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

{“questions”:{“ag48h”:{“id”:”ag48h”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Kaitlin M. Flannery, MD, MPH – Stanford University AND Manchula Navaratnam, MBChB – Stanford University \r\nA 13-year-old, 30 kg, female with hypoplastic left heart syndrome palliated to an extracardiac Fontan with failing Fontan physiology has been listed for heart transplantation. She is highly sensitized resulting in limited compatible donor offers. An acceptable offer becomes available from a 32 kg donor, but the travel time is anticipated to be seven hours. What factor MOST LIKELY limits the use of Organ Care System (OCS) [TransMedics, Andover, MA] preservation in this case?\r\n”,”desc”:”EXPLANATION \r\nHeart transplantation is the gold-standard treatment for pediatric patients with end-stage heart failure. Due to the scarcity of acceptable donor organs, pediatric waitlist mortality remains at 20%. In the United States, an estimated 50% of potential pediatric donor hearts are not utilized, in part because of the limitations with static cold storage preservation. Prior studies have shown increased graft failure and mortality when cold ischemic time exceeds four hours, limiting the geographic distance from which organs can be accepted. Cold storage also does not permit assessment or optimization of organs, a key factor when considering donation after circulatory death (DCD).^{1<\/sup> \r\n\r\nThe Organ Care System (OCS Heart) [TransMedics, Andover, MA] is a portable ex situ perfusion, preservation, and monitoring system. The OCS delivers oxygenated, heated donor blood in a pulsatile fashion, through a cannula placed in the donor aorta, perfusing the myocardium via the coronary arteries. Blood returning to the right heart through the coronary sinus is drained back to the OCS reservoir via a cannula placed in the donor pulmonary artery. The OCS continuously monitors mean aortic pressure, coronary blood flow, and arterial and venous lactate levels.2<\/sup> \r\n\r\nA randomized control trial in adult patients compared risk-adjusted six-month survival between recipients of DCD donor hearts preserved with OCS versus those preserved with traditional cold storage following donation after brain death (DBD). Risk-adjusted six-month survival was 94% in the OCS group compared with 90% in the control group. This demonstrated non-inferiority of DCD heart transplantation using OCS preservation and led to FDA approval of OCS Heart for adult patients in 2022.3<\/sup>\r\n\r\nA prospective study evaluated outcomes of extended criteria donor (ECD) organs preserved with OCS following DBD. Due to concerns for poor outcomes of ECD organs preserved with static cold storage, this was not a randomized control trial. Criteria for ECD include donors \u226555 years old, donors 45-55 years old without a coronary angiogram, anticipated cold ischemic time \u22654 hours, or donors with \u226520 minutes of downtime but stable hemodynamics at procurement. Organs were accepted for transplantation if OCS monitoring demonstrated a downward-trending arterial lactate <5 mmol\/L, stable aortic pressures and coronary flow, and surgeon approval. Of 173 hearts placed on the OCS, 150 were transplanted, yielding an 87% utilization rate. These organs had been declined an average of 51 times before acceptance, suggesting they would likely have been discarded without OCS preservation and assessment. The study\u2019s primary endpoints \u2013 30-day survival and incidence of severe primary graft dysfunction -were 96.6% and 6.7%, respectively.4<\/sup>\r\n\r\nThe first report of pediatric heart transplantation using OCS preservation, was published in 2025 by Duke University Medical Center. Donor weight had to be \u226540 kg to ensure system compatibility. Outcomes of eight recipients were described. The median recipient age was 13 years (range 9-18) and median weight was 58 kg (range 33-127). At the time of transplant offer, two patients were outpatient on ventricular assist device (VAD) support, while six were inpatient (three on VADs and one on ECMO). Of the organs accepted, six were from DBD donors and two from DCD donors. The median OCS duration was 273 minutes (4.5 hours), with an average arterial lactate of 2.42 mmol\/L. Post-operatively, one patient required intra-aortic balloon pump support for one day and one patient required ECMO for three days. All patients survived and demonstrated normal left ventricular systolic function at hospital discharge. Hospital courses, however, were prolonged, with an average ICU stay of 34 days (range 4-101) and hospital stay of 72 days (13-249).1<\/sup> \r\n\r\nOCS Heart is currently FDA approved for preservation of DCD and ECD DBD hearts in adults. Donors must be \u226540 kg to ensure system compatibility. Contraindications to OCS use include moderate to severe aortic insufficiency, visible donor-organ bruising, and known atrial or ventricular septal defects in the donor heart.2<\/sup> Expanded OCS use in pediatric transplantation may be valuable for patients with complex congenital heart disease, such as Fontan physiology, who have limited compatible donor offers and often require prolonged operative times. Current OCS device size limitations remain a barrier for smaller donors. \r\n\r\nRecipient weight of 30kg would not be an absolute contraindication to OCS use for the donor organ, as long as there would not be resultant donor-recipient size mismatch. That would be determined by the transplanting center and the specified donor size range for the patient that is listed for transplant. \r\n\r\nOrgan travel time of seven hours would not be a contraindication and often would be considered an indication for use of OCS.\r\n\r\n\r\n \r\nREFERENCES \r\n1.\tMedina CK, Aykut B, Parker LE, et al<\/em>. Early single-center experience with an ex vivo organ care system in pediatric heart transplantation. J Heart Lung Transplant<\/em>. 2025 Apr;4(4):545-9. \r\n2.\tFDA. Organ Care System (OCS) Heart System \u2013 P180051\/S001. US Food & Drug Administration. Published April 27, 2022. Accessed September 8, 2025. https:\/\/www.fda.gov\/medical-devices\/recently-approved-devices\/organ-care-system-ocs-heart-system-p180051s001. \r\n3.\tSchroder JN, Patel CB, DeVore AD, et al<\/em>. Transplantation outcomes with donor hearts after circulatory death. N Engl J Med<\/em>. 2023 Jun 8;388(23):2121-31.\r\n4.\tSchroder JN, Patel CB, DeVore AD, et al<\/em>. Increasing utilization of extended criteria donor hearts for transplantation: the OCS Heart EXPAND trial. JACC Heart Fail<\/em>. 2024 Mar;12(3):438-47. \r\n”,”hint”:””,”answers”:{“had9k”:{“id”:”had9k”,”image”:””,”imageId”:””,”title”:”A. Travel time of seven hours “},”11u3m”:{“id”:”11u3m”,”image”:””,”imageId”:””,”title”:”B. Recipient weight of 30 kg”},”ixoqj”:{“id”:”ixoqj”,”image”:””,”imageId”:””,”title”:”C. Donor weight of 32 kg “,”isCorrect”:”1″}}}}}}

{“questions”:{“gm6pd”:{“id”:”gm6pd”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Kaitlin M. Flannery, MD, MPH – Stanford University AND Megan Quinn, MD, MPH – Stanford University \r\n\r\nA 10-year-old, 33kg, male with Duchenne Muscular Dystrophy presents for placement of single lead transvenous ICD in the setting of increasing myocardial fibrosis and frequent runs of nonsustained ventricular tachycardia despite treatment with nadolol. His other medications are enalapril and vamorolone. What alteration in management is MOST LIKELY required in patients presenting for anesthesia that are maintained on vamorolone?