Image in Cardiology
Intimal sarcoma of the left atrium – A rare form of mitral valve obstruction
Sarcoma da íntima da aurícula esquerda – uma forma rara de obstrução valvular mitral
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A mean value of 8 mmHg and a peak of 25 mmHg were observed. <span class="elsevierStyleBold">Panel C</span>: Off-axis transesophageal image showing multilobulated mass attached to left atrial posterolateral wall. <span class="elsevierStyleBold">Panel D</span>: 12° Right Anterior Oblique, 15° caudal projection showing high vascularization of the tumor through all three major vascular beds. <span class="elsevierStyleBold">Panel E</span>: 4-chamber MRI T1-weighted image showing mass isointensity. <span class="elsevierStyleBold">Panel F</span>: 4-chamber MRI T2-weighted image depicting mass hyperintensity. <span class="elsevierStyleBold">Panel G</span>: 4-chamber MRI contrast enhanced image revealing heterogeneous uptake after gadolinium contrast administration, with low signal intensity in a central area. <span class="elsevierStyleBold">Panel H</span>: Macroscopic view of the excised tumor. <span class="elsevierStyleBold">Panel I</span>: Histopathology: (H&E, ×100) malignant neoplasm, predominantly undifferentiated, with prominent pleomorphism, with fusiform and epithelioid cells; mitotic activity and necrosis that was easy to find. <span class="elsevierStyleBold">Panel J</span>: (vimentin, ×100) – expression of vimentin in malignant cells (fusiform and epithelioid). <span class="elsevierStyleBold">Panel K</span>: (MDM2, ×100) – nuclear multifocal expression of MDM2 in malignant cells; all other markers negative (SMA, desmin, CD31, CD34, S100, FVIII, CK8/18, HMB45). <span class="elsevierStyleBold">Panel L</span>: Post-operative CT showing tumor relapse, with extensive growth into left atrium and pulmonary vein invasion.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">CT: computed tomography; MRI: magnetic resonance imaging.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Glória Abreu, Alberto Salgado, Nuno Bettencourt, Nuno Salomé, João Ferreira, Susana Guimarães" "autores" => array:6 [ 0 => array:2 [ "nombre" => "Glória" 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(Panel A) Angiographic images depicting the sequential procedural steps from diagnosis to final surgical result. 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Nuno Salomé, João Ferreira, Susana Guimarães" "autores" => array:6 [ 0 => array:4 [ "nombre" => "Glória" "apellidos" => "Abreu" "email" => array:1 [ 0 => "gloriappabreu@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Alberto" "apellidos" => "Salgado" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Nuno" "apellidos" => "Bettencourt" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Nuno" "apellidos" => "Salomé" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "João" "apellidos" => "Ferreira" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 5 => array:3 [ "nombre" => "Susana" "apellidos" => "Guimarães" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "aff0020" ] ] ] ] "afiliaciones" => array:4 [ 0 => array:3 [ "entidad" => "Serviço de Cardiologia, Hospital de Braga, Braga, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Faculdade de Medicina da Universidade do Porto, Porto, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Serviço de Cirurgia Cardiotorácica, Centro Hospital de São João, Porto, Portugal" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Serviço de Anatomia Patológica, Centro Hospitalar de São João, Porto, Portugal" "etiqueta" => "d" "identificador" => "aff0020" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Sarcoma da íntima da aurícula esquerda – uma forma rara de obstrução valvular mitral" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1419 "Ancho" => 1897 "Tamanyo" => 515893 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Panel A</span>: Apical four-chamber view showing a dilated left atrium with a large left atrial mass. <span class="elsevierStyleBold">Panel B</span>: Transmitral gradient recorded from apical approach using continuous wave Doppler across the mitral valve. A mean value of 8 mmHg and a peak of 25 mmHg were observed. <span class="elsevierStyleBold">Panel C</span>: Off-axis transesophageal image showing multilobulated mass attached to left atrial posterolateral wall. <span