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

{“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″}}}}}}

{“questions”:{“davsp”:{“id”:”davsp”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Manal Mirreh, MD AND Asif Padiyath, MD \u2013 Children\u2019s Hospital of Philadelphia, Philadelphia, PA \r\n\r\nA 13-year-old female with Marfan syndrome presents for cardiac MRI. Which of the following class of medications is MOST LIKELY associated with increased risk for aortic dissection and aortic aneurysm in this patient? “,”desc”:”EXPLANATION \r\nAortic dissection is rare in the pediatric population. Prompt diagnosis is crucial due to its high mortality rate of 1% to 2% per hour in the first 48 hours. The risk factors most associated with this condition in the young are inherited disorders of the connective tissue, congenital cardiac disease, severe trauma and chronic hypertension.^{1<\/sup> \r\n\r\nIn addition to these risk factors, various medications and recreational drugs are linked to an increased risk of aortic aneurysm and dissection. One notable medication class that increases risk of aortic dissection are fluoroquinolones. Fluoroquinolones are a class of antibiotics that include ciprofloxacin, levofloxacin, and moxifloxacin, which are prescribed to treat a broad range of infections. Common side effects are nausea, vomiting, peripheral neuropathy, dysglycemia, and arrhythmias, with more serious collagen-disruption-associated complications including tendon rupture and retinal detachment. \r\n \r\nThe association of increased aortic dissection has been highlighted in various studies and safety communications from health authorities. For example, the FDA issued a warning in December 2018 about the potential risks of aortic aneurysm and dissection with fluoroquinolone use.2<\/sup>\r\n\r\nFluoroquinolones may increase the risk of aortic dissection by damaging the collagen and connective tissue in the aortic wall. Fluoroquinolones can damage these components by stimulating matrix metalloproteinases (MMPs), which are gelatinases that have collagenolytic activity. \r\n\r\nBeta-blockers, on the other hand, are protective against aortic dissection. They are routinely used in Marfan patients because they reduce the rate of aortic root dilation by decreasing heart rate and aortic wall stress.3<\/sup> \r\n\r\nLoop diuretics are not linked to an increased risk of aortic aneurysm or dissection. They are used for intravascular volume management in patients with hypertension or heart failure but do not play a direct role in aortic pathology in Marfan syndrome. \r\n\r\nGiven the concern for aortic aneurysm and aortic dissection, some institutions prefer not to use fluoroquinolones in high-risk patients unless no other treatment options are available. \r\n\r\n \r\nREFERENCES \r\n1.\tZalzstein, Eli & Hamilton, Robert & Zucker, Nili & Diamant, Samuel & Webb, Gary. (2003). Aortic dissection in children and young adults: Diagnosis, patients at risk, and outcomes. Cardiology in the young<\/em>. 13. 341-4. 10.1017\/S1047951103000696. \r\n2.\tFDA In Brief: FDA warns that fluoroquinolone antibiotics can cause aortic aneurysm in certain patients. 2018. Available at: https:\/\/www.fda.gov\/news-events\/fda-brief\/fda-brief-fda-warns-fluoroquinolone-antibiotics-can-cause-aortic-aneurysm-certain-patients. \r\n3.\tZhang, Jiawei MDa; Zhang, Zhe MBb,\u2217. Fluoroquinolones increase the risk of aortic aneurysm and dissection: A protocol for meta-analysis.Medicine<\/em> 100(51):p e28081, December 23, 2021. | DOI: 10.1097\/MD.0000000000028081 \r\n”,”hint”:””,”answers”:{“ipl5a”:{“id”:”ipl5a”,”image”:””,”imageId”:””,”title”:”A.\tFluoroquinolones “,”isCorrect”:”1″},”9zr39″:{“id”:”9zr39″,”image”:””,”imageId”:””,”title”:”B.\tBeta-blockers “},”dss08”:{“id”:”dss08″,”image”:””,”imageId”:””,”title”:”C.\tLoop Diuretics “}}}}}}

