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

{“questions”:{“r7qk2”:{“id”:”r7qk2″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Amy Babb, MD, AND Amanpreet Kalsi, MBBS, FRCA – Vanderbilt University Medical Center -\r\nMonroe Carell Jr. Children’s Hospital at Vanderbilt \r\nA 16-year-old female with a history of truncus arteriosus presents for RV-PA conduit change via sternotomy and cardiopulmonary bypass. She recently underwent a cardiac catheterization procedure for attempted percutaneous pulmonary valve placement and had severe post operative nausea and vomiting despite ondansetron and dexamethasone. Aprepitant is chosen for PONV prophylaxis after this surgery. What class of medication does aprepitant belong to? “,”desc”:”EXPLANATION \r\nAprepitant is a neurokinin 1 receptor antagonist used to decrease the risk of nausea and vomiting in adults and children with chemotherapy-induced and post-operative nausea and vomiting (PONV). These drugs target receptors in both central and peripheral nervous systems. In the central nervous system, aprepitant crosses the blood brain barrier inihibiting g-protein coupled signals at the NK1 receptor. Inhibition at the NK1 receptors prevents substance P formation at multiple sites in the brain known to induce nausea and vomiting. In addition, NK1 receptors present in the peripheral nervous system and gut are inihibited by aprepitant leading to an overall deceased vomiting signal to the central nervous system. \r\n\r\nAprepitant can be given orally (tab or liquid) or intravenously. Fosaprepitant is a prodrug of aprepitant that is only available as an IV formulation. Dosing in pediatric patients is limited to the available prescribing information for chemotherapy-induced nausea and vomiting, with few studies describing pediatric patients receiving aprepitant for PONV. Consensus guidelines for PONV published in 2020 by Gan et al recommend a dose of aprepitant 40 mg PO given within 3 hours of anesthesia induction for adults and dosing of aprepitant 3 mg\/kg up to 125 mg for PONV prophylaxis in children^{1<\/sup>.\r\n\r\nA 2019 study evaluating noncardiac pediatric surgical patients from birth to 17 years demonstrated efficacy and safety for PONV with doses of 10 mg, 40 mg and 125 mg2<\/sup>. In 2023, Belk J, et al. described a single-center experience with preoperative aprepitant administration for pediatric cardiac surgical and catheterization patients with a history of severe PONV. This small subset of patients were older, with a mean age of 16 years, and received either 80 mg PO (if over 50 kg) and 40 mg PO (if less than 50 kg)3<\/sup>. \r\n\r\nDescribed risks of aprepitant include decreased INR in patients taking warfarin as well as reduced efficacy of hormonal contraceptives for up to 28 days after administration4<\/sup>. As a general precaution, aprepitant is a weak inhibitor and inducer of CYP3A44. NK1 receptor antagonists have no effect on the QT interval, providing a potential advantage to its use in pediatric cardiac patients with risk factors for PONV. Multimodal management of PONV after pediatric cardiac surgery is an important aspect to any enhanced recovery protocol and NK1 receptor inhibitors may prove to be a useful addition. \r\n\r\n\r\n \r\nREFERENCES \r\n1.\tGan TJ, Belani KG, Bergese S, et al. Fourth Consensus Guidelines for the Management of Postoperative Nausea and Vomiting Anesth Analg<\/em>. 2020;131(2):411-448. doi:10.1213\/ANE.0000000000004833 \r\n2.\tSalman FT, DiCristina C, Chain A, et al. Pharmacokinetics and pharmacodynamics of aprepitant for the prevention of postoperative nausea and vomiting in pediatric subjects. Journal of Pediatric Surgery<\/em>. 2019; 54(7): 1384-1390. https:\/\/doi.org\/10.1016\/j.jpedsurg.2018.09.006. \r\n3.\tBelk JW, Twite MD, Klockau KS, Silveira LJ and Clopton RG (2023) Effects of aprepitant on post-operative nausea and vomiting in patients with congenital heart disease undergoing cardiac surgery or catheterization procedures: a retrospective study with subjects as their own historical control. Front. Anesthesiol<\/em>. 2023; 2:1190383. doi: 10.3389\/fanes.2023.1190383 \r\n4.\tMerck & Co. Emend (Aprepitant). US Food and Drug Administration website. Issued July 2009. https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2007\/021549s012lbl.pdf\r\n”,”hint”:””,”answers”:{“qufqz”:{“id”:”qufqz”,”image”:””,”imageId”:””,”title”:”A.\tNeurokinin 1 receptor antagonist”,”isCorrect”:”1″},”fnlzu”:{“id”:”fnlzu”,”image”:””,”imageId”:””,”title”:”B.\t5-HT3 receptor antagonist”},”chtq9″:{“id”:”chtq9″,”image”:””,”imageId”:””,”title”:”C.\tNeurokinin 1 receptor agonist”}}}}}}

