<!DOCTYPE article PUBLIC "-//NLM//DTD JATS (Z39.96) Journal Publishing DTD v1.1 20151215//EN" "https://jats.nlm.nih.gov/publishing/1.1/JATS-journalpublishing1.dtd">
<article xmlns:xlink="http://www.w3.org/1999/xlink" specific-use="sps-1.9" dtd-version="1.1" xml:lang="pt" article-type="research-article">
  <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">HsR5YDLt9dsDHMC69mCNtFS</article-id>
      <article-id pub-id-type="other">00002</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2005000100002</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(05)08400102</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2005000100002</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Artigos Originais</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Avalia&#231;&#227;o do suprimento sang&#252;&#237;neo vascular pulmonar nos portadores de atresia pulmonar com comunica&#231;&#227;o interventricular e art&#233;rias colaterais sist&#234;mico-pulmonares</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Croti</surname>
            <given-names>Ulisses Alexandre</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Marcial</surname>
            <given-names>Miguel Lorenzo Barbero</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Tanamati</surname>
            <given-names>Carla</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Jatene</surname>
            <given-names>Marcelo Biscegli</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Braile</surname>
            <given-names>Domingo Marcolino</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Oliveira</surname>
            <given-names>Sergio Almeida de</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
      </contrib-group>
      <aff id="aff01">
        <addr-line>
          <named-content content-type="state">S&#227;o Paulo</named-content>
        </addr-line>
        <institution content-type="orgname">Universidade de S&#227;o Paulo</institution>
        <institution content-type="orgdiv1">FM </institution>
        <institution content-type="orgdiv2">Instituto do Cora&#231;&#227;o do Hospital das Cl&#237;nicas</institution>
        <country country="BR">Brazil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>01</month>
        <year>2005</year>
      </pub-date>
      <volume>84</volume>
      <issue>1</issue>
      <fpage>3</fpage>
      <lpage>9</lpage>
      <history>
        <date date-type="accepted">
          <day>11</day>
          <month>02</month>
          <year>2004</year>
        </date>
        <date date-type="received">
          <day>11</day>
          <month>08</month>
          <year>2003</year>
        </date>
      </history>
      <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>
      <abstract>
        <p>OBJETIVO: Analisar as caracter&#237;sticas morfom&#233;tricas das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares, avaliando a morfologia do suprimento sang&#252;&#237;neo vascular pulmonar, procurando estabelecer suas implica&#231;&#245;es no tratamento cir&#250;rgico. M&#201;TODO: Entre janeiro/1990 e junho/2001, foram estudados 40 pacientes, inclu&#237;ndo-se os que apresentavam estudo cineangiocardiogr&#225;fico completo e pr&#233;vio &#224; primeira interven&#231;&#227;o cir&#250;rgica. Analisaram-se as caracter&#237;sticas morfom&#233;tricas das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares, assim como a distribui&#231;&#227;o da irriga&#231;&#227;o sang&#252;&#237;nea nos pulm&#245;es. Calcularam-se os &#237;ndices arterial pulmonar (IAAPP), arterial colateral sist&#234;mico-pulmonar (IACSP) e arterial neopulmonar total (IANPT = IAAPP + IACSP). O tratamento cir&#250;rgico foi considerado paliativo (TP), paliativo definitivo (TPD) e definitivo (TD). RESULTADOS: O TP foi predominante. N&#227;o houve diferen&#231;as estatisticamente significantes entre os pacientes com TP, TPD e TD, em rela&#231;&#227;o ao IAAPP, IACSP e IANPT. Comparando o IAAPP e o IACSP, n&#227;o houve diferen&#231;a entre os &#237;ndices para o TD (p=0,4309), o IACSP foi maior que o IAAPP para o TP (p=0,0176) e descritivamente tamb&#233;m maior para o TPD. O IANPT dos pacientes em TD foi maior que os em TP (p=0,0959). Foram identificados cinco subgrupos morfologicamente semelhantes, denominados: B1, B2, B3, B4 e B5. A mortalidade total foi de 17,5%. CONCLUS&#195;O: A morfologia do suprimento sang&#252;&#237;neo vascular pulmonar das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares mostrou-se soberana na orienta&#231;&#227;o do tratamento cir&#250;rgico. Independentemente da divis&#227;o did&#225;tica em subgrupos, o TP foi predominante. A mortalidade n&#227;o apresentou correla&#231;&#227;o com as caracter&#237;sticas morfom&#233;tricas.</p>
      </abstract>
      <kwd-group xml:lang="pt" kwd-group-type="author-generated">
        <kwd>atresia pulmonar</kwd>
        <kwd>comunica&#231;&#227;o interventricular</kwd>
        <kwd>art&#233;rias colaterais sist&#234;mico-pulmonares</kwd>
      </kwd-group>
      <pub-date publication-format="electronic" date-type="pub">
        <day>14</day>
        <month>02</month>
        <year>2005</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>ARTIGO ORIGINAL</bold>
    </p>
    <p>Avalia&#231;&#227;o do suprimento sang&#252;&#237;neo vascular pulmonar nos portadores de atresia pulmonar com comunica&#231;&#227;o interventricular e art&#233;rias colaterais sist&#234;mico-pulmonares</p>
    <p>Ulisses Alexandre Croti; Miguel Lorenzo Barbero Marcial; Carla Tanamati; Marcelo Biscegli Jatene; Domingo Marcolino Braile; Sergio Almeida de Oliveira </p>
    <p> S&#227;o Paulo, SP</p>
    <p>Instituto do Cora&#231;&#227;o do Hospital das Cl&#237;nicas - FMUSP</p>
    <p>
      <xref ref-type="corresp" rid="back10">Endere&#231;o para correspond&#234;ncia</xref>
    </p>
    <p>
      <bold>RESUMO</bold>
    </p>
    <p><bold>OBJETIVO: </bold>Analisar as caracter&#237;sticas morfom&#233;tricas das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares, avaliando a morfologia do suprimento sang&#252;&#237;neo vascular pulmonar, procurando estabelecer suas implica&#231;&#245;es no tratamento cir&#250;rgico. </p>
    <p><bold>M&#201;TODO: </bold>Entre janeiro/1990 e junho/2001, foram estudados 40 pacientes, inclu&#237;ndo-se os que apresentavam estudo cineangiocardiogr&#225;fico completo e pr&#233;vio &#224; primeira interven&#231;&#227;o cir&#250;rgica. Analisaram-se as caracter&#237;sticas morfom&#233;tricas das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares, assim como a distribui&#231;&#227;o da irriga&#231;&#227;o sang&#252;&#237;nea nos pulm&#245;es. Calcularam-se os &#237;ndices arterial pulmonar (IAAPP), arterial colateral sist&#234;mico-pulmonar (IACSP) e arterial neopulmonar total (IANPT = IAAPP + IACSP). O tratamento cir&#250;rgico foi considerado paliativo (TP), paliativo definitivo (TPD) e definitivo (TD). </p>
    <p><bold>RESULTADOS: </bold>O TP foi predominante. N&#227;o houve diferen&#231;as estatisticamente significantes entre os pacientes com TP, TPD e TD, em rela&#231;&#227;o ao IAAPP, IACSP e IANPT. Comparando o IAAPP e o IACSP, n&#227;o houve diferen&#231;a entre os &#237;ndices para o TD (p=0,4309), o IACSP foi maior que o IAAPP para o TP (p=0,0176) e descritivamente tamb&#233;m maior para o TPD. O IANPT dos pacientes em TD foi maior que os em TP (p=0,0959). Foram identificados cinco subgrupos morfologicamente semelhantes, denominados: B1, B2, B3, B4 e B5. A mortalidade total foi de 17,5%. </p>
    <p><bold>CONCLUS&#195;O: </bold> A morfologia do suprimento sang&#252;&#237;neo vascular pulmonar das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares mostrou-se soberana na orienta&#231;&#227;o do tratamento cir&#250;rgico. Independentemente da divis&#227;o did&#225;tica em subgrupos, o TP foi predominante. A mortalidade n&#227;o apresentou correla&#231;&#227;o com as caracter&#237;sticas morfom&#233;tricas.</p>
    <p><bold>Palavras-chave: </bold>atresia pulmonar, comunica&#231;&#227;o interventricular, art&#233;rias colaterais sist&#234;mico-pulmonares</p>
    <p>Na atresia pulmonar com comunica&#231;&#227;o interventricular e art&#233;rias colaterais sist&#234;mico-pulmonares, o suprimento sang&#252;&#237;neo vascular pulmonar pode ser muito complexo, com grandes varia&#231;&#245;es anat&#244;micas, de tal forma que alguns segmentos dos lobos pulmonares podem ser supridos por fluxo sang&#252;&#237;neo oriundo das art&#233;rias pulmonares centrais e/ou art&#233;rias colaterais sist&#234;mico-pulmonares <sup>1</sup>. Devido a esta extrema heterogeneidade, diversas classifica&#231;&#245;es anat&#244;micas foram propostas com o intuito de estabelecer crit&#233;rios que racionalizassem a conduta cir&#250;rgica. A classifica&#231;&#227;o morfol&#243;gica mais utilizada, atualmente <sup>2-5</sup>, divide os portadores de atresia pulmonar com comunica&#231;&#227;o interventricular em tr&#234;s grupos: A, B e C. No grupo A, todos os segmentos pulmonares s&#227;o supridos pelas art&#233;rias pulmonares centrais. No grupo B, alguns segmentos pulmonares s&#227;o supridos pelas art&#233;rias pulmonares centrais, enquanto outros s&#227;o supridos pelas art&#233;rias colaterais sist&#234;mico-pulmonares. No grupo C, todos os segmentos pulmonares s&#227;o supridos pelas art&#233;rias colaterais sist&#234;mico-pulmonares, encontrando-se ausentes as art&#233;rias pulmonares centrais <sup>5</sup>.</p>
    <p>Com base na an&#225;lise de estudos cineangiocardiogr&#225;ficos de portadores de atresia pulmonar com comunica&#231;&#227;o interventricular e art&#233;rias colaterais sist&#234;mico-pulmonares (grupo B), o presente trabalho visou analisar as caracter&#237;sticas morfom&#233;tricas das art&#233;rias pulmonares centrais e colaterais sist&#234;mico-pulmonares, avaliando a distribui&#231;&#227;o do suprimento sang&#252;&#237;neo vascular pulmonar e procurando estabelecer suas implica&#231;&#245;es no tratamento cir&#250;rgico.</p>
    <p>
      <bold>M&#233;todos</bold>
    </p>
    <p>Foram estudados 40 pacientes com atresia pulmonar, comunica&#231;&#227;o interventricular e art&#233;rias colaterais sist&#234;mico-pulmonares, de janeiro de 1990 a junho de 2001, operados no Instituto do Cora&#231;&#227;o do Hospital das Cl&#237;nicas da Faculdade de Medicina da Universidade de S&#227;o Paulo. O estudo foi aprovado pela Comiss&#227;o Cient&#237;fica e de &#201;tica para An&#225;lise de Projeto de Pesquisa da Institui&#231;&#227;o.</p>
    <p>Neste estudo, a idade na cineangiocardiogr&#225;fica variou de nove dias a 21,6 (mediana de 1,1) ano; nos procedimentos cir&#250;rgicos, de 3,7 meses a 21,6 (com mediana de 1,9) anos. Eram do sexo masculino 21 (52,50%) e 19 (47,50%) do feminino.</p>
    <p>Foram inclu&#237;dos no estudo pacientes com morfologia de <italic>situs solitus</italic> e conex&#245;es atrioventricular e ventriculoarterial concordantes, com estudos cineangiocardiogr&#225;ficos completos, obrigatoriamente pr&#233;vios &#224; 1&#170; interven&#231;&#227;o cir&#250;rgica, exclusivamente realizados na Institui&#231;&#227;o. A an&#225;lise dos prontu&#225;rios foi direcionada aos laudos do 1&#186; estudo cineangiocardiogr&#225;fico e todas as descri&#231;&#245;es das 56 opera&#231;&#245;es realizadas.</p>
