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

{“questions”:{“8aym9”:{“id”:”8aym9″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Michael A. Evans, MD; Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine \r\nAn 18-year-old male adolescent with a history Hypoplastic Left Heart Syndrome palliated with a fenestrated Fontan and who is anticoagulated with rivaroxaban presents to the emergency department with vomiting and altered consciousness. Computed tomography of the head reveals an intracranial hemorrhage. Which of the following agents is the BEST treatment to inhibit the anticoagulative effect of rivaroxaban?”,”desc”:”EXPLANATION \r\nRivaroxaban is a direct Factor Xa inhibitor that is FDA approved for anticoagulation in children over two years of age after the Fontan operation. The brand name of rivaroxaban is Xarelto\u00ae. It is currently the only direct oral anticoagulant (DOAC) that is FDA approved for this indication in the pediatric patient population. Routine blood tests to monitor anticoagulation and dietary restrictions are not required with the use of rivaroxaban, unlike warfarin anticoagulation. Other direct Factor Xa inhibitors include apixaban, enoxaparin, and edoxaban. \r\n\r\nAndexanet alfa (andexanet) is a reversal agent that neutralizes the anticoagulant effects of both direct and indirect factor Xa inhibitors. Andexanet is a catalytically inactive, recombinant modified human factor Xa protein that binds with high affinity to the active site of factor Xa (FXa) inhibitor thereby preventing FXa inhibitor from binding to Factor Xa and thus antagonizing its anticoagulant effect as assessed by measurement of thrombin generation and anti-factor Xa activity. Andexanet antagonizes the anticoagulant activity of apixaban, rivaroxaban, edoxaban, and enoxaparin. Andexanet was FDA approved in 2018, under its Accelerated Approval Program, for the reversal of life-threatening bleeding or uncontrolled bleeding in patients treated with apixaban or rivaroxaban. In the patient described in the question stem, andexanet administration would rapidly reverse systemic anticoagulation. \r\n\r\nThe side effect profile of andexanet alfa is most notable for venous and arterial thrombotic events. In fact, a 2020 meta-analysis of 16 prospective and retrospective studies enrolling patients treated with specific antidotes (idarucizumab and andexanet alfa) for anticoagulation reversal demonstrated a pooled incidence of 5.5% for thrombotic events. A systematic review of the studies on the safety and efficacy of nonvitamin K oral anticoagulants (NOACs) in adult patients with congenital heart disease (CHD) revealed a low annual incidence of thromboembolic events (0.98%) and major bleeding events (1.74%). Although the most common indication for anticoagulation in the adult patient population with congenital heart disease was atrial fibrillation in this study, the majority of both thromboembolic (3.13%) and hemorrhagic (3.17%) events occurred in patients with the Fontan palliation. \r\n\r\nIdarucizumab is a monoclonal antibody fragment used to reverse the effects of dabigatran, a direct thrombin inhibitor. It binds to dabigatran with 350 times greater affinity than thrombin. Idarucizumab would not be effective in reversing the anticoagulant effects of rivaroxaban in this patient. \r\n\r\nVitamin K is utilized for the reversal of coumadin anticoagulation. It would be expected to significantly lower the international normalized ratio (INR) in a 24 to 48 hour time period. It is not utilized as the sole reversal agent in the setting of a life-threatening hemorrhage due to coumadin anticoagulation but is administered concomitantly with fresh frozen plasma or prothrombin complex concentrates. \r\n\r\nPlatelet transfusion could be indicated in the setting of traumatic intracranial hemorrhage, especially to reverse the effects of antiplatelet medications or in the setting of thrombocytopenia. It would not be the first-line intervention in this patient treated with rivaroxaban. \r\n\r\nIn the setting of an intracranial hemorrhage in a patient treated with rivaroxaban, Andexanet alfa is the best initial treatment option. \r\nREFERENCES \r\nSiegal DM, Curnutte JT, Connolly SJ, et al. Andexanet Alfa for the Reversal of Factor Xa Inhibitor Activity. N Engl J Med. <\/em>2015;373(25):2413-2424. doi:10.1056\/NEJMoa1510991\r\n\r\nConnolly SJ, Crowther M, Eikelboom JW, et al. Full Study Report of Andexanet Alfa for Bleeding Associated with Factor Xa Inhibitors. N Engl J Med.<\/em> 2019;380(14):1326-1335. doi:10.1056\/NEJMoa1814051\r\n\r\nStalikas N, Doundoulakis I, Karagiannidis E, et al. Non-Vitamin K Oral Anticoagulants in Adults with Congenital Heart Disease: A Systematic Review. J Clin Med. <\/em>2020;9(6):1794. Published 2020 Jun 9. doi:10.3390\/jcm9061794\r\n\r\nPollack CV Jr, Reilly PA, Eikelboom J, et al. Idarucizumab for Dabigatran Reversal. N Engl J Med.<\/em> 2015;373(6):511-520. doi:10.1056\/NEJMoa1502000\r\n\r\nRodrigues AO, David C, Ferreira JJ, Pinto FJ, Costa J, Caldeira D. The incidence of thrombotic events with idarucizumab and andexanet alfa: A systematic review and meta-analysis. Thromb Res.<\/em> 2020;196:291-296. doi:10.1016\/j.thromres.2020.09.003\r\n”,”hint”:””,”answers”:{“ldtta”:{“id”:”ldtta”,”image”:””,”imageId”:””,”title”:”A.\tIdarucizumab”},”t7yzm”:{“id”:”t7yzm”,”image”:””,”imageId”:””,”title”:”B.\tAndexanet alfa”,”isCorrect”:”1″},”qs0e9″:{“id”:”qs0e9″,”image”:””,”imageId”:””,”title”:”C.\tVitamin K”},”pb1bd”:{“id”:”pb1bd”,”image”:””,”imageId”:””,”title”:”D.\tPlatelet transfusion”}}}}}

