Congenital Cardiac Anesthesia Society
A Section of the the Society for Pediatric Anesthesia

{“questions”:{“r4mpy”:{“id”:”r4mpy”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”This Question of the Week was written by Drs. Sanabria and Campo from Madrid, Spain. English translation by Destiny F Chau, MD. \r\nLa incidencia de complicaciones neurol\u00f3gicas en ni\u00f1os sometidos a reparaci\u00f3n quir\u00fargica de su cardiopat\u00eda es elevada. Los mecanismos de lesi\u00f3n son multifactoriales asociados a diversos factores de riesgo y otras condiciones predisponentes. La monitorizaci\u00f3n cerebral ha demostrado ser una herramienta eficaz para identificar y tratar precozmente los mecanismos fisiopatol\u00f3gicos involucrados en la lesi\u00f3n neurol\u00f3gica. Se\u00f1ale cu\u00e1l de las siguientes estrategias de monitorizaci\u00f3n cerebral intraoperatoria ha demostrado mayor eficacia para identificar los mecanismos de lesi\u00f3n involucrados: \r\n\r\nChildren undergoing surgical repair of congenital heart disease have a high incidence of neurological complications. The mechanisms of injury are multifactorial and are associated with various risk factors and predisposing conditions. Brain monitoring has proven to be an effective tool for the early identification and treatment of pathophysiological mechanisms involved in the neurological injury. What is the MOST EFFECTIVE intraoperative brain monitoring strategy used to identify the mechanism of neurological injury? \r\n”,”desc”:””,”hint”:””,”answers”:{“3akba”:{“id”:”3akba”,”image”:””,”imageId”:””,”title”:”A.\tLa Espectroscopia Cercana al Infrarrojo (NIRS) bifrontal \/ Bifrontal Near Infrared Spectroscopy (NIRS)”},”wn4iy”:{“id”:”wn4iy”,”image”:””,”imageId”:””,”title”:”B.\tLa Electroencefalograf\u00eda (EEG) \/ Electroencephalography (EEG)”},”5xere”:{“id”:”5xere”,”image”:””,”imageId”:””,”title”:”C.\tNeuromonitorizaci\u00f3n multimodal con EEG, Doppler Transcraneal y NIRS \/ Multimodal neuromonitoring with EEG, Transcranial Doppler and NIRS”,”isCorrect”:”1″},”84qm8″:{“id”:”84qm8″,”image”:””,”imageId”:””,”title”:”D.\tLa Ecograf\u00eda Transfontanelar con ultrasonidos \/ Transfontanelle Ultrasonography”}}}},”results”:{“7kygj”:{“id”:”7kygj”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Explicaci\u00f3n: \r\n\r\n\r\nLa incidencia de da\u00f1o neurol\u00f3gico en pacientes pedi\u00e1tricos sometidos a cirug\u00eda card\u00edaca con bypass cardiopulmonar (BCP) se ha descrito en un 25% de los pacientes intervenidos, principalmente cuando la correcci\u00f3n quir\u00fargica se realiza en el per\u00edodo neonatal, provocando aumento de morbimortalidad perioperatoria y diversos grados de d\u00e9ficit neurol\u00f3gico, aunque en ocasiones la repercusi\u00f3n neurocognitiva no se manifiestan hasta a\u00f1os despu\u00e9s, generando un gran impacto social (1-4). \r\nLa incidencia y gravedad de la lesi\u00f3n neurol\u00f3gica se relaciona directamente con la severidad de la cardiopat\u00eda, cuando la correcci\u00f3n se realiza en periodo neonatal y cuando est\u00e1 asociada a s\u00edndromes y\/o malformaciones que afectan al neurodesarrollo (5). El mecanismo de lesi\u00f3n est\u00e1 relacionado con episodios hip\u00f3xico-isqu\u00e9micos que ocurren durante el per\u00edodo perioperatorio, y son de origen multifactorial debidos a hipoxemia, hipocapnia, hipoperfusi\u00f3n, bajo gasto card\u00edaco, disfunci\u00f3n en canulaci\u00f3n arterial y venosa durante el BCP, hemodiluci\u00f3n excesiva, hemorragia intracraneal, microembolias, respuesta inflamatoria, producci\u00f3n de radicales libres (6,7), parada circulatoria con hipotermia profunda y exposici\u00f3n a agentes anest\u00e9sicos durante horas o incluso d\u00edas (8). Sin embargo datos recientes extra\u00eddos de imagenolog\u00eda cerebral neonatal, se ha hecho evidente que existen anomal\u00edas cerebrales, incluso antes de la reparaci\u00f3n quir\u00fargica, secundario a alteraciones del neurodesarrollo en periodo fetal relacionadas con la cardiopat\u00eda, afectando predominantemente a la sustancia blanca, aunque tambi\u00e9n se observan en la sustancia gris cortical y subcortical. Esta inmadurez cerebral puede aumentar la susceptibilidad al riesgo neurol\u00f3gico sobrea\u00f1adido durante la etapa perioperatoria por mecanismos multifactoriales y sin\u00e9rgicos (9), siendo recomendable la neuromonitorizaci\u00f3n, exploraci\u00f3n cl\u00ednica neurol\u00f3gica y pruebas de imagen como la resonancia magn\u00e9tica (RM) (4,10). \r\n\r\nLa monitorizaci\u00f3n neurol\u00f3gica de rutina durante la cirug\u00eda card\u00edaca puede proporcionar informaci\u00f3n que podr\u00eda conducir a mejoras y disminuir el potencial de da\u00f1o neurol\u00f3gico. La oximetr\u00eda transcraneal mediante espectroscopia pr\u00f3xima al infrarrojo (NIRS) permite la medici\u00f3n continua, no invasiva y no puls\u00e1til de la saturaci\u00f3n de ox\u00edgeno cerebral regional (rSO2), lo que permite detectar la hipoxia-isquemia cerebral. Dicha monitorizaci\u00f3n est\u00e1 influenciada por el equilibrio entre el suministro y el consumo de ox\u00edgeno cerebral. Los valores registrados se ven afectados por la oxigenaci\u00f3n, el gasto card\u00edaco y la perfusi\u00f3n, la hipo\/hipercapnia, el hematocrito, la temperatura y la profundidad de la anestesia. Valores bajos de NIRS pueden estar asociados con da\u00f1o neurol\u00f3gico, y ha demostrado utilidad para mantener oxigenaci\u00f3n y perfusi\u00f3n cerebral adecuados en pacientes anestesiados, evitando episodios de desaturaci\u00f3n cerebral cr\u00edtica (2,11). Otras estrategias de neuromonitorizaci\u00f3n como electroencefalograma (EEG), el Doppler transcraneal (DTC) y ultrasonidos transfontanelar han demostrado su utilidad para obtener informaci\u00f3n sobre mecanismos de lesi\u00f3n neurol\u00f3gica