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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">9p3BwYrDDPrZRFmMnJ9xw3k</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2017001400483</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20170152</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Viewpoint</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Limitations in the Diagnosis of Noncompaction Cardiomyopathy by Echocardiography</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Hotta</surname>
            <given-names>Viviane Tiemi</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
          <xref ref-type="aff" rid="aff2">2</xref>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Tendolo</surname>
            <given-names>Sabrina Cunha</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Rodrigues</surname>
            <given-names>Ana Clara Tude</given-names>
          </name>
          <xref ref-type="aff" rid="aff3">3</xref>
          <xref ref-type="aff" rid="aff4">4</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Fernandes</surname>
            <given-names>F&#225;bio</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Nastari</surname>
            <given-names>Luciano</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Mady</surname>
            <given-names>Charles</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
      </contrib-group>
      <aff id="aff1">
        <label>1</label>
        <institution content-type="orgname">Universidade de S&#227;o Paulo</institution>
        <institution content-type="orgdiv1">Faculdade de Medicina</institution>
        <institution content-type="orgdiv2">Instituto do Cora&#231;&#227;o (InCor) do Hospital das Cl&#237;nicas</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) do Hospital das Cl&#237;nicas da Faculdade de Medicina da Universidade de S&#227;o Paulo (HCFMUSP), S&#227;o Paulo, SP - Brazil</institution>
        <institution content-type="normalized">Universidade de S&#227;o Paulo</institution>
      </aff>
      <aff id="aff2">
        <label>2</label>
        <institution content-type="orgname">Fleury Medicina e Sa&#250;de</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">Fleury Medicina e Sa&#250;de, S&#227;o Paulo, SP - Brazil</institution>
        <institution content-type="normalized">Fleury Medicina e Sa&#250;de</institution>
      </aff>
      <aff id="aff3">
        <label>3</label>
        <institution content-type="orgname">USP</institution>
        <institution content-type="orgdiv1">FM</institution>
        <institution content-type="orgdiv2">Hospital das Cl&#237;nicas</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">Hospital das Cl&#237;nicas - FMUSP, S&#227;o Paulo, SP - Brazil</institution>
        <institution content-type="normalized">Universidade de S&#227;o Paulo</institution>
      </aff>
      <aff id="aff4">
        <label>4</label>
        <institution content-type="orgname">Hospital Israelita Albert Einstein</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">Hospital Israelita Albert Einstein, S&#227;o Paulo, SP - Brazil</institution>
        <institution content-type="normalized">Hospital Israelita Albert Einstein</institution>
      </aff>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Viviane Tiemi Hotta;</bold> Unidade Cl&#237;nica de Miocardiopatias do InCor/FMUSP. Av. Doutor En&#233;as Carvalho de Aguiar, 44. Postal Code 05403-000. S&#227;o Paulo, SP - Brazil. E-mail: <email>viviane.hotta@gmail.com</email>, <email>viviane.hotta@grupofleury.com.br</email>
				</corresp>
        <fn fn-type="con">
          <p>
            <bold>Author contributions</bold>
          </p>
          <p>Conception and design of the research: Hotta VT;Writing of the manuscript: Hotta VT, Tendolo SC; Critical revision of the manuscript for intellectual content: Hotta VT, Rodrigues ACT, Fernandes F, Mady C; Perform clinical follow-up of patients with NCM: Nastari L.</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-ppub">
        <month>11</month>
        <year>2017</year>
      </pub-date>
      <volume>109</volume>
      <issue>5</issue>
      <fpage>483</fpage>
      <lpage>488</lpage>
      <history>
        <date date-type="received">
          <day>12</day>
          <month>12</month>
          <year>2016</year>
        </date>
        <date date-type="rev-recd">
          <day>17</day>
          <month>07</month>
          <year>2017</year>
        </date>
        <date date-type="accepted">
          <day>21</day>
          <month>07</month>
          <year>2017</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="https://creativecommons.org/licenses/by/4.0/" xml:lang="en">
          <license-p>This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
        </license>
      </permissions>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Cardiomyopathies</kwd>
        <kwd>Heart Failure</kwd>
        <kwd>Diagnostic Imaging / trends</kwd>
        <kwd>Echocardiography</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <p>Noncompacted myocardium (NCM) was first reported by Grant in 1926 as a heterogeneous myocardial disorder characterized by prominent ventricular trabeculation, intratrabecular recesses, and bilayered myocardium composed by a compacted and a noncompacted layer.<xref ref-type="bibr" rid="B1">1</xref><sup>,</sup><xref ref-type="bibr" rid="B2">2</xref> It can occur isolated or associated with other cardiomyopathies, complex syndromes, metabolic disorders and congenital heart diseases, such as Ebstein's anomaly, left ventricular (LV) or right ventricular outflow tract obstruction, bicuspid aortic valve, cyanotic congenital heart diseases, and coronary artery anomalies. Although NCM usually affects the left ventricle, it can also affect both ventricles or the right ventricle alone.<xref ref-type="bibr" rid="B3">3</xref>
		</p>
    <p>The etiology of LV noncompaction is uncertain, and several etiological bases have been implicated. It is believed to be due to pathogenic mechanisms resulting in a failure in the final phase of myocardial morphogenesis, or myocardial compaction. Increasing evidence has supported a genetic base by identifying mutation in the genes that encode sarcomeric, cytoskeletal and nuclear membrane proteins.<xref ref-type="bibr" rid="B4">4</xref><sup>-</sup><xref ref-type="bibr" rid="B6">6</xref>
