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<front>
<journal-meta>
<journal-id journal-id-type="publisher-id">abcimg</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="publisher-id">abcimg.20240018i</article-id>
<article-id pub-id-type="doi">10.36660/abcimg.20240018i</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Giant Left Atrium due to Probable Rheumatic Disease and Atrial Functional Mitral Insufficiency</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-8044-4485</contrib-id>
<name><surname>Feitosa</surname><given-names>Israel Nilton de Almeida</given-names></name>
<role>Conception and design of the research</role>
<role>acquisition of data</role>
<role>analysis and interpretation of the data</role>
<role>statistical analysis and writing of the manuscript</role>
<role>critical revision of the manuscript for intellectual content</role>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref>
<xref ref-type="corresp" rid="c1"/>
</contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0002-5256-2318</contrib-id>
<name><surname>Guimarães Andrade</surname><given-names>Isaac Newton</given-names></name>
<role>critical revision of the manuscript for intellectual content</role>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref>
</contrib>
<aff id="aff1">
<label>1</label>
<institution content-type="orgname">Empresa Brasileira de Servicos Hospitalares</institution>
<addr-line>
<named-content content-type="city">Campina Grande</named-content>
<named-content content-type="state">PB</named-content>
</addr-line>
<country country="BR">Brazil</country>
<institution content-type="original">Empresa Brasileira de Servicos Hospitalares, Campina Grande, PB – Brazil</institution>
</aff>
</contrib-group>
<author-notes>
<corresp id="c1"><bold>Mailing Address: Israel Nilton de Almeida Feitosa</bold> • Empresa Brasileira de Servicos Hospitalares, UDIDE, Unidade de Imagem. R. Carlos Chagas, s/n. Postal Code: <postal-code>58400-398</postal-code>. São José, Campina Grande, PB – Brazil E-mail: <email>inafeitosa@hotmail.com</email></corresp>
<fn fn-type="edited-by"><p>Editor responsible for the review: Marcelo Tavares</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 publication-format="electronic" date-type="pub">
<day>25</day>
<month>04</month>
<year>2024</year></pub-date>
<pub-date publication-format="electronic" date-type="collection">
<year>2024</year></pub-date>
<volume>37</volume>
<issue>2</issue>
<elocation-id>e20240018</elocation-id>
<history>
<date date-type="received"><day>03</day><month>03</month><year>2024</year></date>
<date date-type="rev-recd"><day>15</day><month>03</month><year>2024</year></date>
<date date-type="accepted"><day>25</day><month>03</month><year>2024</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</license-p>
</license>
</permissions>
<kwd-group xml:lang="en">
<title>Keywords</title>
<kwd>Left atrium</kwd>
<kwd>rheumatic heart disease</kwd>
<kwd>echocardiography</kwd>
</kwd-group>
<counts>
<fig-count count="6"/>
<table-count count="0"/>
<equation-count count="0"/>
<ref-count count="8"/>
</counts>
</article-meta>
</front>
<body>
<sec sec-type="intro">
<title>Introduction</title>
<p>Giant left atrium is a rare condition, and most cases described in the literature are associated with rheumatic disease.<sup><xref ref-type="bibr" rid="B1">1</xref></sup> However, some clinical descriptions suggest a nonrheumatic etiology for this condition, in which characteristic findings of rheumatic disease are not identified or anatomopathological findings do not suggest a specific etiology.</p>
<p>Atrial fibrillation is present in almost all cases and contributes to the maintenance of left atrial remodeling and persistent changes in structure and function.<sup><xref ref-type="bibr" rid="B2">2</xref></sup></p>
<p>Although the mechanism of mitral valve disease may initially be associated with a primary abnormality of the valve apparatus, marked dilatation of the mitral annulus and other changes in valve morphology often led to the development of functional mitral regurgitation.<sup><xref ref-type="bibr" rid="B3">3</xref></sup></p>
</sec>
<sec sec-type="cases">
<title>Case Report</title>
