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  <front>
    <journal-meta>
      <journal-id journal-id-type="nlm-ta">Arq Bras Cardiol</journal-id>
      <journal-id journal-id-type="publisher-id">abc</journal-id>
      <journal-title-group>
        <journal-title>Arquivos Brasileiros de Cardiologia</journal-title>
        <abbrev-journal-title abbrev-type="publisher">Arq. Bras. Cardiol.</abbrev-journal-title>
      </journal-title-group>
      <issn pub-type="ppub">0066-782X</issn>
      <issn pub-type="epub">1678-4170</issn>
      <publisher>
        <publisher-name>Sociedade Brasileira de Cardiologia - SBC</publisher-name>
      </publisher>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v3">Y33pHL743gmD79vcXrjzTyc</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2016000600502</article-id>
      <article-id pub-id-type="publisher-id" specific-use="previous-pid">S0066-782X2016005013103</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20160066</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Original Articles</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Functional Class in Children with Idiopathic Dilated Cardiomyopathy. A pilot Study</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Tavares</surname>
            <given-names>Aline Cristina</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Bocchi</surname>
            <given-names>Edimar Alcides</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Guimar&#227;es</surname>
            <given-names>Guilherme Veiga</given-names>
          </name>
          <xref ref-type="aff" rid="aff1"/>
        </contrib>
        <aff id="aff1">
          <institution content-type="orgname">Hospital S&#237;rio-liban&#234;s</institution>
          <institution content-type="orgdiv1">Instituto do Cora&#231;&#227;o</institution>
          <addr-line>
            <named-content content-type="city">S&#227;o Paulo</named-content>
            <named-content content-type="state">SP</named-content>
          </addr-line>
          <country country="BR">Brazil</country>
          <institution content-type="original">Instituto do Cora&#231;&#227;o (InCor), Hospital S&#237;rio-liban&#234;s (HSL), S&#227;o Paulo, SP - Brazil</institution>
          <institution content-type="normalized">Hospital S&#237;rio-Liban&#234;s</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Aline Cristina Tavares</bold>, Av. Dr. En&#233;as de Carvalho Aguiar, 44. Laborat&#243;rio de Insufici&#234;ncia Card&#237;aca, Bloco 1, 1&#186; andar. Postal Code 05403-900 - InCor, S&#227;o Paulo - Brazil. E-mail: <email>alinet84@gmail.com</email>
				</corresp>
        <fn fn-type="con">
          <p>
            <bold>Author contributions</bold>
          </p>
          <p>Conception and design of the research: Tavares AC, Bocchi EA, Guimar&#227;es GV. Acquisition of data: Tavares AC. Analysis and interpretation of the data: Tavares AC, Guimar&#227;es GV. Statistical analysis: Tavares AC, Guimar&#227;es GV. Obtaining financing: Tavares AC, Guimar&#227;es GV. Writing of the manuscript: Tavares AC, Bocchi EA. Critical revision of the manuscript for intellectual content: Tavares AC, Bocchi EA, Guimar&#227;es GV. Drafting of the figures (dolls): Tavares AC.</p>
        </fn>
        <fn fn-type="conflict">
          <p>
            <bold>Potential Conflict of Interest</bold>
          </p>
          <p>No potential conflict of interest relevant to this article was reported.</p>
        </fn>
      </author-notes>
      <pub-date pub-type="epub">
        <day>06</day>
        <month>05</month>
        <year>2016</year>
      </pub-date>
      <pub-date pub-type="collection">
        <month>6</month>
        <year>2016</year>
      </pub-date>
      <volume>106</volume>
      <issue>6</issue>
      <fpage>502</fpage>
      <lpage>509</lpage>
      <history>
        <date date-type="received">
          <day>10</day>
          <month>07</month>
          <year>2015</year>
        </date>
        <date date-type="rev-recd">
          <day>30</day>
          <month>11</month>
          <year>2015</year>
        </date>
        <date date-type="accepted">
          <day>18</day>
          <month>01</month>
          <year>2016</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/4.0/" xml:lang="en">
          <license-p>This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
        </license>
      </permissions>
      <abstract>
        <title>Abstract</title>
        <sec>
          <title>Background:</title>
          <p>Idiopathic dilated cardiomyopathy (IDCM), most common cardiac cause of pediatric deaths, mortality descriptor: a low left ventricular ejection fraction (LVEF) and low functional capacity (FC). FC is never self reported by children.</p>
        </sec>
        <sec>
          <title>Objective:</title>
          <p>The aims of this study were (i) To evaluate whether functional classifications according to the children, parents and medical staff were associated. (iv) To evaluate whether there was correlation between VO<sub>2</sub> max and Weber's classification.</p>
        </sec>
        <sec>
          <title>Method:</title>
          <p>Prepubertal children with IDCM and HF (by previous IDCM and preserved LVEF) were selected, evaluated and compared. All children were assessed by testing, CPET and functional class classification.</p>
        </sec>
        <sec>
          <title>Results:</title>
          <p>Chi-square test showed association between a CFm and CFp (1, n = 31) = 20.6; p = 0.002. There was no significant association between CFp and CFc (1, n = 31) = 6.7; p = 0.4. CFm and CFc were not associated as well (1, n = 31) = 1.7; p = 0.8. Weber's classification was associated to CFm (1, n = 19) = 11.8; p = 0.003, to CFp (1, n = 19) = 20.4; p = 0.0001and CFc (1, n = 19) = 6.4; p = 0.04).</p>
        </sec>
        <sec>
          <title>Conclusion:</title>
          <p>Drawing were helpful for children's self NYHA classification, which were associated to Weber's stratification.</p>
        </sec>
      </abstract>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Heart Failure</kwd>
        <kwd>Cardiomyopathy</kwd>
        <kwd>Dilated / mortality</kwd>
        <kwd>Stroke Volume</kwd>
        <kwd>Child</kwd>
        <kwd>Pilot Projects</kwd>
      </kwd-group>
      <funding-group>
        <award-group award-type="contract">
          <funding-source>FAPESP</funding-source>
          <award-id>2011/08985-0</award-id>
        </award-group>
        <funding-statement>This study was funded by FAPESP 2011/08985-0.</funding-statement>
      </funding-group>
    </article-meta>
  </front>
  <body>
    <sec sec-type="intro">
      <title>Introduction</title>
      <p>Idiopathic dilated cardiomyopathy (IDCM) - characterized by left ventricular dilatation and systolic dysfunction of undetermined cause,<xref ref-type="bibr" rid="B1">1</xref><sup>-</sup><xref ref-type="bibr" rid="B3">3</xref> has a high incidence among the pediatric population<xref ref-type="bibr" rid="B4">4</xref> and an unfavorable outcome,<xref ref-type="bibr" rid="B2">2</xref><sup>,</sup><xref ref-type="bibr" rid="B5">5</xref><sup>,</sup><xref ref-type="bibr" rid="B6">6</xref> and is thus a target for reasearch.<xref ref-type="bibr" rid="B1">1</xref>
			</p>
      <p>To date, it is known that the only predictors of death or cardiac transplantation in children with IDCM are a low LVEF and low functional capacity.<xref ref-type="bibr" rid="B7">7</xref>
			</p>
      <p>LVEF is easily measured by echocardiography.<xref ref-type="bibr" rid="B8">8</xref> Functional capacity, in turn, may be determined using peak oxygen consumption (VO<sub>2</sub>) in the cardiopulmonary exercise test (CPET)<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B10">10</xref> or scales representing the functional class.<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B11">11</xref><sup>,</sup><xref ref-type="bibr" rid="B12">12</xref> CPET findings provide an objective assessment of the functional capacity,<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref> whereas the scales represent a subjective assessment.<xref ref-type="bibr" rid="B13">13</xref>
			</p>
      <p>However, the scales are not always related to the objective values of CPET,<xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref> and this may impair the communication between parents and the medical team, the stratification, and treatment. Thus, the objective of this study is to fill this gap and evaluate whether there is a correlation between the objective functional capacity (by peak O<sub>2</sub> consumption - peak VO<sub>2</sub> ) and the functional class as proposed by the family, the medical team and the child itself, and whether there is a correlation between peak VO<sub>2</sub> and Weber stratification.<xref ref-type="bibr" rid="B12">12</xref>
			</p>
    </sec>
    <sec sec-type="methods">
      <title>Methods</title>
      <sec>
        <title>Sample</title>
        <p>This is a pilot, cross-sectional, prospective, randomized, consecutive study. Children of both genders with IDCM and children with HF with preserved LVEF (secondary to previous IDCM) were selected from the outpatient clinic of the Medical Unit of Pediatric Cardiology and Congenital Heart Defects of <italic>Instituto do Cora&#231;&#227;o</italic> - InCor, <italic>Hospital das Clinicas da Faculdade de Medicina da Universidade de S&#227;o Paulo</italic> - HCFMUSP.</p>
        <p>The inclusion criteria were: (i) patients diagnosed with current IDCM or HF for previous IDCM with preserved LVEF; (ii) patients clinically stable; (iii) patients receiving drug therapy continuously for the past 3 months; (iv) older than 5 years;<xref ref-type="bibr" rid="B15">15</xref><sup>,</sup><xref ref-type="bibr" rid="B16">16</xref> (v) age equivalent to the prepubertal phase -<italic>Tanner-</italic>Whithouse scale stages 1 to 3;<xref ref-type="bibr" rid="B17">17</xref> (vi) previous echocardiographic study performed at least 6 months earlier. </p>
        <p>Children with complex ventricular arrhythmias or atrial fibrillation; in the postoperative recovery period; with neuromuscular, renal or pulmonary diseases; with diabetes mellitus; and/or those who refused to participate in the study or in the assessments were not included.</p>