que se producen en el pre, intra y postoperatorio. El EEG estandar, EEG procesados como EEG integrado por amplitud (EEGa) o Bispectral Index (BIS) y potenciales evocados somatosensoriales (SEPs) han demostrado utilidad para la determinaci\u00f3n de la profundidad anest\u00e9sica, prevenir despertar intraoperatorio, evitar sobredosis de anest\u00e9sicos y detecci\u00f3n de episodios de isquemia, hipoglucemia y presencia de convulsiones subcl\u00ednicas, brindando oportunidades para su detecci\u00f3n y manejo precoz (12,13). La ecograf\u00eda Doppler transcraneal (DTC) sobre la arteria cerebral media tambi\u00e9n es una herramienta valiosa para identificar flujo cerebral inadecuado durante el BCP. Los ultrasonidos utilizados por ventana transfontanelar son adecuados para uso a pie de cama y detectar complicaciones como la hemorragia intraventricular, sin embargo, no proporciona la resoluci\u00f3n necesaria para detectar la mayor\u00eda de alteraciones estructurales sutiles, s\u00f3lo detectables mediante RM, pero esta \u00faltima presenta limitaciones para desplazar al paciente cr\u00edtico o utilizarla en el intraoperatorio (14). \r\n\r\nLa etiolog\u00eda multifactorial de la lesi\u00f3n neurol\u00f3gica hace que sea menos probable que una \u00fanica modalidad de monitorizaci\u00f3n intraoperatoria sea eficaz para captar todas las posibles amenazas. Sin embargo, la neuromonitorizaci\u00f3n multimodal combina varios aspectos de vigilancia sobre la oxigenaci\u00f3n, hemodin\u00e1mica y electrofisiolog\u00eda cerebrales asociando NIRS, DTC y EEG, permite una aplicaci\u00f3n cl\u00ednica de rutina, reduce las limitaciones de la monitorizaci\u00f3n \u00fanica, ligadas a la variabilidad individual (NIRS, DTC) o a la interacci\u00f3n con las drogas anest\u00e9sicas (EEG) y se obtiene mayor sensibilidad y especificidad en la detecci\u00f3n de los mecanismos de lesi\u00f3n neurol\u00f3gica (15), reduciendo incidencia de complicaciones neurol\u00f3gicas y proporcionando m\u00e1s seguridad al paciente durante la cirug\u00eda (16,17,18,19). \r\n\r\nExplanation: \r\n\r\n\r\nThe incidence of neurological damage in pediatric patients undergoing cardiac surgery with cardiopulmonary bypass (CPB) has been described in about 25% of the operated patients, mainly when surgical correction is performed during the neonatal period, leading to increased perioperative morbidity and mortality and varying degrees of neurological deficit. In some patients, the neurocognitive repercussions may not appear until years later generating associated great social consequences (1-4). \r\n\r\n\r\nThe incidence and severity of the neurological injury is directly correlated to the severity of the heart disease, the timing of correction in the neonatal period, its association with syndromes and \/ or malformations that affect neurodevelopment (5). The mechanism of injury is related to hypoxic-ischemic episodes that occur during the perioperative period which are of multifactorial origin due to hypoxemia, hypocapnia, hypoperfusion, low cardiac output, inadequate arterial and venous cannulation during CPB, excessive hemodilution, intracranial hemorrhage, microembolisms, inflammatory response, production of free radicals (6,7), circulatory arrest with profound hypothermia, and exposure to anesthetic agents for hours or even days (8). However, recent data from neonatal brain imaging have shown evidence that brain abnormalities are present even before surgical repair. The abnormalities are thought to arise from neurodevelopmental alterations in the fetal period related to the heart lesion. They predominantly affect the white matter, although they are also observed in the cortical and subcortical gray matter. \r\n\r\n \r\nThis brain immaturity increases the susceptibility to added neurological risk during the perioperative stage through multifactorial and synergistic mechanisms (9). Thus, it is recommended to perform neuromonitoring, neurological clinical examinations and imaging tests such as magnetic resonance imaging (MRI) (4,10). \r\n\r\n\r\nRoutine neurological monitoring during cardiac surgery can provide information that could lead to improvements and decrease the potential for neurological damage. Transcranial oximetry using near infrared spectroscopy (NIRS) allows continuous, non-invasive and non-pulsatile measurement of regional cerebral oxygen saturation (rSO2), which allows the detection of cerebral hypoxia-ischemia. This monitoring modality is influenced by the balance between brain oxygen supply and consumption. The NIRS values are affected by oxygenation, cardiac output and perfusion, hypo \/ hypercapnia, hematocrit, temperature, and depth of anesthesia. Low NIRS values may be associated with neurological damage, and it has been shown to be useful for maintaining adequate cerebral oxygenation and perfusion in anesthetized patients, avoiding episodes of critical brain desaturation (2,11). Other neuromonitoring strategies such as electroencephalogram (EEG), transcranial Doppler (TCD) and transfontanellar ultrasound have demonstrated their usefulness to obtain information on mechanisms of neurological injury that occur in the pre, intra, and postoperative period. Standard EEG, processed EEG such as amplitude-integrated EEG (EEGa) or Bispectral Index (BIS), and somatosensory evoked potentials (SEPs) have proven useful for determining anesthetic depth, preventing intraoperative awareness, avoiding anesthetic overdose and detection of events of ischemia, hypoglycemia and subclinical seizures, providing opportunities for their early detection and management (12,13). Transcranial Doppler ultrasound (TCD) over the middle cerebral artery is also a valuable tool to identify inadequate cerebral flow during CPB. Transfontanelle ultrasonography is suitable for bedside use to detect complications