Image in Cardiology
The Olympic rings of Duchenne muscular dystrophy – cardiac computed tomography wins gold
Os anéis olímpicos da distrofia muscular de Duchenne – a tomografia computorizada cardíaca ganha a medalha de ouro
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=> "Nicholas" "apellidos" => "Hart" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:4 [ "nombre" => "Stamatis" "apellidos" => "Kapetanakis" "email" => array:1 [ 0 => "Stamatis.Kapetanakis@gstt.nhs.uk" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Department of Cardiology, Guy's and St Thomas’ NHS Foundation Trust, London, United Kingdom" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Respiratory Medicine, Guy's and St Thomas’ NHS Foundation Trust, London, United Kingdom" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Os anéis olímpicos da distrofia muscular de Duchenne – a tomografia computorizada cardíaca ganha a medalha de ouro" ] ] "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" => 2350 "Ancho" => 1000 "Tamanyo" => 246111 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Cardiac computed tomography. (A) Coronal plane with marked herniation of the abdominal contents into the left hemithorax (white arrow); (B) the corresponding axial plane with bowel loops extending up into the thorax to the level of the main pulmonary artery; (C) coronal volume-rendered image of the bilateral diaphragmatic elevation and bowel loops within the thoracic cavity; (D) contrast-filled cardiac chambers with a thin and hypoperfused inferior wall, as typically seen in Duchenne muscular dystrophy. Ao: aorta; Diaph: diaphragm; LV: left ventricle; RA: right atrium; RPA: right pulmonary artery; RV: right ventricle; TTF: transthoracic fat.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">With improvements in respiratory care, patients with Duchenne muscular dystrophy (DMD) are surviving longer, with cardiac morbidity becoming an increasingly common problem. Routine assessment of cardiac function is therefore of increasing importance in this group. We illustrate an unusual case in which marked herniation of the left hemidiaphragm prevented assessment of left ventricular (LV) function, even with the aid of contrast-enhanced echocardiography, highlighting the potential value of cardiac CT imaging in this patient group.</p><p id="par0010" class="elsevierStylePara elsevierViewall">DMD is an X-linked cardiac and skeletal muscle disorder affecting approximately 1 in 6000 boys. The disease process begins in childhood and boys affected with DMD are typically wheelchair-bound before 12 years of age.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">1</span></a> Over 90% of patients with DMD present cardiac involvement with arrhythmias, hypertrophic or dilated cardiomyopathy by the age of 20, accounting for significant morbidity.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Despite extensive cardiac involvement, initiation and up-titration of appropriate therapy has demonstrated prognostic value, and the routine and regular assessment of LV function is therefore of paramount importance. However, echocardiography is not always feasible due to the neuromuscular scoliosis affecting the body and chest wall positioning, and multimodality imaging may be useful in providing accurate cardiac assessment and guiding further management.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">A 32-year-old DMD patient with severe respiratory failure requiring continuous non-invasive ventilation and a background of diabetes mellitus and hypertension attended our multidisciplinary cardiorespiratory clinic for assessment. It was not possible to visualize any cardiac structures with 2D echocardiography and a contrast-enhanced study was arranged. Unfortunately, even with continuous infusion of microsphere contrast, it was not possible to adequately visualize the left ventricle. Appearances suggested possibly significantly reduced LV systolic function, but the study was deemed non-diagnostic.</p><p id="par0025" class="elsevierStylePara elsevierViewall">For further investigation, alternative imaging was sought. Cardiac magnetic resonance imaging was not feasible owing to body habitus, orthopnea and requirement for continuous non-invasive ventilation. Therefore, a retrospective ECG-gated cardiac computed tomography (CT) scan was performed for functional imaging. This showed mildly reduced LV systolic function with left ventricular ejection fraction of 47%, end-diastolic volume of 147 ml and end-systolic volume of 77 ml. The basal and mid inferior wall appeared thin and hypokinetic with a corresponding hypoperfusion pattern seen on first-pass perfusion imaging typical of Xp21 dystrophies (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>).