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

{“questions”:{“zyhh8”:{“id”:”zyhh8″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Meera Gangadharan MBBS, FAAP, FASA, University of Texas Health Science Center at Houston\/Children\u2019s Memorial Hermann Hospital AND Destiny F. Chau MD, University of Arkansas for Medical Sciences\/Arkansas Children\u2019s Hospital, Little Rock, AR \r\n\r\nWhich of the following coronary artery arrangements is MOST commonly associated with D-transposition of the great arteries?\r\n\r\n\r\n\r\n\r\nCreative Commons Licensing from Gittenberger-de Groot et al. J Thorac Cardiovasc Surg<\/em>. 2018;156(6):2260-2269″,”desc”:”EXPLANATION \r\nDextro transposition of the great arteries (D-TGA) is characterized by ventriculoarterial discordance and atrioventricular concordance. D-TGA comprises approximately 5% of all congenital heart lesions and is one of the most common cyanotic congenital heart diseases to present in the newborn period with an incidence of roughly 3 per 10,000 live births. The aorta arises from the morphologic right ventricle and the pulmonary artery from the morphologic left ventricle. Patients usually have levocardia, situs solitus, and D-looped ventricles. The relative position of the aortic valve to the pulmonary valve may vary. Still, the most common arrangement is the aortic valve orifice to the right and anterior to the pulmonary valve orifice. The ventricular septum may be intact or there may be a ventricular septal defect. Left ventricular outflow tract obstruction may also be present. \r\n\r\n\r\nCoronary artery anomalies are another hallmark of transposition of the great arteries and efforts have been made to describe the coronary artery configurations in a standardized manner. Due to variations in the relative positions of the aortic and pulmonary valves to each other, the aortic valve orifice may be side-by-side, right and anterior to, or directly anterior to the pulmonary valve. The origin and subsequent course of the coronary arteries result in several different patterns. The two most common systems for describing the coronary artery configuration in D-TGA are the Yacoub classification described in 1978 and the more common Leiden Convention. The Leiden Convention was originally published in the early 1980s and was modified in 2018. It places the observer in the non-coronary cusp facing the pulmonary artery, with the right hand labeled \u201csinus 1\u201d and the left hand labeled \u201csinus 2\u201d. The classification then describes from which sinus each of the three major coronary arteries arise (Fig.1). \r\n\r\n\r\n \r\n \r\nFigure 1: Illustration of Leiden nomenclature determination. [Creative Commons Licensing from Gittenberger-de Groot et al. J Thorac Cardiovasc Surg<\/em>. 2018;156(6):2260-2269.] \r\n\r\n\r\nThe most common surgery performed for D-TGA in the current era is the Jatene arterial switch operation (ASO). This involves transecting the pulmonary artery and aorta, a few millimeters distal to their valves and anastomosing the pulmonary artery segment to the aortic root and the aorta to the pulmonary root. The operation also requires that the coronaries be translocated to the neo-aortic (pulmonary) root. From a surgical perspective, coronary artery patterns can be grouped into three main categories: (1) the \u201cusual\u201d coronary pattern (70% of cases), as depicted in answer option (A), where the left and right coronaries originate from separate sinuses with the left coronary dividing into left anterior descending and circumflex branches. Coronary transfer is usually straightforward and associated with excellent outcomes; (2) coronary arteries with an anterior or posterior loop (25% of cases), in which the coronary transfer may be difficult; and (3) coronary artery pattern with a single origin or where the coronaries course between the major arterial trunks, which may be associated with an intramural segment. Coronary transfer is usually difficult and represents a high risk for morbidity and mortality. Hence a detailed description of coronary artery anatomy before surgery is critical to avoid kinking, stretching, or placing tension on the coronary arteries when they are translocated. \r\n\r\n\r\nThe most common (\u201cusual\u201d) coronary artery pattern present in approximately 70% of cases of D-TGA is shown in answer option A. The other two patterns are much less commonly associated with D-TGA. \r\n\r\n\r\n\r\n \r\nREFERENCES \r\n\r\nGittenberger-de Groot AC, Koenraadt WMC, Bartelings MM, et al. Coding of coronary arterial origin and branching in congenital heart disease: The modified Leiden Convention. J Thorac Cardiovasc Surg<\/em>. 