such as intraventricular hemorrhage; however, it does not provide the necessary resolution to detect most subtle structural alterations which are only detectable by MRI. MRI requires transporting the critically ill patient to the MRI site thus limiting its intraoperative utility (14). \r\n\r\n\r\nThe multifactorial etiology of neurologic injury makes it less likely that a single intraoperative monitoring modality will be effective in capturing all potential threats. However, multimodal neuromonitoring such as NIRS, TCD and EEG combines several monitoring aspects of brain oxygenation, hemodynamics and electrophysiology. Its routine clinical application reduces the limitations associated to a single monitor, due to its inherent variability (NIRS, TCD) or interactions with anesthetic drugs (EEG). It allows greater sensitivity and specificity in the detection of neurological injury mechanisms (15), reducing the incidence of neurological complications and providing increased safety to the patient during surgery (16,17, 18,19). \r\nReferences \r\n\r\n1.\tGalli KK, Zimmerman RA, Jarvik GP, et al. Periventricular leukomalacia is common after neonatal cardiac surgery. J Thorac Cardiovasc Surg. 2004; 127: 692-704. \r\n2.\tVretzakis G, Georgopoulou S, Stamoulis K, et al. Cerebral oximetry in cardiac anesthesia. J Thorac Dis. 2014; 6(S1): S60-S69. doi: 10.3978\/j.issn.2072-1439.2013.10.22 \r\n3.\tMenache CC, du Plessis AJ, Wessel DL, Jonas RA, Newburger JW. Current incidence of acute neurologic complications after open heart operations in children. Ann Thorac Surg. 2002; 73: 1752-1758. \r\n4.\tMiller S, McQuillen P, Hamrick S, et al. Abnormal Brain Development in Newborns with Congenital Heart Disease. N Engl J Med. 2007; 357: 1928-1938. \r\n5.\tWernovsky G. Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease. Cardiol Young. 2006; 16 (suppl1): 92\u2013104. doi: 10.1017\/ S1047951105002398. \r\n6.\tDominguez TE, Wernovsky G, Gaynor W. Cause and prevention of central nervous system injury in neonates undergoing cardiac surgery. Semin Thorac Cardiovasc Surg. 2007; 19: 269-277. \r\n7.\tAlbers EL, Bichell DP, McLaughlin B. New approaches to neuroprotection in infant heart surgery. Pediatr Res. 2010; 68: 1-9. \r\n8.\tChar D, Ramamoorthy C, Wise-Faberowski L. Cognitive dysfunction in children with heart disease: the role of anesthesia and sedation. Congenit Heart Dis. 2016; 11: 221-229. \r\n9.\tMorton P, Ishibashi N, Jonas R. Neurodevelopmental abnormalities and congenital heart disease insights into altered brain maturation. Circ Res. 2017; 120: 960-977. DOI: 10.1161\/CIRCRESAHA.116.309048. \r\n10.\tPeyvandi S, Latal B, Miller S, McQuillen P. The neonatal brain in critical congenital heart disease: Insights and future directions. Neuroimage. 2018. https:\/\/doi.org\/10.1016\/j.neuroimage.2018.05.045 \r\n11.\tWeber F, Scoones G. A practical approach to cerebral near-infrared spectroscopy (NIRS) directed hemodynamic management in non cardiac pediatric anesthesia. Pediatric Anesthesia. 2019; 29: 993-1001. \r\n12.\tSury M. Brain Monitoring in Children. Anesthesiology Clin. 2014; 32: 115\u2013132. \r\n13.\tBarry A, Chaney M, London M. Anesthetic Management During Cardiopulmonary Bypass: A Systematic Review. Anesth Analg. 2015; 120: 749\u2013769. \r\n14.\tMorton P, Ishibashi N, Jonas R. Neurodevelopmental abnormalities and congenital heart disease Insights Into altered brain maturation. Circ Res. 2017; 120: 960-977. DOI: 10.1161\/CIRCRESAHA.116.309048 \r\n15.\tZanatta P, Benvenuti S, Bosco E, Baldanzi F, Palomba D, Valfr\u00e8 C. Multimodal brain monitoring reduces major neurologic complications in cardiac surgery. Journal of Cardiothoracic and Vascular Anesthesia. 2011; 25: 1076-1085. \r\n16.\tAndropoulos DB, Stayer SA, Diaz LK, Ramamoorthy C. Neurological monitoring for congenital heart surgery. Anesth Analg. 2004; 99: 1365\u20111375. \r\n17.\tThudium M, Heinze I, Ellerkmann R, Hilbert T. Cerebral function and perfusion during cardiopulmonary bypass: A plea for a multimodal monitoring approach. The Heart Surgery Forum. 2018; 21(1): 1894. \r\n18.\tMittnacht A, Rodriguez\u2011Diaz C. Multimodal neuromonitoring in pediatric cardiac anesthesia. Annals of Cardiac Anaesthesia. 2014; 17: 25-32. \r\n19.\tFinucane E, Jooste E, Machovec K. Neuromonitoring Modalities in Pediatric Cardiac Anesthesia: A Review of the Literature. Journal of Cardiothoracic and Vascular Anesthesia. 2020; 34: 3420-3428. \r\n”,”redirect_url”:””}}}

{“questions”:{“ow9nc”:{“id”:”ow9nc”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Jessica Yeh, MBBS and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR\r\n\r\nA 6-month-old infant with a normal karyotype is undergoing repair of a complete atrioventricular canal defect. Compared to an infant with Trisomy 21, what is the MOST LIKELY complication after a full operative repair in a patient with a normal karyotype?\r\n”,”desc”:””,”hint”:””,”answers”:{“2pfwt”:{“id”:”2pfwt”,”image”:””,”imageId”:””,”title”:”A. Left atrioventricular valve regurgitation”,”isCorrect”:”1″},”wna9d”:{“id”:”wna9d”,”image”:””,”imageId”:””,”title”:”B. Complete heart block “},”z0uht”:{“id”:”z0uht”,”image”:””,”imageId”:””,”title”:”C. Junctional ectopic tachycardia”},”ad8hb”:{“id”:”ad8hb”,”image”:””,”imageId”:””,”title”:”D. Right ventricular hypertrophy “}}}},”results”:{“11vkd”:{“id”:”11vkd”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Atrioventricular (AV) septal or canal defects are strongly associated with Trisomy 21. There is a 40-50% risk of Trisomy 21 in fetuses with a prenatal diagnosis of AV canal defect and approximately 40% of fetuses with Trisomy 21 also have an associated AV canal defect. \r\n\r\nTimely surgical repair of AV canal defects can reduce the risk of developing pulmonary vascular disease and subsequent pulmonary hypertension and heart