{“questions”:{“yfux1”:{“id”:”yfux1″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Manal Mirreh, MD, Jennifer M. Lynch, MD, PhD, Lea C. Matthews, MD \u2013 Children\u2019s Hospital of Philadelphia, Philadelphia, PA \r\n\r\nA 4-day-old, 3 kg neonate with HLHS is undergoing a Norwood procedure under hypothermic cardiopulmonary bypass. A few minutes after initiation of antegrade cerebral perfusion (ACP) through the innominate artery, a decrease in bilateral cerebral NIRS values is observed. Which of the following is the MOST LIKELY explanation for the observed drop?\r\n\r\n”,”desc”:”EXPLANATION \r\nNear-infrared spectroscopy (NIRS) offers continuous, non-invasive monitoring of regional tissue oxygen saturation (rSO_{2<\/sub>), reflecting the balance between oxygen delivery and consumption at the tissue level. The NIRS value represents the relative ratio of hemoglobin that remains oxygenated after traversing the local tissue bed. Trending NIRS values can help detect fluctuations in oxygen delivery and\/or utilization. \r\n\r\nNIRS is based on the modified Beer-Lambert Law, which describes how light travels through a scattering medium. NIRS emits near-infrared light through tissues and measures the amount of light at different wavelengths absorbed by oxygenated and deoxygenated hemoglobin. From this information, regional oxygen saturation (rSO2<\/sub>) can be calculated.^{1<\/sup>\r\n\r\nTissue oxygenation is related to oxygen delivery to the tissue (i.e., arterial oxygen content and blood flow) and oxygen consumption by the tissue. Thus, rSO2<\/sub> fluctuates as these parameters change. \r\n\r\nClinically used conventional continuous-wave near-infrared spectroscopy (NIRS) has several limitations that should be considered when interpreting its readings. First, its spatial coverage is limited, as it primarily monitors the superficial anterior brain regions where the sensors are placed\u2014typically the frontal cortex\u2014potentially missing injuries in deeper or posterior areas such as the parieto-occipital regions. Additionally, NIRS provides relative rather than absolute values, which makes clinical guidelines based on absolute rSO2<\/sub> values hard to establish. Furthermore, its accuracy as even a relative trend monitor can be limited depending on factors such as hemoglobin concentration and temperature changes2<\/sup>, which is a crucial point, particularly in this patient population, due to the widespread use of hypothermic cardiopulmonary bypass.3<\/sup>\r\n\r\nBelow is a suggested decision tree guiding management when decreasing cerebral rSO2<\/sub> on CPB is encountered:\r\n\r\n \r\n\r\nA Circle of Willis (cerebral circulation) abnormality (Answer A) would result in an asymmetric drop in rSO2<\/sub> during antegrade cerebral perfusion due to inadequate blood flow to the contralateral brain. It would not result in bilateral drops in cerebral rSO2<\/sub>, assuming adequate flow rate and unimpinged cerebral venous return.\r\n\r\nChanges in temperature do affect rSO2<\/sub> values as cerebral metabolic rate changes. However, periods of antegrade cerebral perfusion typically occur during deep hypothermia, which aims to reduce cerebral metabolic rate. Thus, an increase in cerebral demand due to hypothermia (Answer C) is incorrect.\r\n\r\nThere are many reasons that bilateral cerebral NIRS values may drop during the course of cardiopulmonary bypass, including low hematocrit, arterial cannula malposition, or obstruction to venous drainage. However, of the choices presented, only inadequate flow (Answer B) represents a possible explanation in this case.\r\n\r\n\r\n \r\nREFERENCES \r\n1. Owen-Reece H, Smith M, Elwell CE, Goldstone JC. Near infrared spectroscopy. Br J Anaesth<\/em>. 1999;82(3):418-426. doi:10.1093\/bja\/82.3.418 \r\n2. Kleiser S, Ostojic D, Andresen B, et al. Comparison of tissue oximeters on a liquid phantom with adjustable optical properties: an extension. Biomed Opt Express<\/em>. 