2018;156(6):2260-2269. doi:10.1016\/j.jtcvs.2018.08.009\r\n\r\n\r\nYacoub MH, Radley-Smith R. Anatomy of the coronary arteries in transposition of the great arteries and methods for their transfer in anatomical correction. Thorax<\/em>. 1978;33(4):418-424. doi:10.1136\/thx.33.4.418\r\n\r\n\r\nQureshi AM, Justino H, Heinle JS. Transposition of the Great Arteries. In: Shaddy R, Penny D, Feltes T, Cetta F, Mital S. Moss & Adams heart disease in infants, children, and adolescents: Including the fetus and young adult<\/em>. 10th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2022: 1122-1142. \r\n\r\n\r\nAhlstr\u00f6m L, Odermarsky M, Malm T, Johansson Ramgren J, Liuba P. Preoperative coronary anatomy assessment with echocardiography and morbidity after arterial switch operation of transposition of the great arteries. Pediatr Cardiol<\/em>. 2018;39(8):1620-1626. doi:10.1007\/s00246-018-1939-z\r\n\r\n”,”hint”:””,”answers”:{“m0krq”:{“id”:”m0krq”,”image”:””,”imageId”:””,”title”:”A. Image A Above “,”isCorrect”:”1″},”kptwb”:{“id”:”kptwb”,”image”:””,”imageId”:””,”title”:”B. Image B Above”},”zhdhm”:{“id”:”zhdhm”,”image”:””,”imageId”:””,”title”:”C. Image C Above “}}}}}

{“questions”:{“6kwwk”:{“id”:”6kwwk”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Cori Banerdt, MD – Vanderbilt Children\u2019s Hospital\/Vanderbilt University Medical Center, Nashville, TN AND\r\nDestiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 17-year-old patient with dilated cardiomyopathy is Status 1A on the heart transplantation list. The isohemagglutinin titer, drawn two days prior, is 1:32. According to the Organ Procurement and Transplantation Network (OPTN) policy changes in 2023, which of the following listing criteria MOST likely disqualifies this patient for ABO-incompatible heart transplantation?\r\n”,”desc”:”EXPLANATION \r\nPediatric patients with refractory heart failure placed on the cardiac transplantation waiting list have significant mortality due to long wait times related to the scarcity of donor organs. Furthermore, a proportion of patients with certain ABO blood types have even longer waitlist times. Specifically, recipients with type O blood have the longest waitlist time and can only receive O-type donor organs because of the presence of both anti-A and anti-B antibodies (isohemagglutinins). The immaturity of the immune system in infants prompted the first incompatible blood type (ABOi) heart transplantations in the 1990s. \r\n\r\nCurrently, ABOi heart transplants in young children are routinely performed. Reports in the literature indicate that the long-term outcomes of both compatibility groups are similar. In July 2016, the Organ Procurement and Transplantation Network (OPTN) expanded the candidacy for ABOi heart transplantation from patients younger than one year of age to include patients one to two years of age, as well as increasing the isohemagglutinin titers from 1:4 to 1:16 in this age group. After this policy change, a study using the Scientific Registry of Transplant Recipients database demonstrated that the percentage of ABOi transplantations increased by 2.7-fold, and the waiting times decreased by 68% for children listed for ABOi transplants compared to those listed for ABO compatible (ABOc) transplants. The survival rates were similar in children who received an ABOi versus ABOc heart transplant.\r\n\r\nIn March 2023, the OPTN Executive Committee approved further policy changes, which allowed transplant programs to indicate they were willing to accept an ABOi donor heart and\/or donor heart-lung for status 1A and 1B candidates placed on the waiting list before their 18th birthday. Transplant programs must report isohemagglutinin titers equal to or less than 1:16 to the OPTN every 30 days on behalf of such candidates. Previously, candidates had to be registered before their 2nd birthday. \r\n \r\nPediatric status assignments include status 1A, 1B, 2, and inactive. To be listed status 1A, a pediatric candidate must be less than 18 years of age at the time of registration and require therapy with one or more of the following: 1) continuous mechanical ventilation; 2) an