failure. After surgical repair, hemodynamically significant residual defects may necessitate surgical re-intervention. Several studies have demonstrated that the presence of Trisomy 21 is not a risk factor for re-operation. Patients with a normal karyotype have an increased rate of re-operation after primary AV canal defect repair. A common indication for re-operation is residual left atrioventricular valve regurgitation, presumably due to a higher prevalence of left atrioventricular valve abnormalities such as valve dysplasia. An additional indication for re-operation is significant subaortic stenosis or left ventricular outflow tract obstruction, which is also more prevalent in patients with a normal karyotype. \r\n\r\nTrisomy 21 is a risk factor for the development of pulmonary hypertension even after repair of an AV canal defect. The etiology is multifactorial including a genetic contribution to endothelial cell dysfunction or abnormal lung development as well as the presence of other comorbidities such as obstructive sleep apnea, gastric aspiration, or tracheobronchomalacia. Right ventricular hypertrophy typically develops over time due to increased pulmonary arterial pressure. \r\n\r\n\r\nArrhythmias in the immediate postoperative period after AV canal defect repair occur in approximately 10-15% of patients with 3-4% of patients requiring subsequent implantation of a permanent pacemaker for complete heart block. However, there is no difference in the proportion of patients with heart block relative to the presence of Trisomy 21. \r\n\r\nNeurodevelopmental outcomes after cardiac surgery vary depending on the extent of the congenital cardiac lesion. A child with a normal karyotype and complete atrioventricular canal defect has an increased risk of neurodevelopmental impairment compared to their healthy peers, but the presence of Trisomy 21 increases this risk even further. \r\n\r\n\r\nReferences: \r\n1.\tSt Louis JD, Jodhka U, Jacobs JP, et al. Contemporary outcomes of complete atrioventricular septal defect repair: Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg<\/em>. 2014; 148: 2526-2531. \r\n2.\tXie O, Brizard CP, d\u2019Udekem Y, et al. Outcomes of repair of complete atrioventricular septal defect in the current era. Eur J Cardiothorasc Surg<\/em>. 2014; 45(4): 610-617. \r\n3.\tFormigari R, Di Donato RM, Gargiulo G, et al. Better surgical prognosis for patients with complete atrioventricular septal defect and Down’s syndrome. Ann Thorac Surg<\/em>. 2004; 78(2): 666-672. \r\n4.\tLange R, Guenther T, Busch R, et al. The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair. J Thorac Cardiovasc Surg<\/em>. 2007; 134(2): 304-310. \r\n5.\tMussatto KA, Hoffmann RG, Hoffman GM, et al. Risk and prevalence of developmental delay in young children with congenital heart disease. Pediatrics<\/em>. 2014; 133(3): e570-e577. \r\n”,”redirect_url”:””}}}

{“questions”:{“kr37g”:{“id”:”kr37g”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Wenyu Bai MD, C.S. Mott Children\u2019s Hospital, Michigan Medicine, Ann Arbor\r\n\r\nTrisomy 18 (T18), also known as Edward\u2019s syndrome, is a chromosomal disorder characterized by numerous congenital anomalies including congenital heart disease. Which of the following is the MOST COMMON surgical procedure performed in children with T18?”,”desc”:””,”hint”:””,”answers”:{“jpt4p”:{“id”:”jpt4p”,”image”:””,”imageId”:””,”title”:”A.\tPatent ductus arteriosus (PDA) closure”},”d5d5c”:{“id”:”d5d5c”,”image”:””,”imageId”:””,”title”:”B.\tVentricular septal defect (VSD) repair”,”isCorrect”:”1″},”xp4d6″:{“id”:”xp4d6″,”image”:””,”imageId”:””,”title”:”C.\tTetralogy of Fallot (TOF) repair”},”ovx35″:{“id”:”ovx35″,”image”:””,”imageId”:””,”title”:”D.\tStage I Norwood procedure”}}}},”results”:{“032g6”:{“id”:”032g6″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Trisomy 18 (T18) is the second most common autosomal trisomy disorder surviving to birth, after Trisomy 21 [1, 2]. There are multiple congenital anomalies associated with T18 such as craniofacial defects, respiratory tract abnormalities, neurocognitive disability, and frequent cardiac lesions. Atrial septal defects (ASDs), ventricular septal defects (VSDs), and patent ductus arteriosus (PDA) make up the majority of congenital heart defects (CHD) in T18 patients. More complex congenital heart disease (CHD), such as Tetralogy of Fallot (TOF), double outlet right ventricle (DORV), complete atrioventricular canal defect, and coarctation of the aorta are not uncommon. Although uncommon, the single ventricle lesions are also diagnosed in this patient population [2-4]. The management of patients with T18 has evolved from the traditional non-interventional, palliative approach to a goal-directed, individualized approach. While this remains somewhat controversial, life-sustaining interventions such as intubation, mechanical ventilation, artificial hydration and nutrition, and cardiac and noncardiac surgeries are increasingly being considered and performed in patients with T18. VSD repair is the most common cardiac procedure performed in this patient population. The spectrum of cardiac procedures offered to T18 patients covers all aspects of congenital heart disease and all levels of complexity, from the Stage I Norwood procedure to an ASD repair [5]. \r\n\r\nIn a large descriptive outcome study using data from the Society of Thoracic Surgeons Congenital Heart Surgery Database, the authors found that VSD repair constituted one third of all cardiac surgeries performed in patients with T18, followed by pulmonary artery banding in 18.5% of cases and right ventricle\/right ventricular outflow tract reconstruction in 9.6% of cases. In this study, 28.5% of all cardiac operative procedures were classified into the Society of Thoracic Surgeons\/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality (STAT) category 4 and 5, indicating the highest complexity levels. Postoperative morbidity and mortality were much higher in T18 patients regardless of surgical complexity. The authors also found that preoperative mechanical ventilation was strongly associated with postoperative mortality. The authors then suggested that surgical candidacy of patients with T18 should be carefully considered in those who require preoperative mechanical ventilation [5]. Increased postoperative airway complications and requiring prolonged respiratory support were also described in this patient population[6]. \r\n\r\nReferences\r\n\r\n1.