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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"tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "551" "paginaFinal" => "552" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Argyris Krommydas, Ronak Rajani, Nicholas Hart, Stamatis Kapetanakis" "autores" => array:4 [ 0 => array:3 [ "nombre" => "Argyris" "apellidos" => "Krommydas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:3 [ "nombre" => "Ronak" "apellidos" => "Rajani" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "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" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 839 "Ancho" => 1000 "Tamanyo" => 135412 ] ] "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>" ] ] ] "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" => 2343 "Ancho" => 1001 "Tamanyo" => 286576 ] ] "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" => 839 "Ancho" => 1000 "Tamanyo" => 135412 ] ] "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:7 [ "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" ] ] "itemHostRev" => array:3 [ "pii" => "S0735109703014712" "estado" => "S300" "issn" => "07351097" ] ] ] ] ] ] ] 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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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 8 | 3 | 11 |
| 2024 October | 30 | 29 | 59 |
| 2024 September | 37 | 24 | 61 |
| 2024 August | 37 | 31 | 68 |
| 2024 July | 48 | 37 | 85 |
| 2024 June | 25 | 20 | 45 |
| 2024 May | 39 | 26 | 65 |
| 2024 April | 31 | 21 | 52 |
| 2024 March | 29 | 23 | 52 |
| 2024 February | 29 | 32 | 61 |
| 2024 January | 23 | 34 | 57 |
| 2023 December | 12 | 36 | 48 |
| 2023 November | 36 | 18 | 54 |
| 2023 October | 18 | 15 | 33 |
| 2023 September | 21 | 19 | 40 |
| 2023 August | 24 | 16 | 40 |
| 2023 July | 26 | 7 | 33 |
| 2023 June | 37 | 14 | 51 |
| 2023 May | 46 | 17 | 63 |
| 2023 April | 46 | 11 | 57 |
| 2023 March | 48 | 30 | 78 |
| 2023 February | 23 | 15 | 38 |
| 2023 January | 26 | 22 | 48 |
| 2022 December | 37 | 27 | 64 |
| 2022 November | 41 | 24 | 65 |
| 2022 October | 38 | 29 | 67 |
| 2022 September | 23 | 53 | 76 |
| 2022 August | 34 | 63 | 97 |
| 2022 July | 46 | 34 | 80 |
| 2022 June | 14 | 15 | 29 |
| 2022 May | 21 | 26 | 47 |
| 2022 April | 27 | 27 | 54 |
| 2022 March | 17 | 24 | 41 |
| 2022 February | 14 | 20 | 34 |
| 2022 January | 20 | 18 | 38 |
| 2021 December | 11 | 27 | 38 |
| 2021 November | 27 | 39 | 66 |
| 2021 October | 35 | 37 | 72 |
| 2021 September | 29 | 22 | 51 |
| 2021 August | 26 | 26 | 52 |
| 2021 July | 27 | 30 | 57 |
| 2021 June | 23 | 14 | 37 |
| 2021 May | 28 | 31 | 59 |
| 2021 April | 49 | 41 | 90 |
| 2021 March | 42 | 20 | 62 |
| 2021 February | 35 | 19 | 54 |
| 2021 January | 26 | 15 | 41 |
| 2020 December | 27 | 9 | 36 |
| 2020 November | 35 | 12 | 47 |
| 2020 October | 34 | 18 | 52 |
| 2020 September | 41 | 16 | 57 |
| 2020 August | 23 | 4 | 27 |
| 2020 July | 46 | 10 | 56 |
| 2020 June | 35 | 3 | 38 |
| 2020 May | 42 | 7 | 49 |
| 2020 April | 32 | 13 | 45 |
| 2020 March | 33 | 5 | 38 |
| 2020 February | 90 | 16 | 106 |
| 2020 January | 21 | 4 | 25 |
| 2019 December | 39 | 4 | 43 |
| 2019 November | 28 | 9 | 37 |
| 2019 October | 20 | 9 | 29 |
| 2019 September | 25 | 6 | 31 |
| 2019 August | 32 | 4 | 36 |
| 2019 July | 36 | 7 | 43 |
| 2019 June | 32 | 7 | 39 |
| 2019 May | 50 | 4 | 54 |
| 2019 April | 27 | 15 | 42 |
| 2019 March | 83 | 10 | 93 |
| 2019 February | 59 | 11 | 70 |
| 2019 January | 32 | 4 | 36 |
| 2018 December | 25 | 8 | 33 |
| 2018 November | 61 | 11 | 72 |
| 2018 October | 110 | 15 | 125 |
| 2018 September | 33 | 10 | 43 |
| 2018 August | 15 | 7 | 22 |
| 2018 July | 16 | 1 | 17 |
| 2018 June | 12 | 4 | 16 |
| 2018 May | 33 | 5 | 38 |
| 2018 April | 17 | 5 | 22 |
| 2018 March | 17 | 5 | 22 |
| 2018 February | 15 | 5 | 20 |
| 2018 January | 18 | 7 | 25 |
| 2017 December | 28 | 8 | 36 |
| 2017 November | 21 | 6 | 27 |
| 2017 October | 15 | 11 | 26 |
| 2017 September | 22 | 2 | 24 |
| 2017 August | 24 | 7 | 31 |
| 2017 July | 19 | 4 | 23 |
| 2017 June | 33 | 7 | 40 |
| 2017 May | 24 | 14 | 38 |
| 2017 April | 32 | 2 | 34 |
| 2017 March | 22 | 28 | 50 |
| 2017 February | 27 | 14 | 41 |
| 2017 January | 30 | 4 | 34 |
| 2016 December | 42 | 13 | 55 |
| 2016 November | 38 | 20 | 58 |
| 2016 October | 40 | 19 | 59 |
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