgery between 1995 and 2016, 18 underwent FK, corresponding to 4.3% of the total sample. Of the 18 FK cases, 10 required conversion to TCPC due to signs of failing Fontan circulation, all of which were included in the study analysis. In 9 cases, the main cause of conversion was the presence of uncontrolled arrhythmia and protein-losing enteropathy in 1 case.</p>
    <p>A relevant aspect regarding the presented data concerns the New York Heart Association functional class. Before the surgical conversion, 70% of the patients were in functional class II and III, and after the conversion surgery, approximately 80% of the patients showed functional capacity improvement.</p>
    <p>Although the main indication for conversion was the presence of difficult-to-control arrhythmias, about 44% of the patients continued to have arrhythmias as a clinical manifestation, showing no satisfactory results with the use of surgical cryoablation, different from studies showing favorable results after its use.<xref ref-type="bibr" rid="B5">5</xref> The obtained results also show data that demonstrate the complexity of these patients&#8217; management, such as: prolonged hospitalization time and death rate of 20%. The authors associated the mortality rate found in the study with the period during which the surgeries were performed between 1996 and 2000, and the learning curve of the service related to the described surgical technique. However, the early mortality rate described by other authors was also high, ranging from 0 to 21%.<xref ref-type="bibr" rid="B6">6</xref><sup>,</sup><xref ref-type="bibr" rid="B7">7</xref>
		</p>
    <p>Kreutzer et al.,<xref ref-type="bibr" rid="B8">8</xref> in a review article, assess the five decades of the FK technique, in which they state that late complications would be strongly associated with surgical strategies and procedures no longer used nowadays, such as prolonged use of pulmonary artery banding, classic Blalock-Taussig shunt, late interventions, late diagnosis of significant hemodynamic changes and the use of surgical techniques currently considered to be inadequate, such as the classic Fontan procedure and the original Kreutzer surgery. Kreuzter et al.<xref ref-type="bibr" rid="B8">8</xref> consider that one should be careful when analyzing literature data regarding the surgical technique prior to 1990, as well as when using these results as predictors of long-term outcome in patients submitted to FK surgery today.<xref ref-type="bibr" rid="B8">8</xref>
		</p>
    <p>Regarding the study by Fernandes et al.,<xref ref-type="bibr" rid="B4">4</xref> we do not have this information, which could help us to better understand the late complications observed in this sample.</p>
    <p>For the late survivors of the "old-fashioned" FK technique, Kreutzer et al.<xref ref-type="bibr" rid="B8">8</xref> consider that the conversion to TCPC would be indicated in cases with arrhythmia, symptomatic ones, and those unresponsive to treatment with amiodarone and in the presence of thrombus in the right atrium.<xref ref-type="bibr" rid="B8">8</xref> Heart transplant after Fontan has been considered a therapeutic option in cases of which the main determinant of failure is ventricular dysfunction and some centers have already shown favorable results.<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B10">10</xref> In the study by Fernandes et al.,<xref ref-type="bibr" rid="B4">4</xref> one patient is awaiting a cardiac transplant after a pacemaker was implanted, with an unfavorable evolution.</p>