\tMeyer R, Liu G, Gilboa S, et al. Survival of children with trisomy 13 and trisomy 18: A multi-state population-based study. Am J Med Genet A<\/em>. 2016; 170A(4): 825-837.\t\r\n\r\n2.\tCereda A, Carey J. The trisomy 18 syndrome. Orphanet J Rare Dis<\/em>. 2012; 7: 81. \r\n\r\n3.\tMusewe N, Alexander D, Teshima I, Smallhorn J, Freedom R. Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18. J Am Coll Cardiol<\/em>. 1990; 15(3): 673-677. \r\n\r\n4.\tRosa R, Rosa R, Lorenzen M, et al. Trisomy 18: frequency, types, and prognosis of congenital heart defects in a Brazilian cohort. Am J Med Genet A<\/em>. 2012; 158A(9): 2358-2361. \r\n\r\n5.\tCooper D, Riggs K, Zafar F, et al. Cardiac Surgery in Patients With Trisomy 13 and 18: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. J Am Heart Assoc<\/em>. 2019; 8(13): e012349. DOI: 10.1161\/JAHA.119.012349. \r\n\r\n6.\tSwanson S, Schumacher K, Ohye R, Zampi J. Impact of trisomy 13 and 18 on airway anomalies and pulmonary complications after cardiac surgery. J Thorac Cardiovasc Surg<\/em>. 2020; 162(1): 241-249.”,”redirect_url”:””}}}

{“questions”:{“970sp”:{“id”:”970sp”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Krupa Desai, MD \u2013 Children\u2019s Hospital of Philadelphia; Chinwe Unegbu, MD \u2013 Children\u2019s National Hospital \r\n\r\nA six-year-old child with a secundum atrial septal defect (ASD) presents for a transcatheter ASD device closure. Transthoracic echocardiographic imaging reveals a large ASD with moderate right atrial and ventricular enlargement. What is the MOST LIKELY reason that a Gore Septal Occluder may be preferred versus the Amplatzer Septal Occluder for closure of this defect?\r\n”,”desc”:””,”hint”:””,”answers”:{“zp6ht”:{“id”:”zp6ht”,”image”:””,”imageId”:””,”title”:”A. The Gore Septal Occluder requires a minimal rim for deployment”},”t2say”:{“id”:”t2say”,”image”:””,”imageId”:””,”title”:”B. The Gore Septal Occluder can be placed without echocardiographic guidance”},”u2yaa”:{“id”:”u2yaa”,”image”:””,”imageId”:””,”title”:”C. The Gore Septal Occluder has a low likelihood of device erosion”,”isCorrect”:”1″},”wo88n”:{“id”:”wo88n”,”image”:””,”imageId”:””,”title”:”D. The Gore Septal Occluder does not require aspirin treatment”}}}},”results”:{“l477h”:{“id”:”l477h”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Atrial septal defects (ASD) are one of the most common congenital cardiac defects in the pediatric population. ASD closure is indicated when the resultant left to right shunt leads to right ventricular volume overload and\/or a ratio of total pulmonary blood flow to systemic blood flow (Q_{p<\/sub>:Qs<\/sub>) greater than or equal to 1.5: 1. Additional indications for ASD closure include cyanosis and\/or embolic episodes secondary to right to left shunting. Possible contraindications to ASD closure include evidence of pulmonary hypertension with an elevated pulmonary vascular resistance and reduced right ventricular compliance or function. \r\n\r\nASDs can be closed either surgically or percutaneously via transcatheter intervention. The decision regarding which method of closure to pursue is based on several factors including anatomic criteria, device specific limitations, and possible complications. Transcatheter techniques and device properties have become more refined and as a result, ASD device closure is now accepted as the preferred treatment of choice. Transcatheter closure has excellent clinical efficacy as well as a lower complication rate compared to surgical closure. However, surgical closure is still preferred in patients who have a low body weight, a defect greater than 38mm in diameter, deficient rims, and\/or multiple\/complex defects. \r\n\r\n\r\nTranscatheter device closure of an ASD in the cardiac catheterization lab usually involves the following steps: \r\n\r\n1.\tA hemodynamic cardiac catheterization and assessment of the morphologic characteristics of the defect. \r\n\r\n2.\tEstablishment of a procedural strategy for device implantation including an imaging modality (i.e.TEE, ICE, TTE, etc.). \r\n\r\n3.\t Selection of the optimal device type and size. \r\n\r\n4.\t Device implantation with appropriate surveillance of potential complications. \r\n\r\n5.\t Post implantation assessment. \r\n\r\n\r\nLarge defects represent the main source of challenge for the transcatheter ASD device closure procedure. In this circumstance, problems arise secondary to difficulty in correctly sizing the defect or from the prolapse of left atrial disk of the closure device into the right atrium before proper positioning in the septum. Large defects are frequently associated with rim deficiency. Patients with deficient rims are at risk for device dislodgment, embolization, erosion, and\/or encroachment into nearby cardiac structures as an ASD is surrounded by vital cardiac structures throughout its circumference. \r\n\r\n\r\nBalloon sizing is performed in order to select the correct device. It also provides information about the compliance of the rims. The balloon stretched diameter or balloon occlusive diameter is used to approximate the defect size, but the stop flow diameter (SFD) measurement is standardly recommended to avoid