        <p>The children, as well as their guardians (as established in articles 1634,<xref ref-type="bibr" rid="B18">18</xref> and 1852,<xref ref-type="bibr" rid="B19">19</xref> subsection V of the Civil Code, and in Law 8069/90 and 10406/2002),<xref ref-type="bibr" rid="B20">20</xref> included in any of the groups, were given information on the objectives of the research and the tests participants should undergo. In addition, all children participating and their parents or guardians were informed that the children should keep taking their regular medication throughout the study. All children or guardians gave written informed consent to participate.</p>
        <p>The children were included according to the inclusion criteria and were assessed provided they were cleared by the medical team.</p>
      </sec>
      <sec>
        <title>Assessments</title>
        <p>All children were assessed as regards their functional class, anthropometric data and CPET.</p>
      </sec>
      <sec>
        <title>Functional class</title>
        <p>The modified functional classification used was adapted from a functional classification previously described elsewhere and applied in studies assessing children with cardiomyopathies,<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B21">21</xref> as follows:</p>
        <p>Class I - Heart disease with no limitation of physical activities. Schoolchildren are able to attend physical education classes until the end.</p>
        <p>Class II - Slight limitation of physical activities. Comfortable at rest, but ordinary activities may result in tachycardia, fatigue or dyspnea. Schoolchildren attend physical education classes, but are unable to stay until the end.</p>
        <p>Class III - Marked limitation of physical activities. Less than ordinary activities, such as walking less than a block, may cause fatigue, tachycardia or dyspnea. Schoolchildren are unable to attend physical education classes.</p>
        <p>Class IV - Unable to carry on any physical activity without discomfort. Symptoms are present at rest and increase during activity.</p>
        <p>Based on this description, a graphic representation of the four functional classes was elaborated by this study's author, both for male and female children (<xref ref-type="fig" rid="f1">Figures 1</xref> and <xref ref-type="fig" rid="f2">2</xref>, respectively), so that the guardians and the children could use it. In order to make these drawings, the image taken into consideration was the one with which children in the same age range as those participating in the study could identify themselves.</p>
        <p>
          <fig id="f1">
            <label>Figure 1</label>
            <caption>
              <title>Functional class for male children.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-106-06-0502-60066-gf01.jpg"/></fig>
          <fig id="f2">
            <label>Figure 2</label>
            <caption>
              <title>Functional class for female children.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-106-06-0502-60066-gf02.jpg"/></fig>
        </p>
        <p>Initially, the physician following up the children would give his/her opinion on which functional class the children were in. This baseline assessment was made without the presence of the guardians or the children themselves. This information was expressed as functional class according to the medical team (FCm).</p>
        <p>Next, the parents or guardians would give their opinion on the functional class the children were in, according to <xref ref-type="fig" rid="f1">figures 1</xref> and <xref ref-type="fig" rid="f2">2</xref>. This classification was made without the presence of the physicians or even the children themselves. This information was expressed as functional class according to parents or guardians (FCp).</p>
        <p>Later, the children would perform a self-assessment of their functional class using the graphic representation (<xref ref-type="fig" rid="f1">Figures 1</xref> and <xref ref-type="fig" rid="f2">2</xref>). This self-perceived functional class was expressed as functional class according to the children themselves (FCc).</p>
      </sec>
      <sec>
        <title>Anthropometric data</title>
        <p>Data on age, gender, height, body mass and body mass index (BMI) were collected.</p>
      </sec>
      <sec>
        <title>Echocardiographic data</title>
        <p>Analysis of the cardiac function using echocardiography was considered for studies performed up to six months prior to inclusion.</p>
        <p>Echocardiographic studies were performed according to recommendations from the guidelines for the pediatric population, using the Teicholz method.<xref ref-type="bibr" rid="B22">22</xref> Data on LVEF, end-diastolic left ventricular size, end-systolic left ventricular diameter, and left ventricular wall thickness were collected. Size and thickness values were corrected for body surface area (BSA) using a formula appropriate for children weighing more than 10 kg, as follows: BSA = (weight *4 +7) / (weight + 90),<xref ref-type="bibr" rid="B23">23</xref> in which weight is expressed in kg.</p>
        <p>Children, whose medical record contained a previous echocardiographic study performed no later than six months prior to the collection of the other data, would have their echocardiographic data retrieved from that previous study. Children with no previous echocardiographic study underwent the test, from which the data were further collected.</p>
      </sec>
      <sec>
        <title>Cardiopulmonary exercise test</title>
        <p>The children underwent a cardiopulmonary exercise test (CPET) in a programmable treadmill (Marquette series 2000, Marquette Electronics, Milwaukee, WI, USA), according to the modified Balke ramp protocol.<xref ref-type="bibr" rid="B21">21</xref><sup>,</sup><xref ref-type="bibr" rid="B24">24</xref><sup>-</sup><xref ref-type="bibr" rid="B26">26</xref>
				</p>
        <p>CPET was performed two hours after a caffeine-free light meal, in a room with controlled temperature (21&#176;C to 23&#176;C), after a 2-minute rest, in the upright position on the treadmill.<xref ref-type="bibr" rid="B25">25</xref>
				</p>
        <p>During the beginning of the resting, exercise, and recovery periods, the children had their pulmonary ventilation as well as oxygen and carbon dioxide concentrations in the inhaled and exhaled air volumes continuously monitored (Sensormedics, model Vmax 229, Yorba Linda, CA, USA), breath by breath. During CPET, continuous 12-lead heart rhythm monitoring was performed (Marquette MAX 1, Marquette Electronics, Milwaukee, WI, USA) and systemic blood pressure was measured every minute (HP68S Hewlett-Packard multiparameter monitor, USA, or HP M1008B Hewlett-Packard oscillometric blood pressure transducer, USA).<xref ref-type="bibr" rid="B24">24</xref><sup>-</sup><xref ref-type="bibr" rid="B26">26</xref>
				</p>
        <p>Criteria for exercise termination were the absolute indications recommended by the ACC/AHA Guidelines Update For Exercise Testing, when exhaustion was reached (respiratory quotient &gt; 1.0)<xref ref-type="bibr" rid="B25">25</xref> or in the presence of signs or symptoms that could result in cardiac injury, such as angina, headache, dizziness, syncope, excessive dyspnea, fatigue, ST-segment depression or elevation greater than 3 mm, arrhythmia, supraventricular or ventricular tachycardia, atrioventricular block or progressive decrease in blood pressure (BP).<xref ref-type="bibr" rid="B25">25</xref>
				</p>
      </sec>
      <sec>
        <title>Statistical Analysis</title>
        <p>The statistical analysis was carried out using the SPSS 12.0 software program for Windows (SPSS Inc., Chicago, IL, USA).</p>
        <p>The Shapiro-Wilk test was used to check the normality of data in the population.</p>
        <p>Patient demographics were expressed in a descriptive manner, in absolute numbers, percentages or mean and standard deviation. Functional classes were presented as absolute numbers. Quantitative variables regarding the cardiopulmonary exercise test were expressed as mean and standard deviation.</p>
        <p>The chi-square test (&#967;<sup>2</sup>) was used to analyze the association between categorical variables of the functional class, as assessed by the medical team, guardians and children.</p>
        <p>The Pearson correlation coefficient was used for normal data, and the Spearman correlation, for non-parametric data, in order to correlate quantitative data. These correlations were interpreted as directly proportional (if +) or inversely proportional (if -), and weak (if 0.1 to 0.29), moderate (if 0.3 to 0.59), strong (if 0.6 to 0.79), very strong (if 0.8 to 0,99) or perfect (if 1).<xref ref-type="bibr" rid="B27">27</xref>
				</p>
      </sec>
    </sec>
    <sec sec-type="results">
      <title>Results</title>
      <p>Initially, 77 children were screened to comprise the sample. Only 31 met all inclusion criteria; however, only 19 agreed to participate in the study. The post-hoc Bonferroni test showed that there was no significant effect for gender among the children.</p>
      <p>None of the 19 children presented any hemodynamic instability during the cardiopulmonary exercise test.</p>
      <p>The children were using the following medications: acetyl salicylic acid, captopril, carvedilol, digoxin, enalapril, spironolactone, furosemide, and topimarate.</p>
      <p><xref ref-type="table" rid="t1">Table 1</xref> shows the characterization of the overall sample, with details on its demographics and echocardiographic data.</p>
      <table-wrap id="t1">
        <label>Table 1</label>
        <caption>
          <title>Sample characterization</title>
        </caption>
        <table frame="hsides" rules="groups">
          <colgroup>
            <col width="1%"/>
            <col width="49%"/>
            <col width="49%"/>
          </colgroup>
          <thead>
            <tr>
              <th align="left" colspan="2">
							</th>
              <th align="center">Total (19) </th>
            </tr>
          </thead>
          <tbody>
            <tr>
              <td align="left" colspan="3">
                <bold>Demographics</bold>
              </td>
            </tr>
            <tr>
              <td align="left" colspan="2">Age (years) </td>
              <td align="center">8.7 &#177; 1.9 </td>
            </tr>
            <tr>
              <td align="left" colspan="2">Gender (F/M) </td>
              <td align="center">10/9 </td>
            </tr>
            <tr>
              <td align="left" colspan="2">Body mass (kg) </td>