<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> Note was also made of severe herniation of the abdominal contents into the left hemithorax (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>), accounting for the non-diagnostic echocardiogram and contrast echocardiogram studies. The left lung was almost completely collapsed with a minimally aerated left lower lobe and a small amount of aerated left upper lobe as a result of marked elevation of the left hemidiaphragm. There was herniation of the stomach and small and large bowel as well as the tail of the pancreas into the left thoracic cavity. In short-axis view of the left ventricle, the herniated bowel loops gave the appearance of the Olympic rings (hitherto termed the “Kapetanakis-Rajani” sign) (<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">The incidence of dilated cardiomyopathy in patients with DMD has been reported as 25% by six years of age and up to 60% by 10 years of age. For these patients, echocardiography with or without the use of contrast agents remains the mainstay of cardiac assessment. This technique however is susceptible to suboptimal image quality as a consequence of body habitus, structural diaphragmatic changes and resultant abdominal herniation into the thoracic cavity. In such instances alternative imaging techniques such as multiple gated acquisition scanning and cardiac magnetic resonance imaging have been proposed.</p><p id="par0035" class="elsevierStylePara elsevierViewall">In the case presented, we firstly show an advanced case of DMD in which significant diaphragmatic paralysis and resultant abdominal visceral herniation restricted the use of echocardiography. Secondly we show the clinical utility of gated cardiac computed tomography to assess cardiac size, function and perfusion when other techniques were not possible.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical disclosures</span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Protection of human and animal subjects</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Confidentiality of data</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Right to privacy and informed consent</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article.</p></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:3 [ "identificador" => "sec0005" "titulo" => "Ethical disclosures" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0010" "titulo" => "Protection of human and animal subjects" ] 1 => array:2 [ "identificador" => "sec0015" "titulo" => "Confidentiality of data" ] 2 => array:2 [ "identificador" => "sec0020" "titulo" => "Right to privacy and informed consent" ] ] ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflicts of interest" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2015-11-20" "fechaAceptado" => "2015-12-26" "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2350 "Ancho" => 1000 "Tamanyo" => 246111 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Cardiac computed tomography. (A) Coronal plane with marked herniation of the abdominal contents into the left hemithorax (white arrow); (B) the corresponding axial plane with bowel loops extending up into the thorax to the level of the main pulmonary artery; (C) coronal volume-rendered image of the bilateral diaphragmatic elevation and bowel loops within the thoracic cavity; (D) contrast-filled cardiac chambers with a thin and hypoperfused inferior wall, as typically seen in Duchenne muscular dystrophy. Ao: aorta; Diaph: diaphragm; LV: left ventricle; RA: right atrium; RPA: right pulmonary artery; RV: right ventricle; TTF: transthoracic fat.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 671 "Ancho" => 800 "Tamanyo" => 95444 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Cardiac computed tomography, multiplanar reformatted image showing the left and right ventricles in short-axis view among three loops of large bowel, giving the appearance of the Kapetanakis-Rajani/Olympic rings sign.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0025" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Duchenne muscular dystrophy: patterns of clinical progression and effects of supportive therapy" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "M.H. 