    <p>Fontan conversion strategy has been described since 1991.<xref ref-type="bibr" rid="B9">9</xref> Worldwide, there is limited experience with this procedure, largely restricted to a small number of centers, and even in services with a greater number of surgical procedures, they have usually followed only a few dozen patients.<xref ref-type="bibr" rid="B6">6</xref> The long-term evolution and the best time to perform the conversion is still a matter of debate.<xref ref-type="bibr" rid="B8">8</xref><sup>,</sup><xref ref-type="bibr" rid="B9">9</xref> In a retrospective analysis of ten years of follow-up of patients undergoing Fontan conversion, using Australian and New Zealand registries, Poh CL et al.,<xref ref-type="bibr" rid="B5">5</xref> demonstrated that patients submitted to an earlier conversion had more favorable long-term outcomes, with a heart transplant-free 10-year survival of 86%.<xref ref-type="bibr" rid="B5">5</xref>
		</p>
    <p>A systematic review carried out by Brida et al.,<xref ref-type="bibr" rid="B7">7</xref> which analyzed 1,182 patients, concluded that Fontan conversion has a high mortality risk and the combination with arrhythmia surgery seems to be associated with lower early mortality, especially when patients are referred at an early age and treated in centers of expertise.<xref ref-type="bibr" rid="B7">7</xref>
		</p>
    <p>Regardless of the sample size analyzed in the article, the results are comparable to the literature data, demonstrating the complexity of the procedure and the importance of its performance in more experienced centers.<xref ref-type="bibr" rid="B11">11</xref>
		</p>
  </body>
  <back>
    <fn-group>
      <fn fn-type="other">
        <p>Short Editorial related to the article: Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation</p>
      </fn>
    </fn-group>
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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>Minieditorial</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Convers&#227;o da Cirurgia de Fontan-Kreutzer para Cavo-Pulmonar Total devido a Fal&#234;ncia da Circula&#231;&#227;o Univentricular. Op&#231;&#227;o Terap&#234;utica Vi&#225;vel?</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <contrib-id contrib-id-type="orcid">0000-0003-3367-5779</contrib-id>
          <name>
            <surname>Guimar&#227;es</surname>
            <given-names>Isabel Cristina Britto</given-names>
          </name>
          <xref ref-type="aff" rid="aff3">1</xref>
          <xref ref-type="aff" rid="aff4">2</xref>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
      </contrib-group>
      <aff id="aff3">
        <label>1</label>
        <institution content-type="original">Universidade Federal da Bahia - Faculdade de Medicina da Bahia, Salvador, BA - Brasil</institution>
      </aff>
      <aff id="aff4">
        <label>2</label>
        <institution content-type="original">Hospital Ana Nery - Universidade Federal da Bahia, Salvador, BA - Brasil</institution>
      </aff>
      <author-notes>
        <corresp id="c2"><bold>Correspond&#234;ncia: Isabel Cristina Britto Guimar&#227;es,</bold> Pra&#231;a Ramos de Queiros, s/n. CEP 40026-010, Largo do Terreiro de Jesus, Salvador, BA - Brasil. E-mail: <email>isabelcbguimaraes@gmail.com</email>
				</corresp>
      </author-notes>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Cardiopatias Cong&#234;nitas</kwd>
        <kwd>Atresia Tric&#250;spide/cirurgia</kwd>
        <kwd>T&#233;cnica de Fontan/m&#233;todos</kwd>
        <kwd>Procedimento de Fontan-Kreutzer</kwd>
        <kwd>Deriva&#231;&#227;o Card&#237;aca Direita</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>A cirurgia de Fontan, opera&#231;&#227;o paliativa para pacientes com cora&#231;&#245;es de fisiologia de &#8220;ventr&#237;culo &#250;nico&#8221;, sofreu v&#225;rias modifica&#231;&#245;es desde o primeiro procedimento realizado em 1971, para indiv&#237;duos com diagn&#243;stico de atresia tric&#250;spide.<xref ref-type="bibr" rid="B1">1</xref>