class="elsevierStyleBold">Panel D</span>: 12° Right Anterior Oblique, 15° caudal projection showing high vascularization of the tumor through all three major vascular beds. <span class="elsevierStyleBold">Panel E</span>: 4-chamber MRI T1-weighted image showing mass isointensity. <span class="elsevierStyleBold">Panel F</span>: 4-chamber MRI T2-weighted image depicting mass hyperintensity. <span class="elsevierStyleBold">Panel G</span>: 4-chamber MRI contrast enhanced image revealing heterogeneous uptake after gadolinium contrast administration, with low signal intensity in a central area. <span class="elsevierStyleBold">Panel H</span>: Macroscopic view of the excised tumor. <span class="elsevierStyleBold">Panel I</span>: Histopathology: (H&E, ×100) malignant neoplasm, predominantly undifferentiated, with prominent pleomorphism, with fusiform and epithelioid cells; mitotic activity and necrosis that was easy to find. <span class="elsevierStyleBold">Panel J</span>: (vimentin, ×100) – expression of vimentin in malignant cells (fusiform and epithelioid). <span class="elsevierStyleBold">Panel K</span>: (MDM2, ×100) – nuclear multifocal expression of MDM2 in malignant cells; all other markers negative (SMA, desmin, CD31, CD34, S100, FVIII, CK8/18, HMB45). <span class="elsevierStyleBold">Panel L</span>: Post-operative CT showing tumor relapse, with extensive growth into left atrium and pulmonary vein invasion.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">CT: computed tomography; MRI: magnetic resonance imaging.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 70-year-old woman was referred to the emergency department due to mild effort dyspnea, weight loss and night sweats. Transthoracic and transesophageal echocardiography revealed dilated left atrium with an extensive multilobulated mass infiltrating the left atrial posterolateral wall. It prolapsed through the mitral valve during diastole, resulting in elevated mean and peak pressure gradients (8 mmHg and 25 mmHg, respectively [Panels A-C]). Coronary angiography revealed a highly vascularized mass (Panel D, <a class="elsevierStyleCrossRef" href="#sec0010">Video 1</a>). Cardiac magnetic resonance (CMR) evidenced the full extension of the mass – measuring 10 cm – in relation to the left atrial posterolateral wall. It extended to both inferior pulmonary veins and revealed tissue characteristics in T1- (isointense) and T2-weighted (hyperintense) images. No contrast uptake was found during first-pass perfusion, but progressive and heterogeneous uptake was observed in the early and late gadolinium enhancement (LGE) images, with a low signal intensity central area (Panels E-G, <a class="elsevierStyleCrossRef" href="#sec0010">Video 2 and 3</a>). Computed tomography (CT) staging was negative for metastases. The patient was referred for surgery, but only partial resection was possible (Panel H). Histopathology revealed a malignant neoplasm, with high cellularity, which was predominantly undifferentiated and pleomorphic, with fusiform and epithelioid cells; necrosis areas and a high mitotic index (Panel I); vimentin (Panel J) and nuclear multifocal MDM2 expression (Panel K). These findings are consistent with intimal sarcoma. The patient was not considered to be a candidate for chemotherapy due to the tumor characteristics. One month later, congestive symptoms relapsed. A CT scan confirmed mass growth and pulmonary vein invasion (Panel L). The patient died within a month (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Primary cardiac tumors are rare (incidence <0.03%) and only one quarter is malignant. Intimal sarcomas are mesenchymal tumors and are more commonly encountered in great vessels, so the heart is rarely involved. To our knowledge, only eight cases have been reported to date. These tumors are locally aggressive and proliferate rapidly. Although surgical excision with tumor-free margins is the main treatment, complete surgical excision may often not be possible. The overall prognosis is poor, with a median survival of 3 to 12 months. The effectiveness of chemotherapy and radiotherapy is the subject of debate. In most cases, the cause of death is local tumor enlargement and recurrence.