\r\n”,”desc”:”EXPLANATION \r\nDuchenne Muscular Dystrophy (DMD) is an X-linked, recessive, neuromuscular disease with an incidence of 1:3000 males. The mutation results in absence of the dystrophin protein, which plays an essential role for muscle cell membrane maintenance and signaling. The disease is typically diagnosed around three years of age when progressive weakness of lower extremity muscles begins. Patients are typically wheelchair bound by 7-13 years. Respiratory failure in the 2nd or 3rd decade is the most common cause of death. Dilated cardiomyopathy is present in 25% of patients by age six. Bradyarrhythmia and tachyarrhythmias are common due to progressive fibrosis of cardiac muscle putting patients at increased risk of sudden cardiac death. Glucocorticoids have been a cornerstone of DMD treatment since the Clinical Investigation of Duchenne Dystrophy (CIDD) group was formed in 1981 and investigated the potential benefit of multiple medications. At the time, prednisone was the only medication with beneficial effect, reducing inflammation in the muscle and slowing loss in ambulation by a few years. Unfortunately, there are significant side effects of chronic glucocorticoids including obesity, osteoporosis, glucose intolerance, hypertension, mood change, immunosuppression, and adrenal insufficiency.^{1,2 <\/sup>\r\n\r\n\r\nVamorolone was FDA-approved in 2023 for treatment of patients two years and older with DMD. It is a partial glucocorticoid agonist with anti-mineralocorticoid activity. It is classified as a dissociative glucocorticoid as it possesses anti-inflammatory and immunosuppressive properties but lacks some of the classic adverse side effects. A randomized controlled trial performed from 2018-2021 divided 120 participants into four categories: placebo, prednisone 0.75mg\/kg\/day, vamorolone 2mg\/kg\/day, and vamorolone 6mg\/kg\/day. Over 24 weeks, patients on both doses of vamorolone had improvements in time to stand velocity and distance covered in a 6-minute walk test compared to their baseline, whereas those on placebo demonstrated decline in these parameters over the 24 weeks. Height percentile declined and measures of bone turnover increased in patients on prednisone, but not in patients on vamorolone. Therefore, vamorolone was found to be effective in slowing muscular decline in patients with DMD while decreasing detrimental effects to bone health. Patients on vamorolone had similar increases in BMI as patients on prednisone as well as evidence of adrenal suppression by morning cortisol levels and ACTH stimulation testing.3<\/sup>This seemed to be dose-dependent, as the lower-dose group showed less adrenal suppression than the higher-dose group. \r\n\r\n\r\nIt is important to note that, prior to initiating any treatment in the vamorolone trial, patients with DMD had a high incidence of adrenal insufficiency based on morning cortisol levels and ACTH stimulation testing. This may be explained by the proximity of the DMD and NR0B1<\/em> genes on the X-chromosome, as mutations in NR0B1<\/em> result in congenital adrenal hypoplasia.3<\/sup>\r\n\r\n\r\nIn patients on vamorolone, administration of stress dose steroids should be considered in the perioperative period due to chronic adrenal suppression by the medication. Continuing nadolol is likely to be beneficial in the context of ventricular ectopy, and enalapril may lead to perioperative hypotension, but those concerns remain unrelated to vamorolone as it will not result in significant changes in blood pressure, heart rate or rhythm. \r\n\r\n\r\n \r\nREFERENCES \r\n\r\n1.\tAmes WA, Hayes JA, Crawford MW. The role of corticosteroids in Duchenne muscular dystrophy: a review for the anesthetist. Paediatr Anaesth<\/em>. 2005 Jan;15(1):3-8. \r\n2.\tParchmont E, Vender S. Anesthetic considerations for patients with Duchenne Muscular Dystrophy and Becker Muscular Dystrophy: management of a 15-year-old male with muscular dystrophy for a cystourethroscopy, laser lithotripsy and stent placement \u2013 a case study. CCAS E-News<\/em>. 2021; https:\/\/www2.ccasociety.org\/newsletters\/2021summer\/interesting%20case.html. \r\n3.\tGuglieri M, Clemens PR, Perlman SJ, et al. Efficacy and safety of vamorolone vs placebo and prednisone among boys with Duchenne Muscular Dystrophy. A randomized control trial. JAMA Neurol<\/em>. 2022 Oct 1;79(10):1005-14. \r\n”,”hint”:””,”answers”:{“r08mw”:{“id”:”r08mw”,”image”:””,”imageId”:””,”title”:”A.\tHolding medication for 24 hours to avoid profound hypotension”},”srtth”:{“id”:”srtth”,”image”:””,”imageId”:””,”title”:”B.\tAdministration of stress-dose steroids to manage adrenal suppression”,”isCorrect”:”1″},”7rdnm”:{“id”:”7rdnm”,”image”:””,”imageId”:””,”title”:”C.\tContinuing medication to avoid increase in ectopy “}}}}}}