oversizing. In devices such as the Amplatzer Septal Occluder (ASO) (St. Jude Medical, St. Paul, MN, USA), the recommended device size is usually the same or slightly larger (<2 mm) than the SFD. Ultimately, the selection for device size should be individualized by assessing for rim deficiency and evaluating the spatial relationship of nearby structures. \r\n\r\n\r\nThere are many commercially available devices for percutaneous ASD closure. The first device developed was the Amplatzer Septal Occluder (ASO). More recent devices include the Gore Cardioform Septal Occluder (GSO) (WL Gore & Associates, Inc., Flagstaff, AZ, USA), the Figulla Flexible Occlutech device, the Cardioseal\/Starflex, and the bioabsorbable devices (Biostar or Biotrek). The ASO is a self-centering device with the ability to close defects from 4 to 38mm due to its diameter size while the GSO is a non-self-centering device with the ability to close defects from 15 to 18mm. \r\n\r\n\r\nThe ASO was the first self-expanding double-disk device. The device allows for straightforward deployment as it is self-centering, repositionable, and recapturable. With a self-centering device, the waist sits inside the ASD and completely occupies the defect. The edges of the device remain at a fixed distance from the atrial free wall regardless of the timing of the cardiac cycle. Therefore, an oversized device may tent the atrial free wall throughout the cardiac cycle making it vulnerable to trauma. The self-centering characteristic of the ASO necessitates device selection to closely approximate the defect diameter. A stop-flow diameter sizing technique is recommended for the ASO. \r\n\r\n\r\nAn initial study demonstrated a high success rate of ASD closure (95.7%) with few reported major adverse events (1.6%) with the ASO. The subsequent MAGIC study and IMPACT registry reported compatible success and complication rates. The risk of device erosion has become increasingly concerning as it may be fatal and may be delayed. The exact rate of device erosion is estimated to be 0.1 to 0.3%. \r\n\r\n\r\nThe GSO is a non-self-centering double disk device composed of a platinum filled nitinol wire framework covered with an expanded polytetrafluoroethylene membrane to promote rapid endothelialization. When the GSO is fully deployed it assumes a double-disc configuration that bridges the septal defect to prevent shunting of blood between the right and left atria. To effectively close the ASD, the discs must be approximately twice the diameter of the ASD. The GSO is preferred for closure of small defects, particularly those with aortic rim deficiency, due to its flexibility with minimal metal content which may prevent erosion. Clinical studies have verified the efficacy and safety of GSO in closing ASDs with various morphologies. The GSO can adjust to contraction of the atrial chamber because it is a non-self-centering device. \r\n\r\n\r\nAll of the ASD percutaneous closure devices have excellent efficacy and safety profiles. Serious adverse effects occur in less than 1% of patients and more than 95% of the defects can be closed safely. Major catheterization complications specific to this procedure may appear even after completion of the procedure, such as stroke and AV block. The activated clotting time should be at least 250 seconds throughout the procedure, and heparin should never be reversed at the conclusion of the procedure because it increases the risk of thrombus formation on the device. The most common complication reported to the manufacturers and the FDA is device embolization. Device embolization most commonly occurs in the cardiac catheterization laboratory or during the first 24 hours after device placement. The opposite is true of device-related erosion, which is rarely appreciated in the cardiac catheterization laboratory. However, the risk increases significantly during the first 96 hours with sporadic cases reported after 6 months to several years after implantation. To date, device erosion has been noted with the ASO. It is typically, although not exclusively, seen in cases where either the ASO was oversized or there was deficient anterosuperior or retro-aortic rim. Erosion occurring in cases without device oversizing remains of significant concern. To further minimize the risk of erosion, correct device sizing by carefully and non-aggressively employing the stop-flow balloon diameter method is recommended. Patients with aortic rim deficiency spanning 30\u00ba or more should not be selected for device closure. \r\n\r\n\r\nTo date, there are no known cases of erosion with the GSO, however, long term follow-up data is unavailable due to it recently being commercially approved. There is a new GSO now available that is a hybrid of the ASO and original GSO allowing for decreased incidence of device erosion and the ability to close ASDs measuring greater than or equal to 18mm as demonstrated in the ASSURED clinal study. \r\n\r\n\r\nThe correct answer is that the GSO has a low likelihood of device erosion compared to the ASO. The GSO device has not demonstrated any cases of device erosion to date. GSO does not require minimal rim for deployment and rim deficiency may be a contraindication to placement. ASO and GSO are both placed with guidance by echocardiography. ASO and GSO require anticoagulation due to risk of device thrombosis. The devices have metal and\/or polyester mesh components so most patients are started prophylactically on aspirin and\/or clopidogrel. Dual antiplatelet therapy is typically continued post-procedurally for 3 months and aspirin for up to 6 months (device endothelialization). \r\n\r\n\r\n\r\nReferences \r\n1.\tde Hemptinne Q, Horlick EM, Osten MD, et al. Initial clinical experience with the GORE\u00aeCARDIOFORM ASD occluder for transcatheter atrial septal defect closure. Catheter Cardiovasc Interv<\/em>. 