Case report
Cardiac arrest related to anaesthesia in Williams-Beuren syndrome
Parada cardíaca relacionada con la anestesia en el síndrome Williams-Beuren
J. Lucena Delgadoa,
, P. Sanabria Carreterob, P. Durán la Fuenteb, A. Gónzalez Rocafortc, L. Castro Pargab, F. Reinoso Barberob
Corresponding author
a Departamento de Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitario La Paz, Madrid, Spain
b Departamento de Anestesiología Pediátrica, Reanimación y Terapéutica del Dolor, Hospital Universitario La Paz, Madrid, Spain
c Departamento de Cirugía Cardíaca Pediátrica, Hospital Universitario La Paz, Madrid, Spain
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"apellidos" => "Reinoso Barbero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Departamento de Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitario La Paz, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Anestesiología Pediátrica, Reanimación y Terapéutica del Dolor, Hospital Universitario La Paz, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Departamento de Cirugía Cardíaca Pediátrica, Hospital Universitario La Paz, Madrid, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Parada cardíaca relacionada con la anestesia en el síndrome Williams-Beuren" ] ] "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" => 766 "Ancho" => 2500 "Tamanyo" => 714936 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">(A) Preoperative electrocardiogram, showing repolarisation changes with inverted T wave in lead III and signs of ventricular hypertrophy. The QT interval is within the normal range. (B) Electrocardiogram 4 months after surgery. No significant changes with respect to the previous study.</p> <p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Source: With permission from the University Hospital La Paz of Madrid.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Cases of anaesthesia-induced cardiac arrest and death have been described in patients with Williams-Beuren syndrome (WS). Most of these are associated with coronary ischaemia. However, cases of acute coronary changes due to drug allergies have also been reported, showing that the triggering factor is not entirely clear<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> and there is growing interest in determining the origin of these high morbidity and mortality events. WS is the consequence of a genetic alteration that mainly involves the elastin gene.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> It occurs in about 1/10,000 live births. The result is an array of multisystemic alterations (arterial stenosis, neuropsychological, urinary tract and gastrointestinal abnormalities, hypercalcaemia and diabetes mellitus, among others) with a characteristic phenotype (peculiar facies and mental retardation). During anaesthesia, these alterations can have significant cardiovascular repercussions. We present the case of a boy who presented cardiac arrest associated with anaesthesia induction.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 3-year-old boy weighing 11.6<span class="elsevierStyleHsp" style=""></span>kg, with no known allergies, diagnosed with WS, scheduled for aortic arch enlargement due to severe supravalvular aortic stenosis (SVAS) and pulmonary artery stenosis. He had undergone enlargement angioplasty with a patch 2 years earlier. In the immediate postoperative period of that procedure he presented cardiac arrest, which was resolved with resuscitation for 10<span class="elsevierStyleHsp" style=""></span>min. He was currently under treatment with propranolol due to high blood pressure, and had non-specific chest pain with dyspnoea on moderate exertion, mild general hypotonia and psychomotor retardation of speech and gait. The electrocardiogram showed signs of left ventricular hypertrophy (VH) and repolarisation changes on lead III (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The cardiac ultrasound study showed mild pulmonary, aortic and mitral insufficiency, ejection fraction 48%, SVAS gradient of 72<span class="elsevierStyleHsp" style=""></span>mmHg and a right pulmonary artery <span class="elsevierStyleItalic">z</span>-score of −1.9. The CT scan showed diffuse SVAS with a critical area of 6.18<span class="elsevierStyleHsp" style=""></span>mm (<a class="elsevierStyleCrossRef" href="#fig2">Fig. 2</a>), mild right carotid stenosis and hyperinflation of the right upper lobe of the lung.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig2"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">In the operating room, after fasting for 10<span class="elsevierStyleHsp" style=""></span>h, he was premedicated with intranasal midazolam (3<span class="elsevierStyleHsp" style=""></span>mg), and monitored with pulse oximetry, blood pressure every 3<span class="elsevierStyleHsp" style=""></span>min and continuous electrocardiogram with precordial leads. Anaesthesia was induced gradually with up to 4% sevoflurane, the saphenous vein was cannulated and intravenous fentanyl (1.5<span class="elsevierStyleHsp" style=""></span>μg/kg<span class="elsevierStyleSup">−1</span>) and cisatracurium (0.2<span class="elsevierStyleHsp" style=""></span>mg/kg<span class="elsevierStyleSup">−1</span>) were administered. Three minutes later, with sevoflurane (2%), the patients presented hypotension (60/40<span class="elsevierStyleHsp" style=""></span>mmHg), ST segment depression, arterial oxygen desaturation (SpO<span class="elsevierStyleInf">2</span>: 82%) and progressive bradycardia (70<span class="elsevierStyleHsp" style=""></span>bpm), refractory to atropine, albumin and adrenaline (5<span class="elsevierStyleHsp" style=""></span>μg/kg<span class="elsevierStyleSup">−1</span>), which progressed to electromechanical dissociation. The patient required resuscitation with chest compressions, adrenaline (10<span class="elsevierStyleHsp" style=""></span>μg/kg<span class="elsevierStyleSup">−1</span>) and bicarbonate (according to blood gas after arterial line placement). At 5<span class="elsevierStyleHsp" style=""></span>min, ventricular fibrillation was converted to sinus rhythm with electrical shock (30<span class="elsevierStyleHsp" style=""></span>J) and amiodarone (5<span class="elsevierStyleHsp" style=""></span>mg/kg<span class="elsevierStyleSup">−1</span>). Transient haemodynamic stability was achieved, during which time transoesophageal echocardiography showed severe generalised cardiac dysfunction. Five minutes later, with adrenalin perfusion (0.2<span class="elsevierStyleHsp" style=""></span>μg/kg<span class="elsevierStyleSup">−1</span>/min<span class="elsevierStyleSup">−1</span>), he presented a new episode of hypotension, bradycardia and electromechanical dissociation refractory to chest compressions and adrenalin, and emergency extracorporeal membrane oxygenation (ECMO) with cannulation of the right internal jugular vein and carotid artery was started. Resuscitation up to the start of ECMO lasted around 30–40<span class="elsevierStyleHsp" style=""></span>minutes. Under EMCO, both haemodynamics and blood gas levels stabilised. We decided to continue with the scheduled surgery due to the high risk of the disease, and performed sternotomy, cannulation of the right atrium, selective cerebral perfusion (right brachiocephalic trunk), cardiopulmonary bypass and placement of an enlargement patch on the ascending aorta and arch. During the bypass, bilateral cerebral near infrared spectroscopy was maintained between 55 and 65, with no significant asymmetries. At the end of surgery, with the patient normothermic and with adrenaline (0.1<span class="elsevierStyleHsp" style=""></span>μg/kg<span class="elsevierStyleSup">−1</span>/min<span class="elsevierStyleSup">−1</span>) and milrinone (0.8<span class="elsevierStyleHsp" style=""></span>μg/kg<span class="elsevierStyleSup">−1</span>/min<span class="elsevierStyleSup">−1</span>) perfusion, we decided to delay sternal closure due to 2 episodes of bradycardia and electromechanical dissociation when attempting closure, and to leave the jugular-carotid lines in place for 36<span class="elsevierStyleHsp" style=""></span>h. Hypothermia at 34<span class="elsevierStyleHsp" style=""></span>°C was induced to protect the brain (72<span class="elsevierStyleHsp" style=""></span>h). After withdrawal of sedation and mechanical ventilation (fifth postoperative day), we observed left hemiparesis and moderate-severe global hypotonia. The magnetic resonance scan (fifteenth postoperative day) identified lesions compatible with hypoxic-ischaemic encephalopathy in the basal ganglia and mesencephalon. After 4 months, however, neurological functions had returned to baseline levels and the patient received motor rehabilitation for deambulation.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0020" class="elsevierStylePara elsevierViewall">WS is a multisystemic syndrome that significantly affects the cardiovascular system.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> It is caused by the <span class="elsevierStyleItalic">de novo</span> deletion of 26–28 genes from the 7 q11.23 chromosome, which includes the elastin gene that encodes the elastin protein.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,3</span></a> This causes rigid thickening of the tunica media, mainly due to smooth muscle hypertrophy, and supravalvular (aortic or pulmonary) occlusion which, in turn, produces VH, reduces vascular distensibility and the <span class="elsevierStyleItalic">windkessel</span> effect.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> These abnormalities also affect the coronary arteries and cause stenosis, dilation and ostial obstruction of the aortic valve, which can compromise coronary blood flow. WS can also be associated with pulmonary artery stenosis, usually peripheral.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> In contrast to pulmonary stenosis, SVAS can progress over time. It is considered a risk factor for sudden death during anaesthesia<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,4</span></a> and surgical correction has been strongly associated with increased mortality.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> Coronary stenosis also increases mortality,<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> but is hard to diagnose. Imaging studies (angiography, computed tomography, magnetic resonance imaging), for which children need to be sedated, are also risky in this population.