<p>A 35-year-old man was referred for outpatient echocardiographic follow-up after an appointment with a cardiologist for complaints of chest pain. He reported no history of exertional dyspnea, orthopnea, or paroxysmal nocturnal dyspnea. Previous surgical correction of atrial septal defect at the age of 22. At that time, a chest radiograph showed marked enlargement of the cardiac silhouette and suggested the presence of a pericardial effusion. The electrocardiogram showed an atrial fibrillation rhythm. In previous consultations, he had been prescribed losartan, carvedilol, and spironolactone; the use of spironolactone was discontinued by the patient on his own initiative.</p>
<p>Two-dimensional transthoracic echocardiography showed a dilated left atrium with an anteroposterior diameter of 10.2 mm. The volume of the left atrium was estimated to be approximately 1,200 ml, with an indexed volume of 615 ml/m<sup>2</sup> (<xref ref-type="fig" rid="f2">Figure 2</xref>). The mitral valve had a short posterior leaflet with limited mobility (hamstringing). The anterior leaflet was elongated with a displacement of its tip toward the left atrium (i.e., pseudoprolapse), resulting in significant coaptation failure and marked eccentric regurgitation (<xref ref-type="fig" rid="f1">Figure 1</xref>). No thrombi were identified. Although the subvalvular apparatus did not show typical features of rheumatic disease (e.g., chordae tendineae without significant shortening or thickening) and no calcifications were observed, three-dimensional transthoracic examination revealed bicommissural fusion (<xref ref-type="fig" rid="f3">Figure 3</xref>). The other valves showed no relevant morphofunctional changes. The left ventricle appeared significantly dilated, with a diastolic diameter of 86 mm, a diastolic volume of 427 ml, and a reduced ejection fraction (44%), which was estimated by using the Simpson method.</p>
<fig id="f1">
<label>Figure 1</label>
<caption><title>A) Chest X-ray showing an enlargement of the cardiac area mainly due to the left atrium, which touches the right lateral border of the thoracic wall; B) Three-dimensional (3D) bidimensional echocardiogram (ventricular aspect) revealing a short/tractioned posterior mitral leaflet; C) Subvalvular mitral apparatus in the three-chamber apical view; D) Significant mitral regurgitation observed during color flow mapping.</title></caption>
<graphic xlink:href="2675-312X-abcimg-37-02-e20240018-gf01.tif"/>
</fig>
<fig id="f2">
<label>Figure 2</label>
<caption><title>Two-dimensional transthoracic echocardiography with measurements of the left atrial volume in apical 2- and 4-chamber views</title></caption>
<graphic xlink:href="2675-312X-abcimg-37-02-e20240018-gf02.tif"/>
</fig>
<fig id="f3">
<label>Figure 3</label>
<caption><title>Three-dimensional transthoracic echocardiography of the mitral valve. On the left, the atrial aspect; on the right, the ventricular aspect.</title></caption>
<graphic xlink:href="2675-312X-abcimg-37-02-e20240018-gf03.tif"/>
</fig>
<p>At a follow-up appointment, anticoagulant therapy was prescribed along with a request for evaluation for cardiac surgery.</p>
</sec>
<sec sec-type="discussion">
<title>Discussion</title>
<p>The definition of a giant left atrium is variable. It has been described on chest radiographs as extending to the right lateral aspect of the thoracic wall.<sup><xref ref-type="bibr" rid="B1">1</xref>,<xref ref-type="bibr" rid="B4">4</xref></sup> Furthermore, it may be defined by a cardiothoracic ratio of &gt; 0.7 combined with an anteroposterior diameter of the left atrium greater than 80 mm, as assessed by transthoracic echocardiography.<sup><xref ref-type="bibr" rid="B5">5</xref></sup></p>
<p>Imaging techniques such as cardiac magnetic resonance imaging and computed tomography may be needed to more accurately assess the size of the left atrium and its relationship to surrounding structures.<sup><xref ref-type="bibr" rid="B6">6</xref></sup></p>
<p>Few cases of giant left atrium are asymptomatic; when symptomatic, they are usually related to either mitral valve disease or compression of the esophagus and airway because of enlargement of the posterior wall of the left atrium, resulting in dysphagia and respiratory dysfunction. Enlargement of the left atrium also facilitates thrombus formation and predisposes to thromboembolic events.<sup><xref ref-type="bibr" rid="B7">7</xref></sup></p>