2017; 90(3): 495-503. doi:10.1002\/ccd.26907 ——————–\r\n\r\n2.\tFaccini A, Butera G. Atrial septal defect (ASD) device trans-catheter closure: limitations. J Thorac Dis<\/em>. 2018; 10(Suppl 24): S2923-S2930. doi:10.21037\/jtd.2018.07.128 ——————–\r\n\r\n3.\tDu ZD, Hijazi ZM, Kleinman CS, et al. Comparison between transcatheter and surgical closure of secundum atrial septal defect in children and adults: results of a multicenter nonrandomized trial. J Am Coll Cardiol<\/em>. 2002; 39: 1836-1844.——————–\r\n\r\n4.\tFraisse A, Latchman M, Sharma SR, et al. Atrial septal defect closure: indications and contraindications. J Thorac Dis<\/em>. 2018; 10(Suppl 24): S2874-S2881. doi:10.21037\/jtd.2018.08.111 ———————\r\n\r\n5.\tOlasinska-Wisniewska A, Grygier M. Antithrombotic\/Antiplatelet Treatment in Transcatheter Structural Cardiac Interventions-PFO\/ASD\/LAA Occluder and Interatrial Shunt Devices. Front Cardiovasc Med<\/em>. 2019; 6: 75. Published 2019 Jun 7. doi:10.3389\/fcvm.2019.00075 ——————–\r\n\r\n6.\tS\u00f8ndergaard L, Loh PH, Franzen O, et al. The first clinical experience with the new GORE\u00ae septal occluder (GSO). EuroIntervention<\/em> 2013; 9: 959-963. ——————–\r\n\r\n\r\n7.\tSmith B, Thomson J, Crossland D, et al. UK multicenter experience using the Gore septal occluder (GSO(TM)) for atrial septal defect closure in children and adults. Catheter Cardiovasc Interv<\/em> 2014; 83: 581-586. ——————–\r\n\r\n8.\tSantoro G, Castaldi B, Cuman M, et al. Trans-catheter atrial septal defect closure with the new GORE\u00ae Cardioform ASD occluder: First European experience. Int J Cardiol<\/em>. 2021; 327: 68-73. doi:10.1016\/j.ijcard.2020.11.029 ——————–\r\n\r\n9.\tSommer RJ, Love BA, Paolillo JA, et al. ASSURED clinical study: New GORE\u00ae CARDIOFORM ASD occluder for transcatheter closure of atrial septal defect. Catheter Cardiovasc Interv<\/em>. 2020; 95(7): 1285-1295. doi:10.1002\/ccd.28728 ——————–\r\n\r\n10.\tYang MC, Wu JR. Recent review of transcatheter closure of atrial septal defect. Kaohsiung J Med Sci<\/em>. 2018; 34(7): 363-369. doi:10.1016\/j.kjms.2018.05.001 ———————\r\n\r\n11.\tAmin Z, Hijazi ZM, Bass JL, et al. Erosion of Amplatzer septal occluder device after closure of secundum atrial septal defects: review of registry of complications and recommendations to minimize future risk. Catheter Cardiovasc Interv<\/em>. 2004; 63: 496-502. ——————–\r\n\r\n12.\tKrumsdorf U, Ostermayer S, Billinger K, et al. Incidence and clinical course of thrombus formation on atrial septal defect and patent foramen ovale closure devices in 1,000 consecutive patients. J Am Coll Cardiol<\/em>. 2004; 43: 302-309. ——————–\r\n\r\n13.\tLevi DS, Moore JW. Embolization and retrieval of the Amplatzer septal occluder. Catheter Cardiovasc Interv<\/em>. 2004; 61: 543-547. ———————\r\n\r\n14.\tEverett AD, Jennings J, Sibinga E, et al. Community use of the amplatzer atrial septal defect occluder: results of the multicenter MAGIC atrial septal defect study. Pediatr Cardiol<\/em> 2009; 30: 240-247. ———————\r\n\r\n15.\tMoore JW, Vincent RN, Beekman RH 3rd, et al. Procedural results and safety of common interventional procedures in congenital heart disease: initial report from the National Cardiovascular Data Registry. J Am Coll Cardiol<\/em> . 2014; 64: 2439-2451. ———————-\r\n\r\n16.\tDiab K, Kenny D, Hijazi ZM. Erosions, erosions, and erosions! Device closure of atrial septal defects: how safe is safe? Catheter Cardiovasc Interv<\/em>. 2012; 80: 168-174. ——————–\r\n\t\r\n17.\tCrawford GB, Brindis RG, Krucoff MW, et al. Percutaneous atrial septal occluder devices and cardiac erosion: a review of the literature. Catheter Cardiovasc Interv<\/em> . 2012; 80: 157-167. ——————–\r\n\r\n18.\tMcElhinney DB, Quartermain MD, Kenny D, et al. Relative Risk Factors for Cardiac Erosion Following Transcatheter Closure of Atrial Septal Defects: A Case-Control Study. Circulation<\/em>. 2016; 133: 1738-1746. “,”redirect_url”:””}}}}

{“questions”:{“ue4cq”:{“id”:”ue4cq”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Krupa Desai, MD \u2013 Children\u2019s Hospital of Philadelphia; Chinwe Unegbu, MD \u2013 Children\u2019s National Hospital\r\n\r\nA 3 year-old-child with hypoplastic left heart syndrome (HLHS) status post hybrid palliation and subsequent comprehensive stage II repair presents for cardiac magnetic resonance imaging (MRI). The imaging cardiologist plans to utilize ferumoxytol instead of gadolinium contrast. Which of the following is the MOST LIKELY indication for ferumoxytol contrast versus gadolinium contrast? \r\n”,”desc”:””,”hint”:””,”answers”:{“8olpx”:{“id”:”8olpx”,”image”:””,”imageId”:””,”title”:”A. Iron overload secondary to frequent blood transfusions “},”ck6ku”:{“id”:”ck6ku”,”image”:””,”imageId”:””,”title”:”B. History of anaphylaxis to amoxicillin “},”t7eag”:{“id”:”t7eag”,”image”:””,”imageId”:””,”title”:”C. High resolution imaging of aortopulmonary collaterals “,”isCorrect”:”1″},”k18ov”:{“id”:”k18ov”,”image”:””,”imageId”:””,”title”:”D. Liver dysfunction”}}}},”results”:{“vylzp”:{“id”:”vylzp”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Ferumoxytol (Feraheme, AMAG Pharmaceuticals, Waltham, MA) is an intravenous formulation of enteral iron classically used to treat severe iron deficiency anemia in adult patients with chronic kidney disease. Ferumoxytol has been shown to be an effective and safe magnetic resonance imaging (MRI) contrast agent. Ferumoxytol allows for contrast enhanced imaging in patients with contraindications to gadolinium-based contrast agents (GBCA), such as renal failure. Ferumoxytol has recently been used with increasing frequency in smaller and more medically fragile children. \r\n\r\nDue to the long half-life, ferumoxytol does not have to be administered during the cardiac MRI exam itself. Rather, it can be