    <p>Foram exclu&#237;dos aqueles onde n&#227;o foi poss&#237;vel obter o estudo cineangiocardiogr&#225;fico pr&#233;vio ao tratamento cir&#250;rgico inicial e aqueles onde n&#227;o foram poss&#237;veis t&#233;cnicas para demonstra&#231;&#227;o do suprimento sang&#252;&#237;neo vascular pulmonar, tornando o exame incompleto.</p>
    <p>Considerou-se estudo cineangiocardiogr&#225;fico completo quando inclu&#237;a: inje&#231;&#245;es de contraste na aorta ascendente, descendente e arco a&#243;rtico, ventriculografias direita e esquerda, inje&#231;&#245;es seletivas no canal arterial, art&#233;rias pulmonares centrais, art&#233;rias colaterais sist&#234;mico-pulmonares, coron&#225;rias e venografia retr&#243;grada. As inje&#231;&#245;es de contraste, principalmente as seletivas nas art&#233;rias pulmonares centrais, art&#233;rias colaterais sist&#234;mico-pulmonares e venografias retr&#243;gradas deveriam identificar as diferentes varia&#231;&#245;es anat&#244;micas e segmentos supridos.</p>
    <p>Na an&#225;lise morfom&#233;trica quantitativa, utilizou-se o <italic>software CAAS II</italic> (<italic>cardiovascular angiography analysis system II</italic>). O computador foi ligado ao <italic>Laser videodisc player LVA-7000 (JVC), Videocassette recorder SVO-9500 MD (SONY)</italic> e ao <italic>Tagarno</italic> c<italic>ineviodeo camera</italic>, a fim de permitir an&#225;lise de filmes gravados em disco digital, fita VHS e em filme 35 mm.</p>
    <p>O sistema foi calibrado baseando-se no di&#226;metro da por&#231;&#227;o distal do cateter, calculado pelo <italic>software CAAS II</italic> de acordo com os dados fornecidos previamente sobre o cateter utilizado naquele exame, como por exemplo 5Fr ou 6Fr.</p>
    <p>As imagens selecionadas para medi&#231;&#227;o foram em p&#243;stero-anterior, com o quadro em que o segmento vascular de interesse estivesse bem contrastado e em s&#237;stole ventricular. Ap&#243;s esta calibra&#231;&#227;o mediam-se os di&#226;metros das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares. As primeiras medidas imediatamente proximal &#224; origem do 1&#186; ramo lobar <sup>6</sup> e as &#250;ltimas distalmente ao ponto em que idealmente seria poss&#237;vel a unifocaliza&#231;&#227;o cir&#250;rgica <sup>7</sup>. Com essas medidas obtidas, calculou-se a &#225;rea dos vasos sang&#252;&#237;neos.</p>
    <p>Conhecendo-se o peso e a altura dos pacientes no momento do exame, calculou-se a superf&#237;cie corp&#243;rea, utilizando-se a f&#243;rmula de Mosteller <sup>8</sup>. Assim, foi poss&#237;vel calcular &#237;ndices denominados: &#237;ndice arterial pulmonar, &#237;ndice arterial colateral sist&#234;mico-pulmonar e &#237;ndice arterial neopulmonar total (<xref ref-type="fig" rid="fig1">fig. 1</xref>).</p>
    <fig id="fig1">
      <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gf01.jpg"/></fig>
    <p>Como alguns segmentos pulmonares eram supridos pelos ramos das art&#233;rias pulmonares centrais e outros supridos pelas art&#233;rias colaterais sist&#234;mico-pulmonares, calculou-se o &#237;ndice arterial neopulmonar total.</p>
    <p>A &#225;rea foi obtida medindo-se as art&#233;rias pulmonares centrais imediatamente antes da bifurca&#231;&#227;o dos ramos lobares, como j&#225; descrito <sup>6</sup>. A art&#233;ria pulmonar foi considerada hipopl&#225;sica quando sua &#225;rea apresentava diminui&#231;&#227;o de 25% ou mais com rela&#231;&#227;o &#224; m&#233;dia das &#225;reas das art&#233;rias pulmonares centrais. Quando o &#237;ndice arterial pulmonar era &lt;100 mm<sup>2</sup>/m<sup>2</sup>, as art&#233;rias pulmonares centrais foram consideradas hipopl&#225;sicas <sup>6</sup>. Quando o &#237;ndice arterial neopulmonar total era &lt;100 mm<sup>2</sup>/m<sup>2</sup>, considerou-se a necessidade de avalia&#231;&#227;o da rela&#231;&#227;o do &#237;ndice arterial pulmonar e do &#237;ndice arterial colateral sist&#234;mico-pulmonar.</p>
    <p>O di&#226;metro das art&#233;rias colaterais sist&#234;mico-pulmonares foi medido distalmente ao ponto em que idealmente seria poss&#237;vel a realiza&#231;&#227;o da unifocaliza&#231;&#227;o, j&#225; descrito anteriormente <sup>7</sup>.</p>
    <p>A presen&#231;a de estenose nas art&#233;rias colaterais sist&#234;mico-pulmonares foi considerada quando havia diminui&#231;&#227;o luminal de mais de 25% <sup>7</sup>: fina quando sua &#225;rea era &lt; 50% da m&#233;dia aritm&#233;tica das &#225;reas de todas as art&#233;rias colaterais sist&#234;mico-pulmonares, m&#233;dia quando &gt; 50% da m&#233;dia aritm&#233;tica e menor que a m&#233;dia aritm&#233;tica, sendo calibrosa quando era maior que a m&#233;dia aritm&#233;tica das &#225;reas de todas as art&#233;rias colaterais sist&#234;mico-pulmonares do paciente.</p>
    <p>Quando o &#237;ndice arterial colateral sist&#234;mico-pulmonar &lt;100 mm<sup>2</sup>/m<sup>2</sup>, as art&#233;rias colaterais sist&#234;mico-pulmonares foram consideradas com calibre globalmente reduzido.</p>
    <p>Na an&#225;lise das &#225;reas de suprimento sang&#252;&#237;neo vascular pulmonar, observou-se quais os lobos pulmonares eram supridos pelas art&#233;rias pulmonares centrais e quais eram supridos pelas art&#233;rias colaterais sist&#234;mico-pulmonares. A rela&#231;&#227;o entre os lobos e segmentos pulmonares foi assim definida: lobo superior esquerdo (segmentos apicoposterior, anterior, lingular superior, lingular inferior), lobo inferior esquerdo (segmentos superior, basal anteromedial, basal lateral, basal posterior), lobo superior direito (segmentos apical, anterior, posterior), lobo m&#233;dio direito (segmentos lateral e medial), lobo inferior direito (segmentos superior, basal m&#233;dio, basal anterior, basal lateral, basal posterior) <sup>9</sup>.</p>
    <p>A conflu&#234;ncia entre as art&#233;rias pulmonares centrais foi considerada, quando foi poss&#237;vel visibilizar inje&#231;&#227;o de contraste em uma das art&#233;rias, enchendo o ramo pulmonar contralateral.</p>
    <p>Foram consideradas art&#233;rias colaterais sist&#234;mico-pulmonares, as art&#233;rias que nutriam pelo menos uma art&#233;ria segmentar intrapulmonar e faziam trajeto independente dos br&#244;nquios pulmonares e das art&#233;rias intercostais.</p>
    <p>Os tratamentos cir&#250;rgicos foram considerados paliativo, paliativo definitivo ou definitivo: paliativo para os pacientes submetidos a interven&#231;&#245;es sem fechamento da comunica&#231;&#227;o interventricular e com perspectivas futuras de conclus&#227;o ou n&#227;o do tratamento e considerados como apresentando-se em transcurso de tratamento; paliativo definitivo para os que foram submetidos a interven&#231;&#245;es sem o fechamento da comunica&#231;&#227;o interventricular e que n&#227;o apresentavam mais op&#231;&#245;es cir&#250;rgicas que possibilitassem a conclus&#227;o do tratamento, com conex&#227;o ventr&#237;culo direito-art&#233;rias pulmonares centrais e fechamento da comunica&#231;&#227;o interventricular, situa&#231;&#227;o que foi considerada quando o di&#226;metro das art&#233;rias pulmonares centrais eram extremamente reduzidos sem possibilidade de desenvolvimento ou quando a &#225;rvore vascular pulmonar distal apresentava sinais de doen&#231;a hipertensiva pulmonar com ou sem trombose ou quando n&#227;o era poss&#237;vel a unifocaliza&#231;&#227;o de pelo menos 10 segmentos pulmonares para conex&#227;o ao ventr&#237;culo direito; definitivo quando foi poss&#237;vel a realiza&#231;&#227;o de conex&#227;o ventr&#237;culo direito-art&#233;rias pulmonares centrais e fechamento da comunica&#231;&#227;o interventricular, permitindo o restabelecimento da fisiologia normal. Os crit&#233;rios para fechamento da comunica&#231;&#227;o interventricular foram a presen&#231;a de art&#233;rias pulmonares centrais com calibres adequados, &#225;rvore vascular pulmonar distal adequada e n&#250;mero m&#237;nimo de 10 segmentos pulmonares poss&#237;veis de conex&#227;o com o ventr&#237;culo direito.</p>
    <p>
      <bold>Resultados</bold>
    </p>
    <p>A an&#225;lise morfom&#233;trica dos estudos cineangiocardiogr&#225;ficos pr&#233;vios ao primeiro procedimento cir&#250;rgico, assim como os est&#225;gios de tratamento de todos os pacientes est&#227;o na <xref ref-type="table" rid="tab1">tabela 1</xref>.</p>
    <table-wrap id="tab1">
      <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gt01.jpg"/></table-wrap>
    <p>Associando-se os dados morfom&#233;tricos obtidos &#224; distribui&#231;&#227;o do suprimento sang&#252;&#237;neo vascular pulmonar, foi poss&#237;vel identificar cinco subgrupos com caracter&#237;sticas semelhantes, denominados: B1, B2, B3, B4 e B5 (<xref ref-type="fig" rid="fig2">fig. 2</xref>). O subgrupo B1 com 8 (20%), B2 com 4 (10%), B3 com 16 (40%), B4 com 4 (10%) e B5 com 8 (20%) pacientes.</p>
    <fig id="fig2">
      <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gf02.jpg"/></fig>
    <p>No subgrupo B1, todos os pacientes apresentavam art&#233;rias pulmonares centrais suprindo os segmentos dos lobos superior esquerdo e inferior direito. Em alguns as art&#233;rias colaterais sist&#234;mico-pulmonares tamb&#233;m estavam presentes nesses segmentos, por&#233;m as art&#233;rias pulmonares centrais eram predominantes. Todos os outros segmentos eram irrigados pelas art&#233;rias colaterais sist&#234;mico-pulmonares.</p>
    <p>No subgrupo B2, todos os pacientes apresentavam art&#233;rias pulmonares centrais suprindo os segmentos dos lobos superior direito e inferior esquerdo. Em alguns casos as art&#233;rias colaterais sist&#234;mico-pulmonares tamb&#233;m estavam presentes nesses segmentos, por&#233;m as art&#233;rias pulmonares centrais eram predominantes. Todos os outros segmentos eram irrigados pelas art&#233;rias colaterais sist&#234;mico-pulmonares.</p>
    <p>No subgrupo B3, todos os pacientes apresentavam art&#233;rias pulmonares centrais suprindo os segmentos dos lobos inferiores esquerdo e direito ou suprindo os segmentos de um dos lobos inferiores e a maioria dos segmentos dos lobos do pulm&#227;o contralateral.</p>
    <p>No subgrupo B4, todos os pacientes apresentavam art&#233;rias pulmonares centrais suprindo os segmentos dos lobos superiores esquerdo e direito ou suprindo os segmentos de um dos lobos superiores e a maioria dos segmentos dos lobos do pulm&#227;o contralateral.</p>
    <p>No subgrupo B5, os pacientes apresentavam grande diversidade na distribui&#231;&#227;o das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares, com grande dificuldade para defini&#231;&#227;o do suprimento sang&#252;&#237;neo dos segmentos pulmonares. Desta forma, este subgrupo foi constitu&#237;do por uma miscel&#226;nea. Tr&#234;s pacientes apresentavam agenesia de art&#233;ria pulmonar esquerda, dois de art&#233;ria pulmonar direita, dois f&#237;stulas do tronco coronariano esquerdo para o tronco pulmonar e um tinha as art&#233;rias pulmonares centrais hipopl&#225;sicas. Em todos estes pacientes n&#227;o foi poss&#237;vel identificar de forma adequada quais os segmentos pulmonares eram supridos por art&#233;rias pulmonares centrais ou art&#233;rias colaterais sist&#234;mico-pulmonares.</p>