		</p>
    <p>Although considered rare by some authors, NCM incidence and prevalence are uncertain. Ritter et al.<xref ref-type="bibr" rid="B7">7</xref> have reported a 0.05% prevalence in all echocardiographic exams of a large institution. Patients with heart failure (HF) have been reported to have a 4% prevalence of NCM.<xref ref-type="bibr" rid="B8">8</xref>
		</p>
    <p>Currently, it is controversial whether NCM is a distinct cardiomyopathy or a morphological characteristic shared by different heart diseases. Thus, while the World Health Organization/International Society and Federation of Cardiology considers NCM an unclassified cardiomyopathy, the American Heart Association considers it a primary genetic cardiomyopathy.<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B10">10</xref> The most recent classification of cardiomyopathies proposed by the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases considers NCM an unclassified familial cardiomyopathy.<xref ref-type="bibr" rid="B11">11</xref>
		</p>
    <p>The clinical presentation can occur at any age, being highly variable. Patients can be asymptomatic or have symptoms of severe HF, associated or not with lethal arrhythmias, sudden cardiac death and thromboembolic events.<xref ref-type="bibr" rid="B12">12</xref>
		</p>
    <p>Many asymptomatic patients are identified incidentally by an echocardiography performed for assessment of cardiac murmur or for familial screening after identifying an index case.<xref ref-type="bibr" rid="B12">12</xref>
		</p>
    <p>Symptoms of HF occur in more than half of the patients with NCM, LV dysfunction being reported in up to 84% of them. In addition, arrhythmias are common: atrial fibrillation can affect 25% of adult patients, and ventricular tachyarrhythmias, up to 47% of patients. The occurrence of thromboembolic events, such as stroke, transient ischemic attack, pulmonary embolism and mesenteric ischemia, ranges from 0 to 38%, according to studies published.<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref>
		</p>
    <p>Electrocardiographic abnormalities can be present in up to 90% of patients, being, however, unspecific. The most common findings include intraventricular conduction delay, LV hypertrophy, ventricular repolarization changes, and Wolff-Parkinson-White syndrome.<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref>
		</p>
    <p>The increasing advancement in imaging techniques, in addition to the increasing application of genetic tests for the diagnosis of NCM, significantly impacts on the understanding of the mechanisms involved in the NCM genesis and its clinical treatment.</p>
    <p>Of the cardiac imaging techniques, echocardiography and cardiac magnetic resonance imaging (CMR) are the major diagnostic tools. Because of its large availability and easy access, in addition to no need for contrast agents, no radiation exposure, and mainly its low cost as compared to CMR, echocardiography is the first choice and most commonly used method for the diagnosis of NCM.<xref ref-type="bibr" rid="B8">8</xref><sup>,</sup><xref ref-type="bibr" rid="B12">12</xref><sup>-</sup><xref ref-type="bibr" rid="B14">14</xref>
		</p>
    <p>Usually the diagnosis of NCM should be considered in the presence of a bilayered myocardium composed by one thinner epicardial layer and one thick endocardial layer with prominent trabeculations and deep intraventricular recesses. The trabeculations are mainly identified on two-dimensional (2D) mode, but can be evidenced on one-dimensional or M mode. Color Doppler imaging shows blood flow in those recesses in continuity with the left ventricle.<xref ref-type="bibr" rid="B8">8</xref><sup>,</sup><xref ref-type="bibr" rid="B12">12</xref><sup>-</sup><xref ref-type="bibr" rid="B15">15</xref>
		</p>
    <p>Different echocardiographic criteria have been used to diagnose NCM, and the main ones used in clinical practice are as follows (<xref ref-type="table" rid="t1">Table 1</xref>):</p>
    <table-wrap id="t1">
      <label>Table 1</label>
      <caption>
        <title>Criteria proposed for the diagnosis of noncompacted myocardium.<xref ref-type="bibr" rid="B6">6</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref>
				</title>
      </caption>
      <table frame="hsides" rules="groups">
        <colgroup>
          <col width="25%"/>
          <col width="25%"/>
          <col width="25%"/>
          <col width="25%"/>
        </colgroup>
        <thead>
          <tr>
            <th align="left">Criterion</th>
            <th align="center">Chin et al.<xref ref-type="bibr" rid="B2">2</xref>
						</th>
            <th align="center">Jenni et al.<xref ref-type="bibr" rid="B16">16</xref>
						</th>
            <th align="center">St&#246;llberger et al.<xref ref-type="bibr" rid="B17">17</xref>
						</th>
          </tr>
        </thead>
        <tbody>
          <tr>
            <td align="left">Number of patients</td>
            <td align="center">8</td>
            <td align="center">7</td>
            <td align="center">104</td>
          </tr>
          <tr>
            <td align="left">Phase of the cardiac cycle</td>
            <td align="center">End-diastole</td>
            <td align="center">End-systole</td>
            <td align="center">Trabeculations assessed at end-diastole; NCM and CM assessed at end-systole</td>
          </tr>
          <tr>
            <td align="left">View</td>
            <td align="center">Short axis parasternal views and/or apical views</td>
            <td align="center">Short axis parasternal</td>
            <td align="center">Conventional and modified views</td>
          </tr>
          <tr>
            <td align="left">NCM/CM ratio</td>
            <td align="center">-</td>
            <td align="center">&gt;2</td>
            <td align="center">-</td>
          </tr>
        </tbody>
      </table>
    </table-wrap>
    <list list-type="order">
      <list-item>
        <p>Jenni et al.<xref ref-type="bibr" rid="B16">16</xref> consider for the diagnosis of NCM the presence of two myocardial layers, a thin compacted one (compacted myocardium - CM), and another thicker, noncompacted layer (NCM), with deep endomyocardial recesses filled with blood flow on color Doppler, in the absence of other cardiac abnormalities. A NCM/CM ratio &gt; 2 is considered diagnostic. The measures should be acquired at end-systole on short axis parasternal view<xref ref-type="bibr" rid="B16">16</xref> (<xref ref-type="fig" rid="f1">Figure 1</xref>).</p>