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The risk of sudden death in WS is between 25 and 100 times greater than in the general population,<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> particularly during anaesthesia<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,4</span></a> and major and minor surgery.<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">6,7</span></a> Although morbidity and mortality have been associated with SVAS and coronary stenosis,<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> it has also been associated with repolarization changes and long QT intervals,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> which are common in patients with WS. There are reports of cases of SW associated with Kounis syndrome<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> that present with allergy-induced coronary vasospasm, pulmonary eosinophilia, skin reactions and elevated serum tryptase in reaction to certain drugs (cisatracurium, etomidate, midazolam, latex, iodinated contrast media). The existence of cases with an as yet unclarified cardiac event has been dubbed “the SW mystery”.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Some authors have developed anaesthesia risk stratification tables for SW.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">8,9</span></a> High-risk criteria include: children under 3 years of age, history of cardiovascular events or arrhythmias, moderate-severe bilateral ventricular outflow tract obstruction, supravalvar gradient >40<span class="elsevierStyleHsp" style=""></span>mmHg, left/right VH with signs of ischaemia, coronary involvement, diffuse stenosis of the thoracic aorta, electrocardiographic repolarization changes and long QT.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> Our patient met most of the foregoing criteria.</p><p id="par0035" class="elsevierStylePara elsevierViewall">In view of the potential risks, even during minor procedures, anaesthesiologists must understand the disease, weigh up the risks and benefits of the procedure, maintain haemodynamic stability and an adequate oxygen uptake-to-delivery ratio, optimise preload with preoperative liquids (minimum fasting period), and reduce preoperative anxiety with psychological preparation and carefully chosen premedication.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">8,9</span></a> Some authors recommend skin prick, radioallergosorbent, and antibody tests for hypersensitivity to products and drugs that might be used during the procedure.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> It is essential to preserve inotropism and sinus rhythm, avoid tachycardia, abrupt changes in systemic vascular resistance, and increase pulmonary vascular resistance.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,8</span></a> It is also essential to monitor the ST interval, use capnography, echocardiography (transoesophageal in the case of major surgery<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">8,9</span></a>) and prepare for adverse events (medication, resuscitation, defibrillator, ECMO). During ventilation, hypercapnia and hypoxaemia with low airway pressure must be avoided due to the risk of pulmonary hypertension associated with pulmonary artery stenosis that could cause right ventricular failure.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> There is no ideal anaesthetic agent; anaesthetic drugs should be dosed, combined and titrated according to the procedure. Short-acting, dose-dependent and reversible agents should preferably be used.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> Moderate doses (up to 50%) of nitrous oxide, dexmedetomidine and etomidate, depending on the procedure, have given good results.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">8,9</span></a> Intramuscular ketamine has been used for anaesthetic induction,<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> although oxygen demand may increase due to positive chronotropism and increased afterload. Guidelines recommend that procedures be carried out in tertiary hospitals equipped to perform ECMO and provide optimal postoperative care and experienced, fully prepared multidisciplinary teams.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,6,8,9</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In the case of our patient, preoperative fasting was longer than usual due to his refusal to take liquids. We used slow, gradual inhalation induction with up to 4% sevoflurane in order to minimise the loss of systemic vascular resistance. Once intravenous access has been placed, the synergic effect of fentanyl and cisatracurium in combination with sevoflurane could have facilitated the loss of systemic vascular resistance. Although it was not performed in this case, preoperative hydration with water up to 2<span class="elsevierStyleHsp" style=""></span>h before the procedure or the administration of intravenous fluids if possible can prevent low preload. Although there was no conclusive evidence of coronary stenosis on imaging studies (cardiac CT scan), the patient showed signs of stress and presented VH together with repolarisation changes on the electrocardiogram. It is essential to prepare for coronary artery compromise. Ventricular outflow tract obstruction and ST segment depression are predictors of perioperative myocardial ischaemia and sudden death, according to Horowitz et al. An immediate request for help, prior experience, preparation for adverse events and early start of ECMO will stabilise the patient. In the postoperative period, it is advisable to delay sternal closure and leave the cannulas in place to facilitate the immediate correction of any adverse events. Similarly, early induction of hypothermia to protect the brain minimised the risk of neurological damage secondary to post-cardiac arrest reperfusion and improve the final outcome.