    <p>Na an&#225;lise estat&#237;stica, admitiu-se erro alfa de 5%. Utilizou-se o teste param&#233;trico t de Student e os n&#227;o param&#233;tricos de Kruskal-Wallis e Wilcoxon. Foram realizadas correla&#231;&#245;es entre os subgrupos e a presen&#231;a de estenoses, o &#237;ndice arterial neopulmonar total (&#237;ndice arterial pulmonar e &#237;ndice arterial colateral sist&#234;mico-pulmonar), o n&#250;mero de procedimentos cir&#250;rgicos e o est&#225;gio de tratamento. Houve mais pacientes do subgrupo B2 que chegaram ao tratamento definitivo do que nos demais subgrupos do grupo B, por&#233;m sem significado estat&#237;stico. Os &#250;nicos pacientes com tratamento paliativo definitivo encontravam-se no subgrupo B1. Nenhum paciente que realizou tr&#234;s procedimentos teve tratamento definitivo. A maioria dos pacientes com tratamento definitivo realizou dois procedimentos. Todos aqueles com estenose em pelo menos uma das art&#233;rias pulmonares centrais tiveram tratamento paliativo. A presen&#231;a de estenose nas art&#233;rias colaterais sist&#234;mico-pulmonares n&#227;o apresentou rela&#231;&#227;o com o est&#225;gio de tratamento dos pacientes.</p>
    <p>O n&#250;mero de pacientes com um, dois e tr&#234;s procedimentos realizados nos subgrupos B1 e B3 &#233; muito parecido. O mesmo p&#244;de ser observado com os subgrupos B2 e B4. A maioria dos pacientes do subgrupo B5 realizou apenas um procedimento, propor&#231;&#227;o menor do que em todos os outros subgrupos.</p>
    <p>N&#227;o houve diferen&#231;as estatisticamente significantes entre os pacientes com os tratamentos paliativo, paliativo definitivo e definitivo, em rela&#231;&#227;o ao &#237;ndice arterial pulmonar, &#237;ndice arterial colateral sist&#234;mico-pulmonar e &#237;ndice arterial neopulmonar total. Comparando o &#237;ndice arterial pulmonar e &#237;ndice arterial colateral sist&#234;mico-pulmonar para cada um dos est&#225;gios de tratamento, observa-se: n&#227;o houve diferen&#231;a entre os &#237;ndices para o est&#225;gio definitivo (p=0,4309); o &#237;ndice arterial colateral sist&#234;mico-pulmonar foi estatisticamente maior do que o &#237;ndice arterial pulmonar para o est&#225;gio paliativo (p=0,0176); descritivamente, tem-se que o &#237;ndice arterial colateral sist&#234;mico-pulmonar foi maior do que o &#237;ndice arterial pulmonar para o est&#225;gio paliativo definitivo. O &#237;ndice arterial neopulmonar total dos pacientes em tratamento definitivo foi maior que o &#237;ndice dos em tratamento paliativo (p=0,0959) (<xref ref-type="table" rid="tab2">tabela II</xref>).</p>
    <table-wrap id="tab2">
      <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gt02.jpg"/></table-wrap>
    <p>A maior propor&#231;&#227;o de pacientes falecidos foi encontrada nos subgrupos B1, B2 e B4, enquanto nos subgrupos B3 e B5 esta propor&#231;&#227;o foi menor. No subgrupo B1 2 (25%) faleceram, no B2 1 (25%), no B3 2 (12,5%), no B4 1 (25%) e no B5 1 (12,5%). No grupo total 7 (17,5%) faleceram. Quanto maior o n&#250;mero de procedimentos realizados, maior a propor&#231;&#227;o observada de pacientes falecidos.</p>
    <p>Apesar de n&#227;o ser estatisticamente significante, os &#237;ndices dos pacientes falecidos foram menores do que o daqueles que sobreviveram. A rela&#231;&#227;o entre a m&#233;dia dos &#237;ndices e o est&#225;gio de tratamento para os pacientes falecidos e os que sobreviveram encontra-se na <xref ref-type="table" rid="tab3">tabela III</xref>.</p>
    <table-wrap id="tab3">
      <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gt03.jpg"/></table-wrap>
    <p>
      <bold>Discuss&#227;o</bold>
    </p>
    <p>Em 1970, Somerville classificou a atresia pulmonar com comunica&#231;&#227;o interventricular em tipos A, B, C e D, baseada no grau de desenvolvimento das art&#233;rias pulmonares centrais distalmente ao segmento atr&#233;sico <sup>10</sup>. Comparando-se essa classifica&#231;&#227;o &#224; utilizada em nosso estudo, podemos considerar que o tipo C (atresia da valva e tronco pulmonar e uma das art&#233;rias pulmonares) corresponde ao nosso grupo B, onde os segmentos pulmonares s&#227;o irrigados pelas art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares <sup>5</sup>.</p>
    <p>A investiga&#231;&#227;o dos aspectos hemodin&#226;micos da circula&#231;&#227;o pulmonar, utilizando a cateteriza&#231;&#227;o seletiva e oclus&#227;o com bal&#227;o para visibiliza&#231;&#227;o das art&#233;rias colaterais sist&#234;mico-pulmonares, demonstraram que alguns segmentos pulmonares s&#227;o supridos por fluxo sang&#252;&#237;neo oriundo das art&#233;rias pulmonares centrais e outros pelas art&#233;rias colaterais sist&#234;mico-pulmonares <sup>11</sup>. Desta forma, foi introduzido o importante conceito de suprimento sang&#252;&#237;neo unifocal ou multifocal para a circula&#231;&#227;o pulmonar, que proporcionaria grande avan&#231;o no tratamento da atresia pulmonar com comunica&#231;&#227;o interventricular e art&#233;rias colaterais sist&#234;mico-pulmonares, com t&#233;cnicas cir&#250;rgicas de unifocaliza&#231;&#227;o <sup>12</sup> e utilizadas em alguns pacientes deste grupo.</p>
    <p>Quando o suprimento sang&#252;&#237;neo &#233; unifocal, pode-se aplicar o m&#233;todo proposto por Nakata e cols<italic>.</italic>, que permite o c&#225;lculo das &#225;reas das art&#233;rias pulmonares centrais e, conseq&#252;entemente, a obten&#231;&#227;o do &#237;ndice arterial pulmonar <sup>6</sup>.</p>
    <p>Semelhante ao m&#233;todo de Nakata e cols. <sup>6</sup>, Reddy e cols. calcularam as &#225;reas das art&#233;rias colaterais sist&#234;mico-pulmonares, para os pacientes com suprimento sang&#252;&#237;neo multifocal submetidos a unifocaliza&#231;&#245;es em um &#250;nico est&#225;gio, fechamento da comunica&#231;&#227;o interventricular e conex&#227;o entre o ventr&#237;culo direito e a &#225;rvore pulmonar, obtendo o &#237;ndice arterial colateral sist&#234;mico-pulmonar. Com a somat&#243;ria do &#237;ndice arterial pulmonar e &#237;ndice arterial colateral sist&#234;mico-pulmonar determinaram o &#237;ndice arterial neopulmonar total utilizado em nosso estudo <sup>7</sup>.</p>
    <p>A proposta de corre&#231;&#227;o uniestagiada foi realizada em um &#250;nico paciente do subgrupo B2, falecido por s&#237;ndrome de baixo d&#233;bito card&#237;aco. Reddy e cols. propuseram tamb&#233;m o estudo do fluxo sang&#252;&#237;neo intra-operat&#243;rio, ou seja, ap&#243;s completa unifocaliza&#231;&#227;o e anastomose do tubo extracard&#237;aco, enquanto o paciente ainda est&#225; em circula&#231;&#227;o extracorp&#243;rea, coloca-se uma c&#226;nula no tubo e injeta-se um fluxo predeterminado (d&#233;bito card&#237;aco) de at&#233; 2,5 l/min/m<sup>2</sup>, aspirando vigorosamente o &#225;trio esquerdo. Nesse momento, com um cateter nas art&#233;rias pulmonares aferem-se as medidas press&#243;ricas, que sugerem a possibilidade ou n&#227;o do fechamento da comunica&#231;&#227;o interventricular <sup>7</sup>. Esta comunica&#231;&#227;o pode ser fechada, completando o tratamento definitivo, com mais seguran&#231;a nos pacientes que apresentam &#237;ndice arterial neopulmonar total &gt;200mm<sup>2</sup>/m<sup>2</sup>. Quando o &#237;ndice arterial neopulmonar total &#233; &lt;200mm<sup>2</sup>/m<sup>2</sup>, devem ser associados outros dados fisiol&#243;gicos para corre&#231;&#227;o total em um &#250;nico est&#225;gio <sup>7</sup>. Outros autores consideram pior progn&#243;stico quando o &#237;ndice arterial neopulmonar total &#233; &lt;150mm<sup>2</sup>/m<sup>2</sup><sup>13</sup>.</p>
    <p>Dentre os cinco subgrupos com caracter&#237;sticas semelhantes identificados: B1, B2, B3, B4 e B5, o &#237;ndice arterial neopulmonar total m&#233;dio dos pacientes que realizaram tratamento definitivo foi de 308,66 e nos subgrupos B1, B2, B3, B5, respectivamente 156,65, 230,03, 431,02, 128,50. No subgrupo B4 nenhum paciente realizou tratamento definitivo.</p>
    <p>No subgrupo B1 dos oito pacientes, apenas um tinha todos os segmentos dos lobo superior esquerdo e lobo inferior direito supridos exclusivamente pelas art&#233;rias pulmonares centrais; os outros tinham um ou dois lobos supridos principalmente pelas art&#233;rias pulmonares centrais, por&#233;m com presen&#231;a de art&#233;rias colaterais sist&#234;mico-pulmonares. Estes pacientes tinham pelo menos nove segmentos com a presen&#231;a de art&#233;rias pulmonares centrais, fato n&#227;o muito favor&#225;vel para corre&#231;&#227;o cir&#250;rgica definitiva <sup>14</sup>.</p>
    <p>Apenas um paciente realizou tratamento definitivo e apresentava o &#237;ndice arterial pulmonar expressivamente maior que o &#237;ndice arterial colateral sist&#234;mico-pulmonar, mostrando que nesses pacientes a presen&#231;a de art&#233;rias pulmonares centrais desenvolvidas era um fator de fundamental import&#226;ncia para o adequado tratamento cir&#250;rgico <sup>15</sup>.</p>
    <p>O &#237;ndice arterial neopulmonar total m&#233;dio dos pacientes que realizaram tratamento definitivo (156,65) foi menor do que o daqueles que realizaram tratamento paliativo definitivo (278,84), devido aos altos valores do &#237;ndice arterial colateral sist&#234;mico-pulmonar. Nos dois pacientes que realizaram tratamento paliativo definitivo, as art&#233;rias pulmonares centrais eram extremamente hipopl&#225;sicas e n&#227;o se desenvolveram ap&#243;s o primeiro procedimento cir&#250;rgico.</p>
    <p>No subgrupo B2, dos quatro pacientes, dois tinham os lobo superior direito e lobo inferior esquerdo supridos exclusivamente por art&#233;rias pulmonares centrais e dois apresentavam tamb&#233;m art&#233;rias colaterais sist&#234;mico-pulmonares, por&#233;m com predom&#237;nio de art&#233;rias pulmonares centrais. Estes pacientes tinham apenas sete segmentos pulmonares irrigados por art&#233;rias pulmonares centrais, justificando maior m&#233;dia de art&#233;rias colaterais sist&#234;mico-pulmonares suprindo os segmentos pulmonares desses pacientes.</p>
    <p>Dois chegaram ao tratamento definitivo e dois ao paliativo. N&#227;o houve uma correla&#231;&#227;o com o &#237;ndice arterial neopulmonar total, que foi &gt;200mm<sup>2</sup>/m<sup>2</sup> em todos <sup>7</sup>. Apenas um teve &#237;ndice arterial pulmonar maior que o &#237;ndice arterial colateral sist&#234;mico-pulmonar e este realizou tratamento paliativo, mostrando claramente que n&#227;o houve rela&#231;&#227;o morfom&#233;trica com a morfol&#243;gica para defini&#231;&#227;o de qual paciente realizou tratamento definitivo.</p>
    <p>Um &#250;nico paciente foi submetido &#224; unifocaliza&#231;&#227;o bilateral e fechamento da comunica&#231;&#227;o interventricular em est&#225;gio &#250;nico <sup>15</sup>. Apesar do &#237;ndice arterial neopulmonar total ter sido de 236,27 mm<sup>2</sup>/m<sup>2</sup>, ou seja, concordante com a orienta&#231;&#227;o de corre&#231;&#227;o total quando &gt;200mm<sup>2</sup>/m<sup>2</sup>, o paciente evoluiu com baixo d&#233;bito card&#237;aco e cianose, falecendo em 3h na UTI.</p>
    <p>No subgrupo B3, cinco pacientes apresentavam menos de 10 segmentos supridos pelas art&#233;rias pulmonares centrais e os demais 13 ou 14. Quatro pacientes chegaram ao tratamento definitivo com &#237;ndice arterial neopulmonar total m&#233;dio de 431,02. Um apresentava &#237;ndice arterial pulmonar menor que o &#237;ndice arterial colateral sist&#234;mico-pulmonar, por&#233;m com &#237;ndice arterial neopulmonar total de 211,60. Os demais apresentavam &#237;ndice arterial pulmonar evidentemente maior que o &#237;ndice arterial colateral sist&#234;mico-pulmonar. Isto sugeria que dentre os demais pacientes que estavam em tratamento paliativo (&#237;ndice arterial neopulmonar total m&#233;dio de 233,45), os que possu&#237;am &#237;ndice arterial pulmonar &gt; que &#237;ndice arterial colateral sist&#234;mico-pulmonar teriam maior chance de chegar ao tratamento definitivo, observado tamb&#233;m por Carotti e cols<italic>.</italic> <sup>13</sup>.</p>