{“questions”:{“skoam”:{“id”:”skoam”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Kaitlin M. Flannery, MD, MPH – Stanford University AND Amy Babb MD, Monroe Carell Jr. Children\u2019s Hospital – Vanderbilt University \r\nA 2-day-old, ex-34 week, 1.8kg, neonate with hypoplastic left heart syndrome is scheduled for transcatheter placed pulmonary flow restrictors to balance circulation and delay stage 1 Norwood surgery due to prematurity and size. What is the MOST common complication of transcatheter placed pulmonary flow restrictors?”,”desc”:”EXPLANATION \r\nPulmonary flow restriction via a transcatheter placed modified microvascular plug (MVP) [Medtronic, Minneapolis, MN] is a novel, percutaneous approach to control pulmonary blood flow (PBF) compared with pulmonary artery banding (PAB) via sternotomy. A swine model, published in 2018, demonstrated the feasibility of modified MVPs as retrievable pulmonary flow restrictors (PFR).^{1<\/sup> Previous attempts at percutaneous PFR with other devices were plagued with concerns regarding large delivery systems, unpredictable PBF, and uncertainty regarding removal. The current MVP device is only FDA-approved for peripheral vascular embolization but has been used \u201coff label\u201d for PDA closure. It is available in four sizes, delivered through a microcatheter, is re-sheathable, and has reliability in predicting PBF.\r\n\r\nRetrospective, single center studies have shown the feasibility of utilizing modified MVPs as PFRs to stabilize high-risk neonates with pulmonary overcirculation and defer surgery for weeks to months.2,3<\/sup> The most common complication seen in these initial studies is device embolization. Other complications include device thrombosis, device rupture, persistent overcirculation, and access site complications.\r\n\r\nA 2023 article describes Boston Children\u2019s Hospital\u2019s experience with MVP PFRs.2<\/sup> A total of 17 high-risk neonates, predominantly with hypoplastic left heart syndrome (HLHS), underwent placement of modified MVPs instead of hybrid stage 1 with sternotomy and PAB placement. Throughout the reported 2-year experience, many modifications were made in device fenestration technique and location of device placement. Over 40% required concomitant procedures including PDA stenting or enlargement of atrial communication. One third of the patients experienced device embolization\/migration with many embolization events occurring after procedure completion and without acute symptoms. Two patients (5%) developed device thrombosis. Compared with a historic cohort of high-risk neonates undergoing hybrid procedure, the 6-month all-cause mortality risk was significantly lower in the PFR group.2<\/sup> \r\n\r\nA 2024 article describes the experience at Children\u2019s Memorial Hermann Hospital with MVP PFRs.3<\/sup> A total of seven neonates with HLHS or Shone\u2019s complex with contraindications to immediate surgical stage 1 palliation, most commonly prematurity or low birth weight, underwent placement of PFRs in the bilateral branch pulmonary arteries. None of the procedures had acute complications, with no device embolization events in this cohort. One patient, with larger pulmonary arteries ultimately required sternotomy with PAB placement 5 days after PFR placement for persistent overcirculation. All devices were removed at the time of surgery with no need for pulmonary artery augmentation. One patient with MVPs in for 67 days had a challenging and protracted device removal.3<\/sup> \r\n\r\nTo conclude, pulmonary flow restriction with modified MVP devices exists as one potential option for controlling PBF in congenital heart disease. The patient numbers in the published series remain small, but it remains a potential avenue forward for some of the most high-risk patients. \r\n\r\nDevice embolization\/migration is currently the most common complication described in the literature compared to device thrombosis or pulmonary artery stenosis. \r\n\r\n\r\n \r\nREFERENCES \r\n1.