        <p>
          <fig id="f1">
            <label>Figure 1</label>
            <caption>
              <title>Criteria proposed for the diagnosis of noncompacted myocardium. NCM: noncompacted myocardium; CM: compacted myocardium; LV: left ventricle; LA: left atrium.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-109-05-0483-70152-gf01.jpg"/></fig>
        </p>
      </list-item>
      <list-item>
        <p>Chin et al.<xref ref-type="bibr" rid="B2">2</xref> define NCM as the presence of excessively prominent ventricular trabeculations and progressively increased total thickness of the myocardial wall from the mitral valve and towards the apical region, characterized by CM/(NCM + CM) &#8804; 0.5, assessed at end-diastole on short-axis parasternal views and/or apical views<xref ref-type="bibr" rid="B17">17</xref> (<xref ref-type="fig" rid="f2">Figure 2</xref>).</p>
        <p>
          <fig id="f2">
            <label>Figure 2</label>
            <caption>
              <title>Criteria proposed by Chin for the diagnosis of noncompacted myocardium. NCM: noncompacted myocardium; CM: compacted myocardium; LV: left ventricle; LA: left atrium.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-109-05-0483-70152-gf02.jpg"/></fig>
        </p>
      </list-item>
      <list-item>
        <p>St&#246;llberger et al.<xref ref-type="bibr" rid="B17">17</xref> define NCM as the presence of three or more trabeculations along the LV endocardial borders, different from the papillary muscles, false tendons and aberrant muscle bands, which move synchronized with the CM. In that study, the trabeculations were better visualized at end-diastole, while the bilayered myocardium was better assessed at end-systole<xref ref-type="bibr" rid="B18">18</xref> (<xref ref-type="fig" rid="f1">Figure 1</xref>).</p>
      </list-item>
      <list-item>
        <p>Recently, Paterick et al.<xref ref-type="bibr" rid="B13">13</xref> (Wisconsin) have proposed the diagnosis of NCM as a ratio NCM/CM &gt; 2, with measures taken at end-diastole on short-axis parasternal view. This criterion requires clinical validation<xref ref-type="bibr" rid="B13">13</xref> (<xref ref-type="fig" rid="f1">Figure 1</xref>).</p>
      </list-item>
    </list>
    <sec>
      <title>Critical analysis</title>
      <p>The diagnostic criteria described by Jenni et al.<xref ref-type="bibr" rid="B16">16</xref> and Chin et al.<xref ref-type="bibr" rid="B2">2</xref> are based on the measurement of NCM and CM thicknesses. However, the criteria differ regarding the cardiac cycle point at which the measurements should be taken. Chin et al.<xref ref-type="bibr" rid="B2">2</xref> propose the measurements of NCM and CM thickness be performed at end-diastole, while Jenni et al. propose them at end-systole.<xref ref-type="bibr" rid="B16">16</xref>
			</p>
      <p>The criteria proposed by Jenni et al.<xref ref-type="bibr" rid="B16">16</xref> used an NCM/CM ratio &gt; 2 at end-systole, generating higher specificity and lower sensitivity as compared to the criteria by Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> who use the CM/(NCM + CM) ratio &#8804; 0.5. However, the increase in sensitivity is due to a decrease in specificity, as compared to the criteria by Jenni et al.<xref ref-type="bibr" rid="B16">16</xref>
			</p>
      <p>A recent study has assessed the accuracy of the echocardiographic criteria described by Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> Jenni et al.<xref ref-type="bibr" rid="B16">16</xref> and St&#246;llberger et al.<xref ref-type="bibr" rid="B17">17</xref> for the diagnosis of NCM in patients with HF as compared to a control group of normal individuals. The size and the number of the trabeculations identified on apical view at end-diastole were assessed, as were the measurements of the NCM layer thickness on short-axis parasternal view at end-systole. Only concordant cases assessed by two reviewers were considered positive.<xref ref-type="bibr" rid="B18">18</xref>
			</p>
      <p>In that study, the percentages of patients meeting the diagnostic criteria for NCM were as follows: Chin et al.<xref ref-type="bibr" rid="B2">2</xref> criteria, 79%; Jenni et al.<xref ref-type="bibr" rid="B16">16</xref> criteria, 64%; and St&#246;llberger et al.<xref ref-type="bibr" rid="B17">17</xref> criteria, 53%. In that study, the Chin et al. criteria had higher sensitivity, however with a higher percentage of false-positive diagnoses. The correlation between the three echocardiographic criteria applied was weak, with only 30% of patients meeting all three criteria. All individuals of the control group had preserved ventricular dimensions and systolic function. Five control group individuals (4 black and 1 white) met at least one criterion for the diagnosis of NCM. This result emphasizes the limitation of the echocardiographic criteria to diagnose NCM, particularly in black individuals, leading to an excessive diagnosis of NCM.<xref ref-type="bibr" rid="B18">18</xref> In that study, if the control group was formed by individuals with HF, those results might have been even more discrepant.</p>
      <p>The criterion proposed by Paterick et al.<xref ref-type="bibr" rid="B13">13</xref> showed good correlation with the CMR findings, and, according to those authors, that criterion provided more accurate measurements of NCM and CM layer thickness. However, those criteria have not been validated, requiring additional confirmation and comparison with other populations with cardiac structural disease before they are adopted as a feasible diagnostic option.<xref ref-type="bibr" rid="B13">13</xref>
			</p>