    <p>No subgrupo B4 apenas um paciente apresentava sete segmentos supridos pela art&#233;rias pulmonares centrais, todos os outros apresentavam 14 e mesmo assim nenhum realizou tratamento definitivo. O &#237;ndice arterial neopulmonar total m&#233;dio foi de 150,57mm<sup>2</sup>/m<sup>2</sup>, com &#237;ndice arterial pulmonar menor que seus respectivos &#237;ndices arterial colateral sist&#234;mico-pulmonar. As caracter&#237;sticas morfol&#243;gicas foram mais importantes e significativas para a escolha do tratamento. Todos os pacientes encontravam-se em est&#225;gio de tratamento paliativo, podendo evoluir para paliativo definitivo ou definitivo, mas as caracter&#237;sticas morfom&#233;tricas n&#227;o s&#227;o favor&#225;veis ao tratamento definitivo <sup>13</sup>.</p>
    <p>No subgrupo B5 apenas um paciente realizou tratamento definitivo, com um &#250;nico procedimento, com pelo menos 14 segmentos pulmonares supridos por art&#233;rias pulmonares centrais e apresentava &#237;ndice arterial neopulmonar total de 128,50 com &#237;ndice arterial pulmonar &gt; que o seu &#237;ndice arterial colateral sist&#234;mico-pulmonar. Os demais realizaram tratamento paliativo, independentemente dos seus &#237;ndices, mostrando que as caracter&#237;sticas morfol&#243;gicas s&#227;o mais importantes do que as morfom&#233;tricas neste subgrupo.</p>
    <p>Dentre os 40 pacientes do grupo B apenas dois foram definidos como tratamento paliativo definitivo e por prov&#225;vel coincid&#234;ncia estavam no subgrupo B1, ambos com art&#233;rias pulmonares centrais hipopl&#225;sicas. Um com &#237;ndice arterial neopulmonar total de 48,79 e o outro de 508,89, sendo que este &#250;ltimo apresentava grandes art&#233;rias colaterais sist&#234;mico-pulmonares com sinais de hipertens&#227;o e doen&#231;a vascular pulmonar irrevers&#237;vel, sendo considerado sem op&#231;&#245;es cir&#250;rgicas para tratamento definitivo.</p>
    <p>&#201; importante observar que n&#227;o houve diferen&#231;a entre o &#237;ndice arterial pulmonar e o &#237;ndice arterial colateral sist&#234;mico-pulmonar para os pacientes que realizaram tratamento definitivo. Mas, para os pacientes em est&#225;gios paliativo e paliativo definitivo, o &#237;ndice arterial colateral sist&#234;mico-pulmonar foi maior, demonstrando que quando as art&#233;rias colaterais sist&#234;mico-pulmonares predominam existe maior chance de um tratamento paliativo.</p>
    <p>Assim, independentemente do subgrupo did&#225;tico ao qual o paciente possa pertencer, torna-se claro que as medidas morfom&#233;tricas podem ajudar na orienta&#231;&#227;o do tratamento. Contudo, as caracter&#237;sticas de irriga&#231;&#227;o dos segmentos pulmonares pelas art&#233;rias pulmonares centrais e/ou art&#233;rias colaterais sist&#234;mico-pulmonares s&#227;o imperativas na conduta e devem ser analisadas para cada paciente individualmente, segundo outros autores <sup>14</sup>.</p>
    <p>A mortalidade total foi de 17,50%, sendo que no subgrupo B1 dois pacientes faleceram, um com &#237;ndice arterial neopulmonar total de 48,79 e outro com &#237;ndice arterial neopulmonar total de 174,69. O 1&#186; paciente apresentava &#237;ndice arterial pulmonar de 2,80, ou seja, art&#233;rias pulmonares centrais extremamente hipopl&#225;sicas. Assim, ap&#243;s realiza&#231;&#227;o de Blalock-Taussig &#224; direita e &#224; esquerda o paciente continuava em hip&#243;xia. Como as art&#233;rias colaterais sist&#234;mico-pulmonares apresentavam anatomia desfavor&#225;vel, foi imposs&#237;vel a unifocaliza&#231;&#227;o, evoluindo com oclus&#227;o do Blalock-Taussig e falecimento por hip&#243;xia. No 2&#186; paciente foi realizada conex&#227;o entre ventr&#237;culo direito - art&#233;rias pulmonares centrais, com tubo de PTFE; como foi deixada a comunica&#231;&#227;o interventricular aberta, faleceu no 2&#186; p&#243;s-operat&#243;rio, por baixo d&#233;bito card&#237;aco.</p>
    <p>No subgrupo B2 houve um &#243;bito com &#237;ndice arterial neopulmonar total de 236,27. O paciente foi submetido &#224; toracotomia transversal bilateral com unifica&#231;&#227;o bilateral <sup>16</sup>, art&#233;rias colaterais sist&#234;mico-pulmonares &#224; esquerda com atresia pulmonar esquerda e duas art&#233;rias colaterais sist&#234;mico-pulmonares &#224; direita com atresia pulmonar direita, ligadura de art&#233;rias colaterais sist&#234;mico-pulmonares &#224; direita e amplia&#231;&#227;o da via de sa&#237;da do ventr&#237;culo direito com peric&#225;rdio bovino, optando-se por realiza&#231;&#227;o de um Blalock-Taussig &#224; esquerda e soltura de uma das art&#233;rias colaterais sist&#234;mico-pulmonares ligadas. Faleceu com 19h de p&#243;s-operat&#243;rio por hipoxemia.</p>
    <p>No subgrupo B3 dois faleceram, um com &#237;ndice arterial neopulmonar total de 322,23 e outro com &#237;ndice arterial neopulmonar total de 409,95. O 1&#186; com Blalock-Taussig &#224; direita associado &#224; unifica&#231;&#227;o de art&#233;rias colaterais sist&#234;mico-pulmonares na atresia pulmonar direita e ligadura de duas art&#233;rias colaterais sist&#234;mico-pulmonares; evoluiu com importante hipoxemia com 48h de p&#243;s-operat&#243;rio. O estudo cineangiocardiogr&#225;fico demonstrou Blalock-Taussig p&#233;rvio, confirmado na necropsia, onde foi encontrada uma microcircula&#231;&#227;o arterial pulmonar rarefeita com arter&#237;olas de paredes muito delgadas. O 2&#186; paciente apresentou oclus&#227;o do Blalock-Taussig com dois meses de p&#243;s-operat&#243;rio; evoluindo com hipoxemia foi realizada angioplastia no local da anastomose. Sem sucesso, foi utilizado novo enxerto e ampliadas as art&#233;rias pulmonares centrais com peric&#225;rdio aut&#243;logo, por&#233;m o paciente faleceu com 24h devido a importante hipoxemia.</p>
    <p>No subgrupo B4, um faleceu com &#237;ndice arterial neopulmonar total de 107,43. Com Blalock-Taussig &#224; esquerda e unifica&#231;&#227;o de uma art&#233;rias colaterais sist&#234;mico-pulmonares em atresia pulmonar esquerda, evoluiu no p&#243;s-operat&#243;rio imediato com oclus&#227;o do enxerto, o qual foi substitu&#237;do, por&#233;m no 3&#186; dia de p&#243;s-operat&#243;rio apresentou hipoxemia e fal&#234;ncia de m&#250;ltiplos &#243;rg&#227;os.</p>
    <p>No subgrupo B5 o paciente que faleceu apresentava &#237;ndice arterial neopulmonar total de 295,66. Com Blalock-Taussig &#224; esquerda e ligadura de art&#233;rias colaterais sist&#234;mico-pulmonares. As art&#233;rias pulmonares centrais eram extremamente hipopl&#225;sicas e o paciente evoluiu com quadro de septicemia (<italic>Serratia marcescens</italic>) e trombose do enxerto no 56&#186; dia de p&#243;s-operat&#243;rio.</p>
    <p>O &#237;ndice arterial neopulmonar total m&#233;dio dos pacientes que faleceram foi de 227,86 e dos que sobreviveram 216,68, mostrando que as caracter&#237;sticas morfom&#233;tricas n&#227;o foram importantes. A morfologia da &#225;rvore pulmonar foi fundamental e deve ser analisada para cada paciente, independentemente do subgrupo que perten&#231;a.</p>
    <p>Concluindo, as caracter&#237;sticas morfom&#233;tricas s&#227;o importantes, por&#233;m a morfologia do suprimento sang&#252;&#237;neo vascular pulmonar das art&#233;rias pulmonares centrais e art&#233;rias colaterais sist&#234;mico-pulmonares mostrou-se soberana na orienta&#231;&#227;o do tratamento cir&#250;rgico, o qual deve ser orientado de forma individualizada, independentemente da divis&#227;o did&#225;tica em subgrupos. O tratamento paliativo foi predominante e a mortalidade n&#227;o apresentou correla&#231;&#227;o com as caracter&#237;sticas morfom&#233;tricas.</p>
    <p>
      <fn id="back10" fn-type="corresp">
        <p>Endere&#231;o para correspond&#234;ncia</p>
        <p> Ulisses Alexandre Croti </p>
        <p> Rua Benjamin Constant, 4035/114 </p>
        <p> Cep 15015-600 - S&#227;o Jos&#233; do Rio Preto, SP </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="uacroti@cardiol.br">uacroti@cardiol.br</email>
        </p>
      </fn>
    </p>
    <p>Recebido para publica&#231;&#227;o em 11/8/03 </p>
    <p> Aceito em 11/2/04</p>
  </body>
  <back>
    <ref-list>
      <ref id="B1">
        <mixed-citation>1. Haworth SG, Macartney FJ. Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Br Heart J 1980; 44: 14-24.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries</article-title>
          <source>Br Heart J</source>
          <date>
            <year>1980</year>
          </date>
          <fpage>14</fpage>
          <lpage>24</lpage>
          <volume>44</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Haworth</surname>
              <given-names>SG</given-names>
            </name>
            <name>
              <surname>Macartney</surname>
              <given-names>FJ</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B2">
        <mixed-citation>2. Barbero Marcial M, Jatene AD. Surgical management of the anomalies of the pulmonary arteries in the tetralogy of Fallot with pulmonary atresia. Semin Thorac Cardiovasc Surg 1990; 2: 93-107.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Surgical management of the anomalies of the pulmonary arteries in the tetralogy of Fallot with pulmonary atresia</article-title>
          <source>Semin Thorac Cardiovasc Surg</source>
          <date>
            <year>1990</year>
          </date>
          <fpage>93</fpage>
          <lpage>107</lpage>
          <volume>2</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Barbero Marcial</surname>
              <given-names>M</given-names>
            </name>
            <name>
              <surname>Jatene</surname>
              <given-names>AD</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B3">
        <mixed-citation>3. Mac&#233; L, Dervanian P, Losay J, et al. D&#233;fauts d'arborisation pulmonaire des formes complexes d'atr&#233;sie pulmonaire &#224; septum ouvert: unification, unifocalisation et r&#233;paration compl&#232;te. Arch Mal Coeur 1996; 89: 561-8.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>D&#233;fauts d'arborisation pulmonaire des formes complexes d'atr&#233;sie pulmonaire &#224; septum ouvert: unification, unifocalisation et r&#233;paration compl&#232;te</article-title>
          <source>Arch Mal Coeur</source>
          <date>
            <year>1996</year>
          </date>
          <fpage>561</fpage>
          <lpage>8</lpage>
          <volume>89</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Mac&#233;</surname>
              <given-names>L</given-names>
            </name>
            <name>
              <surname>Dervanian</surname>
              <given-names>P</given-names>
            </name>
            <name>
              <surname>Losay</surname>
              <given-names>J</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B4">