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Echocardiography is the screening method of choice for cardiac tumors (sensitivity 93.3-96%) and is useful for differentiating malignant neoplasms from benign ones. However, due to a limited field of view, the tumor burden may remain underdiagnosed. CMR, which offers multiplanar imaging without restrictions on the field of view, enables the tumor to be accurately located, the extent of involvement to be assessed and the functional impact of the lesion and tissue characterization to be evaluated. In this case, pulmonary vein involvement, broad-based attachment, size greater than 5 cm, ill-defined margins, heterogeneous signal on T1-and T2-weighted images and heterogeneous LGE all led to suspicion of malignancy. However, CMR features are non-specific, with intermediate T1 and high T2 signal intensities and varying amounts of first-pass uptake and LGE. An accurate diagnosis could only be made by histopathology.</p><p id="par0020" class="elsevierStylePara elsevierViewall">In this rare case of mitral valve obstruction, multimodality imaging was crucial to guide diagnosis and treatment.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:1 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-01-12" "fechaAceptado" => "2017-03-15" "apendice" => array:1 [ 0 => array:1 [ "seccion" => array:1 [ 0 => array:4 [ "apendice" => "<p id="par0035" class="elsevierStylePara elsevierViewall"><elsevierMultimedia ident="upi0005"></elsevierMultimedia><elsevierMultimedia ident="upi0010"></elsevierMultimedia><elsevierMultimedia ident="upi0015"></elsevierMultimedia></p>" "etiqueta" => "Appendix A" "titulo" => "Supplementary material" "identificador" => "sec0015" ] ] ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1419 "Ancho" => 1897 "Tamanyo" => 515893 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Panel A</span>: Apical four-chamber view showing a dilated left atrium with a large left atrial mass. <span class="elsevierStyleBold">Panel B</span>: Transmitral gradient recorded from apical approach using continuous wave Doppler across the mitral valve. A mean value of 8 mmHg and a peak of 25 mmHg were observed. <span class="elsevierStyleBold">Panel C</span>: Off-axis transesophageal image showing multilobulated mass attached to left atrial posterolateral wall. <span class="elsevierStyleBold">Panel D</span>: 12° Right Anterior Oblique, 15° caudal projection showing high vascularization of the tumor through all three major vascular beds. <span class="elsevierStyleBold">Panel E</span>: 4-chamber MRI T1-weighted image showing mass isointensity. <span class="elsevierStyleBold">Panel F</span>: 4-chamber MRI T2-weighted image depicting mass hyperintensity. <span class="elsevierStyleBold">Panel G</span>: 4-chamber MRI contrast enhanced image revealing heterogeneous uptake after gadolinium contrast administration, with low signal intensity in a central area. <span class="elsevierStyleBold">Panel H</span>: Macroscopic view of the excised tumor. <span class="elsevierStyleBold">Panel I</span>: Histopathology: (H&E, ×100) malignant neoplasm, predominantly undifferentiated, with prominent pleomorphism, with fusiform and epithelioid cells; mitotic activity and necrosis that was easy to find. <span class="elsevierStyleBold">Panel J</span>: (vimentin, ×100) – expression of vimentin in malignant cells (fusiform and epithelioid). <span class="elsevierStyleBold">Panel K</span>: (MDM2, ×100) – nuclear multifocal expression of MDM2 in malignant cells; all other markers negative (SMA, desmin, CD31, CD34, S100, FVIII, CK8/18, HMB45). <span class="elsevierStyleBold">Panel L</span>: Post-operative CT showing tumor relapse, with extensive growth into left atrium and pulmonary vein invasion.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">CT: computed tomography; MRI: magnetic resonance imaging.</p>" ] ] 1 => array:7 [ "identificador" => "upi0005" "etiqueta" => "Video 1" "tipo" => "MULTIMEDIAECOMPONENTE" "mostrarFloat" => false "mostrarDisplay" => true "Ecomponente" => array:3 [ "fichero" => "mmc1.mp4" "ficheroTamanyo" => 3026344 "Video" => array:2 [ "mp4" => array:5 [ "fichero" => "mmc1.m4v" "poster" => "mmc1.jpg" "tiempo" => 0 "alto" => 0 "ancho" => 0 ] "flv" => array:5 [ "fichero" => "mmc1.flv" "poster" => "mmc1.jpg" "tiempo" => 0 "alto" => 0 "ancho" => 0 ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Coronary angiography – 12° Right Anterior Oblique, 15° caudal projection and 9° Right Anterior Oblique, 36° cranial projection; showing intense vascularization of the mass through the three major beds.