2017;9(1):86-101. Published 2017 Dec 5. doi:10.1364\/BOE.9.000086 \r\n3. Lynch JM, Mavroudis CD, Ko TS, et al. Association of Ongoing Cerebral Oxygen Extraction During Deep Hypothermic Circulatory Arrest With Postoperative Brain Injury. Semin Thorac Cardiovasc Surg<\/em>. 2022;34(4):1275-1284. doi:10.1053\/j.semtcvs.2021.08.026\r\n”,”hint”:””,”answers”:{“6k6gr”:{“id”:”6k6gr”,”image”:””,”imageId”:””,”title”:”A.\tCircle of Willis abnormality\r\n”},”auc22″:{“id”:”auc22″,”image”:””,”imageId”:””,”title”:”B.\tInadequate flow during ACP”,”isCorrect”:”1″},”itw5n”:{“id”:”itw5n”,”image”:””,”imageId”:””,”title”:”C.\tIncreased cerebral metabolic demand “}}}}}}}

{“questions”:{“99moa”:{“id”:”99moa”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Manal Mirreh, MD, Lindsey Weidmann, MD, and Lea Matthews, MD \u2013 Children\u2019s Hospital of Philadelphia, Philadelphia, PA \r\n\r\nA 14-year-old female with a history of ANCA-associated vasculitis, end-stage renal disease requiring dialysis, and severe left ventricular dilation is undergoing left ventricular assist device (LVAD) implantation. Cardiopulmonary bypass (CPB) is initiated uneventfully, and the patient is maintained normothermic with no use of aortic cross-clamp or cardioplegia. During CPB, her serum potassium level is noted to rise to 6.5 mEq\/L. Given the risk of worsening hyperkalemia following transfusion of red blood cells, which of the following intraoperative ultrafiltration strategies is most appropriate to manage her electrolytes prior to separation from CPB?”,”desc”:”EXPLANATION \r\nHyperkalemia is defined as a serum potassium higher than the upper limit of normal, commonly considered to be 5.5 mEq\/L. Homeostatic mechanisms regulate potassium balance to maintain high intracellular levels required for cellular functions (eg, metabolism and growth) and low extracellular concentration to preserve the steep concentration gradient across the cell membrane needed for nerve excitation and muscle contraction.^{1<\/sup>\r\n\r\nPreoperative chronic kidney disease and the administration of potassium-rich cardioplegic solutions are common causes of hyperkalemia during CPB. Additional sources of exogenous potassium include the transfusion of older units of packed red blood cells or irradiated blood products, due to red cell membrane injury, which increases extracellular potassium.2<\/sup>\r\n\r\nCardiopulmonary bypass during pediatric cardiac surgery is linked to a robust inflammatory response, fluid overload, and end-organ dysfunction, all of which contribute to postoperative morbidity and mortality. To address these complications, a range of intraoperative ultrafiltration strategies\u2014including conventional ultrafiltration, modified ultrafiltration (MUF), zero-balance ultrafiltration (ZBUF), and hybrid approaches such as ZBUF-MUF\u2014have been employed over the years to reduce these adverse effects and enhance postoperative recovery. However, no clear consensus exists on which ultrafiltration technique provides the greatest benefit for infants and children undergoing open-heart surgery.\r\n\r\nUltrafiltration is the process of running blood through a device with a semipermeable membrane to remove \u201cfree water\u201d (water, electrolytes and substances with a molecular size smaller than the membrane pore size). It can be performed before, during, and\/or after CPB. The goals of ultrafiltration during cardiopulmonary bypass include the removal of excess crystalloid volume, hemoconcentration to increase hematocrit, and the clearance of electrolytes and inflammatory mediators.2<\/sup> \r\n\r\nUltrafiltration performed during cardiopulmonary bypass, known as conventional ultrafiltration (CUF), is primarily used to remove excess fluid that accumulates from various sources, including pre-bypass fluid administration, cardioplegia solutions, valve testing saline, and crystalloid added to the venous reservoir during periods of reduced venous return.3<\/sup>\r\n\r\nZero-balance ultrafiltration (ZBUF), also referred to as dilutional ultrafiltration (DUF), was initially introduced during the rewarming phase in pediatric patients. With ZBUF, ultrafiltrate is continuously removed and simultaneously replaced with an equivalent volume of crystalloid