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Belmont" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1161/CIRCULATIONAHA.104.528281" "Revista" => array:6 [ "tituloSerie" => "Circulation" "fecha" => "2005" "volumen" => "112" "paginaInicial" => "2799" "paginaFinal" => "2804" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16246949" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0035" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Cardiac assessment of patients with late stage Duchenne muscular dystrophy" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "E.A. van Bockel" 1 => "J.S. Lind" 2 => "J.G. Zijlstra" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Neth Heart J" "fecha" => "2009" "volumen" => "17" "paginaInicial" => "232" "paginaFinal" => "237" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19789685" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0040" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The pathology of the heart in progressive muscular dystrophy: epimyocardial fibrosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "K.A. Frankel" 1 => "R.J. 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| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 7 | 4 | 11 |
| 2024 Outubro | 47 | 33 | 80 |
| 2024 Setembro | 65 | 26 | 91 |
| 2024 Agosto | 53 | 33 | 86 |
| 2024 Julho | 47 | 42 | 89 |
| 2024 Junho | 45 | 29 | 74 |
| 2024 Maio | 66 | 18 | 84 |
| 2024 Abril | 59 | 30 | 89 |
| 2024 Maro | 55 | 28 | 83 |
| 2024 Fevereiro | 43 | 30 | 73 |
| 2024 Janeiro | 27 | 32 | 59 |
| 2023 Dezembro | 30 | 30 | 60 |
| 2023 Novembro | 59 | 19 | 78 |
| 2023 Outubro | 26 | 12 | 38 |
| 2023 Setembro | 29 | 20 | 49 |
| 2023 Agosto | 34 | 22 | 56 |
| 2023 Julho | 22 | 13 | 35 |
| 2023 Junho | 35 | 16 | 51 |
| 2023 Maio | 42 | 29 | 71 |
| 2023 Abril | 19 | 4 | 23 |
| 2023 Maro | 44 | 22 | 66 |
| 2023 Fevereiro | 30 | 28 | 58 |
| 2023 Janeiro | 22 | 8 | 30 |
| 2022 Dezembro | 37 | 17 | 54 |
| 2022 Novembro | 39 | 22 | 61 |
| 2022 Outubro | 31 | 15 | 46 |
| 2022 Setembro | 21 | 37 | 58 |
| 2022 Agosto | 33 | 32 | 65 |
| 2022 Julho | 34 | 39 | 73 |
| 2022 Junho | 21 | 23 | 44 |
| 2022 Maio | 28 | 32 | 60 |
| 2022 Abril | 30 | 24 | 54 |
| 2022 Maro | 29 | 34 | 63 |
| 2022 Fevereiro | 31 | 25 | 56 |
| 2022 Janeiro | 28 | 18 | 46 |
| 2021 Dezembro | 29 | 33 | 62 |
| 2021 Novembro | 44 | 35 | 79 |
| 2021 Outubro | 42 | 42 | 84 |
| 2021 Setembro | 49 | 29 | 78 |
| 2021 Agosto | 136 | 35 | 171 |
| 2021 Julho | 247 | 29 | 276 |
| 2021 Junho | 30 | 11 | 41 |
| 2021 Maio | 25 | 24 | 49 |
| 2021 Abril | 39 | 41 | 80 |
| 2021 Maro | 64 | 22 | 86 |
| 2021 Fevereiro | 58 | 13 | 71 |
| 2021 Janeiro | 24 | 10 | 34 |
| 2020 Dezembro | 21 | 17 | 38 |
| 2020 Novembro | 37 | 19 | 56 |
| 2020 Outubro | 16 | 13 | 29 |
| 2020 Setembro | 62 | 11 | 73 |
| 2020 Agosto | 27 | 10 | 37 |
| 2020 Julho | 47 | 9 | 56 |
| 2020 Junho | 22 | 9 | 31 |
| 2020 Maio | 32 | 4 | 36 |
| 2020 Abril | 33 | 11 | 44 |
| 2020 Maro | 24 | 6 | 30 |
| 2020 Fevereiro | 54 | 15 | 69 |
| 2020 Janeiro | 21 | 7 | 28 |
| 2019 Dezembro | 24 | 16 | 40 |
| 2019 Novembro | 23 | 7 | 30 |
| 2019 Outubro | 39 | 4 | 43 |
| 2019 Setembro | 17 | 7 | 24 |
| 2019 Agosto | 18 | 9 | 27 |
| 2019 Julho | 37 | 9 | 46 |
| 2019 Junho | 28 | 7 | 35 |
| 2019 Maio | 41 | 2 | 43 |
| 2019 Abril | 30 | 12 | 42 |
| 2019 Maro | 40 | 8 | 48 |
| 2019 Fevereiro | 63 | 12 | 75 |
| 2019 Janeiro | 38 | 7 | 45 |
| 2018 Dezembro | 79 | 11 | 90 |
| 2018 Novembro | 98 | 7 | 105 |
| 2018 Outubro | 207 | 18 | 225 |
| 2018 Setembro | 45 | 14 | 59 |
| 2018 Agosto | 22 | 8 | 30 |
| 2018 Julho | 12 | 4 | 16 |
| 2018 Junho | 37 | 3 | 40 |
| 2018 Maio | 40 | 9 | 49 |
| 2018 Abril | 46 | 3 | 49 |
| 2018 Maro | 64 | 11 | 75 |
| 2018 Fevereiro | 44 | 6 | 50 |
| 2018 Janeiro | 25 | 6 | 31 |
| 2017 Dezembro | 55 | 13 | 68 |
| 2017 Novembro | 31 | 9 | 40 |
| 2017 Outubro | 26 | 15 | 41 |
| 2017 Setembro | 35 | 10 | 45 |
| 2017 Agosto | 30 | 15 | 45 |
| 2017 Julho | 28 | 7 | 35 |
| 2017 Junho | 23 | 10 | 33 |
| 2017 Maio | 30 | 9 | 39 |
| 2017 Abril | 15 | 2 | 17 |
| 2017 Maro | 15 | 7 | 22 |
| 2017 Fevereiro | 19 | 4 | 23 |
| 2017 Janeiro | 22 | 9 | 31 |
| 2016 Dezembro | 49 | 15 | 64 |
| 2016 Novembro | 38 | 12 | 50 |
| 2016 Outubro | 57 | 54 | 111 |
| 2016 Setembro | 27 | 43 | 70 |
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