</p>" ] ] 2 => array:7 [ "identificador" => "upi0010" "etiqueta" => "Video 2" "tipo" => "MULTIMEDIAECOMPONENTE" "mostrarFloat" => false "mostrarDisplay" => true "Ecomponente" => array:3 [ "fichero" => "mmc2.mp4" "ficheroTamanyo" => 1450880 "Video" => array:2 [ "flv" => array:5 [ "fichero" => "mmc2.flv" "poster" => "mmc2.jpg" "tiempo" => 0 "alto" => 0 "ancho" => 0 ] "mp4" => array:5 [ "fichero" => "mmc2.m4v" "poster" => "mmc2.jpg" "tiempo" => 0 "alto" => 0 "ancho" => 0 ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">4-chamber, 3-chamber and 2-chamber MRI cines, characterizing the mass, showing bascule movement of the mass. It prolapsed through the mitral valve during diastole.</p>" ] ] 3 => array:7 [ "identificador" => "upi0015" "etiqueta" => "Video 3" "tipo" => "MULTIMEDIAECOMPONENTE" "mostrarFloat" => false "mostrarDisplay" => true "Ecomponente" => array:3 [ "fichero" => "mmc3.mp4" "ficheroTamanyo" => 1068551 "Video" => array:2 [ "flv" => array:5 [ "fichero" => "mmc3.flv" "poster" => "mmc3.jpg" "tiempo" => 0 "alto" => 0 "ancho" => 0 ] "mp4" => array:5 [ "fichero" => "mmc3.m4v" "poster" => "mmc3.jpg" "tiempo" => 0 "alto" => 0 "ancho" => 0 ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Dynamic contrast-enhanced MRI demonstrating no first pass perfusion.</p>" ] ] ] ] "idiomaDefecto" => "en" "url" => "/08702551/0000003700000006/v2_201806270452/S0870255117300215/v2_201806270452/en/main.assets" "Apartado" => array:4 [ "identificador" => "29264" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Imagens em Cardiologia" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/08702551/0000003700000006/v2_201806270452/S0870255117300215/v2_201806270452/en/main.pdf?idApp=UINPBA00004E&text.app=https://revportcardiol.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255117300215?idApp=UINPBA00004E" ]
| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 7 | 4 | 11 |
| 2024 Outubro | 48 | 30 | 78 |
| 2024 Setembro | 71 | 22 | 93 |
| 2024 Agosto | 74 | 34 | 108 |
| 2024 Julho | 49 | 32 | 81 |
| 2024 Junho | 30 | 17 | 47 |
| 2024 Maio | 41 | 21 | 62 |
| 2024 Abril | 55 | 32 | 87 |
| 2024 Maro | 59 | 23 | 82 |
| 2024 Fevereiro | 65 | 31 | 96 |
| 2024 Janeiro | 37 | 24 | 61 |
| 2023 Dezembro | 37 | 25 | 62 |
| 2023 Novembro | 79 | 29 | 108 |
| 2023 Outubro | 55 | 19 | 74 |
| 2023 Setembro | 51 | 18 | 69 |
| 2023 Agosto | 43 | 24 | 67 |
| 2023 Julho | 61 | 12 | 73 |
| 2023 Junho | 50 | 15 | 65 |
| 2023 Maio | 75 | 31 | 106 |
| 2023 Abril | 49 | 11 | 60 |
| 2023 Maro | 38 | 23 | 61 |
| 2023 Fevereiro | 34 | 20 | 54 |
| 2023 Janeiro | 42 | 24 | 66 |
| 2022 Dezembro | 51 | 22 | 73 |
| 2022 Novembro | 68 | 30 | 98 |
| 2022 Outubro | 55 | 33 | 88 |
| 2022 Setembro | 57 | 43 | 100 |
| 2022 Agosto | 51 | 29 | 80 |
| 2022 Julho | 45 | 32 | 77 |
| 2022 Junho | 34 | 25 | 59 |
| 2022 Maio | 51 | 42 | 93 |
| 2022 Abril | 44 | 35 | 79 |
| 2022 Maro | 42 | 40 | 82 |
| 2022 Fevereiro | 28 | 36 | 64 |
| 2022 Janeiro | 38 | 33 | 71 |
| 2021 Dezembro | 24 | 30 | 54 |
| 2021 Novembro | 31 | 39 | 70 |
| 2021 Outubro | 38 | 49 | 87 |
| 2021 Setembro | 33 | 28 | 61 |
| 2021 Agosto | 37 | 39 | 76 |
| 2021 Julho | 20 | 19 | 39 |
| 2021 Junho | 23 | 23 | 46 |
| 2021 Maio | 45 | 35 | 80 |
| 2021 Abril | 91 | 52 | 143 |
| 2021 Maro | 58 | 24 | 82 |
| 2021 Fevereiro | 91 | 18 | 109 |
| 2021 Janeiro | 31 | 14 | 45 |
| 2020 Dezembro | 43 | 12 | 55 |
| 2020 Novembro | 30 | 13 | 43 |
| 2020 Outubro | 18 | 7 | 25 |
| 2020 Setembro | 58 | 15 | 73 |
| 2020 Agosto | 35 | 9 | 44 |
| 2020 Julho | 64 | 15 | 79 |
| 2020 Junho | 38 | 13 | 51 |
| 2020 Maio | 31 | 2 | 33 |
| 2020 Abril | 37 | 13 | 50 |
| 2020 Maro | 54 | 14 | 68 |
| 2020 Fevereiro | 120 | 40 | 160 |
| 2020 Janeiro | 36 | 11 | 47 |
| 2019 Dezembro | 42 | 8 | 50 |
| 2019 Novembro | 41 | 12 | 53 |
| 2019 Outubro | 63 | 13 | 76 |
| 2019 Setembro | 85 | 14 | 99 |
| 2019 Agosto | 58 | 9 | 67 |
| 2019 Julho | 56 | 12 | 68 |
| 2019 Junho | 66 | 23 | 89 |
| 2019 Maio | 54 | 11 | 65 |
| 2019 Abril | 40 | 16 | 56 |
| 2019 Maro | 115 | 14 | 129 |
| 2019 Fevereiro | 66 | 14 | 80 |
| 2019 Janeiro | 19 | 9 | 28 |
| 2018 Dezembro | 26 | 11 | 37 |
| 2018 Novembro | 54 | 14 | 68 |
| 2018 Outubro | 77 | 26 | 103 |
| 2018 Setembro | 30 | 18 | 48 |
| 2018 Agosto | 28 | 13 | 41 |
| 2018 Julho | 54 | 29 | 83 |
| 2018 Junho | 42 | 35 | 77 |
| 2018 Maio | 11 | 22 | 33 |
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