        <mixed-citation>4. Barbero Marcial M. Classification of pulmonary atresia with ventricular septal defect. Ann Thorac Surg 2001; 72: 316-7.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Classification of pulmonary atresia with ventricular septal defect</article-title>
          <source>Ann Thorac Surg</source>
          <date>
            <year>2001</year>
          </date>
          <fpage>316</fpage>
          <lpage>7</lpage>
          <volume>72</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Barbero Marcial</surname>
              <given-names>M</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B5">
        <mixed-citation>5. Croti UA, Barbero Marcial ML, Oliveira SA. Atresia pulmonar com comunica&#231;&#227;o interventricular. Arq Bras Cardiol 2002; 78: 521-3.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Atresia pulmonar com comunica&#231;&#227;o interventricular</article-title>
          <source>Arq Bras Cardiol</source>
          <date>
            <year>2002</year>
          </date>
          <fpage>521</fpage>
          <lpage>3</lpage>
          <volume>78</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Croti</surname>
              <given-names>UA</given-names>
            </name>
            <name>
              <surname>Barbero Marcial</surname>
              <given-names>ML</given-names>
            </name>
            <name>
              <surname>Oliveira</surname>
              <given-names>SA</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B6">
        <mixed-citation>6. Nakata S, Imai Y, Takanashi Y, et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984; 88: 610-9.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow</article-title>
          <source>J Thorac Cardiovasc Surg</source>
          <date>
            <year>1984</year>
          </date>
          <fpage>610</fpage>
          <lpage>9</lpage>
          <volume>88</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Nakata</surname>
              <given-names>S</given-names>
            </name>
            <name>
              <surname>Imai</surname>
              <given-names>Y</given-names>
            </name>
            <name>
              <surname>Takanashi</surname>
              <given-names>Y</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B7">
        <mixed-citation>7. Reddy VM, Petrossian E, Mc Elhinney DB, Moore P, Teitel DF, Hanley FL. One-stage complete unifocalization in infants: When should the ventricular septal defect be closed? J Thorac Cardiovasc Surg 1997; 113: 858-68.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>One-stage complete unifocalization in infants: When should the ventricular septal defect be closed?</article-title>
          <source>J Thorac Cardiovasc Surg</source>
          <date>
            <year>1997</year>
          </date>
          <fpage>858</fpage>
          <lpage>68</lpage>
          <volume>113</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Reddy</surname>
              <given-names>VM</given-names>
            </name>
            <name>
              <surname>Petrossian</surname>
              <given-names>E</given-names>
            </name>
            <name>
              <surname>Mc</surname>
              <given-names>Elhinney DB</given-names>
            </name>
            <name>
              <surname>Moore</surname>
              <given-names>P</given-names>
            </name>
            <name>
              <surname>Teitel</surname>
              <given-names>DF</given-names>
            </name>
            <name>
              <surname>Hanley</surname>
              <given-names>FL</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B8">
        <mixed-citation>8. Behrman RE, Kliegman RM, Arvin AM, Nelson WE. Laboratory medicine and reference tables. In: Behrman RE, Kliegman RM, Arvin AM, Nelson WE, eds. Textbook of Pediatrics. 15th Ed. Philadelphia: WB Saunders, 1996: 2079.</mixed-citation>
        <element-citation publication-type="book">
          <source>Textbook of Pediatrics</source>
          <date>
            <year>1996</year>
          </date>
          <fpage>2079</fpage>
          <person-group person-group-type="author">
            <name>
              <surname>Behrman</surname>
              <given-names>RE</given-names>
            </name>
            <name>
              <surname>Kliegman</surname>
              <given-names>RM</given-names>
            </name>
            <name>
              <surname>Arvin</surname>
              <given-names>AM</given-names>
            </name>
            <name>
              <surname>Nelson</surname>
              <given-names>WE</given-names>
            </name>
            <name>
              <surname>Behrman</surname>
              <given-names>RE</given-names>
            </name>
            <name>
              <surname>Kliegman</surname>
              <given-names>RM</given-names>
            </name>
            <name>
              <surname>Arvin</surname>
              <given-names>AM</given-names>
            </name>
            <name>
              <surname>Nelson</surname>
              <given-names>WE</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B9">
        <mixed-citation>9. Tchervenkov    CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary    atresia  ventricular septal defect. Ann Thorac Surg 2000; 69: S97-105.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia - ventricular septal defect</article-title>
          <source>Ann Thorac Surg</source>
          <date>
            <year>2000</year>
          </date>
          <fpage>S97</fpage>
          <lpage>105</lpage>
          <volume>69</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Tchervenkov</surname>
              <given-names>CI</given-names>
            </name>
            <name>
              <surname>Roy</surname>
              <given-names>N</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B10">
        <mixed-citation>10. Somerville J. Management of pulmonary atresia. Br Heart J 1970; 32: 641-51.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Management of pulmonary atresia</article-title>
          <source>Br Heart J</source>
          <date>
            <year>1970</year>
          </date>
          <fpage>641</fpage>
          <lpage>51</lpage>
          <volume>32</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Somerville</surname>
              <given-names>J</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B11">
        <mixed-citation>11. Macartney FJ, Scott O, Deverall PB. Haemodynamic and anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect - including a case of persistent fifth aortic arch. Br Heart J 1974; 36: 1049-60.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Haemodynamic and anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect: including a case of persistent fifth aortic arch</article-title>
          <source>Br Heart J</source>
          <date>
            <year>1974</year>
          </date>
          <fpage>1049</fpage>
          <lpage>60</lpage>
          <volume>36</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Macartney</surname>
              <given-names>FJ</given-names>
            </name>
            <name>
              <surname>Scott</surname>
              <given-names>O</given-names>
            </name>
            <name>
              <surname>Deverall</surname>
              <given-names>PB</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B12">
        <mixed-citation>12. Sullivan ID,    Wren C, Stark J, De Leval MR, Macartney FJ, Deanfield JE. Surgical unifocalization    in pulmonary atresia and ventricular septal defect. A realistic goal? Circulation    1988; 78: III-5-13.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Surgical unifocalization in pulmonary atresia and ventricular septal defect: A realistic goal?</article-title>
          <source>Circulation</source>
          <date>
            <year>1988</year>
          </date>
          <fpage>III</fpage>
          <volume>78</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Sullivan</surname>
              <given-names>ID</given-names>
            </name>
            <name>
              <surname>Wren</surname>
              <given-names>C</given-names>
            </name>
            <name>
              <surname>Stark</surname>
              <given-names>J</given-names>
            </name>
            <name>
              <surname>De Leval</surname>
              <given-names>MR</given-names>
            </name>
            <name>
              <surname>Macartney</surname>
              <given-names>FJ</given-names>
            </name>
            <name>
              <surname>Deanfield</surname>
              <given-names>JE</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B13">
        <mixed-citation>13. Carotti A, Di Donato RM, Squitieri C, Guccione P, Catena G. Total repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: an integrated approach. J Thorac Cardiovasc Surg 1998; 116: 914-23.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Total repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: an integrated approach</article-title>
          <source>J Thorac Cardiovasc Surg</source>
          <date>
            <year>1998</year>
          </date>
          <fpage>914</fpage>
          <lpage>23</lpage>
          <volume>116</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Carotti</surname>
              <given-names>A</given-names>
            </name>
            <name>
              <surname>Di Donato</surname>
              <given-names>RM</given-names>
            </name>
            <name>
              <surname>Squitieri</surname>
              <given-names>C</given-names>
            </name>
            <name>
              <surname>Guccione</surname>
              <given-names>P</given-names>
            </name>
            <name>
              <surname>Catena</surname>
              <given-names>G</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B14">
        <mixed-citation>14. Shimazaki Y, Tokuan Y, Lio M, et al. Pulmonary artery pressure and resistance late after repair of tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 1990; 100: 425-40.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Pulmonary artery pressure and resistance late after repair of tetralogy of Fallot with pulmonary atresia</article-title>
          <source>J Thorac Cardiovasc Surg</source>
          <date>
            <year>1990</year>
          </date>
          <fpage>425</fpage>
          <lpage>40</lpage>
          <volume>100</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Shimazaki</surname>
              <given-names>Y</given-names>
            </name>
            <name>
              <surname>Tokuan</surname>
              <given-names>Y</given-names>
            </name>
            <name>
              <surname>Lio</surname>
              <given-names>M</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B15">
        <mixed-citation>15. Puga FJ, Leoni FE, Julsrud PR, Mair DD. Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries: experience with preliminary unifocalization procedures in 38 patients. J Thorac Cardiovasc Surg 1989; 98: 1018-29.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries: experience with preliminary unifocalization procedures in 38 patients</article-title>
          <source>J Thorac Cardiovasc Surg</source>