              <td align="center">30.7 &#177; 8.5 </td>
            </tr>
            <tr>
              <td align="left" colspan="2">Height (m) </td>
              <td align="center">1.26 &#177; 0.45 </td>
            </tr>
            <tr>
              <td align="left" colspan="2">BMI (kg/m<sup>2</sup>) </td>
              <td align="center">30.7 &#177; 8.5 </td>
            </tr>
            <tr>
              <td align="left" colspan="2">BSA (m<sup>2</sup>) </td>
              <td align="center">111.2 &#177; 41.5 </td>
            </tr>
            <tr>
              <td align="left" colspan="3">
                <bold>Echocardiographic data</bold>
              </td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; LVEF (%) </td>
              <td align="center">46.7 &#177; 13.8 </td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; Systolic LV size </td>
              <td align="center">48.3 &#177; 9.8 </td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; Diastolic LV size </td>
              <td align="center">37.5 &#177; 12.2 </td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; Relative LV wall thickness </td>
              <td align="center">0.26 &#177; 0.06 </td>
            </tr>
          </tbody>
        </table>
        <table-wrap-foot>
          <fn id="TFN01">
            <p>BSA: body surface area; IDCM: children with idiopathic dilated cardiomyopathy and LVEF &lt; 40%; LVEF: left ventricular ejection fraction; HF: heart failure; BMI: body mass index.</p>
          </fn>
        </table-wrap-foot>
      </table-wrap>
      <p>According to the medical team, 13 children were classified as FC I, five as FC II, one as FC III, and none as FC IV.</p>
      <p>According to parents, 13 children were classified as FC I, four as FC II, one as FC III and one as FC IV.</p>
      <p>According to the self-assessment, 11 children classified themselves as FC I, six as FC II, two as FC III. No children classified themselves as FC IV.</p>
      <p><xref ref-type="table" rid="t2">Table 2</xref> shows FCm, FCp, FCc, and peak VO<sub>2</sub> reached in the cardiopulmonary exercise test for each participant.</p>
      <table-wrap id="t2">
        <label>Table 2</label>
        <caption>
          <title>Cardiopulmonary exercise test data</title>
        </caption>
        <table frame="hsides" rules="groups">
          <colgroup>
            <col width="1%"/>
            <col width="49%"/>
            <col width="49%"/>
          </colgroup>
          <thead>
            <tr>
              <th align="left" colspan="2">
							</th>
              <th align="center">All (19)</th>
            </tr>
          </thead>
          <tbody>
            <tr>
              <td align="left" colspan="3">
                <bold>SBP (mmHg)</bold>
              </td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; rest</td>
              <td align="center">102.2 &#177; 12.4</td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; peak</td>
              <td align="center">120.5 &#177; 18.1</td>
            </tr>
            <tr>
              <td align="left" colspan="3">
                <bold>DBP (mmHg)</bold>
              </td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; rest</td>
              <td align="center">59.2 &#177; 10.6</td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; peak</td>
              <td align="center">69.7 &#177; 13.7</td>
            </tr>
            <tr>
              <td align="left" colspan="3">
                <bold>HR (bpm)</bold>
              </td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; rest</td>
              <td align="center">91.2 &#177; 10.8</td>
            </tr>
            <tr>
              <td align="left">
							</td>
              <td align="left">&#8211; maximum</td>
              <td align="center">162.1 &#177; 18.7</td>
            </tr>
            <tr>
              <td align="left" colspan="2">VO<sub>2-peak</sub> (mL/kg/min)</td>
              <td align="center">25.5 &#177; 6.7</td>
            </tr>
            <tr>
              <td align="left" colspan="2">VE/VCO<sub>2</sub> slope</td>
              <td align="center">37.4 &#177; 6.4</td>
            </tr>
            <tr>
              <td align="left" colspan="2">RER</td>
              <td align="center">1.02 &#177; 0.04</td>
            </tr>
            <tr>
              <td align="left" colspan="2">PetO2</td>
              <td align="center">54.3 &#177; 30.3</td>
            </tr>
            <tr>
              <td align="left" colspan="2">Time (min)</td>
              <td align="center">10.9 &#177; 4.3</td>
            </tr>
          </tbody>
        </table>
        <table-wrap-foot>
          <fn id="TFN02">
            <p>HC: heart rate; DBP: diastolic blood pressure; SBP: systolic blood pressure; PetO2: pressure of end-tidal O<sub>2</sub>; RER: respiratory exchange ratio; VE/VCO<sub>2</sub> slope: slope of the line between ventilation (VE) and carbon dioxide production (VCO<sub>2</sub>), VO<sub>2</sub> peak: peak oxygen consumption.</p>
          </fn>
        </table-wrap-foot>
      </table-wrap>
      <p>The chi-square test showed an association between FCm and FCp (1, n = 31) = 20.6; p = 0.002. No significant association was found between FCp and FCc (1, n = 31) = 6.7; p = 0.4. FCm and FCc were not associated either (1, n = 31) = 1.7; p = 0.8.</p>
      <p>According to the peak VO<sub>2</sub> found in CPET, Weber classification was significantly associated with the three functional classes described in this study, using the &#967;<sup>2</sup> test: Weber classification and FCm (1, n = 19) = 11.8; p = 0.003; Weber classification and FCp (1, n = 19) = 20.4; p = 0.0001; Weber classification and FCc (1, n = 19) = 6.4; p = 0.04. (<xref ref-type="fig" rid="f3">Figure 3</xref>).</p>
      <p>
        <fig id="f3">
          <label>Figure 3</label>
          <caption>
            <title>Functional class, Weber classification, and peak oxygen consumption. c: child; FC: functional class; m: medical team; p: parents or guardians; VO<sub>2</sub> peak: peak oxygen consumption. <sup>a</sup>p = 0.003; <sup>b</sup>p = 0.0001; <sup>c</sup>p = 0.04.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-106-06-0502-60066-gf03.jpg"/></fig>
      </p>
      <p>Children from the sample reached 84% of the maximum HR, according to the formula proposed by Tanaka (maximum HR = 208 - [0.7 x age]),<xref ref-type="bibr" rid="B28">28</xref> with this maximum HR being approximately 35 bpm lower than that proposed.</p>
      <p>Peak VO<sub>2</sub> and LVEF values showed a weak non-significant correlation between each other (r = 0.27; p = 0.25). Likewise, LVEF was not related to the other data obtained from CPET.</p>
      <p>Finally, <xref ref-type="table" rid="t3">Table 3</xref> shows all data from the present study, including data on the functional classes (FCm, FCp, FCc) and peak VO<sub>2</sub> as measured by cardiopulmonary exercise test, for each study subject.</p>
      <table-wrap id="t3">
        <label>Table 3</label>
        <caption>
          <title>Data on functional classes and peak oxygen consumption on cardiopulmonary exercise test</title>
        </caption>
        <table frame="hsides" rules="groups">
          <colgroup width="20%">
            <col/>
            <col/>
            <col/>
            <col/>
            <col/>
          </colgroup>
          <thead>
            <tr>
              <th align="left">Subject</th>
              <th align="center">FCm</th>
              <th align="center">FCp</th>
              <th align="center">FCc</th>
              <th align="center">VO<sub>2</sub> peak</th>
            </tr>
          </thead>
          <tbody>
            <tr>
              <td align="left">1</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">34.6</td>
            </tr>
            <tr>
              <td align="left">2</td>
              <td align="center">2</td>
              <td align="center">1</td>
              <td align="center">3</td>
              <td align="center">32</td>
            </tr>
            <tr>
              <td align="left">3</td>
              <td align="center">2</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">17.6</td>
            </tr>
            <tr>
              <td align="left">4</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">30.3</td>
            </tr>
            <tr>
              <td align="left">5</td>
              <td align="center">2</td>
              <td align="center">1</td>
              <td align="center">2</td>
              <td align="center">27</td>
            </tr>
            <tr>
              <td align="left">6</td>
              <td align="center">2</td>
              <td align="center">2</td>
              <td align="center">1</td>
              <td align="center">25</td>
            </tr>
            <tr>
              <td align="left">7</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">2</td>
              <td align="center">23.4</td>
            </tr>
            <tr>
              <td align="left">8</td>
              <td align="center">1</td>
              <td align="center">4</td>
              <td align="center">2</td>
              <td align="center">15.6</td>
            </tr>
            <tr>
              <td align="left">9</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">3</td>
              <td align="center">31</td>
            </tr>
            <tr>
              <td align="left">10</td>
              <td align="center">2</td>
              <td align="center">2</td>
              <td align="center">1</td>
              <td align="center">36.9</td>
            </tr>
            <tr>
              <td align="left">11</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">35.8</td>
            </tr>
            <tr>
              <td align="left">12</td>
              <td align="center">1</td>
              <td align="center">2</td>
              <td align="center">1</td>
              <td align="center">26</td>
            </tr>
            <tr>
              <td align="left">13</td>
              <td align="center">1</td>
              <td align="center">2</td>
              <td align="center">1</td>
              <td align="center">15.6</td>
            </tr>
            <tr>
              <td align="left">14</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">2</td>
              <td align="center">14.8</td>
            </tr>
            <tr>