      <p>Despite the increasingly frequent diagnosis of NCM, the echocardiographic criteria applied for that purpose are based on studies with limited numbers of patients and different methodologies. The point of the cardiac cycle at which the measurements of NCM and CM thickness are taken influences directly the relationship between the two layers assessed. Myocardial thickness is maximal at systole, and minimal at diastole, which directly affects the ratio between NCM and CM. In addition, the echocardiographic view on which those measurements are taken should be considered. Most criteria suggest that the measurements be taken on short-axis parasternal view; however, in daily clinical practice, measurements are more often taken on apical 4- and 2-chamber views. Finally, there is no consensus on the ratio between NCM and CM to be adopted as the diagnostic criterion, because of the lack of uniformity accepted for diagnosis.</p>
      <p>In addition, some studies have shown a considerable number of young athletes meeting the NCM diagnostic criteria, emphasizing the lack of specificity of the current diagnostic criteria when applied to highly-trained athletes.<xref ref-type="bibr" rid="B19">19</xref>
			</p>
      <p>Although infrequent, NCM has been reported in the right ventricle. However, in such cases, the diagnostic criteria are even more restricted as compared to those applied to the left ventricle on echocardiography, because of the limitation of the right ventricle echocardiographic analysis due to its complex geometry, which cannot be contemplated on only one echocardiographic view.<xref ref-type="bibr" rid="B20">20</xref><sup>,</sup><xref ref-type="bibr" rid="B21">21</xref>
			</p>
      <p>Thus, despite the increasing knowledge on NCM by echocardiography professionals, the diagnostic bases of the criteria applied are frail. Therefore, studies with a larger number of patients diagnosed with NCM are required, in addition to uniformization of the views used for the measurements, and the identification of the most suitable point in the cardiac cycle for that purpose. Such studies should ideally compare healthy individuals and patients with HF, because some "normal" patients can meet the echocardiographic criteria for the diagnosis of NCM, with no apparent clinical finding and benign prognosis, considering that the prevalence of NCM seems higher in patients with HF. In addition, the definition of the diagnostic criteria should take into consideration the particularities of specific populations, such as highly-trained athletes and black individuals, as well as the right ventricular morphological characteristics.</p>
    </sec>
    <sec sec-type="conclusions">
      <title>Conclusions</title>
      <p>Echocardiography is the first choice and most commonly used cardiac imaging method for the diagnosis of NCM. Higher knowledge and understanding of NCM is the first step to increase diagnostic accuracy in echocardiography laboratories. However, the echocardiographic criteria used so far for that purpose are highly varied and have been based on studies with a reduced number of patients.</p>
      <p>Advanced techniques, such as three-dimensional echocardiography, use of contrast agents to better define the endocardial borders, mainly in the apical region of patients with limited acoustic window, as well the analysis of myocardial strain by use of speckle tracking, are promising methods, with potential to increase the diagnostic accuracy of echocardiography in patients with NCM. The use of such techniques in clinical practice has increased in past years; however, the improvement of imaging methods requires study and constant redefinition of the echocardiographic criteria for the diagnosis of NCM.<xref ref-type="bibr" rid="B22">22</xref><sup>-</sup><xref ref-type="bibr" rid="B25">25</xref>
			</p>
      <p>The high prevalence of NCM in low-risk populations, such as athletes and normal black individuals, suggests that the increase in LV trabeculations and recesses can represent a pattern of response to the chronic increment of preload. Thus, because of the current limitations for the diagnosis of NCM, integration of clinical and electrocardiographic assessments, as well as a multimodality approach with echocardiography and CMR, is suggested.<xref ref-type="bibr" rid="B26">26</xref><sup>-</sup><xref ref-type="bibr" rid="B28">28</xref>
			</p>
      <p>In addition to the multimodality imaging approach, future perspectives include changes that suit different ethnicities and functional assessment based on multicenter and international collaboration, incorporating genetic data for a more accurate diagnosis of NCM.<xref ref-type="bibr" rid="B26">26</xref><sup>-</sup><xref ref-type="bibr" rid="B28">28</xref>
			</p>
    </sec>
  </body>
  <back>
    <fn-group>
      <fn fn-type="financial-disclosure">
        <p>
          <bold>Sources of Funding</bold>
        </p>
        <p>There were no external funding sources for this study.</p>
      </fn>
      <fn fn-type="other">
        <p>
          <bold>Study Association</bold>
        </p>
        <p>This study is not associated with any thesis or dissertation work.</p>
      </fn>
    </fn-group>
    <ref-list>
      <title>References</title>
      <ref id="B1">
        <label>1</label>
        <mixed-citation>1 Grant RT. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart. 1926;13:273-83.</mixed-citation>
        <element-citation publication-type="journal">
          <person-group person-group-type="author">
            <name>
              <surname>Grant</surname>
              <given-names>RT</given-names>
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  <sub-article article-type="translation" id="s1" xml:lang="pt">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Ponto de Vista</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Limita&#231;&#245;es no Diagn&#243;stico de Mioc&#225;rdio n&#227;o Compactado pela Ecocardiografia</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Hotta</surname>
            <given-names>Viviane Tiemi</given-names>
          </name>
          <xref ref-type="aff" rid="aff5">1</xref>
          <xref ref-type="aff" rid="aff6">2</xref>
          <xref ref-type="corresp" rid="c2"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Tendolo</surname>
            <given-names>Sabrina Cunha</given-names>
          </name>
          <xref ref-type="aff" rid="aff6">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Rodrigues</surname>
            <given-names>Ana Clara Tude</given-names>
          </name>
          <xref ref-type="aff" rid="aff7">3</xref>
          <xref ref-type="aff" rid="aff8">4</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Fernandes</surname>