{“questions”:{“ocrjy”:{“id”:”ocrjy”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Manal Mirreh, MD \u2013 Children’s Hospital of Philadelphia, Philadelphia, PA \r\n\r\nA 2-month-old full-term boy with polysyndactyly\/syndactyly\/nail hypoplasia of the left foot, aplasia cutis, and cutis marmorata telangiectasia congenita presents to cardiology clinic for routine follow-up of a patent ductus arteriosus (PDA). His initial echocardiogram on day of life two showed bidirectional flow in his PDA, with normal RV pressure. His new echocardiogram now indicates suprasystemic right ventricular pressure, RV dysfunction and possible multivessel pulmonary vein disease. Given the physical exam and echocardiogram findings, which of the following genetic syndromes is the MOST likely present in this patient? “,”desc”:”EXPLANATION \r\nIf a patient presents with skin (scalp) and limb defects plus signs of pulmonary hypertension, Adams-Oliver syndrome (AOS) should be considered. \r\n \r\nAdams-Oliver Syndrome is a rare genetic disorder characterized by a distinct combination of congenital anomalies. The hallmark features include aplasia cutis congenita<\/strong>, most often presenting as areas of missing skin on the scalp, and transverse limb defects<\/strong>, such as missing digits, hypoplastic nails, or syndactyly. Many patients also exhibit cutaneous and vascular abnormalities<\/strong>, including cutis marmorata telangiectatica congenita (CMTC). CMTC is a rare congenital disorder characterized by discolored patches of skin caused by widened (dilated) surface blood vessels. As a result, the skin has a purple or blue \u201cmarbled\u201d or \u201cfishnet\u201d appearance (cutis marmorata). Importantly, cardiovascular involvement<\/strong> is common and may include congenital heart defects as well as pulmonary hypertension<\/strong>, which is thought to arise from underlying vascular dysplasia.^{1<\/sup> The syndrome can follow an autosomal dominant or recessive inheritance pattern, with mutations in genes such as ARHGAP31, DOCK6,<\/em> and NOTCH1<\/em> identified in affected individuals.2<\/sup> \r\n \r\nCutis marmorata is a common benign reticulate marbling of the skin described in neonates. It represents a physiologic phenomenon, with exacerbation on exposure to cold and resolution with re-warming.\u202f \r\n\r\nIt remains somewhat unclear as to whether the prognosis of AOS without lethal anomalies alters the lifespan. However, it is very likely that limited forms of the disease with only skin and skeletal abnormalities are likely to be associated with a better prognosis and no reduction in lifespan as compared to those patients with systemic involvement.3<\/sup> \r\n \r\nHolt-Oram syndrome is caused by mutations in the TBX5<\/strong> gene and follows an autosomal dominant inheritance pattern. It is characterized primarily by abnormalities of the upper limbs<\/strong>, which can range from missing or malformed thumbs to more severe reductions of the long bones, such as phocomelia. These limb anomalies are usually bilateral but often asymmetric. A hallmark of Holt-Oram syndrome is its strong association with cardiac defects<\/strong>, especially atrial and ventricular septal defects, as well as cardiac conduction abnormalities<\/strong> ranging from first-degree heart block to more serious rhythm disturbances. Notably, patients with Holt-Oram syndrome do not exhibit scalp defects or intellectual disability. \r\n\r\nAlagille syndrome is an autosomal dominant disorder consisting of bile duct paucity, cholestasis, \u201ctriangular\u201d facies, widespread vascular anomalies, and congenital heart disease. The congenital heart disease commonly presents as peripheral pulmonary arterial stenosis or hypoplasia or pulmonary valve stenosis.\r\n\r\n \r\nREFERENCES \r\n1.\tZapata, H.H., Sletten, L.J. and Pierpont, M.E.M. (1995), Congenital cardiac malformations in Adams-Oliver syndrome. Clinical Genetics<\/em>, 47: 80-84.\u202fhttps:\/\/doi.org\/10.1111\/j.1399-0004.1995.tb03928.x \r\n2.\tMedlinePlus Genetics. Adams-Oliver Syndrome<\/em>. National Library of Medicine. Updated February 28, 2024. Accessed May 10, 2025. https:\/\/medlineplus.gov\/genetics\/condition\/adams-oliver-syndrome\/ \r\n3.\tSeo JK, Kang JH, Lee D, Sung HS, Hwang SW. A case of Adams-Oliver syndrome. Ann Derm<\/em> 2010; 22:96-8. \r\n”,”hint”:””,”answers”:{“cpm3c”:{“id”:”cpm3c”,”image”:””,”imageId”:””,”title”:”A. Alagille Syndrome “},”wcvcl”:{“id”:”wcvcl”,”image”:””,”imageId”:””,”title”:”B. Adams-Oliver Syndrome “,”isCorrect”:”1″},”zcr6i”:{“id”:”zcr6i”,”image”:””,”imageId”:””,”title”:”C. Holt-Oram Syndrome “}}}}}}