              <td align="left">15</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">2</td>
              <td align="center">27.3</td>
            </tr>
            <tr>
              <td align="left">16</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">24</td>
            </tr>
            <tr>
              <td align="left">17</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">2</td>
              <td align="center">18.4</td>
            </tr>
            <tr>
              <td align="left">18</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">30</td>
            </tr>
            <tr>
              <td align="left">19</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">1</td>
              <td align="center">31.3</td>
            </tr>
          </tbody>
        </table>
        <table-wrap-foot>
          <fn id="TFN03">
            <p>FCc: functional class according to the children themselves. FCm: functional class according to the medical team; FCp: functional class according to parents or guardians; VO<sub>2</sub> peak: peak oxygen consumption.</p>
          </fn>
        </table-wrap-foot>
      </table-wrap>
    </sec>
    <sec sec-type="discussion">
      <title>Discussion</title>
      <p>Although the study sample had a small number of participants, our findings show that the cardiopulmonary exercise test is safe in the populations described; that peak VO<sub>2</sub> findings are related to the stratification data using Weber classification;<xref ref-type="bibr" rid="B12">12</xref> and that drawings can be an additional resource for the assessment of children with IDCM and HF (for previous IDCM) with preserved LVEF.</p>
      <p>As regards the anthropometric data, all children enrolled were in the prepubertal phase,<xref ref-type="bibr" rid="B17">17</xref> thus there was no influence of hormones on the results obtained.<xref ref-type="bibr" rid="B29">29</xref>
			</p>
      <p>Although all children included in the present study had been in the same age range in which linear growth occurs (from 7 to 11 years of age),<xref ref-type="bibr" rid="B30">30</xref> children with IDCM were shorter than those with HF. This may have resulted from low weight gain during childhood<xref ref-type="bibr" rid="B31">31</xref> because of a low systemic supply secondary to impaired cardiac output that children with more severely affected hearts show.<xref ref-type="bibr" rid="B32">32</xref>
			</p>
      <p>The medications used were consistent with those described in the literature for the treatment of IDCM or HF in the pediatric population, including angiotensin-converting enzyme inhibitors (ACEI),<xref ref-type="bibr" rid="B33">33</xref> betablockers and diuretics<xref ref-type="bibr" rid="B1">1</xref>.<xref ref-type="bibr" rid="B26">26</xref>
			</p>
      <p>The cause of short stature in children with IDCM and of high drug doses may be similar to that of nocturnal dip. The latter, in turn , is related to severity of symptoms and greater sympathetic activity.<xref ref-type="bibr" rid="B34">34</xref> In this regard, further studies are probably necessary to establish these associations.</p>
      <p>Like for adults, exercise tolerance is known to be predictive of mortality in children with heart failure.<xref ref-type="bibr" rid="B7">7</xref> Additionally, the experience with cardiopulmonary exercise test<xref ref-type="bibr" rid="B21">21</xref><sup>,</sup><xref ref-type="bibr" rid="B25">25</xref> in healthy children<xref ref-type="bibr" rid="B16">16</xref> and in those with HF for IDCM<xref ref-type="bibr" rid="B25">25</xref> older than 6 years,<xref ref-type="bibr" rid="B16">16</xref><sup>,</sup><xref ref-type="bibr" rid="B25">25</xref><sup>,</sup><xref ref-type="bibr" rid="B32">32</xref> show that the cardiovascular and metabolic responses are similar to those observed in adults with the same clinical characteristics.<xref ref-type="bibr" rid="B17">17</xref>
			</p>
      <p>In our CPET assessments, we observed that both groups of children with IDCM and those with HF with preserved LVEF are unable to reach the maximum age-predicted HR in the exercise test. These findings are corroborated by results of studies conducted in adults with HF<xref ref-type="bibr" rid="B35">35</xref> and in children with IDCM,<xref ref-type="bibr" rid="B17">17</xref> in which 80% of the maximum HR in the mean for age was reached, and are similar to those found in the present study, in which the values are between 82% and 84% of the maximum HR.</p>
      <p>Peak VO<sub>2</sub> values found in the present study were different in the two groups. This probably resulted from the fact that peak VO<sub>2</sub> is believed to occur between 13 and 14 years of age,<xref ref-type="bibr" rid="B16">16</xref><sup>,</sup><xref ref-type="bibr" rid="B21">21</xref><sup>,</sup><xref ref-type="bibr" rid="B29">29</xref> i.e., the parameters related to this indicator are expected to be rising during the prepubertal period, phase in which the participants were assessed.<xref ref-type="bibr" rid="B29">29</xref><sup>,</sup><xref ref-type="bibr" rid="B36">36</xref> Although a systematic review by the present study's author had shown that peak VO<sub>2</sub> values in prepubertal girls are, on average, 20% lower than those found in prepubertal boys,<xref ref-type="bibr" rid="B37">37</xref> because of the influence of hormones and body fat,<xref ref-type="bibr" rid="B37">37</xref><sup>,</sup><xref ref-type="bibr" rid="B38">38</xref> this finding was not observed here after post-hoc Bonferroni test. We can suppose that the small sample size had a negative influence on the analysis of this variable.</p>
      <p>Even with peak VO<sub>2</sub> values lower than expected,<xref ref-type="bibr" rid="B37">37</xref> all children reached the maximum test according to the criteria of exercise termination mentioned by ACC/AHA Guidelines Update for Exercise Testing,<xref ref-type="bibr" rid="B39">39</xref> because the modified Balke protocol used is appropriate to the study population, and the protocol-demanded response to exercise is similar to the physiological response to exercise in children. That is, the time to reach 50% of peak VO<sub>2</sub> values in children is shorter than that for adults; children are less dependent on the glycogenic pathway to meet the demands than adults; the use of fatty acids as an energy source is greater during childhood; and children show lower levels of blood lactate (which makes it more difficult to reach exhaustion), lower pulmonary ventilation (VE, L/min) and lower carbon dioxide production (VCO<sub>2</sub>, mL/min).<xref ref-type="bibr" rid="B29">29</xref><sup>,</sup><xref ref-type="bibr" rid="B39">39</xref>
			</p>
      <p>Since the information on the functional class as assessed by the children, their guardians and the medical team was not correlated, the data prove to be subjective, which is corroborated by previous studies.<xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref> However, it was correlated with peak VO<sub>2</sub> values on CPET,<xref ref-type="bibr" rid="B40">40</xref> according to Weber's criteria, which are very frequently used for stratification and prognosis in adults.<xref ref-type="bibr" rid="B12">12</xref> Since, to date, no such prognostic assessment exists in the scientific literature regarding children with IDCM and HF, the measurement will probably continue to be subjective, corroborating previous findings from 2001, in which objective values on CPET did not correlate with the functional class as assessed by the medical team.<xref ref-type="bibr" rid="B13">13</xref>
			</p>
    </sec>
    <sec sec-type="conclusions">
      <title>Conclusion</title>
      <p>Peak VO<sub>2</sub> peak are related to stratification data by Weber classification, and the drawings shown to prepubertal children may be an additional resource for the assessment of children with IDCM and HF (for previous IDCM) and preserved LVEF.</p>
    </sec>
  </body>
  <back>
    <fn-group>
      <fn fn-type="financial-disclosure">
        <p>
          <bold>Sources of Funding</bold>
        </p>
        <p>This study was funded by FAPESP 2011/08985-0.</p>
      </fn>
      <fn fn-type="other">
        <p>
          <bold>Study Association</bold>
        </p>
        <p>This article is part of the thesis of doctoral submitted by Aline Cristina Tavares, from Instituto do Cora&#231;&#227;o (INCOR), Hospital das cl&#237;nicas da Faculdade de Medicina da USP.</p>
      </fn>
    </fn-group>
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  <sub-article article-type="translation" id="s1" xml:lang="pt">
    <front-stub>
      <title-group>
        <article-title>Classe Funcional em Crian&#231;as Portadoras de Miocardiopatia Dilatada Idiop&#225;tica. Estudo Piloto</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Tavares</surname>
            <given-names>Aline Cristina</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">
            <sup>1</sup>
          </xref>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Bocchi</surname>
            <given-names>Edimar Alcides</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">
            <sup>1</sup>
          </xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Guimar&#227;es</surname>
            <given-names>Guilherme Veiga</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">
            <sup>1</sup>
          </xref>
        </contrib>
        <aff id="aff2">
          <label>1</label>
          <institution content-type="original">Instituto do Cora&#231;&#227;o (InCor), Hospital S&#237;rio-liban&#234;s (HSL), S&#227;o Paulo, SP - Brasil</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c2"><bold>Correspond&#234;ncia: Aline Cristina Tavares</bold>, Av. Dr. En&#233;as de Carvalho Aguiar, 44. Laborat&#243;rio de Insufici&#234;ncia Card&#237;aca, Bloco 1, 1&#186; andar. CEP: 05403-900. InCor, S&#227;o Paulo - Brasil. E-mail: <email>alinet84@gmail.com</email>
				</corresp>
        <fn fn-type="con">
          <p>
            <bold>Contribui&#231;&#227;o dos autores</bold>
          </p>
          <p>Concep&#231;&#227;o e desenho da pesquisa: Tavares AC, Bocchi EA, Guimar&#227;es GV. Obten&#231;&#227;o de dados: Tavares AC. An&#225;lise e interpreta&#231;&#227;o dos dados: Tavares AC, Guimar&#227;es GV. An&#225;lise estat&#237;stica: Tavares AC, Guimar&#227;es GV. Obten&#231;&#227;o de financiamento: Tavares AC, Guimar&#227;es GV. Reda&#231;&#227;o do manuscrito: Tavares AC, Bocchi EA. Revis&#227;o cr&#237;tica do manuscrito quanto ao conte&#250;do intelectual importante: Tavares AC, Bocchi EA, Guimar&#227;es GV. Elabora&#231;&#227;o das figuras (bonecos): Tavares AC.</p>