            <given-names>F&#225;bio</given-names>
          </name>
          <xref ref-type="aff" rid="aff6">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Nastari</surname>
            <given-names>Luciano</given-names>
          </name>
          <xref ref-type="aff" rid="aff6">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Mady</surname>
            <given-names>Charles</given-names>
          </name>
          <xref ref-type="aff" rid="aff6">2</xref>
        </contrib>
      </contrib-group>
      <aff id="aff5">
        <label>1</label>
        <institution content-type="original">Instituto do Cora&#231;&#227;o (InCor) do Hospital das Cl&#237;nicas da Faculdade de Medicina da Universidade de S&#227;o Paulo (HCFMUSP), S&#227;o Paulo, SP - Brasil</institution>
      </aff>
      <aff id="aff6">
        <label>2</label>
        <institution content-type="original">Fleury Medicina e Sa&#250;de, S&#227;o Paulo, SP - Brasil</institution>
      </aff>
      <aff id="aff7">
        <label>3</label>
        <institution content-type="original">Hospital das Cl&#237;nicas - FMUSP, S&#227;o Paulo, SP - Brasil</institution>
      </aff>
      <aff id="aff8">
        <label>4</label>
        <institution content-type="original">Hospital Israelita Albert Einstein, S&#227;o Paulo, SP - Brasil</institution>
      </aff>
      <author-notes>
        <corresp id="c2"><bold>Correspond&#234;ncia: Viviane Tiemi Hotta</bold> Unidade Cl&#237;nica de Miocardiopatias do InCor/FMUSP. Av. Doutor En&#233;as Carvalho de Aguiar, 44. CEP 05403-000. S&#227;o Paulo, SP - Brasil. E-mail: <email>viviane.hotta@gmail.com</email>, <email>viviane.hotta@grupofleury.com.br</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: Hotta VT; Reda&#231;&#227;o do manuscrito: Hotta VT, Tendolo SC; Revis&#227;o cr&#237;tica do manuscrito quanto ao conte&#250;do intelectual importante: Hotta VT, Rodrigues ACT, Fernandes F, Mady C; Realiza acompanhamento cl&#237;nico de pacientes com mioc&#225;rdio n&#227;o compactado: Nastari L.</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>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Cardiomiopatias</kwd>
        <kwd>Insufici&#234;ncia Card&#237;aca</kwd>
        <kwd>Diagn&#243;stico por Imagem / tend&#234;ncias</kwd>
        <kwd>Ecocardiograma</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>O mioc&#225;rdio n&#227;o compactado (MNC) foi descrito pela primeira vez por Grant em 1926 como uma desordem heterog&#234;nea do mioc&#225;rdio caracterizada por trab&#233;culas proeminentes, recessos intratrabeculares e um mioc&#225;rdio composto por duas camadas distintas: compactada e n&#227;o compactada.<xref ref-type="bibr" rid="B1">1</xref><sup>,</sup><xref ref-type="bibr" rid="B2">2</xref> Pode ocorrer de forma isolada ou em associa&#231;&#227;o com outras miocardiopatias, s&#237;ndromes complexas, dist&#250;rbios metab&#243;licos e cardiopatias cong&#234;nitas, como a anomalia de Ebstein, obstru&#231;&#227;o da via de sa&#237;da do ventr&#237;culo esquerdo (VE) ou direito, valva a&#243;rtica bivalvular, cardiopatias cong&#234;nitas cian&#243;ticas e anomalias das art&#233;rias coron&#225;rias. Embora acometa geralmente o VE, pode-se encontrar comprometimento biventricular ou isolado do ventr&#237;culo direito.<xref ref-type="bibr" rid="B3">3</xref>
			</p>
      <p>A etiologia da n&#227;o compacta&#231;&#227;o do VE ainda &#233; incerta e diversas bases etiol&#243;gicas t&#234;m sido implicadas. Acredita-se que seja decorrente de mecanismos patog&#234;nicos resultando em uma defici&#234;ncia na fase final da morfog&#234;nese mioc&#225;rdica, ou compacta&#231;&#227;o mioc&#225;rdica. Evid&#234;ncias crescentes t&#234;m suportado uma base gen&#233;tica por meio da identifica&#231;&#227;o de muta&#231;&#227;o nos genes que codificam as prote&#237;nas sarcom&#233;ricas, do citoesqueleto e das membranas nucleares.<xref ref-type="bibr" rid="B4">4</xref><sup>-</sup><xref ref-type="bibr" rid="B6">6</xref>
			</p>
      <p>Embora seja considerada rara por alguns autores, sua incid&#234;ncia e preval&#234;ncia s&#227;o incertas. Ritter et al.<xref ref-type="bibr" rid="B7">7</xref> reportaram uma preval&#234;ncia de 0,05% entre todos os exames ecocardiogr&#225;ficos de uma institui&#231;&#227;o de grande porte. J&#225; em pacientes com insufici&#234;ncia card&#237;aca (IC), a preval&#234;ncia de MNC foi descrita ao redor de 4%.<xref ref-type="bibr" rid="B8">8</xref>
			</p>
      <p>Atualmente, h&#225; controv&#233;rsias se o MNC consiste em uma miocardiopatia distinta ou uma caracter&#237;stica morfol&#243;gica compartilhada por diferentes cardiopatias. Assim, enquanto &#233; considerado como uma miocardiopatia n&#227;o classificada pela <italic>World Health Organization/International Society and Federation of Cardiology,</italic> a <italic>American Heart Association</italic> considera o MNC uma miocardiopatia gen&#233;tica prim&#225;ria.<xref ref-type="bibr" rid="B9">9</xref><sup>,</sup><xref ref-type="bibr" rid="B10">10</xref> A mais recente classifica&#231;&#227;o das miocardiopatias proposta pela <italic>European Society of Cardiology Working Group on Myocardial and Pericardial Diseases</italic> considera-o como uma miocardiopatia familiar n&#227;o classificada.<xref ref-type="bibr" rid="B11">11</xref>
			</p>
      <p>A apresenta&#231;&#227;o cl&#237;nica pode ocorrer em qualquer idade, sendo altamente vari&#225;vel. Os pacientes podem ser assintom&#225;ticos ou apresentar sintomas de IC grave, associados ou n&#227;o a arritmias letais, morte s&#250;bita card&#237;aca e eventos tromboemb&#243;licos.<xref ref-type="bibr" rid="B12">12</xref>
			</p>
      <p>Muitos pacientes assintom&#225;ticos s&#227;o identificados incidentalmente pela ecocardiografia realizada para avalia&#231;&#227;o de sopro card&#237;aco ou para rastreio familiar ap&#243;s identifica&#231;&#227;o de um caso &#237;ndice.<xref ref-type="bibr" rid="B12">12</xref>
			</p>
      <p>Os sintomas de IC ocorrem em mais da metade dos pacientes e a disfun&#231;&#227;o ventricular esquerda foi reportada em at&#233; 84% dos pacientes. As arritmias tamb&#233;m s&#227;o comuns, sendo que a fibrila&#231;&#227;o atrial pode ocorrer em cerca de 25% dos pacientes adultos e as taquiarritmias ventriculares em at&#233; 47% dos pacientes. A ocorr&#234;ncia de eventos tromboemb&#243;licos, como acidente vascular encef&#225;lico, ataque isqu&#234;mico transit&#243;rio, embolia pulmonar e isquemia mesent&#233;rica, apresenta grande varia&#231;&#227;o (de 0 a 38%) de acordo com estudos publicados at&#233; o momento.<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref>