			</p>
      <p>A t&#233;cnica da anastomose &#225;trio-pulmonar, Fontan-Kreutzer (FK), foi amplamente utilizada na d&#233;cada de 80. Publica&#231;&#245;es referentes ao seguimento tardio dos pacientes submetidos &#224; t&#233;cnica antes de 1990, demonstraram maior frequ&#234;ncia de complica&#231;&#245;es como: insufici&#234;ncia card&#237;aca, arritmias, fen&#244;menos tromboemb&#243;licos, enteropatia perdedora de prote&#237;nas, bronquite pl&#225;stica, morte s&#250;bita e insufici&#234;ncia hep&#225;tica.<xref ref-type="bibr" rid="B2">2</xref><sup>,</sup><xref ref-type="bibr" rid="B3">3</xref>
			</p>
      <p>O estudo de Fernandes et al.,<xref ref-type="bibr" rid="B4">4</xref> publicado nesse n&#250;mero da revista, teve como objetivo analisar os resultados da convers&#227;o do FK em Conex&#227;o Cavo-Pulmonar Total (CCPT) dos pacientes com sinais de fal&#234;ncia da circula&#231;&#227;o univentricular, submetidos a cirurgia em uma &#250;nica institui&#231;&#227;o.</p>
      <p>Dos 420 pacientes submetidos &#224; cirurgia de Fontan entre 1995 a 2016, 18 realizaram FK, correspondendo a 4,3% do total da amostra. Dos 18 casos de FK, em 10 houve a necessidade de convers&#227;o para CCPT por conta de sinais de falha na circula&#231;&#227;o do Fontan, sendo todos inclu&#237;dos na an&#225;lise do trabalho. Em 9 casos, a principal causa de convers&#227;o foi a presen&#231;a de arritmia n&#227;o controlada e em 1 foi a enteropatia perdedora de prote&#237;nas.</p>
      <p>Aspecto relevante em rela&#231;&#227;o aos dados apresentados, diz respeito a classe funcional pela <italic>New York Heart Association</italic>. Antes da convers&#227;o cir&#250;rgica, 70% estavam em classe funcional II e III, e ap&#243;s a cirurgia de convers&#227;o, cerca de 80% dos pacientes apresentaram melhora da capacidade funcional.</p>
      <p>Apesar da principal indica&#231;&#227;o da convers&#227;o ter sido a presen&#231;a de arritmias de dif&#237;cil controle, cerca de 44% dos pacientes continuaram apresentando arritmias como manifesta&#231;&#227;o cl&#237;nica, n&#227;o demonstrando resultados satisfat&#243;rios na utiliza&#231;&#227;o da crioabla&#231;&#227;o cir&#250;rgica, diferente de estudos que mostram resultados favor&#225;veis na sua utiliza&#231;&#227;o.<xref ref-type="bibr" rid="B5">5</xref> Observa-se tamb&#233;m nos resultados apresentados, dados que demonstram a complexidade na condu&#231;&#227;o destes pacientes, como: tempo de interna&#231;&#227;o prolongado e taxa de &#243;bito de 20%. Os autores associaram a taxa de mortalidade encontrada, com o per&#237;odo em que as cirurgias foram realizadas, entre 1996 e 2000, a curva de aprendizado do servi&#231;o relativo &#224; t&#233;cnica cir&#250;rgica descrita. Contudo, a taxa de mortalidade precoce descrita por outros autores, tamb&#233;m tem sido elevada, variando de 0 a 21%.<xref ref-type="bibr" rid="B6">6</xref><sup>,</sup><xref ref-type="bibr" rid="B7">7</xref>
			</p>
      <p>Kreutzer et al.,<xref ref-type="bibr" rid="B8">8</xref> em artigo de revis&#227;o faz uma reflex&#227;o sobre as cinco d&#233;cadas da t&#233;cnica de FK, no qual defendem que as complica&#231;&#245;es tardias, estariam fortemente associadas as estrat&#233;gias cir&#250;rgicas e procedimentos n&#227;o mais utilizados nos dias atuais, como: uso prolongado de bandagem da art&#233;ria pulmonar, Blalock-Taussig cl&#225;ssico, interven&#231;&#245;es tardias, diagn&#243;stico tardio das altera&#231;&#245;es hemodin&#226;micas significativas e o uso de t&#233;cnicas cir&#250;rgicas atualmente consideradas inadequadas como o Fontan cl&#225;ssico e a cirurgia de Kreutzer original. Kreuzter et al.,<xref ref-type="bibr" rid="B8">8</xref> consideram que devemos ser cautelosos ao analisar dados da literatura em rela&#231;&#227;o a t&#233;cnica cir&#250;rgica antes de 1990, como tamb&#233;m ao utilizar esses resultados como preditores de desfecho a longo prazo nos pacientes submetidos &#224; cirurgia de FK na atualidade.<xref ref-type="bibr" rid="B8">8</xref>