to maintain a net-zero fluid balance. The primary goal is to continuously clear electrolytes such as potassium and lactate, as well as inflammatory mediators, thereby producing a circulating volume with a composition closer to that of the replacement solution. Several crystalloids are utilized for ZBUF, including lactated ringers, Plasma-Lyte, and normal saline. In patients with chronic kidney disease, normal saline is preferred due to lack of potassium in the solution. However, use of normal saline is not ideal due to its high chloride content and may also exacerbate hyperkalemia due to potential for hyperchloremic acidosis. Case reports have described the use of Dialysate solution, in an attempt to avoid this occurrence, as it contains a lower potassium content than lactated ringers or Plasma-Lyte.4<\/sup>\r\n\r\nModified ultrafiltration (MUF) is performed after cardiopulmonary bypass to remove free water and concentrate the blood, resulting in increased hematocrit. It also allows recovery of whole blood from the bypass circuit, unlike cell salvage systems that return only red blood cells. While proposed benefits such as reduced edema, lower inotropic needs, and improved pulmonary function remain debated, MUF is widely accepted for its efficiency in raising hematocrit and reducing transfusion requirements.5<\/sup> In conclusion, among the available ultrafiltration techniques, zero-balance ultrafiltration (ZBUF) is the most appropriate choice in this setting, as it effectively reduces serum potassium levels and is particularly beneficial for managing hyperkalemia during cardiopulmonary bypass. \r\n\r\n \r\nREFERENCES \r\n1.\tKremen, J, Matoo TK, Somers, M. Hyperkalemia in children: Causes, clinical manifestations, diagnosis, and evaluation. In: UpToDate, Post TW (Ed). UpToDate. [Accessed May 10th, 2025]. Available from: https:\/\/www.uptodate.com \r\n2.\tOzdemir D, Chan R. The Challenges of Hyperkalemia on Cardiopulmonary Bypass. The Academy Newsletter. Summer 2012;(Summer):6\u20119 \r\n3.\tAndersen ND, Meza JM, Turek JW, Mavroudis C, Backer CL. Management of pediatric cardiopulmonary bypass. In: Mavroudis C, Backer CL, eds. Pediatric Cardiac Surgery<\/em>. 5th ed. John Wiley & Sons Ltd; 2023:161-189. doi:10.1002\/9781119282327.ch \r\n4.\tHeath M, Raghunathan K, Welsby I, Maxwell C. Using Zero Balance Ultrafiltration with Dialysate as a Replacement Fluid for Hyperkalemia during Cardiopulmonary Bypass. J Extra Corpor Technol<\/em>. 2014;46(3):262-266 \r\n5.\tMatte GS. The Bypass Plan. In: Perfusion for Congenital Heart Surgery: Notes on Cardiopulmonary Bypass for a Complex Patient Population<\/em>. Wiley Blackwell; 2015:55-59\r\n\r\n”,”hint”:””,”answers”:{“doo1j”:{“id”:”doo1j”,”image”:””,”imageId”:””,”title”:”A. Zero-balance ultrafiltration (ZBUF) “,”isCorrect”:”1″},”wbiwj”:{“id”:”wbiwj”,”image”:””,”imageId”:””,”title”:”B. Modified ultrafiltration (MUF) “},”ob7t8”:{“id”:”ob7t8″,”image”:””,”imageId”:””,”title”:”C. Conventional ultrafiltration (CUF)”}}}}}}

{“questions”:{“y5t9v”:{“id”:”y5t9v”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Morgan Ulloa, MD – Children\u2019s Hospital Los Angeles – Los Angeles, CA \r\n\r\nA 12 year old, non-diabetic, 45kg female patient with Hx of hypoplastic left heart syndrome s\/p palliation to Fontan underwent an uneventful cardiac catheterization. The next morning, she is found to be nauseated and confused. Physical examination demonstrates tachypnea with deep shallow breathing, tachycardia, and abdominal pain with palpation. Laboratory evaluation reveals a pH 7.3, serum glucose 105mg\/dl, and serum positive for ketones. Which of the following home medications continued in the perioperative period is MOST LIKELY<\/strong> associated with her current presentation?\r\n\r\n\r\n”,”desc”:”EXPLANATION \r\nEmpagliflozin is a member of the Sodium Glucose Cotransporter 2 (SGLT-2) inhibitors, colloquially referred to as \u201cflozins\u201d. The earliest US FDA approved medication of this class, dapagliflozin, has been in clinical use since 2012.