          <date>
            <year>1989</year>
          </date>
          <fpage>1018</fpage>
          <lpage>29</lpage>
          <volume>98</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Puga</surname>
              <given-names>FJ</given-names>
            </name>
            <name>
              <surname>Leoni</surname>
              <given-names>FE</given-names>
            </name>
            <name>
              <surname>Julsrud</surname>
              <given-names>PR</given-names>
            </name>
            <name>
              <surname>Mair</surname>
              <given-names>DD</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
      <ref id="B16">
        <mixed-citation>16. Reddy VM, Liddicoat    JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary    atresia with ventricular septal defect and major aortopulmonary collaterals.    J Thorac Cardiovasc Surg 1995; 109: 832-45.</mixed-citation>
        <element-citation publication-type="journal">
          <article-title>Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals</article-title>
          <source>J Thorac Cardiovasc Surg</source>
          <date>
            <year>1995</year>
          </date>
          <fpage>832</fpage>
          <lpage>45</lpage>
          <volume>109</volume>
          <person-group person-group-type="author">
            <name>
              <surname>Reddy</surname>
              <given-names>VM</given-names>
            </name>
            <name>
              <surname>Liddicoat</surname>
              <given-names>JR</given-names>
            </name>
            <name>
              <surname>Hanley</surname>
              <given-names>FL</given-names>
            </name>
          </person-group>
        </element-citation>
      </ref>
    </ref-list>
  </back>
  <sub-article article-type="translation" id="TRen" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Original Articles</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title xml:lang="en">Assessment of the pulmonary vascular blood supply in patients with pulmonary atresia, ventricular septal defect, and aortopulmonary collateral arteries</article-title>
      </title-group>
      <abstract xml:lang="en">
        <p>OBJECTIVE: To study the morphometric characteristics of the central pulmonary arteries and aortopulmonary collateral arteries by assessing the morphology of the pulmonary vascular blood supply, to determine their significance in surgical treatment. METHOD: From January 1990 to June 2001, 40 patients were studied. Those who had the complete cineangiocardiographic study prior to the first surgical intervention were included in the study. The morphometric characteristics of the central pulmonary arteries (PPAA) and aortopulmonary collateral arteries (APCA) were analyzed, as was the distribution of blood irrigation to the lungs. The following indices were calculated: pulmonary arterial index (PPAAI), aortopulmonary collateral arterial index (APCAI), and total neopulmonary arterial index (TNPAI = PPAAI + APCAI). The surgical treatment was considered palliative (PT), palliative definitive (PDT), and definitive (DT). RESULTS: The palliative treatment predominated. No statistically significant differences were observed in the patients undergoing PT, PDT, and DT, in regard to PPAAI, APCAI, and TNPAI. Comparing PPAAI and APCAI, no difference was observed for DT (P=0.4309); APCAI was greater than PPAAI for PT (P=0.0176); and APCAI was descriptively greater for PDT. The TNPAI of patients undergoing DT was greater than that of patients undergoing PT (P=0.0959). Five morphologically similar subgroups were identified and designated as B1, B2, B3, B4, and B5. Overall mortality was 17.5%. CONCLUSION: The morphometric characteristics are important, but the morphology of the pulmonary vascular blood supply of the PPAA and APCA proved to be better for guiding the surgical treatment. Independently of the didactical division into subgroups, PT predominated. Mortality was not correlated with the morphometric characteristics.</p>
      </abstract>
      <kwd-group xml:lang="en">
        <kwd>pulmonary atresia</kwd>
        <kwd>ventricular septal defect</kwd>
        <kwd>aortopulmonary collateral arteries</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>
        <bold>ORIGINAL ARTICLE</bold>
      </p>
      <p>Assessment of the pulmonary vascular blood supply in patients with pulmonary atresia, ventricular septal defect, and aortopulmonary collateral arteries</p>
      <p>Ulisses Alexandre Croti; Miguel Lorenzo Barbero Marcial; Carla Tanamati; Marcelo Biscegli Jatene; Domingo Marcolino Braile; Sergio Almeida de Oliveira</p>
      <p> S&#227;o Paulo, SP - Brazil</p>
      <p>Instituto do Cora&#231;&#227;o of the Hospital das Cl&#237;nicas of the Medical School of the Universidade de S&#227;o Paulo</p>
      <p>
        <xref ref-type="corresp" rid="back10-body2">Correspondence</xref>
      </p>
      <p>
        <bold>ABSTRACT</bold>
      </p>
      <p><bold>OBJECTIVE: </bold>To study the morphometric characteristics of the central pulmonary arteries and aortopulmonary collateral arteries by assessing the morphology of the pulmonary vascular blood supply, to determine their significance in surgical treatment.</p>
      <p><bold>METHOD: </bold>From January 1990 to June 2001, 40 patients were studied. Those who had the complete cineangiocardiographic study prior to the first surgical intervention were included in the study. The morphometric characteristics of the central pulmonary arteries (PPAA) and aortopulmonary collateral arteries (APCA) were analyzed, as was the distribution of blood irrigation to the lungs. The following indices were calculated: pulmonary arterial index (PPAAI), aortopulmonary collateral arterial index (APCAI), and total neopulmonary arterial index (TNPAI = PPAAI + APCAI). The surgical treatment was considered palliative (PT), palliative definitive (PDT), and definitive (DT).</p>
      <p><bold>RESULTS:</bold> The palliative treatment predominated. No statistically significant differences were observed in the patients undergoing PT, PDT, and DT, in regard to PPAAI, APCAI, and TNPAI. Comparing PPAAI and APCAI, no difference was observed for DT (P=0.4309); APCAI was greater than PPAAI for PT (P=0.0176); and APCAI was descriptively greater for PDT. The TNPAI of patients undergoing DT was greater than that of patients undergoing PT (P=0.0959). Five morphologically similar subgroups were identified and designated as B1, B2, B3, B4, and B5. Overall mortality was 17.5%.</p>
      <p><bold>CONCLUSION: </bold> The morphometric characteristics are important, but the morphology of the pulmonary vascular blood supply of the PPAA and APCA proved to be better for guiding the surgical treatment. Independently of the didactical division into subgroups, PT predominated. Mortality was not correlated with the morphometric characteristics.</p>
      <p><bold>Key words: </bold>pulmonary atresia, ventricular septal defect, aortopulmonary collateral arteries</p>
      <p>In pulmonary atresia (PA) with ventricular septal defect (VSD) and aortopulmonary collateral arteries (APCA), the pulmonary vascular blood supply may be very complex with great anatomical variations, so that some segments of the pulmonary lobes may be supplied by blood flow from the central pulmonary arteries (PPAA) or APCA, or both <sup>1</sup>. Due to this extreme heterogeneity, several anatomical classifications have been proposed, aiming at establishing criteria that rationalize the surgical approach. The currently most-used morphological classification <sup>2-5</sup> divides the patients with PA and VSD into 3 different groups: A, B, and C. In group A, all pulmonary segments are supplied by PPAA. In group B, some pulmonary segments are supplied by PPAA, while others are supplied by APCA. In group C, all pulmonary segments are supplied by APCA. PPAA are absent <sup>5</sup>.</p>
      <p>Based on the analysis of the cineangiocardiographic studies of patients with pulmonary atresia, ventricular septal defect, and aortopulmonary collateral arteries (group B), the present study aimed at assessing the morphometric characteristics of the central pulmonary and aortopulmonary collateral arteries, through the assessment of the distribution of the pulmonary vascular blood supply, in an attempt to determine their significance in surgical treatment.</p>
      <p>
        <bold>Methods</bold>
      </p>
      <p>This study comprised 40 patients with PA, VSD, and APCA operated upon at the Instituto do Cora&#231;&#227;o of the Hospital das Cl&#237;nicas of the Medical School of the University of S&#227;o Paulo from January 1990 to June 2001.</p>
      <p>This study was approved by the Scientific and Ethical Committee for Analysis of Research Projects of the Instituto do Cora&#231;&#227;o of the Hospital das Cl&#237;nicas of the Medical School of the Universidade de S&#227;o Paulo.</p>
      <p>The age in the cineangiocardiographic studies ranged from 9 days to 21.6 years (median of 1.1 year). The age in the surgical procedures ranged from 3.7 months to 21.6 years (median of 1.9 years). Twenty-one (52.50%) patients were males and 19 (47.50%) were females.</p>
      <p>Patients with morphology of <italic>situs solitus</italic> and concordant atrioventricular and ventriculoarterial connections with complete cineangiocardiographic studies prior to the first surgical intervention exclusively performed at our institution were included in the study. The medical record analysis focused on the reports of the first cineangiocardiographic study and the descriptions of all 56 surgeries performed.</p>
      <p>The following patients were excluded from the study: those in whom the results were not available of the cineangiocardiographic study performed before the initial surgical treatment and those in whom not all the possible techniques for demonstrating the pulmonary vascular blood supply were used, resulting in an incomplete examination.</p>
      <p>The complete cineangiocardiographic study comprised the following: injections of contrast medium into the ascending and descending aorta and aortic arch; right and left ventriculographies; selective injections into the ductus arteriosus, PPAA, APCA, and coronary arteries; and retrograde venography. The injections of contrast medium, mainly the selective one into the PPAA and APCA, and retrograde venographies should identify the different anatomical variations and segments supplied.</p>
      <p>For quantitative morphometric analysis, CAAS II (cardiovascular angiography analysis system II) software was used. The computer was coupled to an LVA-7000 laser videodisc player (JVC), an SVO-9500 MD videocassette recorder (SONY), and a Tagarno cinevideo camera to allow the analysis of films recorded on digital disk, VHS tape, and 35 mm film.</p>
      <p>The system was calibrated based on the diameter of the distal portion of the catheter, which was calculated with CAAS II software, according to the data previously provided regarding the catheter used during that examination, as for example 5Fr or 6Fr.</p>
      <p>The images selected for measurement were in the posteroanterior view, with the vascular segment of interest well contrasted and in ventricular systole.</p>