\tKhan AH, Hoskoppal D, Kumar TKS, et al. Utility of the Medtronic microvascular plug as a transcatheter implantable and explantable pulmonary artery flow restrictor in a swine model. Catheter Cardiovasc Interv<\/em>. 2019 Jun 1;93(7):1320-8. \r\n2.\tSperotto F, Lang N, Nathan M, et al. Transcatheter Palliation with pulmonary flow restrictors in neonates with congenital heart disease: feasibility, outcomes, and comparison with a historical hybrid stage 1 cohort. Circ Cardiovasc Interv<\/em>. 2023 Dec;16(12):e013383. \r\n3.\tWarren M, Choy AV, Khan M, et al. Percutaneous pulmonary flow restriction in infants with congenital heart disease. JACC Adv<\/em>. 2024 Jun 7;3(7):101031. \r\n”,”hint”:””,”answers”:{“67wbu”:{“id”:”67wbu”,”image”:””,”imageId”:””,”title”:”A.\tDevice thrombosis”},”e46fa”:{“id”:”e46fa”,”image”:””,”imageId”:””,”title”:”B.\tPulmonary artery stenosis “},”ux3c2”:{“id”:”ux3c2″,”image”:””,”imageId”:””,”title”:”C.\tDevice embolization\/migration”,”isCorrect”:”1″}}}}}}

{“questions”:{“hng79”:{“id”:”hng79″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2025\/10\/CCAS-102-QOW-Pic-1.jpg”,”imageId”:”9113″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Kaitlin M. Flannery, MD, MPH – Stanford University AND Catherine Dietrich, MD – Stanford University \r\nA 5kg, 4-month-old infant with feeding intolerance and failure to thrive is noted to have a murmur on pediatrician follow up visit. A 4-chamber view of his transthoracic echocardiogram is shown below. What is the MOST LIKELY diagnosis identified in this image?”,”desc”:”EXPLANATION \r\nCor triatriatum (from the Latin for a \u201cheart with three atria\u201d) is a rare cardiac anomaly that results from abnormal septation of either the right (dexter \u2013 from the Latin for \u201cright-sided or right-handed\u201d) or left (sinister \u2013 from the Latin for \u201cleft-sided or left-handed\u201d) atrium. It occurs in less than 0.1% of all congenital heart disease. In classic cor triatriatum sinister (CTS), which was present in the patient above, a fibromuscular membrane divides the proximal atrium, which accepts pulmonary venous return, from the distal atrium which contains the atrial appendage and mitral valve. The membrane may be complete, incomplete, or fenestrated. The severity of the membrane gradient, and presence or absence of other cardiac anomalies, will determine the age and status at presentation.^{1<\/sup> \r\n \r\n\r\nCTS symptoms and management emulate that of mitral stenosis. Restriction of flow across the membrane results in elevated left atrial pressure which leads to elevated pulmonary venous pressure and pulmonary edema. The elevated pulmonary venous pressure will cause elevated pulmonary arterial pressure that can become permanent if left untreated. High left atrial pressure can result in left atrial dilation increasing risk for atrial arrhythmias and thrombus formation. If the flow gradient significantly limits left ventricular filling, a low cardiac output state may develop.1<\/sup> \r\n\r\nInduction of anesthesia for surgical correction requires maintenance of preload with judicious fluid administration, maintenance of sinus rhythm and avoidance of tachycardia to allow ventricular filling, and avoidance of increases in pulmonary vascular resistance (PVR) that will put additional strain on the right ventricle. Prior to surgical removal of the membrane, therapies that decrease PVR should be limited as they might cause pulmonary congestion. After removal of the membrane, PVR-lowering therapies can be employed.1<\/sup> \r\n\r\nAs cor triatriatum remains a rare anomaly, there are limited large studies assessing surgical outcomes. The largest one to date is a descriptive retrospective review of 65 patients treated from 1963-2010 at Boston Children\u2019s Hospital. The median age at surgical repair was 6.9 months with a range from 2 days to 47 years. Over half of the patients were infants. Additional cardiac anomalies were found in 75% of the patients, with atrial septal