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	<front>
		<journal-meta>
			<journal-id journal-id-type="publisher-id">dic</journal-id>
			<journal-title-group>
				<journal-title>ABC Imagem Cardiovascular</journal-title>
				<abbrev-journal-title abbrev-type="publisher">Arq Bras Cardiol: Imagem cardiovasc.</abbrev-journal-title>
			</journal-title-group>
			<issn pub-type="epub">2675-312X</issn>
			<publisher>
				<publisher-name>Sociedade Brasileira de Cardiologia</publisher-name>
			</publisher>
		</journal-meta>
		<article-meta>
			<article-id pub-id-type="doi">10.47593/2675-312X/20223503ecard07</article-id>
			<article-categories>
				<subj-group subj-group-type="heading">
					<subject>O Que o Cardiologista Espera</subject>
				</subj-group>
			</article-categories>
			<title-group>
				<article-title>O que o cardiologista espera do Ecocardiograma na avaliação de miocárdio não compactado</article-title>
			</title-group>
			<contrib-group>
				<contrib contrib-type="author">
					<name>
						<surname>Nastari</surname>
						<given-names>Luciano</given-names>
					</name>
					<xref ref-type="aff" rid="aff1"><sup>1</sup></xref>
				</contrib>
			</contrib-group>
			<aff id="aff1">
				<label>1</label>
				<institution content-type="orgdiv2">Unidade Clínica de Miocardiopatias e Doenças da Aorta</institution>
				<institution content-type="orgname">HCFMUSP</institution>
				<institution content-type="orgdiv1">InCor</institution>
				<addr-line>
					<named-content content-type="city">São Paulo</named-content>
					<named-content content-type="state">SP</named-content>
				</addr-line>
				<country country="BR">Brasil</country>
				<institution content-type="original">Unidade Clínica de Miocardiopatias e Doenças da Aorta - Instituto do Coração - HCFMUSP (InCor), São Paulo, SP, Brasil.</institution>
			</aff>
			<author-notes>
				<corresp id="c01"> Correspondência: Luciano Nastari • Av. Dr. Avenida Enéas de Carvalho Aguiar, 44, Cerqueira Cesar, CEP: 05403-000 – São Paulo, SP, Brazil. E-mail: <email>luciano-nastari@uol.com.br</email>
				</corresp>
				<fn fn-type="conflict">
					<p>Conflito de interesses</p>
					<p>O autor declara não ter conflito de interesse.</p>
				</fn>
			</author-notes>
			<pub-date date-type="pub" publication-format="electronic">
				<day>23</day>
				<month>09</month>
				<year>2022</year>
			</pub-date>
			<pub-date date-type="collection" publication-format="electronic">
				<year>2022</year>
			</pub-date>
			<volume>35</volume>
			<issue>3</issue>
			<elocation-id>ecard07</elocation-id>
			<history>
				<date date-type="received">
					<day>24</day>
					<month>6</month>
					<year>2022</year>
				</date>
				<date date-type="rev-recd">
					<day>8</day>
					<month>7</month>
					<year>2022</year>
				</date>
				<date date-type="accepted">
					<day>15</day>
					<month>7</month>
					<year>2022</year>
				</date>
			</history>
			<permissions>
				<license license-type="open-access" xlink:href="https://creativecommons.org/licenses/by-nc/4.0/">
					<license-p> This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. </license-p>
				</license>
			</permissions>
			<kwd-group xml:lang="pt">
				<kwd>Miocárdio Ventricular não Compactado Isolado</kwd>
				<kwd>Ecocardiografía</kwd>
				<kwd>Ressonância Nuclear Magnética</kwd>
			</kwd-group>
			<counts>
				<fig-count count="0"/>
				<table-count count="0"/>
				<equation-count count="0"/>
				<ref-count count="12"/>
			</counts>
		</article-meta>
	</front>
	<body>
		<p>O ecocardiograma é o método de primeira escolha para o rastreamento diagnóstico do miocárdio não compactado (MNC), por ser de fácil realização, baixo custo e risco reduzido para o paciente. Ainda, trata-se de exame bastante disponível no nosso meio. Entretanto, é também um método operador-dependente, com limitações de janela ecocardiográfica dependente, principalmente para a visualização do ápice ventricular, além de difícil determinação com precisão da separação entre as camadas compactadas e não compactadas dos ventrículos.</p>
		<p>Outra limitação importante do método é a presença de maior trabeculação, já constatada e determinada na literatura em populações saudáveis de indivíduos negros, atletas e gestantes, que podem superestimar o diagnóstico.</p>
		<p>Assim, alguns autores determinaram critérios para o diagnóstico de MNC pelo ecocardiograma. Os critérios mais aceitos atualmente são os de Chin et al.<sup><xref ref-type="bibr" rid="B1">1</xref></sup>, que analisaram pacientes com MNC, sendo cinco do sexo masculino, com avaliação de corte paraesternal, eixo curto e apical, parede livre do ventrículo esquerdo (VE) em diástole máxima, da distância da superfície epicárdica até o recesso trabecular (X) e a distância da superfície epicárdica até o pico das trabéculas (Y). O diagnóstico de MNC estaria determinado quando X/Y &lt;0,5.</p>