			</p>
      <p>Anormalidades eletrocardiogr&#225;ficas podem ser encontradas em at&#233; 90% dos pacientes, por&#233;m, s&#227;o inespec&#237;ficas. Os achados mais comuns incluem atrasos na condu&#231;&#227;o intraventricular, hipertrofia ventricular esquerda, altera&#231;&#245;es da repolariza&#231;&#227;o ventricular e achados de s&#237;ndrome de Wolff-Parkinson-White.<xref ref-type="bibr" rid="B3">3</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref><sup>,</sup><xref ref-type="bibr" rid="B14">14</xref>
			</p>
      <p>De qualquer modo, as crescentes evolu&#231;&#245;es dos m&#233;todos de imagem, al&#233;m do aumento da aplica&#231;&#227;o dos testes gen&#233;ticos para o diagn&#243;stico do MNC, t&#234;m impacto significativo na compreens&#227;o dos mecanismos envolvidos na g&#234;nese do MNC e tamb&#233;m na terap&#234;utica cl&#237;nica.</p>
      <p>Dentre os m&#233;todos de imagem card&#237;aca, a ecocardiografia e a resson&#226;ncia magn&#233;tica card&#237;aca (RMC) s&#227;o as principais ferramentas diagn&#243;sticas. Devido a sua ampla disponibilidade, custo e facilidade de acesso, al&#233;m de n&#227;o necessitar de uso de agentes de contraste, n&#227;o expor o paciente a radia&#231;&#227;o e principalmente pelo baixo custo quando comparada &#224; RMC, a ecocardiografia &#233; o m&#233;todo inicial e mais comumente utilizado para o diagn&#243;stico de MNC.<xref ref-type="bibr" rid="B8">8</xref><sup>,</sup><xref ref-type="bibr" rid="B12">12</xref><sup>-</sup><xref ref-type="bibr" rid="B14">14</xref>
			</p>
      <p>Usualmente, o diagn&#243;stico de MNC deve ser considerado na presen&#231;a de um mioc&#225;rdio composto por duas camadas, sendo uma camada epic&#225;rdica mais fina e uma camada endoc&#225;rdica espessa, com trabecula&#231;&#245;es proeminentes na presen&#231;a de profundos recessos intraventriculares. As trabecula&#231;&#245;es s&#227;o identificadas principalmente ao modo 2D ou bidimensional, mas tamb&#233;m podem ser evidenciadas ao Modo M ou unidimensional. O estudo Doppler colorido demonstra a presen&#231;a de fluxo sangu&#237;neo nesses recessos em continuidade com o VE.<xref ref-type="bibr" rid="B8">8</xref><sup>,</sup><xref ref-type="bibr" rid="B12">12</xref><sup>-</sup><xref ref-type="bibr" rid="B15">15</xref>
			</p>
      <p>At&#233; o momento, t&#234;m sido empregados diferentes crit&#233;rios ecocardiogr&#225;ficos para o diagn&#243;stico de MNC. Os principais crit&#233;rios ecocardiogr&#225;ficos utilizados na pr&#225;tica cl&#237;nica s&#227;o descritos a seguir (<xref ref-type="table" rid="t2">Tabela 1</xref>):</p>
      <table-wrap id="t2">
        <label>Tabela 1</label>
        <caption>
          <title>Crit&#233;rios propostos para o diagn&#243;stico de mioc&#225;rdio n&#227;o compactado.<xref ref-type="bibr" rid="B6">6</xref><sup>,</sup><xref ref-type="bibr" rid="B13">13</xref>
					</title>
        </caption>
        <table frame="hsides" rules="groups">
          <colgroup>
            <col width="25%"/>
            <col width="25%"/>
            <col width="25%"/>
            <col width="25%"/>
          </colgroup>
          <thead>
            <tr>
              <th align="left">Crit&#233;rio</th>
              <th align="center">Chin et al.<xref ref-type="bibr" rid="B2">2</xref>
							</th>
              <th align="center">Jenni et al.<xref ref-type="bibr" rid="B16">16</xref>
							</th>
              <th align="center">St&#246;llberger et al.<xref ref-type="bibr" rid="B17">17</xref>
							</th>
            </tr>
          </thead>
          <tbody>
            <tr>
              <td align="left">N&#250;mero de pacientes</td>
              <td align="center">8</td>
              <td align="center">7</td>
              <td align="center">104</td>
            </tr>
            <tr>
              <td align="left">Fase do ciclo card&#237;aco</td>
              <td align="center">Final da di&#225;stole</td>
              <td align="center">Final da s&#237;stole</td>
              <td align="center">Trabecula&#231;&#245;es avaliadas ao final da di&#225;stole e MNC e MC avaliados ao final da s&#237;stole</td>
            </tr>
            <tr>
              <td align="left">Corte</td>
              <td align="center">Paraesternais transversais e/ou apicais</td>
              <td align="center">Paraesternal transversal</td>
              <td align="center">Cortes convencionais e modificados</td>
            </tr>
            <tr>
              <td align="left">Rela&#231;&#227;o MNC/MC</td>
              <td align="center">-</td>
              <td align="center">&gt; 2</td>
              <td align="center">-</td>
            </tr>
          </tbody>
        </table>
      </table-wrap>
      <list list-type="order">
        <list-item>
          <p>Jenni et al.,<xref ref-type="bibr" rid="B16">16</xref> consideram para o diagn&#243;stico a presen&#231;a de mioc&#225;rdio composto por duas camadas, sendo uma fina compactada (C) e outra mais espessa n&#227;o compactada (NC) com profundos recessos endomioc&#225;rdicos preenchidos por fluxo sangu&#237;neo ao Doppler colorido, na aus&#234;ncia de outras anormalidades card&#237;acas. &#201; considerada diagn&#243;stica uma rela&#231;&#227;o NC/C &gt; 2. As medidas devem ser adquiridas ao final da s&#237;stole no corte paraesternal transversal<xref ref-type="bibr" rid="B16">16</xref> (<xref ref-type="fig" rid="f3">Figura 1</xref>).</p>
          <p>
            <fig id="f3">
              <label>Figura 1</label>
              <caption>
                <title>Representa&#231;&#227;o esquem&#225;tica dos crit&#233;rios propostos para o diagn&#243;stico de mioc&#225;rdio n&#227;o compactado. MNC: mioc&#225;rdio n&#227;o compactado; MC: mioc&#225;rdio compactado; VE: ventr&#237;culo esquerdo; AE: &#225;trio esquerdo.</title>
              </caption>
              <graphic xlink:href="1678-4170-abc-109-05-0483-70152-gf01-pt.jpg"/></fig>
          </p>
        </list-item>
        <list-item>