defect, ventricular septal defect, and partial anomalous pulmonary venous return being the most common. The two deaths that occurred in the early postoperative period were in infants operated on in the 1970\u2019s. In the remaining patients, there was no recurrence of obstruction in the atrium. However, approximately 10% developed pulmonary vein stenosis that required interventional or surgical management.2<\/sup> \r\n\r\nA more recent retrospective review describes 16 patients treated from 2000-2020 at the University of Minnesota. The median age at diagnosis was 4.3 months with a range from 1 day to 7 years. Additional cardiac anomalies were found in 81% of the patients. The CTS created a clinically significant obstruction in 50% of patients. Twelve patients underwent surgical repair. Two patients had early postoperative surgical mortality. These patients had additional congenital cardiac anomalies, one with heterotaxy and complex single ventricle anatomy and one with total anomalous pulmonary venous return. The remaining 10 surgical patients did well with no recurrence of obstruction in the atrium on follow-up.3<\/sup> \r\n\r\nThe image in the scenario clearly shows a left atrial membrane, effectively separating it in proximal and distal portions. Cor triatriatum dexter would result from a right atrium membrane, and no membrane would be seen in context of pure mitral stenosis. \r\n\r\n \r\nREFERENCES \r\n\r\n1.\tKumar Jha A, Makhija N. Cor Triatriatum: a review. Semin Cardiothorac Vasc Anesth<\/em>. 2017 Jun;21(2):178-85. \r\n2.\tKazanci SY, Emani S, McElhinney DB. Outcome after repair of cor triatriatum. Am J Cardiol<\/em>. 2012 Feb 1;109(3):412-6. \r\n3.\tMashadi AH, Narasimhan SL, Said SM. Cor triatriatum sinister: long-term surgical outcomes in children and a proposal for a new classification.J Card Surg<\/em>. 2022 Dec;37(12):4526-33.\r\n”,”hint”:””,”answers”:{“bkkc4”:{“id”:”bkkc4″,”image”:””,”imageId”:””,”title”:”A.\tCor triatriatum dexter “},”uovkd”:{“id”:”uovkd”,”image”:””,”imageId”:””,”title”:”B.\tCor triatriatum sinister”,”isCorrect”:”1″},”zvgdv”:{“id”:”zvgdv”,”image”:””,”imageId”:””,”title”:”C.\tMitral stenosis “}}}}}}

{“questions”:{“jj06y”:{“id”:”jj06y”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Anila B. Elliott, MD – University of Michigan, C.S. Mott Children\u2019s Hospital \r\n\r\nA 5-day-old male with d-Transposition of the Great Arteries (TGA) and ventricular septal defect presents for arterial switch operation. Which one of the following perioperative factors would INCREASE the risk of poor neurodevelopmental outcomes in this patient? “,”desc”:”EXPLANATION \r\nOver the last few decades, survival for those with congenital heart disease (CHD) has improved as advancements in management have led to innovative treatment for even those with more complex anatomy. However, despite a survival improvement, neurodevelopmental outcomes have not improved in the same manner. Children with CHD are at increased risk for a variety of neurodevelopmental issues including motor delays, impaired language development, and reduced cognitive performance. Some of these deficits are more pronounced in those with more complex congenital heart disease, such as a lower intelligence quotient (IQ) in those with hypoplastic left heart syndrome (HLHS) compared to those with atrial septal defects (ASD)^{1<\/sup>.\r\n\r\nIn the latest American Heart Association (AHA) guidelines, new research has led to improved understanding regarding perioperative risk factors that impact neurodevelopment1<\/sup>. Genetic predisposition, perinatal, socioeconomic, and perioperative factors all play a role in long-term neurodevelopmental outcomes 1,2<\/sup>. \r\n\r\nA summary of major risk factors for neurodevelopmental impairment can be found below:1,2<\/sup>: \r\n1.\tGenetic predisposition \r\na.\tSyndromes (Down syndrome, 22q11.2 deletions, William syndrome, Turner syndrome)\r\nb.\tMale gender\r\nc.\tExtracardiac anomalies\r\n2.\tFetal and perinatal factors \r\na.\tCyanosis with hypoxia and reduced nutrient delivery \r\nb.