        </fn>
        <fn fn-type="conflict">
          <p>
            <bold>Potencial Conflito de Interesses</bold>
          </p>
          <p>Declaro n&#227;o haver conflito de interesses pertinentes.</p>
        </fn>
      </author-notes>
      <abstract>
        <title>Resumo</title>
        <sec>
          <title>Fundamento:</title>
          <p>A cardiomiopatia dilatada idiop&#225;tica (CMDid) possui poucos preditores de mortalidade descritos: a baixa fra&#231;&#227;o de eje&#231;&#227;o de ventr&#237;culo esquerdo (FEVE) e a baixa capacidade funcional, sendo esta subjetiva.</p>
        </sec>
        <sec>
          <title>Objetivo:</title>
          <p>Os objetivos desse estudo foram (i) Avaliar se as classes funcionais propostas pela NYHA, modificada para crian&#231;as, estiveram associadas entre a percep&#231;&#227;o m&#233;dica (CFm), dos pais ou representantes (CFp) e das pr&#243;prias crian&#231;as avaliadas (CFc). (ii) Avaliar se houve correla&#231;&#227;o entre VO<sub>2</sub> max e a classifica&#231;&#227;o proposta por Weber.</p>
        </sec>
        <sec>
          <title>M&#233;todo:</title>
          <p>Crian&#231;as com CMDid e com IC por CMDid pr&#233;via com FEVE preservada, na fase pr&#233;-puberdade foram selecionadas submetidas a avalia&#231;&#245;es de ergoespirometria e classifica&#231;&#227;o da classe funcional. As crian&#231;as utilizaram uma representa&#231;&#227;o gr&#225;fica para se intitular quanto &#224; classe funcional.</p>
        </sec>
        <sec>
          <title>Resultado:</title>
          <p>O teste Chi-quadrado mostrou que houve associa&#231;&#227;o ente a CFm e CFp (1, n = 31) = 20,6; p = 0,002. N&#227;o houve associa&#231;&#227;o significativa entre CFp e CFc (1, n = 31) = 6,7; p = 0,4. As CF segundo m&#233;dico e CFc n&#227;o foram, tampouco, associadas (1, n = 31) = 1,7; p = 0,8. A classifica&#231;&#227;o de Weber foi significativamente associada &#224;s tr&#234;s classes funcionais (classifica&#231;&#227;o de Weber e CFm (1, n = 19) = 11,8; p = 0,003; classifica&#231;&#227;o de Weber e CFp (1, n = 19) = 20,4; p = 0,0001; classifica&#231;&#227;o de Weber e CFc (1, n = 19) = 6.4; p = 0.04.).</p>
        </sec>
        <sec>
          <title>Conclus&#227;o:</title>
          <p>A representa&#231;&#227;o gr&#225;fica serviu para que as crian&#231;as pudessem se classificar segundo a NYHA, que se demonstrou associada com a estratifica&#231;&#227;o de Weber.</p>
        </sec>
      </abstract>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Insufici&#234;ncia Card&#237;aca</kwd>
        <kwd>Cardiomiopatia Dilatada / mortalidade</kwd>
        <kwd>Volume Sist&#243;lico</kwd>
        <kwd>Crian&#231;a</kwd>
        <kwd>Projetos Piloto</kwd>
      </kwd-group>
      <funding-group>
        <award-group award-type="contract">
          <funding-source>FAPESP</funding-source>
          <award-id>2011/08985-0</award-id>
        </award-group>
        <funding-statement>O presente estudo foi financiado por FAPESP 2011/08985-0.</funding-statement>
      </funding-group>
    </front-stub>
    <body>
      <sec sec-type="intro">
        <title>Introdu&#231;&#227;o</title>
        <p>A cardiomiopatia dilatada idiop&#225;tica (CMDid), caracterizada pela dilata&#231;&#227;o do ventr&#237;culo esquerdo e disfun&#231;&#227;o sist&#243;lica sem causa determinada,<xref ref-type="bibr" rid="B1">1</xref><sup>-</sup><xref ref-type="bibr" rid="B3">3</xref> tem alta incid&#234;ncia popula&#231;&#227;o pedi&#225;trica,<xref ref-type="bibr" rid="B4">4</xref> possui evolu&#231;&#227;o desfavor&#225;vel,<xref ref-type="bibr" rid="B2">2</xref><sup>,</sup><xref ref-type="bibr" rid="B5">5</xref><sup>,</sup><xref ref-type="bibr" rid="B6">6</xref> assim &#233; alvo das pesquisas na &#225;rea.<xref ref-type="bibr" rid="B1">1</xref>
				</p>
        <p>At&#233; o momento, sabe-se que, em crian&#231;as com CMDid, os &#250;nicos preditores para &#243;bito ou transplante card&#237;aco s&#227;o a baixa FEVE e a baixa capacidade funcional nesta popula&#231;&#227;o espec&#237;fica.<xref ref-type="bibr" rid="B7">7</xref>
				</p>
        <p>A FEVE &#233; facilmente observada pelo exame de ecocardiograma.<xref ref-type="bibr" rid="B8">8</xref> J&#225; a capacidade funcional pode ser observada pelo valor do consumo de oxig&#234;nio (VO<sub>2</sub>) m&#225;ximo no teste ergoespirom&#233;trico (TCP)<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B10">10</xref> ou por escalas que representam a classe funcional.<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B11">11</xref><sup>,</sup><xref ref-type="bibr" rid="B12">12</xref> Os resultados do TCP apresentam uma avalia&#231;&#227;o objetiva da capacidade funcional<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref> enquanto que as escalas representam sua forma de avalia&#231;&#227;o subjetiva.<xref ref-type="bibr" rid="B13">13</xref>
				</p>
        <p>No entanto as escalas nem sempre est&#227;o relacionadas com os valores objetivos do TCP,<xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref> o que pode limitar a comunica&#231;&#227;o entre pais e equipe cl&#237;nica, a estratifica&#231;&#227;o e o tratamento das crian&#231;as. Assim, a proposta deste estudo est&#225; no preenchimento dessa lacuna para avaliar se existe correla&#231;&#227;o entre a capacidade funcional objetiva (pelo consumo m&#225;ximo de O<sub>2</sub> - VO<sub>2</sub> max) e a classe funcional propostas pelo familiar, equipe m&#233;dica e pela pr&#243;pria crian&#231;a e se h&#225; correla&#231;&#227;o entre VO<sub>2</sub> max e a estratifica&#231;&#227;o proposta por Weber.<xref ref-type="bibr" rid="B12">12</xref>
				</p>
      </sec>
      <sec sec-type="methods">
        <title>M&#233;todo</title>
        <sec>
          <title>Amostra</title>
          <p>Trata-se de um estudo piloto, transversal, prospectivo, aleat&#243;rio, consecutivo. Crian&#231;as de ambos os sexos portadores de CMDid e crian&#231;as com IC com FEVE preservada (por CMDid pr&#233;via) foram selecionadas a partir do ambulat&#243;rio da Unidade Cl&#237;nica de Cardiologia Pedi&#225;trica e de Cardiopatias Cong&#234;nitas do Instituto do Cora&#231;&#227;o - InCor do Hospital das Clinicas da Faculdade de Medicina da Universidade de S&#227;o Paulo - HCFMUSP.</p>
          <p>Os crit&#233;rios de inclus&#227;o para compor a amostra foram: (i) diagn&#243;stico de CMDId atual ou de IC por CMDid pr&#233;via com FEVE preservada; (ii) estabilidade cl&#237;nica; (iii) terap&#234;utica medicamentosa mantida durante os &#250;ltimos 3 meses; (iv) idade superior a 5 anos;<xref ref-type="bibr" rid="B15">15</xref><sup>,</sup><xref ref-type="bibr" rid="B16">16</xref> (v) idade equivalente &#224; fase pr&#233;-puberdade - est&#225;gios 1 a 3 da escala de <italic>Tanner</italic> -Whithouse;<xref ref-type="bibr" rid="B17">17</xref> (vi) portar de exame ecocardiogr&#225;fico datado de, no m&#237;nimo, 6 meses.</p>
          <p>N&#227;o foram inclu&#237;dos no estudo crian&#231;as com arritmias ventriculares complexas ou fibrila&#231;&#227;o atrial, em recupera&#231;&#227;o p&#243;s-operat&#243;ria, portadores de doen&#231;as neuromusculares, doen&#231;a renal, doen&#231;a pulmonar, diabetes mellitus, e/ou que se recusassem a participar do estudo ou das avalia&#231;&#245;es.</p>
          <p>As crian&#231;as bem como seus respons&#225;veis legais (conforme estabelecido nos artigos 1.634,<xref ref-type="bibr" rid="B18">18</xref> e 1.852,<xref ref-type="bibr" rid="B19">19</xref> inciso V C&#243;digo Civil, e na Lei 8.069/90e 10.406/2002),<xref ref-type="bibr" rid="B20">20</xref> pertencentes a qualquer dos grupos, foram informados sobre os objetivos da pesquisa e exames aos quais os participantes foram submetidos. Ainda, todas as crian&#231;as participantes, e seus respectivos representantes legais, pais ou respons&#225;veis, foram orientados a manter normalmente a terapia medicamentosa durante todo o estudo. Estando esclarecidos os termos do presente estudo e persistindo o interesse em sua participa&#231;&#227;o, foi assinado um termo de consentimento demonstrando a livre iniciativa em sua participa&#231;&#227;o ou de quem, por for&#231;a de lei, esteja representando.</p>
          <p>As crian&#231;as foram selecionadas segundo os crit&#233;rios de inclus&#227;o da amostra, e foram avaliadas segundo libera&#231;&#227;o por parte da equipe m&#233;dica.</p>
        </sec>
        <sec>
          <title>Avalia&#231;&#245;es</title>
          <p>Todas as crian&#231;as foram avaliadas quanto &#224; classe funcional, dados antropom&#233;tricos e ergoespirometria.</p>
        </sec>
        <sec>
          <title>Classe funcional</title>
          <p>A classe funcional modificada utilizada foi uma adapta&#231;&#227;o da classifica&#231;&#227;o funcional j&#225; descrita na literatura e aplicada em estudos que avaliam crian&#231;as portadoras de cardiomiopatia,<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B21">21</xref> como segue:</p>
          <p>Classe I - doen&#231;a card&#237;aca sem limita&#231;&#227;o de atividade f&#237;sica. Crian&#231;as em fase escolar realizam as aulas de gin&#225;stica e continuam at&#233; o fim dela.</p>
          <p>Classe II - limita&#231;&#227;o leve da atividade f&#237;sica. Confort&#225;veis no repouso, por&#233;m atividades comuns podem trazer taquicardia, fadiga ou dispneia. Crian&#231;as em fase escolar realizam as aulas de gin&#225;stica, mas n&#227;o continuam at&#233; o fim dela.</p>
          <p>Classe III - limita&#231;&#227;o severa da atividade. Atividades inferiores &#224;s comuns, como andar menos que um quarteir&#227;o podem causar fadiga, taquicardia ou dispneia. Crian&#231;as em fase escolar n&#227;o conseguem realizar as aulas de gin&#225;stica.</p>
          <p>Classe IV - sem condi&#231;&#245;es de realizar qualquer atividade sem apresentar desconforto. Sintomas est&#227;o presentes no repouso e se exacerbam na atividade.</p>
          <p>A partir dessa descri&#231;&#227;o, uma representa&#231;&#227;o gr&#225;fica das quarto classes funcionais foi elaborada pelo autor desse trabalho, seguindo essas descri&#231;&#245;es, para as crian&#231;as do sexo masculino e feminino (<xref ref-type="fig" rid="f4">Figuras 1</xref> e <xref ref-type="fig" rid="f5">2</xref>, respectivamente), de forma que os respons&#225;veis legais e as crian&#231;as pudessem utiliz&#225;-la. Para a elabora&#231;&#227;o desses desenhos, foi levada em considera&#231;&#227;o a imagem que crian&#231;as, a partir faixa et&#225;ria da inclus&#227;o do estudo, pudessem se visualizar.</p>