^{1<\/sup> As their class name implies, these medications inhibit the sodium dependent glucose transport protein SGLT-2 which is in the first segment of the proximal convoluted tubule within the kidney, and is responsible for ~90% of glucose reabsorption filtered by the glomeruli. Inhibition of this protein leads to glucosuria and resultant lowering of the serum glucose concentration. In this role, these medications are primarily and predominantly utilized for the management of type 2 diabetes mellitus. \r\n\r\n\r\nSince 2022, empagliflozin has been FDA approved for the management of adults with heart failure with preserved ejection fraction (HFpEF) following the results of the EMPEROR-Preserved clinical trial.2<\/sup> This randomized, double blinded study compared the use of daily empagliflozin versus placebo on the primary outcome of composite cardiovascular death or hospitalization for heart failure in an adult population. This investigation demonstrated a statistically significant reduction of composite cardiovascular death or hospitalization for the empagliflozin group. This effect was primarily due to the lower risk of hospitalization and was notably independent of diabetes status.3<\/sup> Subsequent investigations have also demonstrated likely benefit to the patient population with heart failure with reduced ejection fraction. The mechanisms of this benefit are as of yet not entirely understood, but hypotheses include direct effects on cardiac myocyte metabolism, growth, and function that may diminish pathophysiologic remodeling and optimize energy substrate utilization, as well as protective effects on the endothelial glycocalyx from reduced damage associated with hyperglycemia, and its role as a diuretic normalizing preload for patients with congestive heart failure. 4<\/sup>\r\n\r\n\r\nAlthough empagliflozin received FDA approval in June of 2023 for the management of pediatric patients 10 years and older, it has not received specific FDA approval for pediatric patients with heart failure. Nonetheless, empagliflozin is being prescribed to pediatric patients with heart failure both with and without congenital heart disease and will likely become more commonly encountered in clinical settings for the pediatric cardiac anesthesiologist. The perioperative concerns surrounding \u201cFlozins\u201d center on the increased risk of euglycemic diabetic ketoacidosis (EDKA). This risk of developing EDKA exists regardless of duration of exposure. Since SGLT-2 inhibitors lead to diuresis and loss of glucose, a state of carbohydrate starvation, hypovolemia, and an imbalance of metabolic regulatory mechanisms may lead to dehydration and ketosis despite normoglycemia, which are more likely to occur in the perioperative setting. For this reason, several societies and regulatory agencies have recommended perioperative cessation of SGLT-2 inhibitors.5<\/sup> The current FDA recommendations are to hold SGLT-2 inhibitors for 3 days prior to scheduled procedures\/surgery or for 4 days in the case of ertugliflozin (Steglatro). All patients taking SGLT-2 inhibitors should be monitored with an increased index of suspicion for the development of EDKA, especially for patients unable to hold their dosages secondary to emergent\/urgent circumstances. \r\n\r\n\r\nThe diagnosis of EDKA should be suspected when signs\/symptoms akin to DKA present and further confirmed with laboratory evaluation demonstrating high anion-gap metabolic acidosis and ketosis in the setting of capillary blood glucose <250mg\/dL.5<\/sup> In the scenario presented, the most likely drug to induce EDKA is the SLGT-2 inhibitor. GLP-1 receptor agonists may also cause EDKA, but less commonly so. Sacubitril is a neprilysin inhibitor which is commercialized as a combination drug with valsartan to treat heart failure, and does not significantly impact glucose metabolism.\r\n\r\n\r\n\r\n \r\nREFERENCES \r\n\r\n1.\tGrube PM, Beckett RD. Clinical studies of dapagliflozin in pediatric patients: a rapid review. Ann Pediatr Endocrinol Metab<\/em>. 2022;27:265-72. \r\n2.\tAnker SD, Butler J, Filippatos G, et al. Empagliflozin in Heart Failure with a Preserved Ejection Fraction. N Engl J Med<\/em>. 2021;385(16):1451-1461. doi:10.1056\/NEJMoa2107038 \r\n3.\tTeo YH, Teo YN, Syn NL, et al. Effects of Sodium\/Glucose Cotransporter 2 (SGLT2) Inhibitors on Cardiovascular and Metabolic Outcomes in Patients Without Diabetes Mellitus: A Systematic Review and Meta-Analysis of Randomized-Controlled Trials. J Am Heart Assoc<\/em>. 