      <p>After this calibration, the PPAA and APCA diameters were measured. The PPAA were measured immediately proximal to the origin of the first lobar branch <sup>6</sup>, and the APCA were measured distally to the point where surgical unifocalization would be ideally possible <sup>7</sup>.</p>
      <p>With these measurements obtained, the area of the blood vessels was calculated.</p>
      <p>Body surface was calculated by applying the Mosteller formula <sup>8</sup> to the patients' weights and heights known on the occasion of the examination.</p>
      <p>Therefore, the following indices could be calculated: PPAAI (pulmonary arterial index), APCAI (aortopulmonary collateral arterial index), and TNPAI (total neopulmonary arterial index), as shown in <xref ref-type="fig" rid="fig1-body2">figure 1</xref>.</p>
      <fig id="fig1-body2">
        <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gf01-en.jpg"/></fig>
      <p>Because some pulmonary segments were supplied by the branches of the PPAA while others were supplied by the APCA, the TNPAI was calculated.</p>
      <p>The area was obtained by measuring the PPAA immediately before bifurcation of the lobar branches, as already described <sup>6</sup>.</p>
      <p>The pulmonary artery was considered hypoplastic when its area had a 25% or greater reduction as compared with the mean areas of PPAA.</p>
      <p>When PPAAI was smaller than 100 mm<sup>2</sup>/m<sup>2</sup>, the PPAA were considered hypoplastic <sup>6</sup>.</p>
      <p>When TNPAI was smaller than 100 mm<sup>2</sup>/m<sup>2</sup>, the need for assessing the ratio between PPAAI and APCAI was considered.</p>
      <p>The diameter of the APCA was measured distally to the point, in which surgical unifocalization would be ideally possible, as previously described <sup>7</sup>.</p>
      <p>Stenosis of the APCA was characterized by a luminal reduction greater than 25% <sup>7</sup>. The vessel was considered thin when its area was smaller than 50% of the arithmetic mean of the areas of all APCA; the vessel was considered medium when its area was greater than 50% of the arithmetic mean and smaller than the arithmetic mean of all APCA; and the vessel was considered wide when its area was greater than the arithmetic mean of the areas of all APCA of the patient.</p>
      <p>When APCAI was smaller than 100 mm<sup>2</sup>/m<sup>2</sup>, the APCA were considered to have an overall reduced caliber.</p>
      <p>Analyzing the areas of pulmonary vascular blood supply, the pulmonary lobes irrigated by the PPAA and those irrigated by the APCA were identified. The relation between the pulmonary lobes and segments was defined as follows: left superior lobe (apicoposterior, anterior, superior lingular, inferior lingular segments); left inferior lobe (superior, anteromedial basal, lateral basal, posterior basal segments); right superior lobe (apical, anterior, posterior segments); right middle lobe (lateral and medial segments); right inferior lobe (superior, middle basal, anterior basal, lateral basal, posterior basal segments) <sup>9</sup>.</p>
      <p>When the injection of contrast medium into one of the arteries filled the contralateral pulmonary branch, confluence between the PPAA was considered.</p>
      <p>The arteries that supplied at least one intrapulmonary segmentary artery and had a trajectory independent of the pulmonary bronchi and intercostal arteries were considered APCA.</p>
      <p>The surgical treatments were classified as palliative, "palliative definitive," or definitive.</p>
      <p>Palliative treatment was that in which the patients underwent interventions without closure of the VSD and had future perspectives of conclusion or not of the treatment. They were considered as being under treatment.</p>
      <p>"Palliative definitive" treatment was that in which the patients underwent interventions without closure of the VSD and had no other surgical option that enabled the conclusion of the treatment, with RV-PPAA connection and VSD closure. This was the case when the diameter of the PPAA was extremely reduced with no possibility of development, or when the distal pulmonary vascular tree showed signs of pulmonary hypertensive disease with or without thrombosis, or when unifocalization of at least 10 pulmonary segments for connection to RV was not possible <sup>14</sup>.</p>
      <p>The definitive treatment consisted of RV-PPAA connection and closure of the VSD, allowing reestablishment of the normal physiology. The criteria for closure of the VSD were as follows: presence of PPAA with appropriate calibers, adequate distal pulmonary vascular tree, and presence of at least 10 pulmonary segments that could be connected to the RV.</p>
      <p>
        <bold>Results</bold>
      </p>
      <p>The morphometric analysis of the cineangiocardiographic studies performed before the first surgical procedure and the stages of treatment of all patients are described in <xref ref-type="table" rid="tab1-body2">table 1</xref>.</p>
      <table-wrap id="tab1-body2">
        <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gt01-en.jpg"/></table-wrap>
      <p>Associating the morphometric data obtained with the distribution of the pulmonary vascular blood supply, 5 subgroups with similar characteristics were identified as follows: B1, B2, B3, B4, and B5 (<xref ref-type="fig" rid="fig2-body2">figure 2</xref>). Subgroup B1 comprised 8 (20%) patients, subgroup B2 comprised 4 (10%) patients, subgroup B3 comprised 16 (40%) patients, subgroup B4 comprised 4 (10%) patients, and subgroup B5 comprised 8 (20%) patients.</p>
      <fig id="fig2-body2">
        <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gf02-en.jpg"/></fig>
      <p>All subgroup B1 patients had PPAA supplying the segments of the left superior and right inferior lobes. In some patients, the APCA were also present in those segments; the PPAA, however, predominated. All other segments were irrigated by the APCA.</p>
      <p>All subgroup B2 patients had PPAA supplying the segments of the right superior and left inferior lobes. In some patients, the APCA were also present in those segments; the PPAA, however, predominated. All other segments were irrigated by the APCA.</p>
      <p>All subgroup B3 patients had PPAA supplying the segments of the left and right inferior lobes or supplying the segments of one of the inferior lobes and most of the segments of the lobes of the contralateral lung.</p>
      <p>All subgroup B4 patients had PPAA supplying the segments of the left and right superior lobes or supplying the segments of one of the superior lobes and most of the segments of the lobes of the contralateral lung.</p>
      <p>The subgroup B5 patients varied greatly in regard to the distribution of the PPAA and APCA, which resulted in great difficulty in defining their blood supply to the pulmonary segments. Therefore, that was a miscellaneous subgroup. Three patients had agenesis of the left pulmonary artery, 2 had agenesis of the right pulmonary artery, 2 had fistulas from the left main coronary artery to the pulmonary trunk, and one had hypoplastic PPAA. Adequate identification of which pulmonary segments were supplied by PPAA or APCA could not be performed in all those patients.</p>
      <p>An alpha error of 5% was admitted in the statistical analysis. The parametric Student <italic>t</italic> test and the nonparametric Kruskal-Wallis and Wilcoxon tests were used. The subgroups were correlated with the presence of stenoses, TNPAI (PPAAI and APCAI), the number of surgical procedures, and the stage of the treatment.</p>
      <p>More subgroup B2 patients underwent the definitive treatment than the patients in the other subgroups B; this, however, had no statistical significance.</p>
      <p>The only patients undergoing palliative definitive treatment were in subgroup B1.</p>
      <p>No patient undergoing 3 procedures underwent definitive treatment. Most patients who had definitive treatment underwent 2 procedures.</p>
      <p>All patients with stenosis in at least one PPAA underwent palliative treatment.</p>
      <p>The presence of stenosis in the APCA showed no relation to the stage of treatment of the patients.</p>
      <p>The number of patients undergoing one, 2, and 3 procedures in the subgroups B1 and B3 is very similar. The same was observed in subgroups B2 and B4. Most patients in subgroup B5 underwent only one procedure, a smaller proportion than that in all other subgroups.</p>
      <p>No statistically significant difference between the patients undergoing the palliative, palliative definitive, and definitive treatments was observed in regard to the indices PPAAI, APCAI, and TNPAI. Comparing PPAAI and APCAI for each stage of the treatment, the following was observed: no difference occurred between the indices in the definitive stage (P=0.4309); the APCAI was statistically greater than the PPAAI in the palliative stage (P=0.0176); descriptively, the APCAI was greater than the PPAAI in the palliative definitive stage. The TNPAI of patients undergoing definitive treatment was greater than that of patients undergoing palliative treatment (P=0.0959), as shown in <xref ref-type="table" rid="tab2-body2">table 2</xref>.</p>
      <table-wrap id="tab2-body2">
        <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gt02-en.jpg"/></table-wrap>
      <p>The greatest proportion of patients who died was found in subgroups B1, B2, and B4; in subgroups B3 and B5, that proportion was smaller. In subgroups B, the number of patients who died was as follows: subgroup B1, 2 (25%) patients; subgroup B2, one (25%) patient; subgroup B3, 2 (12.5%) patients; subgroup B4, one (25%) patient; and subgroup B5, one (12.5%) patient. Considering the entire group, 7 (17.5%) patients died. The greater the number of procedures performed, the greater the proportion of dead patients.</p>
      <p>Although lacking statistical significance, the indices of the patients who died were lower than those of the patients who survived. The relation between the mean of the indices and the stage of the treatment for the patients who died and those who survived is shown in <xref ref-type="table" rid="tab3-body2">table 3</xref>.</p>
      <table-wrap id="tab3-body2">
        <graphic xlink:href="1678-4170-abc-84-01-0003-00002-gt03-en.jpg"/></table-wrap>
      <p>
        <bold>Discussion</bold>
      </p>
      <p>In 1970, Somerville classified pulmonary atresia with VSD as types A, B, C, and D, based on the degree of the development of the PPAA distal to the atresic segment <sup>10</sup>. Comparing that classification with the one adopted in our study, type C (atresia of the pulmonary valve and trunk and one of the pulmonary arteries) in the Somerville classification corresponds to our group B, in which the pulmonary segments are irrigated by the PPAA and APCA <sup>5</sup>.</p>
      <p>Investigation of the hemodynamic aspects of the pulmonary circulation, using selective catheterization and occlusion with a balloon for visualizing APCA, showed that some pulmonary segments are supplied by blood flow originating from the PPAA, while others are supplied by the APCA <sup>11</sup>. Based on this, the important concept of unifocal or multifocal blood supply to the pulmonary circulation was introduced. This knowledge provided great advancement in the treatment of PA with VSD and APCA with the new surgical techniques of unifocalization <sup>12</sup>, which were used in some patients in the group.</p>