intra-aortic balloon pump; 3) a stent or prostaglandin infusion to maintain ductal-dependent circulation; 4) multiple inotropic infusions or a single inotrope at high dose to treat hemodynamically unstable heart failure; or 5) a mechanical circulatory support device. Pediatric status 1A must be recertified every 14 days. \r\n\r\nThe correct answer is B. The patient described in the stem is 17 years old, status 1A, and has a recent isohemagglutinin titer of 1:32. Although the other criteria set by the most current OPTN policy are met, the isohemagglutinin titer is greater than 1:16. Therefore, this patient is a candidate for an ABOc, but not ABOi heart transplantation. \r\n\r\n \r\nREFERENCES \r\nBansal N, West LJ, Simmonds J, Urschel S. ABO-incompatible heart transplantation-evolution of a revolution. J Heart Lung Transplant<\/em>. 2024. 43(9):1514-1520. \r\n\r\nMilligan C, Daly KP. ABO-Incompatible heart transplantation: where science, society, and policy collide. J Card Fail<\/em>. 2024;30(3):486-487. \r\n\r\nAmdani S, Deshpande SR, Liu W, Urschel S. Impact of the pediatric ABO policy change on listings, transplants, and outcomes for children younger than 2 years listed for heart transplantation in the United States. J Card Fail<\/em>. 2024;30(3):476-485. \r\n\r\nOrgan Procurement and Transplantation Network. Notice of OPTN Policy Changes. Modify heart policy for intended incompatible blood type (ABOi) offers to pediatric candidates. Updated June 26, 2023. Accessed July 27 2024.\r\nhttps:\/\/optn.transplant.hrsa.gov\/media\/05vnqa0k\/optn_heart_aboi-offers_pn_june-2023.pdf\r\n\r\nOrgan Procurement and Transplantation Network Policies. Updated July 25, 2024. Accessed July 30, 2024. https:\/\/optn.transplant.hrsa.gov\/media\/eavh5bf3\/optn_policies.pdf\r\n\r\n”,”hint”:””,”answers”:{“wceai”:{“id”:”wceai”,”image”:””,”imageId”:””,”title”:”A.\tAge”},”zz0ks”:{“id”:”zz0ks”,”image”:””,”imageId”:””,”title”:”B.\tIsohemagglutinin titer “,”isCorrect”:”1″},”iiq2x”:{“id”:”iiq2x”,”image”:””,”imageId”:””,”title”:”C.\tListing status”}}}}}

{“questions”:{“elmq8”:{“id”:”elmq8″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Vera Winograd-Gomez, MD \u2013 Cincinnati Children\u2019s Hospital and Medical Center, Cincinnati, OH AND\r\nDestiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 2-month-old infant with cor triatriatum sinister is to undergo surgical repair. Which of the following hemodynamic goals is MOST appropriate during the pre-bypass period? \r\n”,”desc”:”EXPLANATION \r\nCor triatriatum (\u201ctriatrial heart\u201d), a rare cardiac anomaly found in approximately 1 in 1000 patients with congenital heart disease, describes the anatomical finding of a membrane dividing the left atrium (sinister) or right atrium (dexter) into two chambers. Cor triatriatum sinister results from an inadequate merging of the common pulmonary vein with the left atrium during heart development. In this malformation, a fibromuscular membrane with one or more orifices separates the atrium into an upper chamber, with the pulmonary veins, and a lower chamber with the left atrial appendage and the mitral valve. The degree of obstruction to blood flow through the orifices in the membrane determines the severity of the patient\u2019s symptoms. Significant obstruction may result in severe left atrial hypertension, low cardiac output, and pulmonary venous hypertension. The symptoms and pathophysiology are similar to severe mitral stenosis. Most patients develop symptoms during the first year of life, or earlier if severe obstruction is present. There are also reports of completely asymptomatic adult patients with multiperforated membranes. Cor triatriatum sinister is usually associated with other congenital cardiac malformations including hypoplastic left heart syndrome, left superior vena cava, and anomalous pulmonary venous drainage.