{“questions”:{“ixq5l”:{“id”:”ixq5l”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2025\/06\/CCAS-QOW-Pic-6-4-2025.jpg”,”imageId”:”8741″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Kaitlin M. Flannery, MD, MPH – Stanford University \r\nA 2-day-old, 3.1kg, term neonate has required non-invasive positive pressure ventilation and oxygen administration since delivery due to increased work of breathing and low oxygen saturation. A transthoracic echocardiogram was performed and subsequently a CT scan of the chest with contrast. An image from the CT is displayed. What is the diagnosis? “,”desc”:”EXPLANATION \r\nVascular rings are congenital malformations that result from abnormal embryologic development and regression of the ventral and dorsal aortae and the six paired pharyngeal arches. The malformation results in an encircling and possible compression of the trachea and esophagus. Some present with severe symptoms at birth while others are incidentally found.^{1<\/sup> \r\n\r\nThe Society of Thoracic Surgeons (STS) utilizes the Backer and Mavroudis classifications system. This system divides vascular rings into four categories. These categories account for about 95% of pathology seen. The categories with incidence are:2<\/sup>\r\n\r\n1.\tDouble Aortic Arch (0.53%) \u2013 presence of both left and right-sided aortic arches encircling the trachea and esophagus \r\n2.\tRight aortic arch with left ligamentum (0.05%) \u2013 presence of aberrant right-sided aortic arch with left ligamentum arteriosum attached to left subclavian artery or descending aorta, encircling the trachea and esophagus \r\n3.\tInnominate artery compression syndrome (0.004%) \u2013 not a true vascular ring; occurs mostly when innominate artery origin is more distal or posterior, thus passing across the front of the trachea \r\n4.\tPulmonary artery sling (0.006%) \u2013 not a true vascular ring; aberrant origin of one pulmonary artery arising from the other; passes between the trachea and esophagus at a level close to the carina4<\/sup> \r\n\r\nThe CT shows the left pulmonary artery arising from the right pulmonary artery and coursing between the trachea and esophagus thereby creating a pulmonary artery sling (PAS). Patients with PAS typically present after a few months of life with significant respiratory symptoms as the disease is associated with tracheal stenosis from complete tracheal rings.1<\/sup> The severity of symptoms and management depend on the degree and length of tracheobronchial narrowing. The largest case series on surgical management of PAS was published in 2012.3<\/sup> The series reports the outcomes of 34 patients repaired on CPB from 1985-2012. The surgical approach to tracheal repair changed over the years with slide tracheoplasty utilized since 2002. Slide tracheoplasty is mostly used in cases of long-segment stenosis, and consists in transection and oblique division of the proximal and distal trachea, with subsequent anastomosis of each end, thereby increasing lumen size. Short segment stenosis may be managed with excision of diseased segment and end-to-end anastomosis. The aberrant pulmonary artery is often reimplanted during the same surgery. In this series, 79% of patients had tracheal stenosis from complete tracheal rings. There were no early deaths in this series. The mean percent of LPA blood flow was 41% with only one patient requiring reintervention for stenosis. There were four late deaths in this series, two were unrelated PAS, and two were due to complications from older surgical techniques for complete tracheal rings that resulted in dehiscence during follow-up bronchoscopy.3<\/sup>\r\n\r\n\r\n \r\nREFERENCES \r\n1.\tRagalie WS, Mitchell ME. Chapter 44: Vascular rings and pulmonary artery slings. In: Ungerleider RM, Meliones JN, McMillan KN, Cooper DS, Jacobs JP, eds. Critical Heart Disease in Infants and Children. 3rd ed<\/em>. Elsevier; 2019: 544-50. \r\n2.\tWadle M, Joffe D, Backer C, Ross F. Perioperative and anesthetic considerations in vascular rings and slings. Semin Cardiothorac Vasc Anesth<\/em>. 2024 Sep;28(3):152-64. \r\n3.\tBacker CL, Russel HM, Kaushal S, et al<\/em>. Pulmonary artery sling: current results with cardiopulmonary bypass. J Thorac Cardiovasc Surg<\/em>. 2012 Jan;143(1):144-51. \r\n4.\t McKenzie I, Zestos MM, Stayer SA, Kaminski E, Davies P, Andropoulos DB. Anesthesia for miscellaneous cardiac lesions. In: Andropoulos DB, Mossad EB, Gottlieb EA. Eds. Anesthesia for Congenital Heart Disease. 4th Ed<\/em>. Wiley Blackwell; 2023:781-831. \r\n”,”hint”:””,”answers”:{“lbw7e”:{“id”:”lbw7e”,”image”:””,”imageId”:””,”title”:”A.\tDouble aortic arch”},”70r1i”:{“id”:”70r1i”,”image”:””,”imageId”:””,”title”:”B.\tInnominate artery compression”},”6afqo”:{“id”:”6afqo”,”image”:””,”imageId”:””,”title”:”C.\tPulmonary artery sling “,”isCorrect”:”1″}}}}}}