2021;10(5):e019463. doi:10.1161\/JAHA.120.019463 \r\n4.\tHernandez VK, Parks Melville BT, Siwaju K. How Does It Work? Unraveling the Mysteries by Which Empagliflozin Helps Diabetic and Non-diabetic Patients With Heart Failure. Cureus<\/em>. 2023;15(9):e45290. Published 2023 Sep 15. doi:10.7759\/cureus.45290 \r\n5.\tHwang SM, Abcejo AS, Jacob AK, et al. Editorial: Euglycemic ketoacidosis concerns in perioperative use of SGLT2 inhibitors: re-examining current recommendations. APSF Newsletter<\/em>. 2025:13\u201315.\r\n\r\n”,”hint”:””,”answers”:{“ppbjl”:{“id”:”ppbjl”,”image”:””,”imageId”:””,”title”:”A.\tEmpagliflozin (SGLT-2 inhibitor) “,”isCorrect”:”1″},”ep57e”:{“id”:”ep57e”,”image”:””,”imageId”:””,”title”:”B.\tSemaglutide (GLP-1 receptor agonist) “},”ihins”:{“id”:”ihins”,”image”:””,”imageId”:””,”title”:”C.\tSacubitril \r\n\r\n”}}}}}}

{“questions”:{“p6d1n”:{“id”:”p6d1n”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Manal Mirreh, MD AND Divya Madhusudhan, MD – Children\u2019s Hospital of Philadelphia, Philadelphia, PA\r\n\r\nA 6-month-old, 5 kg girl with history of severe biventricular dysfunction secondary to perinatal myocardial infarction managed initially with VA ECMO and now supported with Berlin VAD is awaiting heart transplantation. Which of the following patient characteristics is associated with the BEST long-term survival after pediatric heart transplantation? \r\n\r\n”,”desc”:”EXPLANATION \r\nSignificant advancements in anesthetic management, immunosuppression and postoperative management have improved outcomes in pediatric heart transplant recipients, with 1 year survival rates now over 90%. \r\n\r\nAccording to data from the International Society of Heart and Lung Transplantation ISHLT), leading causes of early mortality<\/em> are graft failure, acute rejection, coronary artery vasculopathy and infection. \r\n\r\nThe Kaplan-Meier survival curves for patients of differing ages after transplant are presented below (Figure 1). The youngest recipients have the longest median survival to 24.5 years; however, infants are also at the highest risk of dying in the early period post-transplant.^{1<\/sup> \r\n\r\nFigure 1: Kaplan-Meier Survival Curve from ISHLT \r\n\r\nKaplan-Meier survival curve generated from the International Society of Heart and Lung Transplantation (J Heart Lung Transplant<\/em>. 2019;38(10):1028-1041. DOI: 10.1016\/j.healun.2019.08.002)\r\nThis work is openly licensed via CC BY 4.0.\r\n\r\nThere is an inverse relationship between age at transplant and survival.\r\n\r\nA study in Circulation<\/em> reported that the actuarial survival after infant heart transplantation was 84% at 1 month and 70% at 1 year with greatest hazard for death occurring within the first 3 months.2<\/sup> \r\n \r\nDiagnosis of cardiomyopathy (especially dilated cardiomyopathy) is associated with better outcomes than congenital heart disease, especially those with complex anatomy or multiple prior surgeries. \r\n\r\nPatients transplanted while on ECMO support had significantly decreased survival post-transplant compared with patients on VAD support or no mechanical support.3<\/sup> \r\n\r\n \r\nREFERENCES \r\n1.\tRossano JW, Singh TP, Cherikh WS, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-second pediatric heart transplantation report – 2019; Focus theme: Donor and recipient size match. J Heart Lung Transplant<\/em>. 2019;38(10):1028-1041. doi:10.1016\/j.healun.2019.08.002 \r\n2.\tCanter C, Naftel D, Caldwell R, et al. Survival and risk factors for death after cardiac transplantation in infants. A multi-institutional study. The Pediatric Heart Transplant Study. Circulation<\/em>. 1997;96(1):227-231. doi:10.1161\/01.cir.96.1.227 \r\n3.\tConway J, Cantor R, Koehl D, et al. Survival After Heart Transplant Listing for Infants on Mechanical Circulatory Support [published correction appears in J Am Heart Assoc<\/em>. 2020 Dec 15;9(24):e014641. doi: 10.1161\/JAHA.119.014641.]. \r\n”,”hint”:””,”answers”:{“cguh4”:{“id”:”cguh4″,”image”:””,”imageId”:””,”title”:”A.\tPrimary diagnosis of congenital heart disease “},”juigj”:{“id”:”juigj”,”image”:””,”imageId”:””,”title”:”B.\tPretransplant need for ECMO “},”pi5za”:{“id”:”pi5za”,”image”:””,”imageId”:””,”title”:”C.\tInfant age at time of transplant “,”isCorrect”:”1″}}}}}}