administered hours prior to the cardiac MRI. This is particularly beneficial in tenuous cardiac patients in order to decrease the time spent outside of the intensive care unit (ICU) by administration of ferumoxytol at bedside prior to transport. Ferumoxytol is impermeable to vascular capillaries and is not filtered by the kidneys. Thus, it remains in the intravascular compartment until it is ingested by macrophages and then excreted by the liver. Due to its large molecular size and dextran coat, ferumoxytol has a long intravascular half-life of approximately 14\u201315 hours, which provides a long and stable time window for vascular enhancement and imaging. As the uptake and metabolism of ferumoxytol is by the reticuloendothelial system, the risk of nephrogenic systemic fibrosis and central nervous system deposition is nil compared to GBCA. \r\n\r\nIn pediatric patients, the additional benefits of ferumoxytol are quite noteworthy. Ferumoxytol allows for patients with renal dysfunction to have contrast-enhanced MRI imaging when needed due to a lack of renal metabolism and excretion. Administration of ferumoxytol is quite beneficial in patients with cardiac lesions whose residual disease burden can be best addressed by contrast enhanced imaging. This includes patients with Fontan physiology, anomalous coronaries, and those with aortopulmonary collaterals. Use of ferumoxytol negates the need for suspended respirations during cardiac MRI studies due to prolonged vascular enhancement. Suspended respirations are typically requested to obtain better-quality imaging with GBCA and to minimize motion artifact related to respiratory efforts. As a result, patients undergoing cardiac MRI with GBCA are more likely to be intubated and receive neuromuscular blocking agents to facilitate suspended respirations during MRI gating. Imaging parameters are enhanced with the use of ferumoxytol negating the need for respiratory pauses. Ultimately, this results in a reduced time needed for the cardiac MRI imaging and general anesthesia when compared to imaging obtained with GBCA. \r\n\r\nFerumoxytol has an excellent safety profile with a long history of use. Due to its long half-life, however, anaphylactic reactions (0.03% aggregate rate in post-market surveillance of > 8000 administrations) can be serious and difficult to treat. The majority of anaphylactic reactions are recognizable within 5 minutes of starting the infusion. Thirty percent of individuals who develop anaphylatic reactions have had at least one prior allergic reaction to a medication. Due to the long half-life of ferumoxytol, some consider a history of anaphylaxis to any other medication a \u201csoft\u201d contraindication to ferumoxytol. Hypersensitivity reactions resulting in hypotension and death have been reported with the use of ferumoxytol for the treatment of anemia in adults. The reports of hypersensitivity to ferumoxytol in adults led to a black box warning in 2015 by the Food and Drug Administration (FDA). Monitoring for signs of anaphylaxis (hypotension, erythema, rash, bronchospasm, etc.) is prudent. Blood pressure measurements should be performed routinely up until 30 min after completion of the infusion. Early treatment with diphenhydramine and epinephrine has successfully treated anaphylaxis. \r\n\r\nHypotension alone (nonimmunologic) has also been associated with ferumoxytol and is thought to be related to the release of free iron. Other intravenous iron formulations have similar effects on blood pressure (hypotension). As a result, the FDA has recommended dilution and slow administration of ferumoxytol over approximately 15 minutes. \r\n\r\nFerumoxytol is contraindicated in patients with a history of allergic reactions to ferumoxytol or other intravenous iron products and those with iron overload such as hemochromatosis, severe chronic hemolysis, frequent blood transfusion, and prolonged hemodialysis. Additionally, use is cautioned in patients with severe hepatic disease. \r\n\r\nAll MRI images may be altered by ferumoxytol for days to months. This complicates subsequent MRI imaging of other areas of the body such as the brain. As a result, close coordination and communication is needed with respect to the sequence of imaging in patients requiring MRI scans of other regions of the body in addition to the cardiac MRI. \r\n\r\nReferences\r\n1.\tRuangwattanapaisarn N, Hsiao A, Vasanawala SS. Ferumoxytol as an off-label contrast agent in body 3T MR angiography: a pilot study in children. Pediatr Radiol<\/em>. 2015; 45(6): 831-839. doi:10.1007\/s00247-014-3226-3.2. \r\n2.\tCorot C, Robert P, Idee J, Port M. Recent advances in iron oxide nanocrystal technology for medical imaging. Adv Drug Deliv Rev<\/em>. 2006; 58(14): 1471-1504. doi:10.1016\/j.addr.2006.09.013 \r\n3.\tNguyen KL, Yoshida T, Han F, et al. MRI with ferumoxytol: A single center experience of safety across the age spectrum. J Magn Reson Imaging<\/em>. 2017; 45(3): 804-812. doi:10.1002\/jmri.25412. \r\n4.\tVan Wyck DB. Labile iron: manifestations and clinical implications. J Am Soc Nephrol<\/em> 2004; 15: S107\u2013S111. \r\n5.\tWise-Faberowski L, Velasquez N, Chan F, Vasanawala S, McElhinney DB, Ramamoorthy C. Safety of ferumoxytol in children undergoing cardiac MRI under general anaesthesia. Cardiol Young<\/em>. 2018; 28(7): 916-921. doi:10.1017\/S1047951118000306 \r\n6.\tLi W, Tutton S, Vu AT, et al. First-pass contrast-enhanced magnetic resonance angiography in humans using ferumoxytol, a novel ultrasmall superparamagnetic iron. (USPIO)-based blood pool agent. J Magn Reson Imaging<\/em>. 2005; 21: 46\u201352. \r\n7.\tRampton D, Folkersen J, Fishbane S, et al. Hypersensitivity reactions to intravenous iron: guidance for risk minimization and management. Haematologica<\/em>. 2014; 99: 1671\u20131676.\r\n”,”redirect_url”:””}}}