		<p>Existem também os critérios de Jenni et al.<sup><xref ref-type="bibr" rid="B2">2</xref></sup> Nesse estudo, que envolveu 34 pacientes com MNC, sendo 25 do sexo masculino, os autores analisaram na localização das trabéculas em paredes apical, lateral média e médio inferior do VE, a espessura da parece ventricular esquerda na região compactada (C) e da região não compactada (NC). NC/C &gt;2 no eixo curto, no final da sístole, determinava o diagnóstico de MNC.</p>
		<p>Os critérios de Stöllberger et al.<sup><xref ref-type="bibr" rid="B3">3</xref></sup> e Kohli et al.<sup><xref ref-type="bibr" rid="B4">4</xref></sup> definiram, a partir da análise de 62 pacientes com MNC, sendo 49 do sexo masculino, que a presença de mais de três trabeculações na parede ventricular esquerda, com localização apical dos músculos papilares e visível em um único plano da imagem, apical 4 câmaras, definia o diagnóstico de MNC. Ao Doppler colorido, os espaços intertrabeculares são preenchidos por sangue proveniente da cavidade ventricular.</p>
		<p>Recentemente, Paterick et al.<sup><xref ref-type="bibr" rid="B5">5</xref></sup> propuseram o diagnóstico de MNC como MNC/ miocárdio compactado &gt;2, com medidas realizadas no final da diástole no corte paraesternal de eixo curto. O critério proposto por Paterick et al.<sup><xref ref-type="bibr" rid="B5">5</xref></sup>apresentou boa correlação com os achados da Ressonância Nuclear Magnética (RMC) e, segundo eles, esse critério forneceu medidas mais precisas da MNC e da espessura da camada de MC. No entanto, esses critérios não foram validados, exigindo confirmação adicional e comparação com outras populações com doença estrutural cardíaca antes de serem adotados como uma opção diagnóstica viável.</p>
		<p>Em estudo<sup><xref ref-type="bibr" rid="B4">4</xref></sup> de pacientes que foram encaminhados para um centro de avaliação de insuficiência cardíaca referendado, 199 pacientes (143 com fração de ejeção do ventrículo esquerdo &lt;40%) foram comparados com 60 voluntários saudáveis, sendo 30 da raça negra. Os autores utilizaram as três definições ecocardiográficas para identificar o MNC, avaliando o número e o tamanho das trabeculações, bem como a espessura relativa da camada não compactada quando possível. Houve correlação pobre entre as três definições ecocardiográficas, com 29,8% dos pacientes preenchendo todos os três critérios. Outro achado importante foi o fato de que 8,3% dos controles normais também preencheram os critérios para MNC. Cinco indivíduos do grupo controle (quatro negros e um branco) preencheram pelo menos um critério para o diagnóstico de MNC. Esse resultado enfatiza a limitação dos critérios ecocardiográficos para o diagnóstico de MNC, principalmente em indivíduos negros, levando a um diagnóstico excessivo de MNC. Nesse estudo, se o grupo controle fosse formado por indivíduos com insuficiência cardíaca (IC), esses resultados poderiam ter sido ainda mais discrepantes. Esse estudo sugere que os critérios diagnósticos ecocardiográficos atuais são muito sensíveis e resultam em diagnósticos excessivos de MNC em pacientes com disfunção sistólica do VE. Isso parece particularmente verdadeiro em indivíduos negros. Para evitar investigações e tratamentos desnecessários em pacientes e em seus parentes, são necessários estudos amplos e detalhados de indivíduos normais de origem racial diferente, para determinar os limites superiores dos padrões trabeculares normais. A hipertrabeculação também pode ser um fenômeno transitório. Estudo<sup><xref ref-type="bibr" rid="B6">6</xref></sup> com 102 gestantes primigestas submetidas a avaliações ecocardiográficas seriadas mostrou que 26 desenvolveram aumento de trabeculações durante a gestação (sendo que oito destas tinham critérios de MNC). O seguimento dessas pacientes por 2 anos em média mostrou completa redução das trabeculações em 19 e redução acentuada das trabeculações no grupo restante. A incapacidade de distinguir cardiomiopatia não compactada de hipertrabeculação tem implicações clínicas significativas.</p>