\r\n\r\nAnesthetic considerations and goals are similar to mitral stenosis, which include the following: 1) preservation of contractility and normal sinus rhythm; 2) low to low-normal heart rate to enhance left ventricular filling time in the presence of inflow obstruction; 3) maintenance of adequate preload (central venous pressure[CVP]) to minimize the functional obstruction to left ventricular (LV) filling; and 4) adequate afterload for maintenance of systemic and coronary artery perfusion. Maneuvers to decrease pulmonary vascular resistance (PVR) before relieving pulmonary venous obstruction can result in the worsening of pulmonary venous hypertension. Conversely, after repair, pulmonary arterial hypertension may indicate a need for inhaled nitric oxide during the postoperative period. \r\nIn this case scenario, during the pre-bypass period, the hemodynamic goals are normal sinus rhythm, low to low-normal heart rate, maintenance of adequate preload (CVP) and afterload (mean arterial pressure (MAP)), and avoidance of low PVR. Decreased afterload can result in coronary artery hypoperfusion in the setting of a reduced MAP pressure in a patient with impaired left ventricular filling\/stroke volume. Excessive preload or high central venous pressure (CVP) can exacerbate left atrial hypertension and pulmonary edema, whereas low preload and CVP can further reduce stroke volume (LV end-diastolic volume) and cardiac output. Left atrial hypertension and pulmonary venous hypertension with secondary pulmonary arterial hypertension may require initiation of inhaled nitrous oxide post-operatively. \r\n\r\n\r\n \r\nREFERENCES \r\n\r\nSpaeth J, Loepke A. Anesthesia for left-sided obstructive lesions. In: Andropoulos DB, Mossad EB, Gottlieb EA, eds. Anesthesia for Congenital Heart Disease<\/em>. 4th ed. Hoboken, NJ; Wiley-Blackwell. 2023: 513-514.\r\n\r\nStout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA\/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology\/American Heart Association Task Force on Clinical Practice Guidelines [published correction appears in J Am Coll Cardiol. 2019 May 14;73(18):2361]. J Am Coll Cardiol. 2019;73(12):1494-1563. doi:10.1016\/j.jacc.2018.08.1028\r\n”,”hint”:””,”answers”:{“mlgrq”:{“id”:”mlgrq”,”image”:””,”imageId”:””,”title”:”A. Heart rate 100 to 140 bpm”,”isCorrect”:”1″},”1ih89″:{“id”:”1ih89″,”image”:””,”imageId”:””,”title”:”B. Mean arterial pressure 35 to 45 mmHg”},”zauyj”:{“id”:”zauyj”,”image”:””,”imageId”:””,”title”:”C. Central venous pressure 3-5 mmHg”}}}}}

{“questions”:{“74dah”:{“id”:”74dah”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: David Fitzgerald, MD and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 1-month-old boy who is born with multiple intracavitary left ventricular tumors presents for follow-up cardiac evaluation. A transthoracic echocardiogram demonstrates multiple masses of homogeneous appearance without inflow or outflow obstruction that are reduced in size from previous imaging. Delta waves are noted on the electrocardiogram. Which of the following cardiac tumors is the MOST likely diagnosis?”,”desc”:”EXPLANATION \r\nPrimary cardiac tumors in children are rare with a reported incidence of up to 0.4%, excluding inflammatory masses, thrombi, and vegetations. Although most congenital cardiac tumors are benign (90%), the degree of involvement with or invasion into surrounding cardiac structures can lead to detrimental physiologic consequences. \r\n\r\nThe most common type of cardiac tumor in children is rhabdomyoma, accounting for over 60% of cases. Morphologically, these tumors are well circumscribed, multiple, and often located in the ventricles, either in an intramural or intracavitary position. On echocardiographic images, rhabdomyomas appear echogenic and homogenous. As many of these tumors regress spontaneously, an initial conservative, observational approach to management is appropriate unless the tumors cause hemodynamically significant sequelae. Both atrial and ventricular arrhythmias can occur in 40% of cases. Specifically, the incidence of Wolff-Parkinson-White syndrome is ten-fold that of the general population. Rhabdomyomas are known to be associated with tuberous sclerosis, an autosomal dominant multi-systemic disease affecting the brain, kidney, liver, and lungs. The drugs sirolimus and everolimus have been shown to reduce tumor size in the heart and brain, thus offering a potential alternative to surgical resection.