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> As there were no signs of respiratory or cutaneous allergic reaction, we omitted allergy testing and did not determine serum tryptase levels to rule out Kounis syndrome. However, this may be advisable in the case of adverse events in WS.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">To conclude, patients with SW are at high risk of cardiac arrest and death during anaesthetic procedures, particularly during induction. Although coronary ischaemia is the leading cause, arrhythmias, hypersensitivity and possibly other unknown mechanisms may also be involved. It is essential to report adverse events to identify with greater accuracy the risk factors and physiopathological mechanisms involved.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1018456" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec976826" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1018455" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec976825" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflicts of interest" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-08-14" "fechaAceptado" => "2017-11-13" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec976826" "palabras" => array:6 [ 0 => "Williams syndrome" 1 => "Cardiac arrest" 2 => "Anaesthesia" 3 => "Cardiopathy" 4 => "Death sudden" 5 => "Myocardial ischaemia" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec976825" "palabras" => array:6 [ 0 => "Síndrome Williams" 1 => "Parada cardiaca" 2 => "Anestesia" 3 => "Cardiopatía" 4 => "Muerte súbita" 5 => "Isquemia miocárdica" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Williams-Beuren syndrome is the clinical manifestation of a congenital genetic disorder in the elastin gene, among others. There is a history of cardiac arrest refractory to resuscitation manoeuvres in anaesthesia. The incidence of myocardial ischaemia is high during anaesthetic induction, but there are patients who do not have this condition yet also have had very serious cardiac events, and issues that are still to be resolved. Case descriptions will enable the common pathophysiological factors to be defined, and decrease morbidity and mortality. We report the case of a 3-year-old boy with cardiac arrest at induction, rescued with circulatory assistance with extracorporeal membrane oxygenation and hypothermia induced for cerebral protection.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Williams-Beuren es la manifestación clínica de una alteración genética congénita en el gen de la elastina, entre otros. Existen antecedentes de parada cardíaca refractaria a maniobras de resucitación en contexto anestésico. Es alta la incidencia de isquemia miocárdica durante la inducción anestésica, pero existen pacientes que, sin esta causa, también presentan eventos cardíacos muy graves. Quedan cuestiones aún por resolver. La descripción de casos permitirá definir factores fisiopatológicos comunes y disminuir la morbimortalidad. Presentamos el caso de un niño de 3 años con parada cardíaca en la inducción anestésica, rescatado con asistencia circulatoria con membrana de oxigenación extracorpórea e hipotermia inducida como protección cerebral.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Lucena Delgado J, Sanabria Carretero P, Durán la Fuente P, Gónzalez Rocafort A, Castro Parga L, Reinoso Barbero F. Parada cardíaca relacionada con la anestesia en el síndrome Williams-Beuren. Rev Esp Anestesiol Reanim. 2018;65:234–237.</p>" ] ] "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" => 766 "Ancho" => 2500 "Tamanyo" => 714936 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">(A) Preoperative electrocardiogram, showing repolarisation changes with inverted T wave in lead III and signs of ventricular hypertrophy. The QT interval is within the normal range. (B) Electrocardiogram 4 months after surgery. No significant changes with respect to the previous study.</p> <p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Source: With permission from the University Hospital La Paz of Madrid.</p>" ] ] 1 => array:7 [ "identificador" => "fig2" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 625 "Ancho" => 900 "Tamanyo" => 77409 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Coronal plane cardiac CT scan showing a 6-mm supravalvular aortic stenosis (white arrow).</p> <p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Source: With permission from the University Hospital La Paz of Madrid.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The mystery of Williams-Beuren syndrome associated with pulmonary dysfunction, sudden death, and Kounis syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "N.G. 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