		<p>Apesar do diagnóstico cada vez mais frequente de MNC, os critérios ecocardiográficos aplicados para esse fim baseiam-se em estudos com número limitado de pacientes e metodologias diferentes. O ponto do ciclo cardíaco em que são realizadas as medidas de MNC e espessura do miocárdio compactado influencia diretamente na relação entre as duas camadas avaliadas. A espessura miocárdica é máxima na sístole e mínima na diástole, o que afeta diretamente a relação entre MNC e miocárdio compactado. Além disso, a visão ecocardiográfica na qual essas medidas são feitas deve ser considerada. A maioria dos critérios sugere que as medidas sejam feitas na incidência paraesternal de eixo curto; entretanto, na prática clínica diária, as medidas são mais frequentemente realizadas em cortes apicais de duas e quatro câmaras.<sup><xref ref-type="bibr" rid="B7">7</xref></sup> Finalmente, alguns estudos mostraram número considerável de jovens atletas que atendem aos critérios diagnósticos da MNC, ressaltando a falta de especificidade dos critérios diagnósticos atuais quando aplicados a atletas altamente treinados.<sup><xref ref-type="bibr" rid="B8">8</xref></sup></p>
		<p>Assim, não há consenso sobre qual critério seria o mais eficiente para o diagnóstico de MNC. O ideal para essa situação seria a presença dos três critérios para determinação diagnóstica. Na prática clínica atual, os critérios de Jenni et al.<sup><xref ref-type="bibr" rid="B2">2</xref></sup> são os mais utilizados, mas não são incomuns a identificação de aumento da trabeculação ventricular (sem aplicação dos critérios acima definidos) e laudo do ecocardiograma sugerindo a possibilidade de MNC e, assim, esperando do clínico a complementação com outro exame de imagem para confirmação diagnóstica.</p>
		<p>Outro fator importante que deve ser considerado é que talvez a presença da doença de MNC nas formas familiares esteja sendo subestimada, uma vez que o método disponível para checá-la no nosso meio é o ecocardiograma, o qual apresenta alta taxa de não identificação diagnóstica.</p>
		<p>O uso do contraste ecocardiográfico e a ecocardiografia tridimensional permitem melhor avaliação das trabéculas. Há publicações recentes<sup><xref ref-type="bibr" rid="B9">9</xref>-<xref ref-type="bibr" rid="B11">11</xref></sup> com número pequeno de pacientes em suas amostras que objetivam a comparação de pacientes com MNC e indivíduos saudáveis e com miocardiopatia dilatada. Assim, foi identificado na técnica de <italic>speckle tracking</italic><sup><xref ref-type="bibr" rid="B10">10</xref></sup> comprometimento miocárdico subclínico em pacientes com FEVE preservada e MNC, quando comparados a indivíduos normais. Outros autores identificaram a redução do <italic>strain</italic> longitudinal do VE em região média e apical e motilidade normal na região basal, diferentemente dos pacientes com cardiomiopatia dilatada que mostram redução da motilidade nas três regiões em cortes apicais. Assim, os autores sugerem que o <italic>strain</italic> longitudinal pode contribuir para separação dos casos de miocardiopatia dilatada e, portanto, reduzir a situação de excesso de diagnóstico de MNC.<sup><xref ref-type="bibr" rid="B9">9</xref>-<xref ref-type="bibr" rid="B11">11</xref></sup></p>
		<p>Em relação às diretrizes atuais, temos as seguintes orientações sobre o grau de recomendação e nível de evidência<sup><xref ref-type="bibr" rid="B12">12</xref></sup></p>
		<sec>
			<title>Nível de recomendação IIb; nível de evidência B</title>
			<list list-type="bullet">
				<list-item>
					<p>Em indivíduos com suspeita de MNC, os critérios diagnósticos por ecocardiografia ou RNM, medidos com razão eficaz de não compactação para compactação (NC/C), podem ser razoáveis para se estabelecer o diagnóstico.</p>
				</list-item>
				<list-item>
					<p>Em indivíduos com suspeita de MNC e arritmias ventriculares, a RNM ou outra imagem cardíaca avançada, podem ser razoáveis para se estabelecer o diagnóstico e para estratificação de risco.<sup><xref ref-type="bibr" rid="B12">12</xref></sup></p>
				</list-item>
			</list>
		</sec>
	</body>
	<back>
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	<sub-article article-type="translation" id="TRen" xml:lang="en">
		<front-stub>
			<article-categories>
				<subj-group subj-group-type="heading">
					<subject>What do Cardiologists Expect</subject>
				</subj-group>
			</article-categories>
			<title-group>
				<article-title>What do cardiologists expect from Echocardiography in the evaluation of non-compacted myocardium</article-title>
			</title-group>
			<contrib-group>
				<contrib contrib-type="author">
					<name>
						<surname>Nastari</surname>
						<given-names>Luciano</given-names>
					</name>
					<xref ref-type="aff" rid="aff1001"><sup>1</sup></xref>
				</contrib>
			</contrib-group>
			<aff id="aff1001">
				<label>1</label>