			</p>
      <p>Em rela&#231;&#227;o ao estudo de Fernandes et al.,<xref ref-type="bibr" rid="B4">4</xref> n&#227;o dispomos dessas informa&#231;&#245;es, as quais poderiam nos auxiliar num melhor entendimento das complica&#231;&#245;es tardias que ocorreram nessa amostra.</p>
      <p>Para os sobreviventes tardios da t&#233;cnica do FK &#8220;modo antigo&#8221;, Kreutzer et al.,<xref ref-type="bibr" rid="B8">8</xref> consideram que a convers&#227;o para CCPT estaria indicada nos casos com arritmia, sintom&#225;ticos e n&#227;o responsivos ao tratamento com amiodarona e na presen&#231;a de trombo no &#225;trio direito.<xref ref-type="bibr" rid="B8">8</xref> O transplante card&#237;aco ap&#243;s o Fontan, tem sido considerado uma op&#231;&#227;o terap&#234;utica nos casos em que o principal determinante de fal&#234;ncia &#233; a disfun&#231;&#227;o ventricular e alguns centros j&#225; apresentam resultados favor&#225;veis.<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B10">10</xref> No estudo de Fernandes et al.,<xref ref-type="bibr" rid="B4">4</xref> um paciente encontra-se na fila de transplante card&#237;aco, ap&#243;s implante de marca-passo com evolu&#231;&#227;o desfavor&#225;vel.</p>
      <p>A estrat&#233;gia de convers&#227;o do Fontan vem sendo descrita desde 1991.<xref ref-type="bibr" rid="B9">9</xref> Em todo o mundo, h&#225; experi&#234;ncia limitada com esse procedimento, em grande parte restrito a pequeno n&#250;mero de centros, e at&#233; em servi&#231;os com maior volume cir&#250;rgico, geralmente acompanham apenas algumas dezenas de pacientes.<xref ref-type="bibr" rid="B6">6</xref> A evolu&#231;&#227;o a longo prazo e qual o melhor momento para realiza&#231;&#227;o da convers&#227;o ainda &#233; motivo de discuss&#227;o.<xref ref-type="bibr" rid="B8">8</xref><sup>,</sup><xref ref-type="bibr" rid="B9">9</xref> Analisando retrospectivamente dez anos de seguimento dos pacientes submetidos a convers&#227;o do Fontan, utilizando registros da Austr&#225;lia e Nova Zel&#226;ndia, Poh CL et al.,<xref ref-type="bibr" rid="B5">5</xref> demonstraram que pacientes submetidos mais precocemente a convers&#227;o apresentaram desfechos a longo prazo mais favor&#225;veis, com uma sobrevida em 10 anos livre de transplante card&#237;aco de 86%.<xref ref-type="bibr" rid="B5">5</xref>
			</p>
      <p>Revis&#227;o sistem&#225;tica realizada por Brida et al.<xref ref-type="bibr" rid="B7">7</xref> analisando 1.182 pacientes, concluiu que a convers&#227;o do Fontan apresenta elevado risco de mortalidade e que a combina&#231;&#227;o com cirurgia da arritmia parece estar associada a menor mortalidade precoce, principalmente quando os pacientes s&#227;o encaminhados em idade precoce e tratados em centros experientes.<xref ref-type="bibr" rid="B7">7</xref>
			</p>
      <p>Independentemente do tamanho da amostra analisada no artigo, os resultados s&#227;o compar&#225;veis aos dados da literatura, demonstrando a complexidade do procedimento e da import&#226;ncia da sua realiza&#231;&#227;o em centros mais experientes.<xref ref-type="bibr" rid="B11">11</xref>
			</p>
    </body>
    <back>
      <fn-group>
        <fn fn-type="other">
          <p>Minieditorial referente ao artigo: Resultados da Convers&#227;o da Cirurgia de Fontan-Kreutzer para Cavo-Pulmonar Total Devido Fal&#234;ncia da Circula&#231;&#227;o Univentricular</p>
        </fn>
      </fn-group>
    </back>
  </sub-article>
</article>