          <p>
            <fig id="f4">
              <label>Figura 1</label>
              <caption>
                <title>Classe funcional para crian&#231;as do sexo masculino.</title>
              </caption>
              <graphic xlink:href="1678-4170-abc-106-06-0502-60066-gf01-pt.jpg"/></fig>
            <fig id="f5">
              <label>Figura 2</label>
              <caption>
                <title>Classe funcional para crian&#231;as do sexo feminino.</title>
              </caption>
              <graphic xlink:href="1678-4170-abc-106-06-0502-60066-gf02-pt.jpg"/></fig>
          </p>
          <p>Inicialmente, o m&#233;dico que acompanhava as crian&#231;as opinava sobre qual classe funcional a crian&#231;a fazia parte. Essa avalia&#231;&#227;o inicial era respondida sem que os representantes legais ou mesmo as crian&#231;as estivessem presentes. Esse dado foi exposto como classe funcional segundo a equipe m&#233;dica (CFm).</p>
          <p>Em seguida, os pais ou respons&#225;veis legais opinavam qual classe funcional as crian&#231;as faziam parte, segundo as <xref ref-type="fig" rid="f4">figuras 1</xref> e <xref ref-type="fig" rid="f5">2</xref>. Essa classifica&#231;&#227;o era respondida sem que os m&#233;dicos ou mesmo as crian&#231;as estivessem presentes. Esse dado foi exposto como classe funcional segundo pais ou respons&#225;veis legais (CFp).</p>
          <p>Posteriormente, as crian&#231;as realizavam a autoavalia&#231;&#227;o da classe funcional por representa&#231;&#227;o gr&#225;fica (<xref ref-type="fig" rid="f4">Figuras 1</xref> e <xref ref-type="fig" rid="f5">2</xref>). Essa auto-percep&#231;&#227;o foi exposta como classe funcional segundo as pr&#243;prias crian&#231;as (CFc).</p>
        </sec>
        <sec>
          <title>Dados antropom&#233;tricos</title>
          <p>Foram coletados os dados de idade, sexo, estatura, massa corp&#243;rea e &#237;ndice de massa corp&#243;rea (IMC).</p>
        </sec>
        <sec>
          <title>Dados ecocardiogr&#225;ficos</title>
          <p>A an&#225;lise da fun&#231;&#227;o card&#237;aca pelo exame de ecocardiograma foi considerada nos exames datados de at&#233; seis meses pr&#233;vios &#224; inclus&#227;o.</p>
          <p>Os exames de ecocardiograma forma conduzidos conforme recomenda&#231;&#245;es das diretrizes para a popula&#231;&#227;o pedi&#225;trica, segundo m&#233;todo de Teicholz.<xref ref-type="bibr" rid="B22">22</xref> Foram coletados os dados de FEVE, tamanho do ventr&#237;culo esquerdo na di&#225;stole, tamanho do ventr&#237;culo esquerdo na s&#237;stole, e espessura da parede do ventr&#237;culo esquerdo. Os valores de tamanho e espessura foram corrigidos pela &#225;rea de superf&#237;cie corp&#243;rea (ASC) em f&#243;rmula apropriada para crian&#231;as com mais de 10 Kg, a saber: ASC = (peso *4 +7) / (peso + 90)<xref ref-type="bibr" rid="B23">23</xref> na qual o peso &#233; dado em Kg.</p>
          <p>As crian&#231;as que possu&#237;am exame de ecocardiograma em seu prontu&#225;rio, datado de, no m&#225;ximo, seis meses anteriores &#224;s demais coletas de dados, tiveram seus dados coletados. As crian&#231;as que n&#227;o possu&#237;am exame de ecocardiograma tiveram o exame realizado e, assim, seus dados coletados em seguida.</p>
        </sec>
        <sec>
          <title>Ergoespirometria</title>
          <p>As crian&#231;as foram submetidas a teste de esfor&#231;o cardiopulmonar (TCP) em esteira program&#225;vel (Marquette series 2000, Marquette Electronics, Milwaukee, WI, EUA), segundo protocolo de Balke modificado, em rampa.<xref ref-type="bibr" rid="B21">21</xref><sup>,</sup><xref ref-type="bibr" rid="B24">24</xref><sup>-</sup><xref ref-type="bibr" rid="B26">26</xref>
					</p>
          <p>O TCP foi realizado ap&#243;s duas horas de refei&#231;&#227;o leve sem conte&#250;do de cafe&#237;na, em ambiente com temperatura controlada (21&#176;C a 23&#176;C), e ap&#243;s dois minutos em repouso, na posi&#231;&#227;o ereta sobre a esteira.<xref ref-type="bibr" rid="B25">25</xref>
					</p>
          <p>Durante o per&#237;odo inicial de repouso, de exerc&#237;cio e de recupera&#231;&#227;o, as crian&#231;as foram submetidas a monitorizar&#227;o cont&#237;nua de ventila&#231;&#227;o pulmonar, concentra&#231;&#227;o de oxig&#234;nio e de di&#243;xido de carbono nos volumes de ar inspirado e expirado (Sensormedics, modelo Vmax 229, Yorba linda, CA, EUA) respira&#231;&#227;o a respira&#231;&#227;o. Durante o TCP as crian&#231;as tamb&#233;m tiveram monitoriza&#231;&#227;o ritmo card&#237;aco continuamente em 12 deriva&#231;&#245;es (Marquette MAX 1, Marquette Electronics, Milwaukee, WI, EUA) e da press&#227;o arterial sist&#234;mica com medidas a cada minuto (monitor multiparam&#233;trico HP68S Hewlett-Packard, EUA ou transdutor oscilom&#233;trico de press&#227;o arterial HP M1008B Hewlett-Packard, EUA).<xref ref-type="bibr" rid="B24">24</xref><sup>-</sup><xref ref-type="bibr" rid="B26">26</xref>
					</p>
          <p>Os crit&#233;rios de interrup&#231;&#227;o do esfor&#231;o foram as indica&#231;&#245;es absolutas citadas pelo ACC/AHA Guidelines Update For Exercise Testing, ao se atingir crit&#233;rio de exaust&#227;o (quociente respirat&#243;rio &gt; 1,0)<xref ref-type="bibr" rid="B25">25</xref> ou a presen&#231;a de sinais ou sintomas de que pudessem levar &#224; inj&#250;ria card&#237;aca, como sintomas de angina, dor de cabe&#231;a, tontura, s&#237;ncope, dispn&#233;ia excessiva, fadiga, depress&#227;o ou eleva&#231;&#227;o do segmento ST maiores que 3mm, arritmia, taquicardia supraventricular ou ventricular, bloqueio &#225;trio-ventricular ou queda progressiva de press&#227;o arterial (PA).<xref ref-type="bibr" rid="B25">25</xref>
					</p>
        </sec>
        <sec>
          <title>Analise estat&#237;stica</title>
          <p>A an&#225;lise estat&#237;stica foi realizada com o programa SPSS 12.0 para Windows (SPSS Inc., Chicago, IL, USA).</p>
          <p>O teste de Shapiro-Wilk foi utilizado para avaliar a normalidade dos dados na popula&#231;&#227;o.</p>
          <p>As caracter&#237;sticas demogr&#225;ficas das crian&#231;as foram expressas de forma descritiva, em n&#250;mero absoluto, porcentagem ou em m&#233;dia e desvio padr&#227;o. As classes funcionais foram apresentadas em n&#250;mero absoluto. As vari&#225;veis quantitativas do teste de esfor&#231;o cardiopulmonar foram expressas em m&#233;dia e desvio padr&#227;o.</p>
          <p>O teste Chi-quadrado, simbolizado por &#967;<sup>2</sup>,foi utilizado para avaliar a associa&#231;&#227;o entre as vari&#225;veis categ&#243;ricas da classe funcional, representadas pela avalia&#231;&#227;o da equipe cl&#237;nica, respons&#225;veis legais e crian&#231;as.</p>
          <p>Foi utilizado o coeficiente de correla&#231;&#227;o Pearson para dados normais e a correla&#231;&#227;o de Spearman para dados n&#227;o param&#233;tricos, a fim de correlacionar os dados quantitativos. Os achados foram interpretados como correla&#231;&#227;o diretamente proporcional (se +) ou inversamente proporcional (se -), e fraca (se 0,1 a 0,29), moderada (se 0,3 a 0,59), forte (se 0,6 a 0,79),muito forte (se de 0,8 a 0,99) ou perfeita (se 1).<xref ref-type="bibr" rid="B27">27</xref>
					</p>
        </sec>
      </sec>
      <sec sec-type="results">
        <title>Resultados</title>
        <p>Inicialmente, foram triadas 77 crian&#231;as para compor a amostra. Apenas 31 participantes inclu&#237;ram completamente os requisitos da amostra, por&#233;m apenas 19 crian&#231;as aceitaram participar do estudo. A an&#225;lise post hoc Bonferroni indicou que n&#227;o houve efeito significativo para sexo entre as crian&#231;as. </p>
        <p>Nenhuma das 19 crian&#231;as apresentou qualquer instabilidade hemodin&#226;mica durante o teste ergoespirom&#233;trico.</p>
        <p>As crian&#231;as faziam uso das seguintes medica&#231;&#245;es: &#225;cido acetil salic&#237;lico, captopril, carvedilol, digoxina, enalapril, espirinolactona, furosemida e topiramato.</p>
        <p>A <xref ref-type="table" rid="t4">Tabela 1</xref> apresenta a caracteriza&#231;&#227;o da amostra total, com detalhamento de seus dados demogr&#225;ficos e dos dados ecocardiogr&#225;ficos.</p>
        <table-wrap id="t4">
          <label>Tabela 1</label>
          <caption>
            <title>Caracteriza&#231;&#227;o da amostra</title>
          </caption>
          <table frame="hsides" rules="groups">
            <colgroup>
              <col width="1%"/>
              <col width="49%"/>
              <col width="49%"/>
            </colgroup>
            <thead>
              <tr>
                <th align="left" colspan="2">
								</th>
                <th align="center">Total (19)</th>
              </tr>
            </thead>
            <tbody>
              <tr>
                <td align="left" colspan="3">
                  <bold>Dados demogr&#225;ficos</bold>
                </td>
              </tr>
              <tr>
                <td align="left" colspan="2">Idade (anos)</td>
                <td align="center">8,7 &#177; 1,9</td>
              </tr>
              <tr>
                <td align="left" colspan="2">Sexo (F/M)</td>
                <td align="center">10/9</td>
              </tr>
              <tr>
                <td align="left" colspan="2">Massa corporal (kg)</td>
                <td align="center">30,7 &#177; 8,5</td>
              </tr>
              <tr>
                <td align="left" colspan="2">Estatura (m)</td>
                <td align="center">1,26 &#177; 0,45</td>
              </tr>
              <tr>
                <td align="left" colspan="2">IMC (kg/m<sup>2</sup>)</td>
                <td align="center">30,7 &#177; 8,5</td>
              </tr>
              <tr>
                <td align="left" colspan="2">ASC (m<sup>2</sup>)</td>
                <td align="center">111,2 &#177; 41,5</td>
              </tr>
              <tr>
                <td align="left" colspan="3">
                  <bold>Dados ecocardiogr&#225;ficos</bold>
                </td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; FEVE (%)</td>
                <td align="center">46,7 &#177; 13,8</td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; Tamanho do VE na s&#237;stole</td>
