Image in Cardiology
Left pericardial defect: A rare cause of chest pain
Defeito do pericárdio esquerdo: uma causa rara de dor torácica
Ana Rita Pereiraa,
, Ana Rita Almeidaa, Inês Cruza, Luís Rocha Lopesb,c,d, Isabel Joãoa, Hélder Pereiraa
Autor para correspondência
a Cardiology Department, Hospital Garcia de Orta, Almada, Portugal
b Barts Heart Centre, Barts Health NHS Trust, London, United Kingdom
c Institute of Cardiovascular Science, University College London, London, United Kingdom
d Centro Cardiovascular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
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(A-D) Steady-state free precession images. (A) Axial image showing left and posterior heart displacement. (B) Axial image displaying non-visualization of the pericardium and pericardial fat around the left ventricle (between the white arrows). (C) Coronal image showing absence of the pericardium and pericardial fat around the left ventricle and great vessels (between the white arrows), with lung tissue interposition in the several recesses where the pericardium is usually found. (D) Axial image showing lung tissue in the aorto-pulmonary window (white arrow), which is considered a pathognomonic sign of this condition. (E) T1-weighted axial image with no abnormalities, particularly, fat tissue deposition. (F) Delayed gadolinium-enhanced axial image with no macroscopic areas of myocardial fibrosis/necrosis.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital pericardial defects are extremely rare with a reported incidence of <1 in 10<span class="elsevierStyleHsp" style=""></span>000 and a male:female ratio of 3:1. Most patients are asymptomatic and diagnosis is usually incidental. Symptomatic patients present a range of non-specific symptoms, meaning that imaging is crucial for diagnosis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 46-year-old woman was referred to our department with symptoms of palpitations and atypical chest pain. Clinical examination found a palpable apical impulse on the midaxillary line. Electrocardiogram revealed sinus rhythm with a heart rate of 90 bpm, left anterior fascicular block and incomplete right bundle branch block. Chest X-ray (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>) revealed levoposition and a “teardrop” appearance of the heart, superimposition of the right cardiac border over the spine, a prominent main pulmonary artery and a translucent area between the heart and the diaphragm.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Unusual transthoracic echocardiographic windows were required, which showed paradoxical ventricular septal motion, cardiac hypermobility and apparent right ventricular dilation (<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Systemic-to-pulmonary shunt, arrhythmogenic cardiomyopathy and congenital pericardial defect were considered as possible diagnoses.</p><p id="par0025" class="elsevierStylePara elsevierViewall">As the echocardiographic images were suboptimal, cardiac magnetic resonance imaging (MRI) was performed (<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>), confirming leftward displacement of the heart (<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>A) and hypermobility (Video 1). Absence of left ventricular pericardium and pericardial fat (<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>B and C) and interposition of lung tissue between the great vessels (<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>D) were also observed. This last finding is considered a specific diagnostic sign (for some authors even a pathognomonic sign) of congenital pericardial defect.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Therefore, our patient was diagnosed with complete defect of the left pericardium, which is the most common type described in the literature. As it is a form with good prognosis, no treatment was given and the patient was kept under regular follow-up.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0035" 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" => "2017-04-28" "fechaAceptado" => "2017-07-24" "apendice" => array:1 [ 0 => array:1 [ "seccion" => array:1 [ 0 => array:4 [ "apendice" => "<p id="par0045" class="elsevierStylePara elsevierViewall">The following are the supplementary material to this article:<elsevierMultimedia ident="upi0005"></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" => 1181 "Ancho" => 1200 "Tamanyo" => 296755 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Chest X-ray showing marked levoposition and a “teardrop” appearance (dashed white line) of the cardiac silhouette, loss of right heart border due to superimposition over the spine (A), prominent main pulmonary artery (B) and translucent area between the diaphragm and the heart (C).</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" => 601 "Ancho" => 2333 "Tamanyo" => 157000 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Transthoracic echocardiogram images. (A) Parasternal long-axis view, bi-dimensional mode. (B) Parasternal long-axis view, M-mode, showing paradoxical septal motion. (C) Parasternal short-axis view of the base, showing apparent right ventricular dilation.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 2503 "Ancho" => 1867 "Tamanyo" => 368140 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Cardiac MRI images. (A-D) Steady-state free precession images. (A) Axial image showing left and posterior heart displacement. (B) Axial image displaying non-visualization of the pericardium and pericardial fat around the left ventricle (between the white arrows). (C) Coronal image showing absence of the pericardium and pericardial fat around the left ventricle and great vessels (between the white arrows), with lung tissue interposition in the several recesses where the pericardium is usually found. (D) Axial image showing lung tissue in the aorto-pulmonary window (white arrow), which is considered a pathognomonic sign of this condition. (E) T1-weighted axial image with no abnormalities, particularly, fat tissue deposition. (F) Delayed gadolinium-enhanced axial image with no macroscopic areas of myocardial fibrosis/necrosis.</p>" ] ] 3 => array:5 [ "identificador" => "upi0005" "tipo" => "MULTIMEDIAECOMPONENTE" "mostrarFloat" => false "mostrarDisplay" => true "Ecomponente" => array:3 [ "fichero" => "mmc1.mp4" "ficheroTamanyo" => 3774990 "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 ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/08702551/0000003700000009/v2_201810120613/S0870255117302913/v2_201810120613/en/main.assets" "Apartado" => array:4 [ "identificador" => "75834" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Imagem em Cardiologia" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/08702551/0000003700000009/v2_201810120613/S0870255117302913/v2_201810120613/en/main.pdf?idApp=UINPBA00004E&text.app=https://revportcardiol.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255117302913?idApp=UINPBA00004E" ]
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