\r\n\r\n\r\nThe second most common type of cardiac tumor in children is fibroma, accounting for 10-25% of cases. Unlike rhabdomyomas, fibromas are solitary tumors that typically do not regress. They tend to grow slowly, typically reaching maximal size in late gestation and early infancy. They are usually located in the interventricular septum or on the ventricular free wall. Fibromas are echogenic, well circumscribed, and often have areas of calcification or cystic degeneration. Fibromas often grow into the conduction system and may cause ventricular tachycardia or sudden death. \r\n\r\nMyxomas are rare in neonates but account for approximately 10% of cardiac tumors in older children. They are the most common cardiac tumor in adults. They are most commonly located in the left atrium arising from the fossa ovalis. Morphologically they appear sessile or pedunculated and lobular with frond like projections. Myxomas do not recede but rather require resection. The morphological appearance, typical location in the left atrium and later presentation in life tend to make these lesions easily identifiable. Clinically significant, intermittent obstruction of left ventricular inflow across the mitral valve can occur, as can embolization. \r\n\r\nIn the stem, the patient has multiple tumors with a characteristic echocardiographic appearance and history of regression over time that is most consistent with a cardiac rhabdomyoma. Fibromas may have a cystic appearance, are solitary and do not regress. Myxomas are usually present in older children, are typically located in the left atrium, and do not recede.\r\n\r\n\r\n \r\nREFERENCES \r\nUzun O, Wilson DG, Vujanic GM, Parsons JM, De Giovanni JV. Cardiac tumours in children. Orphanet J Rare Dis<\/em>. 2007;2:11. \r\n\r\nMarx G, Moran A. Cardiac tumors. In: Shaddy R, Penny D, Feltes T, Cetta F, Mital S, eds. Moss & Adams\u2019 Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult<\/em>. 10th ed. Philadelphia: Lippincott Williams and Wilkins; 2022:1639-1644\r\n\r\nMcKenzie I, Markakis Zestos M, Stayer S, Kamiski E, Davies P, Andropoulos D. In: Andropoulos D, Mossad E, Gottlieb E, eds. Anesthesia for miscellaneous lesions. Anesthesia for Congenital Heart Disease<\/em>. 4th ed. New Jersey: John Wiley & Sons, Inc.; 2023: 816-820.\r\n\r\n\r\n”,”hint”:””,”answers”:{“f9oqu”:{“id”:”f9oqu”,”image”:””,”imageId”:””,”title”:”A.\tFibroma”},”ldlpv”:{“id”:”ldlpv”,”image”:””,”imageId”:””,”title”:”B.\tRhabdomyoma”,”isCorrect”:”1″},”urfik”:{“id”:”urfik”,”image”:””,”imageId”:””,”title”:”C.\tMyxoma\r\n\r\n”}}}}}

{“questions”:{“di61c”:{“id”:”di61c”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Amy Babb, MD – Vanderbilt University and Kaitlin M. Flannery, MD, MPH – Stanford University \r\nA 16-year-old girl with a history of orthotopic heart transplantation is admitted with one week of nausea, vomiting, and fatigue. She is scheduled for an urgent endomyocardial biopsy due to suspected rejection. Past medical history includes stage 3 chronic kidney disease and type 2 diabetes, and current medications are aspirin, atorvastatin, carvedilol and empagliflozin. Which of the following laboratory values is MOST likely to be associated with euglycemic diabetic ketoacidosis associated with empagliflozin?\r\n”,”desc”:”EXPLANATION \r\nEmpagliflozin, dapagliflozin, canagliflozin, and ertugliflozin are sodium-glucose cotransporter-2 (SGLT2) inhibitors used for managing type 2 diabetes, chronic kidney disease, and heart failure. SGLT2 inhibitors work by blocking reabsorption of glucose and sodium in the proximal renal tubules leading to glucosuria, natriuresis, and diuresis. Adult studies and limited pediatric studies have shown SGLT2 inhibitors can decrease HbA1c, proteinuria, brain natriuretic peptide (BNP), and blood pressure, and modestly improve ejection fraction in heart failure. Adverse effects include urinary tract infection and fungal genital infections (due to glucosuria), headache, diarrhea, vomiting, and euglycemic diabetic ketoacidosis (eDKA). \r\n\r\nThe mechanism of SGLT2-associated eDKA is not completely understood. However, diagnostic criteria of eDKA are similar to classic DKA. The most notable difference is the absence of hyperglycemia in eDKA that is typically seen in classic DKA. DKA occurs during a relative or absolute state of insulin deficiency with increased counter-regulatory hormones, including glucagon, corticosteroids and catecholamines. The imbalance of hormones leads to ketone formation and anion-gap metabolic acidosis. In the presence of SGLT2 inhibitors, glucosuria and diuretic effect lowers the serum glucose and decreases the overall insulin requirement. During periods of stress, such as illness, nausea, vomiting, fasting and surgery, the low insulin\/high glucagon environment can lead to eDKA (see Figure 1 below). \r\n\r\n \r\n \r\n \r\nFigure 1. Proposed role of sodium-glucose cotransporter 2 (SGLT2) inhibition