                <td align="center">48,3 &#177; 9,8</td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; Tamanho do VE na di&#225;stole</td>
                <td align="center">37,5 &#177; 12,2</td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; Espessura relativa da parede do VE</td>
                <td align="center">0,26 &#177; 0,06</td>
              </tr>
            </tbody>
          </table>
          <table-wrap-foot>
            <fn id="TFN04">
              <p>ASC: &#225;rea de superf&#237;cie corporal; CMDid-: crian&#231;as com cardiomiopatia dilatada idiop&#225;tica e FEVE&lt;40%; FEVE:fra&#231;&#227;o de eje&#231;&#227;o do ventriculo esquerdo; IC: insufici&#234;ncia card&#237;aca; IMC: &#237;ndice de massa corp&#243;rea.</p>
            </fn>
          </table-wrap-foot>
        </table-wrap>
        <p>De acordo com a equipe m&#233;dica, 13 crian&#231;as foram classificadas como CF I, cinco crian&#231;as foram classificadas como CF II, uma crian&#231;a foi classificada na CF III e nenhuma crian&#231;a foi classificada como CF IV.</p>
        <p>De acordo com os pais, 13 crian&#231;as foram classificadas como CF I, quatro crian&#231;as foram classificadas como CF II, uma crian&#231;a foi classificada na CF III e uma crian&#231;a foi classificada como CF IV.</p>
        <p>De acordo com a autopercep&#231;&#227;o da classe funcional da pr&#243;pria crian&#231;a, 11 se classificaram como CF I, seis crian&#231;as se classificaram como CF II, duas crian&#231;as se classificaram como CF III. Nenhuma crian&#231;a se autoavaliou como pertencente &#224; CF IV.</p>
        <p>A <xref ref-type="table" rid="t5">tabela 2</xref> apresenta a CFm, CFp, CFc e VO<sub>2</sub> max atingido o teste ergoespirom&#233;trico para cada participante.</p>
        <table-wrap id="t5">
          <label>Tabela 2</label>
          <caption>
            <title>Dados do teste cardiopulmonar</title>
          </caption>
          <table frame="hsides" rules="groups">
            <colgroup>
              <col width="1%"/>
              <col width="49%"/>
              <col width="49%"/>
            </colgroup>
            <thead>
              <tr>
                <th align="left" colspan="2">
								</th>
                <th align="center">Todos (19)</th>
              </tr>
            </thead>
            <tbody>
              <tr>
                <td align="left" colspan="3">
                  <bold>PAS (mmHg)</bold>
                </td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; repouso</td>
                <td align="center">102,2 &#177; 12,4</td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; pico</td>
                <td align="center">120,5 &#177; 18,1</td>
              </tr>
              <tr>
                <td align="left" colspan="3">
                  <bold>PAD (mmHg)</bold>
                </td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; repouso</td>
                <td align="center">59,2 &#177; 10,6</td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; pico</td>
                <td align="center">69,7 &#177; 13,7</td>
              </tr>
              <tr>
                <td align="left" colspan="3">
                  <bold>FC (bpm)</bold>
                </td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; repouso</td>
                <td align="center">91,2 &#177; 10,8</td>
              </tr>
              <tr>
                <td align="left">
								</td>
                <td align="left">&#8211; m&#225;xima</td>
                <td align="center">162,1 &#177; 18,7</td>
              </tr>
              <tr>
                <td align="left" colspan="2">VO<sub>2</sub> max (ml/kg/min)</td>
                <td align="center">25,5 &#177; 6,7</td>
              </tr>
              <tr>
                <td align="left" colspan="2">VE/VCO<sub>2</sub> Slope</td>
                <td align="center">37,4 &#177; 6,4</td>
              </tr>
              <tr>
                <td align="left" colspan="2">RER</td>
                <td align="center">1,02 &#177; 0,04</td>
              </tr>
              <tr>
                <td align="left" colspan="2">PetO2</td>
                <td align="center">54,3 &#177; 30,3</td>
              </tr>
              <tr>
                <td align="left" colspan="2">Tempo (min)</td>
                <td align="center">10,9 &#177; 4,3</td>
              </tr>
            </tbody>
          </table>
          <table-wrap-foot>
            <fn id="TFN05">
              <p>FC: frequ&#234;ncia card&#237;aca; PAD: press&#227;o arterial diast&#243;lica; PAS: press&#227;o arterial sist&#243;lica; PetO2: press&#227;o expirada de oxig&#234;nio; RER: raz&#227;o de troca respirat&#243;ria; VE/VCO2 Slope: inclina&#231;&#227;o da reta entre ventila&#231;&#227;o (VE) e produ&#231;&#227;o de di&#243;xido de carbono (VCO<sub>2</sub>), VO<sub>2</sub> max: consumo m&#225;ximo de oxig&#234;nio.</p>
            </fn>
          </table-wrap-foot>
        </table-wrap>
        <p>O teste Chi-quadrado mostrou que houve associa&#231;&#227;o ente a CFm e CFp (1, n = 31) = 20,6; p = 0,002. N&#227;o houve associa&#231;&#227;o significativa entre CFp e CFc (1, n = 31) = 6,7; p = 0,4. As CFm e CFc n&#227;o foram, tampouco, associadas (1, n = 31) = 1,7; p = 0,8.</p>
        <p>Pelo achado de VO<sub>2</sub> m&#225;ximo do TCP, a classifica&#231;&#227;o de Weber foi significativamente associada &#224;s tr&#234;s classes funcionais descritas no estudo, segundo o teste &#967;<sup>2</sup>: classifica&#231;&#227;o de Weber e CFm (1, n = 19) = 11,8; p = 0,003; classifica&#231;&#227;o de Weber e CFp (1, n = 19) = 20,4; p = 0,0001; classifica&#231;&#227;o de Weber e CFc (1, n = 19) = 6.4; p = 0.04. <xref ref-type="fig" rid="f6">Figura 3</xref>.</p>
        <p>
          <fig id="f6">
            <label>Figura 3</label>
            <caption>
              <title>Classe funcional, classifica&#231;&#227;o de Weber e consumo m&#225;ximo de oxig&#234;nio. c: crian&#231;a; CF: Classe funcional; m: equipe m&#233;dica; p: pais ou respons&#225;veis legais; VO<sub>2</sub> max: consumo m&#225;ximo de oxig&#234;nio. <sup>a</sup>p = 0.003; <sup>b</sup>p = 0.0001; <sup>c</sup>p = 0.04.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-106-06-0502-60066-gf03-pt.jpg"/></fig>
        </p>
        <p>As crian&#231;as da amostra atingiram 84% da FC m&#225;xima. De acordo com a f&#243;rmula proposta por Tanaka (FC m&#225;xima = 208 - [0.7 &#215; idade]),<xref ref-type="bibr" rid="B28">28</xref> sendo esta FC m&#225;xima cerca de 35 bpm menor que a proposta.</p>
        <p>Os valores de VO<sub>2</sub> m&#225;ximo e FEVE apresentaram uma fraca correla&#231;&#227;o entre si, por&#233;m n&#227;o significativa (r = 0,27; p = 0,25). Da mesma forma, a FEVE n&#227;o esteve relacionada aos demais dados obtidos no TCP.</p>
        <p>Por fim, a <xref ref-type="table" rid="t6">Tabela 3</xref> apresenta os dados integrais do presente estudo, incorporando os dados das classes funcionais (CFm, CFp, CFc) e do e VO<sub>2</sub> max pelo teste cardiopulmonar para cada sujeito que comp&#244;s a amostra.</p>
        <table-wrap id="t6">
          <label>Tabela 3</label>
          <caption>
            <title>Dados das classes funcionais e do consumo m&#225;ximo de oxig&#234;nio pelo teste cardiopulmonar</title>
          </caption>
          <table frame="hsides" rules="groups">
            <colgroup width="20%">
              <col/>
              <col/>
              <col/>
              <col/>
              <col/>
            </colgroup>
            <thead>
              <tr>
                <th align="left">Sujeito</th>
                <th align="center">CFm</th>
                <th align="center">CFp</th>
                <th align="center">CFc</th>
                <th align="center">VO<sub>2</sub> max</th>
              </tr>
            </thead>
            <tbody>
              <tr>
                <td align="left">1</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">34,6</td>
              </tr>
              <tr>
                <td align="left">2</td>
                <td align="center">2</td>
                <td align="center">1</td>
                <td align="center">3</td>
                <td align="center">32</td>
              </tr>
              <tr>
                <td align="left">3</td>
                <td align="center">2</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">17,6</td>
              </tr>
              <tr>
                <td align="left">4</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">30,3</td>
              </tr>
              <tr>
                <td align="left">5</td>
                <td align="center">2</td>
                <td align="center">1</td>
                <td align="center">2</td>
                <td align="center">27</td>
              </tr>
              <tr>
                <td align="left">6</td>
                <td align="center">2</td>
                <td align="center">2</td>
                <td align="center">1</td>
                <td align="center">25</td>
              </tr>
              <tr>
                <td align="left">7</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">2</td>
                <td align="center">23,4</td>
              </tr>
              <tr>
                <td align="left">8</td>
                <td align="center">1</td>
                <td align="center">4</td>
                <td align="center">2</td>
                <td align="center">15,6</td>
              </tr>
              <tr>
                <td align="left">9</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">3</td>
                <td align="center">31</td>
              </tr>
              <tr>
                <td align="left">10</td>
                <td align="center">2</td>
                <td align="center">2</td>
                <td align="center">1</td>
                <td align="center">36.9</td>
              </tr>
              <tr>
                <td align="left">11</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">35,8</td>
              </tr>
              <tr>
                <td align="left">12</td>
                <td align="center">1</td>
                <td align="center">2</td>
                <td align="center">1</td>
                <td align="center">26</td>
              </tr>
              <tr>
                <td align="left">13</td>