<p>Surgical management includes isolated mitral valve surgery or mitral valve surgery combined with left atrial volume reduction. The primary indication for volume reduction is the presence of intra- or extracardiac compressive symptoms.<sup><xref ref-type="bibr" rid="B8">8</xref></sup></p>
<p>In the reported case, both two-dimensional and three-dimensional echocardiographic images show a short, narrow posterior leaflet with apparent bicommissural fusion. Chronic rheumatic heart disease, a significant cause of mitral valve disease in Brazil with a variable presentation on echocardiography, is often mentioned as a possible etiological factor.</p>
<p>The history of pathological interatrial communication requiring surgical correction raises the hypothesis that atrial dilatation may have occurred at some point. This condition predisposes to atrial fibrillation and may lead to functional mitral regurgitation.</p>
<p>A significant dilation of the left ventricle is also associated with a slight reduction in ejection fraction. However, the role of these changes as a cause or consequence of mitral valve regurgitation and consequently of left atrial remodeling cannot be determined in the pathological evolution described.</p>
</sec>
<sec sec-type="conclusions">
<title>Conclusion</title>
<p>Giant left atrium is an extremely rare condition. Echocardiography plays a pivotal role in identifying this condition, investigating its underlying causes, and determining appropriate management strategies. Although a rheumatic etiology predominates in this setting, several factors and causes should be considered as contributing to the excessive dilatation of the left atrium.</p>
</sec>
</body>
<back>
<fn-group>
<fn fn-type="financial-disclosure" id="fn1"><p><bold>Sources of Funding</bold></p>
<p>There were no external funding sources for this study</p></fn>
<fn fn-type="other" id="fn2"><p><bold>Study Association</bold></p>
<p>This study is not associated with any thesis or dissertation work.</p></fn>
<fn fn-type="other" id="fn3"><p><bold>Ethics Approval and Consent to Participate</bold></p>
<p>This article does not contain any studies with human participants or animals performed by any of the authors.</p></fn>
</fn-group>
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<sub-article article-type="translation" id="S1" xml:lang="pt">
<front-stub>
<article-id pub-id-type="doi">10.36660/abcimg.20240018</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Relato de Caso</subject></subj-group></article-categories>
<title-group>
<article-title>Átrio Esquerdo Gigante por Provável Doença Reumática e Insuficiência Mitral Funcional Atrial</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-8044-4485</contrib-id>
<name><surname>Feitosa</surname><given-names>Israel Nilton de Almeida</given-names></name>
<role>Concepção e desenho da pesquisa</role>
<role>obtenção de dados</role>
<role>análise e interpretação dos dados</role>
<role>análise estatística e redação do manuscrito</role>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<xref ref-type="aff" rid="aff2"><sup>1</sup></xref>
<xref ref-type="corresp" rid="c2"/>
</contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0002-5256-2318</contrib-id>
<name><surname>Guimarães Andrade</surname><given-names>Isaac Newton</given-names></name>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<xref ref-type="aff" rid="aff2"><sup>1</sup></xref>
</contrib>
<aff id="aff2">
<label>1</label>
<addr-line>
<named-content content-type="city">Campina Grande</named-content>
<named-content content-type="state">PB</named-content>
</addr-line>
<country country="BR">Brasil</country>
<institution content-type="original">Empresa Brasileira de Servicos Hospitalares, Campina Grande, PB – Brasil</institution>
</aff>
</contrib-group>
<author-notes>
<corresp id="c2"><bold>Correspondência: Israel Nilton de Almeida Feitosa</bold> • Empresa Brasileira de Servicos Hospitalares, UDIDE, Unidade de Imagem. R. Carlos Chagas, s/n. CEP: <postal-code>58400-398</postal-code>. São José, Campina