          <p>Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> definem MNC na presen&#231;a de trabecula&#231;&#227;o proeminente excessiva e aumento progressivo da espessura total da parede mioc&#225;rdica a partir da valva mitral em dire&#231;&#227;o &#224; regi&#227;o apical caracterizado por uma propor&#231;&#227;o de MC/(MNC + MC) &#8804; 0,5 avaliada ao final da di&#225;stole nos cortes paraesternais transversais e/ou cortes apicais (<xref ref-type="fig" rid="f4">Figura 2</xref>).</p>
          <p>
            <fig id="f4">
              <label>Figura 2</label>
              <caption>
                <title>Representa&#231;&#227;o esquem&#225;tica dos crit&#233;rios propostos por Chin et al para o diagn&#243;stico de mioc&#225;rdio n&#227;o compactado. MNC: mioc&#225;rdio n&#227;o compactado; MC: mioc&#225;rdio compactado; VE: ventr&#237;culo esquerdo; AE: &#225;trio esquerdo</title>
              </caption>
              <graphic xlink:href="1678-4170-abc-109-05-0483-70152-gf02-pt.jpg"/></fig>
          </p>
        </list-item>
        <list-item>
          <p>St&#246;llberger et al.,<xref ref-type="bibr" rid="B17">17</xref> definem MNC como a presen&#231;a de tr&#234;s ou mais trabecula&#231;&#245;es ao longo das bordas endoc&#225;rdicas do VE, distintas dos m&#250;sculos papilares, falsos tend&#245;es e bandas musculares aberrantes e que se movimentam sincronicamente com o MC. Nesse estudo, as trabecula&#231;&#245;es foram melhor visibilizadas ao final da di&#225;stole, enquanto o mioc&#225;rdio composto por duas camadas foi melhor avaliado ao final da s&#237;stole (<xref ref-type="fig" rid="f3">Figura 1</xref>).</p>
        </list-item>
        <list-item>
          <p>Recentemente, Paterick et al.,<xref ref-type="bibr" rid="B13">13</xref> (Wisconsin) propuseram o diagn&#243;stico de MNC como uma raz&#227;o de MNC/MC &gt; 2, medidas tomadas ao final da di&#225;stole ao corte paraesternal transversal. Esse crit&#233;rio necessita valida&#231;&#227;o cl&#237;nica (<xref ref-type="fig" rid="f3">Figura 1</xref>).</p>
        </list-item>
      </list>
      <sec>
        <title>An&#225;lise cr&#237;tica</title>
        <p>Os crit&#233;rios diagn&#243;sticos descritos por Jenni et al.,<xref ref-type="bibr" rid="B16">16</xref> e Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> baseiam-se nas medidas da espessura do MNC e do MC. Por&#233;m, os crit&#233;rios diferem no momento do ciclo card&#237;aco em que as medidas devem ser realizadas. Enquanto Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> prop&#245;em que as medidas da espessura do MNC e do MC sejam realizadas ao final da di&#225;stole, Jenni et al.,<xref ref-type="bibr" rid="B16">16</xref> prop&#245;em a medida ao final da s&#237;stole.</p>
        <p>Os crit&#233;rios propostos por Jenni et al.,<xref ref-type="bibr" rid="B16">16</xref> utilizam a rela&#231;&#227;o MNC/MC &gt; 2 ao final da s&#237;stole, gerando maior especificidade e menor sensibilidade em compara&#231;&#227;o aos crit&#233;rios de Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> que empregaram a rela&#231;&#227;o MC/(MNC + MC) &#8804; 0,5. O aumento da sensibilidade, por&#233;m, se d&#225; &#224; custa de redu&#231;&#227;o da especificidade, quando comparado aos crit&#233;rios de Jenni et al.<xref ref-type="bibr" rid="B16">16</xref>
				</p>
        <p>Um estudo recente avaliou a acur&#225;cia dos crit&#233;rios ecocardiogr&#225;ficos descritos por Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> Jenni et al.,<xref ref-type="bibr" rid="B16">16</xref> e St&#246;llberger et al.,<xref ref-type="bibr" rid="B17">17</xref> para o diagn&#243;stico de MNC em pacientes com IC comparados a um grupo controle composto de indiv&#237;duos normais. Foram avaliados o tamanho e o n&#250;mero de trabecula&#231;&#245;es identificadas no corte apical ao final da di&#225;stole e a medida da espessura da camada de MNC no corte paraesternal eixo curto ao final da s&#237;stole. Somente casos concordantes avaliados por dois revisores foram considerados positivos.<xref ref-type="bibr" rid="B18">18</xref>
				</p>
        <p>Nesse estudo, 79% dos pacientes preencheram crit&#233;rios diagn&#243;sticos de MNC pelos crit&#233;rios de Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> 64% pelos crit&#233;rios de Jenni et al.,<xref ref-type="bibr" rid="B16">16</xref> e 53% pelos crit&#233;rios de St&#246;llberger et al.<xref ref-type="bibr" rid="B17">17</xref> Nesse estudo, os crit&#233;rios de Chin et al.,<xref ref-type="bibr" rid="B2">2</xref> apresentaram maior sensibilidade, por&#233;m com maior percentagem de diagn&#243;stico falso-positivo. Houve fraca correla&#231;&#227;o entre os tr&#234;s crit&#233;rios ecocardiogr&#225;ficos aplicados, com apenas 30% dos pacientes preenchendo todos os tr&#234;s crit&#233;rios. Todos os indiv&#237;duos do grupo controle apresentaram dimens&#245;es ventriculares e fun&#231;&#227;o sist&#243;lica preservadas. Cinco indiv&#237;duos do grupo controle (4 negros e 1 branco) preencheram um ou mais crit&#233;rios para o diagn&#243;stico de MNC. Esse resultado ressalta a limita&#231;&#227;o dos crit&#233;rios ecocardiogr&#225;ficos para o diagn&#243;stico de MNC, particularmente em indiv&#237;duos negros, resultando em excesso de diagn&#243;sticos de MNC.<xref ref-type="bibr" rid="B18">18</xref> Nesse estudo, foi realizada compara&#231;&#227;o com indiv&#237;duos normais e, talvez se fosse realizada a compara&#231;&#227;o com pacientes com IC, os resultados poderiam ser ainda mais discrepantes.</p>
        <p>O crit&#233;rio proposto por Paterick et al.,<xref ref-type="bibr" rid="B13">13</xref> em 2014 apresentou boa correla&#231;&#227;o com os achados da RMC e, de acordo com os autores, esse crit&#233;rio permitiu medidas mais acuradas da espessura do MNC e do MC. Por&#233;m, esses crit&#233;rios n&#227;o foram validados e necessitam de confirma&#231;&#245;es adicionais e compara&#231;&#227;o com outras popula&#231;&#245;es com doen&#231;a estrutural card&#237;aca, antes de serem adotados como uma op&#231;&#227;o diagn&#243;stica vi&#225;vel.<xref ref-type="bibr" rid="B13">13</xref>
				</p>
