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

{“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″}}}}}}

{“questions”:{“11v76”:{“id”:”11v76″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Omar El Masri, MD, MA – Stanford University AND Kaitlin M. Flannery, MD, MPH – Stanford University \r\nA 5-day-old, 3.2kg, term neonate with severe aortic coarctation and hypoplastic transverse arch (Z-score = -4) is currently managed on a PGE_{1<\/sub> infusion with low lactate and good lower extremity pulses. According to the 2024 Society of Thoracic Surgeons (STS) Clinical Practice Guidelines on the management of neonates and infants with coarctation which is the MOST appropriate next step in management?\r\n\r\n”,”desc”:”EXPLANATION \r\nCoarctation of the aorta accounts for 4-5% of congenital heart disease (CHD). It is the second most common CHD requiring neonatal intervention after patent ductus arteriosus (PDA). In the current era, mortality following surgical repair is low at 0.98%. However, important short and long-term morbidity requiring repeat intervention persists, including re-coarctation and hypertension.^{1, 2<\/sup>\r\n\r\n\r\nDespite the frequency of coarctation, management plans remain heterogenous among institutions and surgeons. In 2024, the STS published practice guidelines for the management of neonates and infants with coarctation of the aorta. Experts in the field reviewed the available literature and using a modified Delphi method established guidelines with classification of the recommendations and level of evidence.1<\/sup> \r\n\r\n\r\nThe following are the practice guidelines for neonates and infants with isolated coarctation1<\/sup>:\r\n\r\n\r\n1.\tSurgical intervention is recommended in the absence of obvious contraindications. (Class I) \r\n2.\tIn patients with prematurity, low weight, or other risk factors for surgical intervention, medical optimization before intervention or primary operation is reasonable. (Class IIa) \r\n3.\tIn patients without associated arch hypoplasia, repair through a thoracotomy is indicated. (Class I) \r\n4.\tIn patients with associated arch hypoplasia that cannot be adequately addressed through a thoracotomy, repair through a sternotomy is preferable. (Class IIa) \r\n5.\tIn patients with bovine arch anatomy, repair through a sternotomy may be reasonable given the potential increased risk of re-coarctation with bovine arch anatomy repaired through a thoracotomy. (Class IIb) \r\n6.\tIn patients undergoing repair through a sternotomy, antegrade cerebral perfusions or limited duration deep hypothermic circulatory arrest may be reasonable. (Class IIb) \r\n7.\tIn patients undergoing repair through a sternotomy, extended end-to-end, arch advancement, and patch augmentation are all reasonable techniques. (Class IIa) \r\n\r\nThe patient presented above has no contraindications to surgical intervention and should undergo surgical repair before onset of complications. Given the patient\u2019s significant transverse arch hypoplasia with a Z-score of -4, a sternotomy with repair on CPB is preferable as the risk of re-coarctation would be high with thoracotomy and repair that did not address the hypoplastic transverse arch. \r\n\r\nThe decision to perform arch repair on CPB with antegrade cerebral perfusion versus coarctation repair via thoracotomy without CPB significantly changes the anesthetic management and postoperative course. However, the literature review to produce these clinical practice guidelines found no significant difference in mortality, global neurologic outcomes, recurrent laryngeal nerve injury, or incidence of chylothorax between the two approaches.1,3<\/sup> \r\n\r\n\r\n \r\nREFERENCES \r\n1.\tStephens EH, Feins EN, Karamlou T, et al<\/em>. The Society of Thoracic Surgeons Clinical Practice Guidelines on the Management of Neonates and Infants with Coarctation.Ann Thorac Surg<\/em>. 2024 Sep;118(3):527-44.\r\n2.\tSpaeth JP, Loepke AW. Chapter 22: Anesthesia for left-sided obstructive lesions. In: Andropoulos DB, Stayer S, Mossad EB, Miller-Hance WC, eds. Anesthesia for Congenital Heart Disease<\/em>. 3rd ed. Wiley Blackwell; 2015: 506-9. \r\n3.\tWaldman JC, Ing RJ, Stone ML. Current Practice Management Guidelines in Neonates and Infants with Isolated Coarctation of the aorta. J Cardiothorac Vasc Anesth<\/em>. 2025 Mar;39(3):573-5.\r\n\r\n”,”hint”:””,”answers”:{“2mnn1”:{“id”:”2mnn1″,”image”:””,”imageId”:””,”title”:”A.\tContinue medical management”},”okp13″:{“id”:”okp13″,”image”:””,”imageId”:””,”title”:”B.\tSurgical repair via sternotomy and cardiopulmonary bypass”,”isCorrect”:”1″},”7ipgr”:{“id”:”7ipgr”,”image”:””,”imageId”:””,”title”:”C.\tSurgical repair via thoracotomy”}}}}}}}