Grande, PB – Brasil E-mail: <email>inafeitosa@hotmail.com</email></corresp>
<fn fn-type="conflict"><p><bold>Potencial Conflito de Interesse</bold></p>
<p>Declaro não haver conflito de interesses pertinentes.</p></fn>
<fn fn-type="edited-by"><p>Editor responsável pela revisão: Marcelo Tavares</p></fn>
</author-notes>
<kwd-group xml:lang="pt">
<title>Palavras-chave</title>
<kwd>Átrio esquerdo gigante</kwd>
<kwd>cardiopatia reumática</kwd>
<kwd>ecocardiografia</kwd>
</kwd-group>
</front-stub>
<body>
<sec sec-type="intro">
<title>Introdução</title>
<p>Átrio esquerdo gigante é uma condição rara e a maioria dos casos descritos na literatura está associada à doença reumática.<sup><xref ref-type="bibr" rid="B1">1</xref></sup> Entretanto, algumas descrições clínicas sugerem uma etiologia não reumática para esta condição, na qual os achados característicos da doença reumática não são identificados ou os achados anatomopatológicos não indicam uma etiologia específica.</p>
<p>A fibrilação atrial está presente em quase todos os casos e contribui para a manutenção do remodelamento atrial esquerdo e alterações persistentes em sua estrutura e função.<sup><xref ref-type="bibr" rid="B2">2</xref></sup></p>
<p>Embora o mecanismo da doença valvar mitral possa inicialmente estar associado a uma anormalidade primária do aparelho valvar, a dilatação acentuada do anel mitral e outras alterações na morfologia valvar muitas vezes levam ao desenvolvimento de insuficiência mitral funcional.<sup><xref ref-type="bibr" rid="B3">3</xref></sup></p>
</sec>
<sec sec-type="cases">
<title>Relato do caso</title>
<p>Um homem de 35 anos foi encaminhado para acompanhamento ecocardiográfico ambulatorial após consultar um cardiologista devido a queixas de dor torácica. Negou apresentar dispneia aos esforços, ortopneia ou dispneia paroxística noturna. Antecedente de correção cirúrgica de comunicação interatrial aos 22 anos. Na ocasião, uma radiografia de tórax revelou acentuado aumento da área cardíaca e sugeriu a presença de um derrame pericárdico. O eletrocardiograma demonstrou ritmo de fibrilação atrial. Em consultas anteriores, foram prescritos losartana, carvedilol e espironolactona; o uso da espironolactona foi interrompido pelo paciente por iniciativa própria.</p>
<p>O ecocardiograma transtorácico bidimensional evidenciou um átrio esquerdo gigante com diâmetro anteroposterior de 10,2 mm. O volume do átrio esquerdo foi estimado em aproximadamente 1.200 ml, com volume indexado de 615 ml/m<sup>2</sup> (<xref ref-type="fig" rid="f5">Figura 2</xref>). A valva mitral apresentava um folheto posterior curto e com mobilidade limitada (<italic>hamstringing</italic>). A cúspide anterior estava alongada, apresentando deslocamento de sua extremidade em direção ao átrio esquerdo (isto é, um pseudoprolapso), o que resultava em significativa falha de coaptação e um refluxo excêntrico de grau importante (<xref ref-type="fig" rid="f4">Figura 1</xref>). Não foram identificados trombos. Embora o aparato subvalvar não apresentasse características típicas de doença reumática (p.ex., cordas tendíneas sem encurtamento ou espessamento significativos) e não se observassem calcificações, o exame transtorácico tridimensional revelou fusão bicomissural (<xref ref-type="fig" rid="f6">Figura 3</xref>). As demais valvas não exibiam alterações morfofuncionais relevantes. O ventrículo esquerdo mostrou-se significantemente dilatado, com diâmetro diastólico de 86 mm e volume diastólico de 427 ml, e apresentou fração de ejeção reduzida (44%, estimada pelo método de Simpson).</p>
<fig id="f4">
<label>Figura 1</label>
<caption><title>A) Radiografia de tórax evidenciando aumento da área cardíaca às custas, principalmente, do átrio esquerdo, que toca a borda lateral direita da parede torácica; B) ecocardiograma bidimensional 3D (face ventricular) mostrando um folheto mitral posterior curto/tracionado; C) aparelho subvalvar mitral em janela apical 3 câmaras; D) refluxo mitral importante ao mapeamento em cores.</title></caption>
<graphic xlink:href="2675-312X-abcimg-37-02-e20240018-gf01-pt.tif"/>
</fig>
<fig id="f5">
<label>Figura 2</label>