      <p>When the blood supply is unifocal, the method proposed by Nakata et al <sup>6</sup> may be used, which allows the calculation of the areas of the PPAA, and, consequently, the obtainment of PPAAI <sup>6</sup>.</p>
      <p>Similarly to the way Nakata et al <sup>6</sup> (1984) calculated the areas of the PPAA (PPAAI), Reddy et al <sup>7</sup> (1997) calculated the areas of the APCA for patients with multifocal blood supply, who underwent unifocalizations in a single stage, closure of the VSD, and connection between RV and the pulmonary tree. Therefore, the aortopulmonary collateral arterial index (APCAI) was obtained. Addition of the PPAAI and the APCAI provided the total neopulmonary arterial index (TNPAI) used in our study.</p>
      <p>The one-stage correction was proposed for only one subgroup B2 patient, who died due to low cardiac output syndrome. Reddy et al <sup>7</sup> (1997) also suggested the intraoperative study of blood flow. This is as follows: after complete unifocalization and anastomosis of the extracardiac tube while the patient is still under extracorporeal circulation, a cannula is placed in the tube, and a predetermined flow (cardiac output) is injected up to 2.5 L/min/m<sup>2</sup>, accompanied by a vigorous aspiration of the left atrium. On that occasion, with a catheter in the pulmonary arteries, pressure measurements are taken. Those measurements may or may not suggest closure of the VSD.</p>
      <p>The ventricular septal defect may be closed, completing the definitive treatment more safely in patients with TNPAI greater than 200 mm<sup>2</sup>/m<sup>2</sup>. When TNPAI is lower than 200 mm<sup>2</sup>/m<sup>2</sup>, other physiological data should be added for one-stage complete correction <sup>7</sup>. Others consider it a worse prognosis when TNPAI is lower than 150 mm<sup>2</sup>/m<sup>2</sup><sup>13</sup>.</p>
      <p>Five subgroups with similar characteristics were identified: B1, B2, B3, B4, and B5. The mean TNPAI of patients undergoing definitive treatment was 308.66, and, in subgroups B1, B2, B3, and B5, the mean TNPAI was, respectively, 156.65, 230.03, 431.02, and 128.50. In subgroup B4, no patient underwent definitive treatment.</p>
      <p>Of the 8 subgroup B1 patients, only one had all segments of the left superior lobe and right inferior lobe exclusively supplied by the PPAA. The others had one or 2 lobes supplied mainly by the PPAA, but with the presence of APCA. These patients had at least 9 segments with PPAA present, a fact that did not favor definitive surgical correction <sup>14</sup>.</p>
      <p>Only one patient underwent definitive treatment. That patient had a PPAAI significantly greater than APCAI, showing that, for this type of patient, the presence of developed PPAA is a factor of fundamental importance for the appropriate surgical treatment <sup>15</sup>.</p>
      <p>The mean TNPAI of the patients undergoing definitive treatment (156.65) was lower than that of patients undergoing palliative definitive treatment (278.84). This occurred due to the high values of APCAI. The 2 patients who underwent palliative definitive treatment had extremely hypoplastic PPAA, which did not develop after the first surgical procedure.</p>
      <p>Of the 4 patients in subgroup B2, 2 had the right superior lobe and left inferior lobe exclusively supplied by PPAA, and 2 also had APCA, but PPAA predominated. Those patients had only 7 pulmonary segments irrigated by PPAA, justifying the greater mean of APCA supplying the pulmonary segments of those patients.</p>
      <p>Two patients underwent definitive treatment and 2 patients underwent palliative treatment. No correlation with TNPAI, which was greater than 200 mm<sup>2</sup>/m<sup>2</sup> in all patients, was observed <sup>7</sup>. Only one patient had PPAAI greater than APCAI. That patient underwent palliative treatment, clearly showing that no relation existed between morphometry and morphology for defining which patient underwent definitive treatment.</p>
      <p>Only one patient underwent bilateral unifocalization and one-stage ventricular septal defect closure <sup>15</sup>. Although his TNPAI was 236.27 mm<sup>2</sup>/m<sup>2</sup>, and, therefore, concordant with the recommendation of total correction when TNPAI is greater than 200 mm<sup>2</sup>/m<sup>2</sup>, the patient evolved with low cardiac output and cyanosis, and died within 3 hours at the intensive care unit.</p>
      <p>Five patients in subgroup B3 had less than 10 segments supplied by the PPAA, all others had 13 or 14. Four patients achieved the definitive treatment with mean TNPAI of 431.02. One patient had PPAAI lower than APCAI, but TNPAI of 211.60. The others had PPAAI clearly greater than APCAI. This suggests that of the other patients undergoing palliative treatment (mean TNPAI of 233.45), those with PPAAI greater than APCAI have a greater chance of reaching definitive treatment, similarly to that reported by Carotti et al <sup>13</sup>.</p>
      <p>In subgroup B4, only one patient had 7 segments supplied by PPAA, all others had 14 segments, and yet none of them underwent definitive treatment. The mean TNPAI was 150.57 mm2/m2, with PPAAI smaller than their respective APCAI. The morphological characteristics were more important and significant in the choosing of the treatment. All patients were in the palliative treatment stage, and could evolve to palliative definitive or definitive, but the morphometric characteristics did not favor the definitive treatment <sup>13</sup>.</p>
      <p>In subgroup B5, only one patient underwent definitive treatment with one single procedure and had at least 14 pulmonary segments supplied by PPAA, with TNPAI of 128.50 and PPAAI greater than its APCAI. The other patients underwent palliative treatment, independently of their indices, showing that the morphological characteristics are more important than the morphometric ones in that subgroup.</p>
      <p>Of the 40 patients in group B, only 2 underwent palliative definitive treatment and were coincidentally in subgroup B1, both with hypoplastic PPAA. Their TNPAI were 48.79 and 508.89. The latter had great APCA with signs of hypertension and irreversible pulmonary vascular disease, which led him to be considered to have no surgical option for definitive treatment.</p>
      <p>It is worth noting that no difference existed between PPAAI and APCAI for the patients undergoing definitive treatment. For those in the palliative and palliative definitive stages, however, APCAI was greater, showing that when APCA predominates, the palliative treatment is more likely to be used.</p>
      <p>Thus, independently of the didactical subgroup to which the patient may belong, it is clear that the morphometric measures may help in guiding the treatment. However, the characteristics of irrigation of the pulmonary segments by PPAA or APCA, or both, are mandatory for patient's management and should be analyzed for each patient individually, as already reported by other authors <sup>14</sup>.</p>
      <p>Overall mortality was 17.50%, and, in subgroup B1, 2 patients died, one with TNPAI of 48.79 and the other with TNPAI of 174.69. The first patient had PPAAI of 2.80, ie, extremely hypoplastic PPAA. Thus, after undergoing the Blalock-Taussig (BT) procedure on the right- and left-hand sides, the patient remained hypoxic. As the APCA had unfavorable anatomy, unifocalization was not possible, and the patient evolved with occlusion of the BT, dying due to hypoxia. In the second patient, a connection between the RV and the PPAA was performed with a PTFE tube; because the VSD was left opened, the patient died on the second postoperative day, due to low cardiac output.</p>
      <p>In subgroup B2, one patient with a TNPAI of 236.27 died. The patient underwent bilateral transversal thoracotomy with bilateral unification <sup>16</sup> (one APCA on the left-hand side with the left pulmonary artery and 2 APCA on the right-hand side), ligature of one APCA on the right-hand side, and enlargement of the right ventricular outflow tract with bovine pericardium. Left BT and release of one of the ligated APCA were chosen to be performed. The patient died 19 hours after the procedure, due to hypoxemia.</p>
      <p>In subgroup B3, 2 patients died; their TNPAI was 322.23 and 409.95. The first patient had BT on the right-hand side associated with unification of APCA with the left pulmonary artery and ligature of 2 APCA. The patient developed significant hypoxemia 48 hours after surgery. The cineangiocardiographic study showed a patent BT, which was confirmed during autopsy, which also evidenced a rarefied pulmonary arterial microcirculation with very thin-walled arterioles. The second patient had occlusion of the BT 2 months after surgery and evolved with hypoxemia. Angioplasty was performed at the site of the anastomosis, but without success. A new graft was used, and the PPAA were widened with autologous pericardium, but the patient died after 24 hours due to significant hypoxemia.</p>
      <p>In subgroup B4, one patient with a TNPAI of 107.43 died. The patient had BT on the left-hand side and unification of one APCA with the left pulmonary artery, and evolved in the immediate postoperative period with occlusion of the graft, which was replaced. On the third postoperative day, however, the patient experienced hypoxemia and multiple organ failure.</p>
      <p>In subgroup B5, the patient who died had a TNPAI of 295.66. The patient had a BT on the left-hand side and ligature of the APCA. The PPAA were extremely hypoplastic, and the patient developed septicemia (<italic>Serratia marcescens</italic>) and thrombosis of the graft on the 56th postoperative day.</p>
      <p>The mean TNPAI of the patients who died was 227.86 and that of those who survived was 216.68, showing that the morphometric characteristics were not important. Morphology of the pulmonary tree is fundamental and should be analyzed for each patient, independently of the patient's subgroup.</p>
      <p>Morphometric characteristics are important, but the morphology of the pulmonary vascular blood supply of the central pulmonary arteries and the aortopulmonary collateral arteries proved to be paramount in guiding surgical treatment, which should be handled in an individualized manner, independently of the didactical division of the patients into subgroups.</p>
      <p>The palliative treatment predominated, and mortality showed no correlation with the morphometric characteristics.</p>
      <p>
        <bold>References</bold>
      </p>
      <p>
        <fn id="back10-body2" fn-type="corresp">
          <p>Correspondece to</p>
          <p> Ulisses Alexandre Croti</p>
          <p> Rua Benjamin Constant, 4035/114</p>
          <p> Cep 15015-600 - S&#227;o Jos&#233; do Rio Preto, SP, Brazil</p>
          <p> E-mail: </p>
          <p>
            <email xlink:href="uacroti@cardiol.br">uacroti@cardiol.br</email>
          </p>
        </fn>
      </p>
      <p>Received for publication: 8/11/03</p>
      <p> Accepted for publication: 2/11/04</p>
      <p>English version by Stela Maris Costalonga</p>
    </body>
  </sub-article>
</article>