				<country country="BR">Brazil</country>
				<institution content-type="original">Assistant Physician - Clinical Unit Cardiomyopathies and Aortic Diseases - Instituto do Coração - HCFMUSP (InCor), São Paulo, SP, Brazil.</institution>
			</aff>
			<author-notes>
				<corresp id="c01001"> Mailing Address: Luciano Nastari • Av. Dr. Avenida Enéas de Carvalho Aguiar, 44, Cerqueira Cesar, CEP: 05403-000 – São Paulo, SP, Brazil.E-mail: luciano-nastari@uol.com.br </corresp>
				<fn fn-type="conflict">
					<p>Conflict of interest</p>
					<p>The author declares that he has no conflict of interest</p>
				</fn>
			</author-notes>
			<kwd-group xml:lang="en">
				<kwd>Isolated Noncompaction of the Ventricular Myocardium</kwd>
				<kwd>Echocardiography</kwd>
				<kwd>Nuclear magnetic resonance</kwd>
			</kwd-group>
		</front-stub>
		<body>
			<p>Echocardiography, the first-line diagnostic screening method for non-compacted myocardium (NCM), is easy to perform and features low cost and low risk for patients. It is also widely available in clinical practice. However, it is also an operator-dependent method, with limitations including a dependent echocardiographic window, primary focus of visualizing the ventricular apex, and operator difficulty accurately distinguishing between compacted and non-compacted ventricular layers.</p>
			<p>Another important limitation of echocardiography is the presence of greater trabeculations among healthy populations of Black people, athletes, and pregnant women, which may overestimate diagnoses. Thus, some authors determined diagnostic criteria for NCM by echocardiography. The currently most accepted criteria were proposed by Chin et al.<sup><xref ref-type="bibr" rid="B1">1</xref></sup>, who analyzed five men with NCM by analyzing the parasternal view, short and apical axes, left ventricular (LV) free wall in maximum diastole, distance from the epicardial surface to the trabecular recess (X), and distance from the epicardial surface to the peak of the trabeculae (Y). The NCM diagnosis is made when X/Y &lt; 0.5.</p>
			<p>Other criteria were proposed by Jenni et al.<sup><xref ref-type="bibr" rid="B2">2</xref></sup>, who studied 34 patients (25 men) with NCM and analyzed the location of the trabeculae in the apical, medial, and inferior medial LV walls and LV wall thicknesses of the compacted (C) versus non-compacted (NC) regions. Using this classification, the NCM diagnosis is made when NC/C &gt; 2 in the short-axis view at the end of systole.</p>
			<p>Stöllberger et al.<sup><xref ref-type="bibr" rid="B3">3</xref></sup> and Kohli et al.<sup><xref ref-type="bibr" rid="B4">4</xref></sup> analyzed 62 patients (49 men) with NCM and identified defined it as the presence of more than three trabeculations in the LV wall in the apical area of the papillary muscles and visible in a single apical four-chamber plane. On color Doppler, intertrabecular spaces are filled with blood from the ventricular cavity.</p>
			<p>Paterick et al.<sup><xref ref-type="bibr" rid="B5">5</xref></sup> recently proposed the diagnosis of NCM as NCM/compacted myocardium &gt; 2, with parasternal short-axis view measurements taken at end-diastole. This criterion showed a good correlation with cardiac magnetic resonance findings and, according to the authors, provided more accurate NCM and myocardium layer thickness measurements. However, this requires validation, additional confirmation, and comparison with other populations with structural heart disease before it can be accepted as a viable diagnostic option.</p>
			<p>Another study<sup><xref ref-type="bibr" rid="B4">4</xref></sup> compared 199 patients, 143 of whom had an LV ejection fraction (LVEF) &lt; 40%, referred to an accredited heart failure assessment center with 60 healthy volunteers (30 Black). The authors used the three echocardiographic definitions to identify NCM, assessed trabeculation number and size as well as the relative thickness of the non-compacted layer whenever possible. The three echocardiographic definitions showed a poor correlation, with only 29.8% of patients fulfilling all three criteria. Also, 8.3% of the normal controls met the criteria for NCM. Five subjects in the control group (four Black, one White) met at least one diagnostic criterion for NCM. This result emphasizes the limitation of echocardiographic criteria for diagnosing NCM, especially in Black individuals, leading to its overdiagnosis. In that study, if the control group included individuals with heart failure, these results could have been even more discrepant. The study findings suggest that current echocardiographic diagnostic criteria are very sensitive and result in NCM overdiagnosis in patients with LV systolic dysfunction. This seems particularly true in the Black population.</p>