<caption><title>Ecocardiografia transtorácica bidimensional com medidas do volume atrial esquerdo em janelas apical 2 e 4 câmaras</title></caption>
<graphic xlink:href="2675-312X-abcimg-37-02-e20240018-gf02-pt.tif"/>
</fig>
<fig id="f6">
<label>Figura 3</label>
<caption><title>Ecocardiografia transtorácica tridimensional da valva mitral. À esquerda, face atrial; à direita, face ventricular.</title></caption>
<graphic xlink:href="2675-312X-abcimg-37-02-e20240018-gf03-pt.tif"/>
</fig>
<p>Em consulta de seguimento, foi prescrita terapia anticoagulante e solicitada avaliação para cirurgia cardíaca.</p>
</sec>
<sec sec-type="discussion">
<title>Discussão</title>
<p>A definição de átrio esquerdo gigante é variável. Na radiografia de tórax, é descrito como aquele que se estende até o lado lateral direito da parede torácica.<sup><xref ref-type="bibr" rid="B1">1</xref>,<xref ref-type="bibr" rid="B4">4</xref></sup> Adicionalmente, pode ser definido por um índice cardiotorácico &gt; 0,7, combinado com um diâmetro anteroposterior do átrio esquerdo superior a 80 mm, conforme avaliação por ecocardiografia transtorácica.<sup><xref ref-type="bibr" rid="B5">5</xref></sup></p>
<p>Métodos de imagem, como ressonância magnética cardíaca e tomografia computadorizada, podem ser necessários para uma avaliação mais precisa do tamanho do átrio esquerdo e de sua relação com as estruturas circunvizinhas.<sup><xref ref-type="bibr" rid="B6">6</xref></sup></p>
<p>Poucos casos de átrio esquerdo gigante são assintomáticos; quando sintomáticos, geralmente estão relacionados à valvopatia mitral ou à compressão do esôfago e das vias aéreas pelo aumento da parede posterior do átrio esquerdo, resultando em disfagia e disfunção respiratória. O aumento do átrio esquerdo também possibilita a formação de trombos, favorecendo a ocorrência de eventos tromboembólicos.<sup><xref ref-type="bibr" rid="B7">7</xref></sup></p>
<p>O manejo cirúrgico envolve a cirurgia valvar mitral isolada ou associada à redução de volume do átrio esquerdo. A principal indicação para redução de volume é a presença de sintomas compressivos intra ou extracardíacos.<sup><xref ref-type="bibr" rid="B8">8</xref></sup></p>
<p>No caso relatado, as imagens bidimensionais, bem como aquelas adquiridas pela ecocardiografia tridimensional, demonstram um folheto posterior curto e restrito e aparente fusão bicomissural. A cardiopatia reumática crônica, uma importante causa de valvopatia mitral no Brasil que possui formas variáveis de apresentação pela ecocardiografia, é comumente associada como uma possibilidade etiológica.</p>
<p>O antecedente de comunicação interatrial patológica, que exigiu correção cirúrgica, levanta a hipótese de que a dilatação dos átrios possa ter ocorrido em algum momento. Essa condição predispõe à fibrilação atrial e pode resultar em insuficiência mitral funcional atrial.</p>
<p>Observa-se também uma significativa dilatação do ventrículo esquerdo associada a uma leve redução da fração de ejeção. No entanto, o papel dessas alterações como causa ou consequência da insuficiência mitral e, consequentemente, do remodelamento atrial esquerdo, não pode ser determinado no contexto da evolução patológica descrita.</p>
</sec>
<sec sec-type="conclusions">
<title>Conclusão</title>
<p>O átrio esquerdo gigante é uma condição extremamente rara. O ecocardiograma é um exame de imagem fundamental para o seu reconhecimento, investigação etiológica e definição de conduta. Embora a etiologia reumática seja predominante neste contexto, é importante considerar uma combinação de fatores e causas como contribuintes para a dilatação excessiva do átrio esquerdo.</p>
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<fn-group>
<fn fn-type="financial-disclosure" id="fn4"><p><bold>Fontes de Financiamento</bold></p>
<p>O presente estudo não teve fontes de financiamento externas.</p></fn>
<fn fn-type="other" id="fn5"><p><bold>Vinculação Acadêmica</bold></p>
<p>Não há vinculação deste estudo a programas de pós-graduação.</p></fn>
<fn fn-type="other" id="fn6"><p><bold>Aprovação Ética e Consentimento Informado</bold></p>
<p>Este artigo não contém estudos com humanos ou animais realizados por nenhum dos autores.</p></fn>
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