in euglycemic diabetic ketoacidosis (eDKA). Classic DKA results from insulin deficiency (absolute or relative) and concurrent increase in counter-regulatory hormones leading to ketosis, hyperglycemia, and osmotic diuresis. In contrast, SGLT2 inhibitor therapy in a well-compensated individual at baseline causes glucosuria, mild volume depletion, and lower serum glucose levels, associated with decreased insulin secretion. During times of intercurrent illness and\/or metabolic stress, such as surgery or gastrointestinal illness, decreased carbohydrate intake coupled with lower serum glucose levels can further depress insulin secretion. This can ultimately lead to eDKA (red box). \u2217Possible pathways of carbohydrate deficiency and causes of insulinopenia. Abbreviations: BP, blood pressure; PO, oral. (From: Wang KM and Isom RT. SGLT2 inhibitor-induced euglycemic diabetic ketoacidosis: A case report. Kidney Med. 2020;2:218-221. Used under Creative Commons License.) \r\n\r\nA diagnosis of eDKA requires the following: \r\n1.\tBlood glucose < 200 mg\/dL\r\n2.\tKetonemia (serum beta-hydroxybutyrate greater than 3 mmol\/L) \r\n3.\tAnion gap metabolic acidosis: \r\na.\tArterial pH < 7.3 \r\nb.\tSerum bicarbonate < 18 mEq\/L \r\nc.\tAnion gap > 10 mmol\/L \r\n\r\n\r\nTreatment of eDKA includes initiation of IV insulin infusion and dextrose solution, as well as IV fluid repletion and electrolyte management. The goal of therapy in eDKA is not to lower the serum glucose, but to replenish the insulin stores necessary to reduce ketone production. Monitoring of urine glucose can help determine residual SGLT2 inhibitor activity, and urine\/serum ketones can guide efficacy of treatment. \r\n\r\nDue to the frequency of reported perioperative eDKA events, the FDA updated its safety labeling in 2020 to recommend that empagliflozin, dapagliflozin, and canagliflozin be held for THREE days and ertugliflozin for FOUR days prior to elective procedures. In addition, a recent study from Massachusetts General Hospital has shown that anion gap metabolic acidosis develops in all patients when SGLT2 inhibitors were not held appropriately. \r\n\r\nWhen patients on SGLT2 inhibitors present for urgent\/emergent procedures, they must be monitored closely for the development of eDKA. Signs and symptoms of eDKA are non-specific and include nausea, vomiting, abdominal pain, fatigue, tachycardia, and tachypnea. Unfortunately, these are also signs of orthotopic heart transplant rejection and heart failure. \r\n\r\nDue to increased evidence in the literature, candidacy and indications for SGLT2 inhibitor use are being expanded to include children ages ten years and older, non-diabetic patients with heart failure and congenital heart disease patients. This trend will increase the likelihood of pediatric cardiac anesthesiologists and pediatric anesthesiologists managing patients treated with SGLT2 inhibitors in the perioperative setting.\r\n\r\nThe correct answer is B. EDKA is characterized by the finding of increased serum and urine ketones, such as serum beta-hydroxybutyrate 5, in the presence of an increased anion-gap metabolic acidosis and relatively normal serum glucose levels. \r\n\r\n \r\nREFERENCES \r\nGrube PM, Beckett RD. Clinical studies of dapagliflozin in pediatric patients: a rapid review. Ann Pediatr Endocrinol Metab<\/em>. 2022;27:265-72.\r\n\r\nSteinhorn B, Wiener-Kronish J. Dose-dependent relationship between SGLT2 inhibitor hold time and risk from postoperative anion gap acidosis: a single-centre retrospective analysis. Br J Anaesth<\/em>. 2023 Oct;131(4):682-6.\r\n\r\nChow E, Clement S, Garg R. Euglycemic diabetic ketoacidosis in the era of SGLT-2 inhibitors. BMJ Open Diab Res Care<\/em>. 2023;11:e003666.\r\n\r\nFDA revises labels of SGLT2 inhibitors for diabetes to include warnings about too much acid in the blood and serious urinary tract infections (fda.gov). 2022. Available at: https:\/\/www.fda.gov\/drugs\/drug-safety-and-availability\/fda-revises-labels-sglt2-inhibitors-diabetes-include-warnings-about-too-much-acid-blood-and-serious. Accessed 7\/17\/2023.\r\n\r\n”,”hint”:””,”answers”:{“qqk3f”:{“id”:”qqk3f”,”image”:””,”imageId”:””,”title”:”A.\tArterial pH 7.35″},”un9x4″:{“id”:”un9x4″,”image”:””,”imageId”:””,”title”:”B.\tSerum beta-hydroxybutyrate 5 mmol\/L “,”isCorrect”:”1″},”gxnjc”:{“id”:”gxnjc”,”image”:””,”imageId”:””,”title”:”C.\tBlood Glucose 350 mg\/dL”}}}}}