\tPrematurity, especially in those with transposition of great arteries (TGA) or single-ventricle physiology\r\nc.\tPost-natal diagnosis of CHD requiring early surgical intervention\r\n3.\tSurgical and perioperative factors \r\na.\tPerioperative seizures in infancy\r\nb.\tBrain injury (white matter injury, stroke) \r\nc.\tIncreased post-operative length of stay (>14 days) \r\nd.\tCardiopulmonary resuscitation or cardiac arrest \r\ne.\tMechanical circulatory support\r\nf.\tHeart transplantation in childhood \r\ng.\tNeed for multiple interventions or complications \r\n4.\tPsychosocial factors \r\na.\tLow socioeconomic status \r\nb.\tLow maternal education \r\nc.\tSignificant parental mental health challenges (anxiety, depression, post-traumatic stress disorder [PTSD]) \r\n5.\tGrowth and developmental factors \r\na.\tFeeding difficulties \r\nb.\tGrowth failure (including low birth weight and failure to thrive) \r\nc.\tHistory of developmental delay \r\n6.\tOther factors \r\na.\tAbnormal fetal cerebral blood flow \r\nb.\tAbnormal placental development \r\nc.\tExposure to environmental neurotoxins \r\n \r\nPerioperative seizures (Answer A) are strongly associated with poor neurodevelopmental outcomes. Seizures are linked to underlying brain injury and predict long-term deficits in cognitive, academic, and functional abilities1,2<\/sup>. \r\n\r\nDelays in surgical correction are also associated with poorer neurodevelopmental outcomes. For example, in neonates with TGA, prolonged time to arterial switch surgery has been shown to increase the risk of white matter ischemia, which is tied to subsequent neurodevelopmental impairments3<\/sup>. This concern is consistent with earlier findings that delayed surgical intervention is linked to impaired brain growth and worse language development2<\/sup>. \r\n\r\nWhile lower hematocrit levels and hypotension during CPB may impair oxygen delivery, and theoretically contribute to cerebral hypoperfusion and injury, the direct long-term impact of this on neurodevelopment has not been well-established. There is no current data to support specific thresholds for mean arterial pressure (MAP) or hematocrit during bypass that is predictive of preventing poor neurodevelopmental outcomes across large populations1,2,4<\/sup>. \r\n\r\nThe correct answer is A, perioperative seizures increase the likelihood of poor neurodevelopmental outcomes based on the most recent AHA guidelines1<\/sup>. Although lower hematocrit and hypotension may play a role, there is no definitive data to recommend specific surgical, hemodynamic, or transfusion practices in the congenital cardiac patient population to prevent neurodevelopmental impairment. \r\n\r\n \r\nREFERNCES \r\n1.\tSood E, Newburger JW, Anixt JS, et al. Neurodevelopmental Outcomes for Individuals With Congenital Heart Disease: Updates in Neuroprotection, Risk-Stratification, Evaluation, and Management: A Scientific Statement From the American Heart Association. Circulation<\/em>. 2024;149(13):e997-e1022. doi:10.1161\/CIR.0000000000001211 \r\n2.\tWernovsky G, Licht DJ. Neurodevelopmental Outcomes in Children With Congenital Heart Disease-What Can We Impact?. Pediatr Crit Care Med<\/em>. 2016;17(8 Suppl 1):S232-S242. doi:10.1097\/PCC.0000000000000800 \r\n3.\tLim JM, Porayette P, Marini D, et al. Associations Between Age at Arterial Switch Operation, Brain Growth, and Development in Infants With Transposition of the Great Arteries. Circulation<\/em>. 2019;139(24):2728-2738. doi:10.1161\/CIRCULATIONAHA.118.037495 \r\n4.\tGaynor JW, Stopp C, Wypij D, et al. Neurodevelopmental outcomes after cardiac surgery in infancy. Pediatrics<\/em>. 2015;135(5):816-825. doi:10.1542\/peds.2014-3825\r\n\r\n”,”hint”:””,”answers”:{“71s2z”:{“id”:”71s2z”,”image”:””,”imageId”:””,”title”:”A.\tPerioperative seizures”,”isCorrect”:”1″},”2w3gg”:{“id”:”2w3gg”,”image”:””,”imageId”:””,”title”:”B.\tMean arterial pressure (MAP) < 50mmHg on cardiopulmonary bypass (CPB)"},"trorh":{"id":"trorh","image":"","imageId":"","title":"C.\tHematocrit < 35% during cardiopulmonary bypass (CPB)\r\n\r\n"}}}}}}