        <p>Apesar do diagn&#243;stico cada vez mais frequente do MNC, os crit&#233;rios ecocardiogr&#225;ficos aplicados para esse fim baseiam-se em estudos com n&#250;mero limitado de pacientes e metodologias distintas. O momento do ciclo card&#237;aco em que s&#227;o realizadas as medidas do MNC e MC tem influ&#234;ncia direta na rela&#231;&#227;o entre as duas camadas avaliadas. A espessura mioc&#225;rdica &#233; m&#225;xima na s&#237;stole e m&#237;nima na di&#225;stole o que impacta diretamente no valor da rela&#231;&#227;o entre o MNC e o MC. Al&#233;m disso, outro ponto a ser considerado &#233; o corte em que s&#227;o realizadas essas medidas. A maioria dos crit&#233;rios sugere que as medidas sejam realizadas no corte paraesternal transversal, por&#233;m, na pr&#225;tica cl&#237;nica di&#225;ria, as medidas s&#227;o feitas frequentemente nos cortes apical 4 e 2-c&#226;maras. Finalmente, n&#227;o h&#225; at&#233; o momento consenso acerca da raz&#227;o entre MNC e MC a ser adotada como crit&#233;rio diagn&#243;stico devido &#224; falta de uniformidade aceita para o diagn&#243;stico.</p>
        <p>Al&#233;m disso, alguns estudos demonstram um n&#250;mero consider&#225;vel de atletas jovens que apresentam crit&#233;rios diagn&#243;sticos de MNC refor&#231;ando a falta de especificidade dos crit&#233;rios diagn&#243;sticos atuais quando aplicados em popula&#231;&#245;es de atletas de elite.<xref ref-type="bibr" rid="B19">19</xref>
				</p>
        <p>Embora infrequente, &#233; descrita em literatura a ocorr&#234;ncia de n&#227;o compacta&#231;&#227;o mioc&#225;rdica em ventr&#237;culo direito. Por&#233;m, nesses casos, os crit&#233;rios diagn&#243;sticos apresentam ainda mais limita&#231;&#245;es quando comparados &#224;queles aplicados ao VE pela ecocardiografia, dada a limita&#231;&#227;o para a an&#225;lise do ventr&#237;culo direito pela ecocardiografia por sua geometria complexa e que n&#227;o pode ser contemplada em apenas um corte ecocardiogr&#225;fico.<xref ref-type="bibr" rid="B20">20</xref><sup>,</sup><xref ref-type="bibr" rid="B21">21</xref>
				</p>
        <p>Assim, apesar do maior reconhecimento do MNC pelos ecocardiografistas, s&#227;o fr&#225;geis as bases diagn&#243;sticas que fundamentam os crit&#233;rios aplicados. Portanto, s&#227;o necess&#225;rios estudos com maior n&#250;mero de pacientes com diagn&#243;stico de MNC, al&#233;m de uniformiza&#231;&#227;o dos cortes a serem utilizados para as medidas, bem como o momento mais adequado para esse fim. Esses estudos idealmente deveriam comparar grupo de indiv&#237;duos saud&#225;veis e grupo de pacientes com IC, uma vez que alguns pacientes "normais" podem preencher os crit&#233;rios ecocardiogr&#225;ficos diagn&#243;sticos de MNC, por&#233;m, sem quadro cl&#237;nico aparente e progn&#243;stico benigno e visto que a preval&#234;ncia de MNC parece ser mais elevada em pacientes com IC. Adicionalmente, para a defini&#231;&#227;o dos crit&#233;rios diagn&#243;sticos, devem ser levadas em considera&#231;&#227;o as particularidades de popula&#231;&#245;es espec&#237;ficas, como atletas de alta performance e indiv&#237;duos da ra&#231;a negra, e tamb&#233;m as caracter&#237;sticas morfol&#243;gicas do ventr&#237;culo direito.</p>
      </sec>
      <sec sec-type="conclusions">
        <title>Conclus&#245;es</title>
        <p>A ecocardiografia &#233; o m&#233;todo de imagem card&#237;aca inicial e tamb&#233;m o mais empregado para o diagn&#243;stico de MNC. O maior conhecimento e entendimento do MNC consistem no primeiro passo para maior acur&#225;cia diagn&#243;stica nos laborat&#243;rios de ecocardiografia. Por&#233;m, os crit&#233;rios ecocardiogr&#225;ficos utilizados at&#233; o momento para essa finalidade apresentam grande varia&#231;&#227;o entre si e foram baseados em estudos que avaliaram um n&#250;mero reduzido de pacientes.</p>
        <p>T&#233;cnicas avan&#231;adas, como a ecocardiografia tridimensional, utiliza&#231;&#227;o de agentes de contraste para melhor defini&#231;&#227;o das bordas endoc&#225;rdicas principalmente da regi&#227;o apical em pacientes com janela ac&#250;stica limitada, bem como a an&#225;lise da deforma&#231;&#227;o mioc&#225;rdica (<italic>strain</italic>) pela t&#233;cnica de <italic>speckle tracking</italic> s&#227;o m&#233;todos promissores e apresentam grande potencial para aumentar a acur&#225;cia diagn&#243;stica da ecocardiografia em pacientes com MNC. O emprego dessas t&#233;cnicas na pr&#225;tica cl&#237;nica tem aumentado nos &#250;ltimos anos, por&#233;m a evolu&#231;&#227;o dos m&#233;todos de imagem exige estudo e redefini&#231;&#227;o constante dos crit&#233;rios ecocardiogr&#225;ficos para o diagn&#243;stico de MNC.<xref ref-type="bibr" rid="B22">22</xref><sup>-</sup><xref ref-type="bibr" rid="B25">25</xref>
				</p>
        <p>A identifica&#231;&#227;o de alta preval&#234;ncia de n&#227;o compacta&#231;&#227;o mioc&#225;rdica em popula&#231;&#245;es de baixo risco como atletas e indiv&#237;duos normais da ra&#231;a negra sugere que o aumento de trabecula&#231;&#227;o e recessos em VE possam representar um padr&#227;o de resposta ao incremento cr&#244;nico da pr&#233;-carga. Assim, devido &#224;s limita&#231;&#245;es atuais para o diagn&#243;stico do MNC, sugere-se uma integra&#231;&#227;o entre avalia&#231;&#227;o cl&#237;nica, eletrocardiogr&#225;fica e abordagem multimodalidade pela ecocardiografia e t&#233;cnicas da RMC.<xref ref-type="bibr" rid="B26">26</xref><sup>-</sup><xref ref-type="bibr" rid="B28">28</xref>
				</p>
        <p>Perspectivas futuras incluem al&#233;m da avalia&#231;&#227;o multimodalidade, modifica&#231;&#245;es que se adequem &#224;s diferentes etnias e avalia&#231;&#227;o funcional embasada por uma colabora&#231;&#227;o multic&#234;ntrica e internacional, incorporando dados gen&#233;ticos para um diagn&#243;stico mais acurado do MNC.<xref ref-type="bibr" rid="B26">26</xref><sup>-</sup><xref ref-type="bibr" rid="B28">28</xref>
				</p>
      </sec>
    </body>
    <back>
      <fn-group>
        <fn fn-type="financial-disclosure">
          <p>
            <bold>Fontes de financiamento</bold>
          </p>
          <p>O presente estudo n&#227;o teve fontes de financiamento externas.</p>
        </fn>
        <fn fn-type="other">
          <p>
            <bold>Vincula&#231;&#227;o acad&#234;mica</bold>
          </p>
          <p>N&#227;o h&#225; vincula&#231;&#227;o deste estudo a programas de p&#243;s-gradua&#231;&#227;o.</p>
        </fn>
      </fn-group>
    </back>
  </sub-article>
</article>