			<p>Extensive and detailed studies including normal populations of different races are necessary to determine the upper limits of normal trabecular patterns to avoid unnecessary investigations and treatments in patients and their relatives. Hypertrabeculation can also be a transient phenomenon. A study<sup><xref ref-type="bibr" rid="B6">6</xref></sup> of 102 primiparous pregnant women undergoing serial echocardiographic tests showed that 26 developed increased trabeculations during pregnancy (8 women met NCM criteria). These patients were followed up for a mean two years, with total trabeculation reductions occurring in 19 women and marked trabeculation reductions occurring in the remaining group. The inability to distinguish noncompaction from hypertrabeculation cardiomyopathy has significant clinical implications.</p>
			<p>Despite the increasingly frequent diagnosis of NCM, the echocardiographic criteria are based on studies of limited numbers of patients and different methods. The point in the cardiac cycle at which NCM and compacted myocardium thickness are measured directly influences the relationship between the two evaluated layers. Myocardial thickness peaks in systole and troughs in diastole, directly affecting the relationship between NCM and compacted myocardium. In addition, we must consider the echocardiographic view in which these measurements are taken. Most criteria suggest that these measurements be taken in the parasternal short-axis view; however, in daily clinical practice, they are often taken in the apical two- and four-chamber views.<sup><xref ref-type="bibr" rid="B7">7</xref></sup> Finally, some studies showed that a considerable number of young athletes met NCM diagnostic criteria, highlighting the non-specificity of the current diagnostic criteria for highly trained athletes.<sup><xref ref-type="bibr" rid="B8">8</xref></sup></p>
			<p>Thus, consensus is lacking about which classification is the most efficient for diagnosing NCM. In this situation, patients fulfilling all three diagnostic criteria would be ideal. In current clinical practice, the Jenni et al.<sup><xref ref-type="bibr" rid="B2">2</xref></sup> criteria are the most widely used, but increased ventricular trabeculation (without the criteria defined above) and echocardiography findings suggestive of possible NCM are also common; thus, the clinician is expected to perform another imaging test to confirm the diagnosis.</p>
			<p>Another important factor worth consideration is the underestimation of familial NCM disease since echocardiography is used to screen for it and has a high misdiagnosis rate. The trabeculae are better assessed with echocardiographic contrast and three-dimensional echocardiography. Some recent publications<sup><xref ref-type="bibr" rid="B9">9</xref></sup> with small sample sizes compared NCM patients with healthy and dilated cardiomyopathy subjects. Thus, subclinical myocardial impairment was identified by speckle tracking<sup><xref ref-type="bibr" rid="B10">10</xref></sup> in patients with a preserved LVEF and NCM versus normal subjects. Other authors identified LV longitudinal strain reductions in the mid and apical regions and normal motility in the basal region, unlike patients with dilated cardiomyopathy, who presented reduced motility in the three regions in apical sections. The authors suggested that longitudinal strain may contribute to the differentiation of cases of dilated cardiomyopathy, therefore reducing NCM overdiagnosis.<sup><xref ref-type="bibr" rid="B9">9</xref></sup></p>
			<p>As for the current guidelines, we have the following recommendations and evidence levels.<sup><xref ref-type="bibr" rid="B12">12</xref></sup></p>
			<sec>
				<title>Recommendation level IIb; evidence level B</title>
				<list list-type="bullet">
					<list-item>
						<p>In individuals with suspected NCM, diagnostic criteria on echocardiography or magnetic resonance imaging may be reasonable for establishing the diagnosis when measured as an effective NC/C.</p>
					</list-item>
					<list-item>
						<p>In individuals with suspected NCM and ventricular arrhythmias, magnetic resonance imaging or other advanced cardiac imaging tests may be able to establish the diagnosis and perform the risk stratification.<sup><xref ref-type="bibr" rid="B12">12</xref></sup></p>
					</list-item>
				</list>
			</sec>
		</body>
	</sub-article>
</article>