                <td align="center">1</td>
                <td align="center">2</td>
                <td align="center">1</td>
                <td align="center">15,6</td>
              </tr>
              <tr>
                <td align="left">14</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">2</td>
                <td align="center">14,8</td>
              </tr>
              <tr>
                <td align="left">15</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">2</td>
                <td align="center">27,3</td>
              </tr>
              <tr>
                <td align="left">16</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">24</td>
              </tr>
              <tr>
                <td align="left">17</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">2</td>
                <td align="center">18,4</td>
              </tr>
              <tr>
                <td align="left">18</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">30</td>
              </tr>
              <tr>
                <td align="left">19</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">1</td>
                <td align="center">31,3</td>
              </tr>
            </tbody>
          </table>
          <table-wrap-foot>
            <fn id="TFN06">
              <p>CFc: classe funcional segundo as pr&#243;prias crian&#231;as. CFm: classe funcional segundo a equipe m&#233;dica; CFp: classe funcional segundo pais ou respons&#225;veis legais; VO<sub>2</sub> max: consumo m&#225;ximo de oxig&#234;nio</p>
            </fn>
          </table-wrap-foot>
        </table-wrap>
      </sec>
      <sec sec-type="discussion">
        <title>Discuss&#227;o</title>
        <p>Embora a amostra do presente estudo tenha tido um n&#250;mero reduzido de participantes, o resultado aponta que a realiza&#231;&#227;o de teste ergoespirom&#233;trico &#233; segura nas popula&#231;&#245;es descritas, que os achados de VO<sub>2</sub> m&#225;ximo est&#227;o relacionados com os dados de estratifica&#231;&#227;o pela classifica&#231;&#227;o de Weber<xref ref-type="bibr" rid="B12">12</xref> e que as figuras podem servir de recurso adicional para avalia&#231;&#227;o das crian&#231;as com CMDid e com IC (por CMDid pr&#233;via) e FEVE preservada.</p>
        <p>Quanto aos dados antropom&#233;tricos, todas as crian&#231;as inclu&#237;das se encontravam na fase de pr&#233;-puberdade,<xref ref-type="bibr" rid="B17">17</xref> sendo assim, n&#227;o houve influ&#234;ncia hormonal dos achados obtidos.<xref ref-type="bibr" rid="B29">29</xref>
				</p>
        <p>Apesar de todas as crian&#231;as inclu&#237;das no presente estudo estarem na faixa et&#225;ria na qual o crescimento linear se manifesta (de 7 e 11 anos de idade),<xref ref-type="bibr" rid="B30">30</xref> as crian&#231;as com CMDid possu&#237;am menor estatura daquelas com IC. Esse fato pode ter sido decorrente do baixo ganho ponderal durante a inf&#226;ncia<xref ref-type="bibr" rid="B31">31</xref> decorrente do baixo aporte sist&#234;mico pelo comprometimento do d&#233;bito card&#237;aco que as crian&#231;as com amior acometiemtno card&#237;aco apresentam.<xref ref-type="bibr" rid="B32">32</xref>
				</p>
        <p>Observou-se que medica&#231;&#245;es se mostraram condizentes com os achados da literatura para a popula&#231;&#227;o pedi&#225;trica com CMDid ou IC, nas quais inibidores da enzima conversor a de angiotensina (IECA),<xref ref-type="bibr" rid="B33">33</xref> beta-bloqueadores e diur&#233;ticos<xref ref-type="bibr" rid="B1">1</xref> s&#227;o empregados como tratamento padr&#227;o para os grupos citados.<xref ref-type="bibr" rid="B26">26</xref>
				</p>
        <p>&#201; poss&#237;vel que a causa para a baixa estatura das crian&#231;as com CMDid e alta dose medicamentosa seja semelhantes &#224; causa da diminui&#231;&#227;o do fen&#244;meno do descenso press&#243;rico noturno. Este, por sua vez, relacionado com severidade dos sintomas e maior atividade simp&#225;tica nervosa.<xref ref-type="bibr" rid="B34">34</xref> Neste sentido, especula-se que se fazem necess&#225;rias novas pesquisas que possam estabelecer esses v&#237;nculos.</p>
        <p>Como preditor de mortalidade para crian&#231;as, sabe-se que, assim como em adultos, a toler&#226;ncia ao exerc&#237;cio tamb&#233;m &#233; preditora de mortalidade em crian&#231;as com insufici&#234;ncia card&#237;aca.<xref ref-type="bibr" rid="B7">7</xref> Al&#233;m disso, na experi&#234;ncia em avalia&#231;&#227;o ergoespirom&#233;trica<xref ref-type="bibr" rid="B21">21</xref><sup>,</sup><xref ref-type="bibr" rid="B25">25</xref> em crian&#231;as saud&#225;veis<xref ref-type="bibr" rid="B16">16</xref> e em portadoras de IC por CMDid<xref ref-type="bibr" rid="B25">25</xref> acima de 6 anos,<xref ref-type="bibr" rid="B16">16</xref><sup>,</sup><xref ref-type="bibr" rid="B25">25</xref><sup>,</sup><xref ref-type="bibr" rid="B32">32</xref> as respostas cardiovasculares e metab&#243;licas s&#227;o semelhantes &#224;quelas observadas em adultos com as mesmas caracter&#237;sticas cl&#237;nicas.<xref ref-type="bibr" rid="B17">17</xref>
				</p>
        <p>Em nossas avalia&#231;&#245;es de TCP, foi observada que, em ambos os grupos de crian&#231;as com CMDid e nas com IC com FEVE preservada, existe a mesma incapacidade em atingir a FC m&#225;xima predita para a idade no teste de esfor&#231;o. Essa mesma situa&#231;&#227;o &#233; corroborada por resultados em pesquisa realizada em adultos com IC<xref ref-type="bibr" rid="B35">35</xref> e em crian&#231;as com CMdid,<xref ref-type="bibr" rid="B17">17</xref> nos quais 80% da FC m&#225;xima na m&#233;dia para a idade foi alcan&#231;ada e s&#227;o similares &#224;queles encontrados neste estudo, onde os valores se situam entre 82 a 84% da FC m&#225;xima.</p>
        <p>Os valores de VO<sub>2</sub> max observado no presente estudo diferiram entre os dois grupos. Isto porque acredita-se que seja decorrente do pico de VO<sub>2</sub> ocorrer entre 13 e 14 anos,<xref ref-type="bibr" rid="B16">16</xref><sup>,</sup><xref ref-type="bibr" rid="B21">21</xref><sup>,</sup><xref ref-type="bibr" rid="B29">29</xref> ou seja, espera-se que os par&#226;metros deste indicador estejam em ascens&#227;o no per&#237;odo da pr&#233;-puberdade, fase em que foram avaliadas.<xref ref-type="bibr" rid="B29">29</xref><sup>,</sup><xref ref-type="bibr" rid="B36">36</xref> Apesar de uma revis&#227;o sistem&#225;tica realizada pelo autor do atual trabalho apontar que meninas na fase pr&#233; puberdade possuem em m&#233;dia 20% menores valores de VO<sub>2</sub> max que meninos na mesma fase,<xref ref-type="bibr" rid="B37">37</xref> por influencia hormonal e da gordura corp&#243;rea,<xref ref-type="bibr" rid="B37">37</xref><sup>,</sup><xref ref-type="bibr" rid="B38">38</xref> este achado n&#227;o foi aqui identificado uma vez que n&#227;o houve efeito significativo do sexo na vari&#225;vel entre as crian&#231;as dos dois grupos, ap&#243;s a an&#225;lise de Post hoc de Bonferroni. Pode-se entender que o baixo n&#250;mero amostral interferiu negativamente na avalia&#231;&#227;o desta vari&#225;vel.</p>
        <p>Mesmo com valores de VO<sub>2</sub> max inferiores ao esperado,<xref ref-type="bibr" rid="B37">37</xref> todas as crian&#231;as chegaram ao teste m&#225;ximo segundo os crit&#233;rios de interrup&#231;&#227;o do esfor&#231;o citados pelo ACC/AHA <italic>Guidelines Update For Exercise Testing</italic><xref ref-type="bibr" rid="B39">39</xref> porque o protocolo utilizado de Balke modificado &#233; adequado &#224; popula&#231;&#227;o inclu&#237;da e a resposta ao esfor&#231;o que o protocolo exige se assemelha &#224;quela fisiol&#243;gica ao exerc&#237;cio em crian&#231;as: o tempo para atingir 50% dos valores de VO<sub>2</sub> max em crian&#231;as &#233; mais r&#225;pido que em adultos; a depend&#234;ncia da via glicog&#234;nica para satisfazer as demandas &#233; menor que em adultos; &#233; maior a utiliza&#231;&#227;o de &#225;cidos graxos como substrato energ&#233;tico na inf&#226;ncia; as crian&#231;as apresentam menores n&#237;veis de lactato no sangue (o que as dificulta chegar &#224; exaust&#227;o), menor ventila&#231;&#227;o pulmonar (VE, l/min) e menor produ&#231;&#227;o de di&#243;xido de carbono (VCO<sub>2</sub>, ml/min).<xref ref-type="bibr" rid="B29">29</xref><sup>,</sup><xref ref-type="bibr" rid="B39">39</xref>
				</p>
        <p>Conforme as informa&#231;&#245;es da classe funcional trazidas pelas crian&#231;as, seus representantes legais e a equipe m&#233;dica n&#227;o estarem correlacionadas, os dados se mostraram mais uma vez subjetivos assim como levantado em trabalhos anteriores.<xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref> No entanto, se mostraram correlacionados aos valores de VO<sub>2</sub> m&#225;ximo do TCP<xref ref-type="bibr" rid="B40">40</xref> segundo os crit&#233;rios de Weber, este extremamente usado para estratifica&#231;&#227;o e progn&#243;stico de adultos.<xref ref-type="bibr" rid="B12">12</xref> Como n&#227;o existe tal avalia&#231;&#227;o progn&#243;stica na literatura cientifica em crian&#231;as com CMDid e IC at&#233; o momento, especula-se que a medida seguir&#225; subjetiva, corroborando com os achados pr&#233;vios de 2001, nos quais os valores objetivos do TCP n&#227;o estiveram correlacionados com a classe funcional avaliada pela equipe m&#233;dica.<xref ref-type="bibr" rid="B13">13</xref>
				</p>
      </sec>
      <sec sec-type="conclusions">
        <title>Conclus&#227;o</title>
        <p>Os valores de VO<sub>2</sub> m&#225;ximo est&#227;o relacionados com os dados de estratifica&#231;&#227;o pela classifica&#231;&#227;o de Weber e que as figuras apresentadas para crian&#231;as pr&#233;-p&#250;beres podem servir de recurso adicional para avalia&#231;&#227;o das crian&#231;as com CMDid e com IC (por CMDid pr&#233;via) e FEVE preservada.</p>
      </sec>
    </body>
    <back>
      <fn-group>
        <fn fn-type="financial-disclosure">
          <p>
            <bold>Fontes de Financiamento</bold>
          </p>
          <p>O presente estudo foi financiado por FAPESP 2011/08985-0.</p>
        </fn>
        <fn fn-type="other">
          <p>
            <bold>Vincula&#231;&#227;o Acad&#234;mica</bold>
          </p>
          <p>Este artigo &#233; parte de tese de Doutorado de Aline Cristina Tavares pelo Instituto do Cora&#231;&#227;o (INCOR), Hospital das cl&#237;nicas da Faculdade de Medicina da USP.</p>
        </fn>
      </fn-group>
    </back>
  </sub-article>
</article>