{“questions”:{“0856e”:{“id”:”0856e”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Destiny F. Chau MD, Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences, Little Rock, AR and Meera Gangadharan MD, Childrens Memorial Hermann Hospital\/McGovern Medical School, Houston, TX \r\n\r\nA 7-day-old, 3.5 kg male infant with a history of Transposition of the Great Arteries (TGA) is scheduled for the arterial switch operation. A transthoracic echocardiogram reports situs solitus, levocardia, and {S,D,D} segmental anatomy. Which of the following MOST accurately describes the segmental anatomy {S,D,D}? “,”desc”:”EXPLANATION \r\nThe growth in knowledge surrounding the embryologic basis of cardiac development necessitates a consistent classification system to accurately describe and classify the many variations in cardiovascular defects. Standardization of the cardiac nomenclature and a universally accepted classification system is critical for appropriately diagnosing and disseminating accurate information across professional specialties caring for congenital heart disease (CHD) patients. Unfortunately, at present time, there is not one universally accepted classification system. \r\n\r\nThe sequential segmental approach is a widely accepted diagnostic evaluation of CHD. There are many publications describing this approach to the evaluation and diagnosis of CHD and the steps involved in a structural assessment. The segmental approach was initially described by Van Praagh and colleagues and rests upon an examination of cardiac anatomy in segments. The anatomy of each segment and the relationship of each segment to the others represents the underpinning of segmental analysis. A second system described by Robert Anderson and colleagues added to this concept but minimized the focus on the relationships between segments by using a model based on blood flow through the heart, which focused on characterizing the connections between the segments, termed a sequential segmental approach. In each segment, right and left-sided structures are evaluated based on the morphology, relative orientations, proximal and distal connections, and presence of abnormalities such as shunts and stenosis. Generally, clinicians have been segregated into followers of the Van Praagh or Anderson approach though many medical professionals have opted for a position in the middle. The chosen classification system for use at a particular institution is often based on preference. \r\n\r\n\r\nDefining the thoraco-abdominal organ position and cardiac position\/apex orientation are important components of the cardiac evaluation and should precede the sequential segmental analysis. Embryologically, all major organ systems are initially positioned in the midline and have mirror image symmetry. During normal development, the cardiovascular, respiratory and gastrointestinal systems become asymmetric, referred to as visceral situs solitus.<\/em> Abnormal situs development can result in mirror-image visceral situs (visceral situs inversus)<\/em> or an ambiguous visceral situs (visceral situs ambiguous).<\/em> Mirror image arrangement describes reversed left-right position and orientation of the organs. Situs ambiguous refers to elements of situs solitus and situs inversus in the same patient. \r\n\r\n\r\nCardiac position is often described as the thoracic position where the majority of the cardiac mass is located: levocardia (left-hemithorax), dextrocardia (right-hemithorax) and mesocardia (mid-thorax). Although these prior terms have also been used to describe the base-to-apex orientation, the apex orientation is often, although not always, in agreement to the cardiac position within the chest. Dextrocardia describes the cardiac apex pointing towards the right side of the chest, mesocardia indicates apex pointing inferiorly, and levocardia pointing to the left side of the chest. Displacement of the heart into the right or left thorax should be indicated by the terms dextropositioning or levopositioning, respectively. \r\n\r\n\r\nThe Van Praagh style uses a unique three-letter notation, or code, inside curly brackets such as {X,X,X}. The letters are abbreviations that represent the sidedness or anatomic arrangement (situs) of the three main cardiac segments of the heart in venoarterial sequence (atria, ventricles, and great arteries). A normal heart is coded {S,D,S}. Atrial situs describes the arrangement of the atria as ascertained by the position of the morphologic right and left atria. When the atria are identified, their situs can then be defined. Similarly the same approach is followed for the ventricles. In the Van Praagh shorthand notation, the types of atrial situs are \”S\” for situs solitus <\/em>(normal arrangement), \”I\” for situs inversus <\/em>and \”A\” for situs ambiguous <\/em>(indeterminate arrangement). \r\n\r\n\r\nThe ventricular segment is described by the type of ventricular loop (handedness of the ventricular mass) and the relationship between the atria and ventricles in three-dimensional space. In the normal D-loop (\u201cD\u201d) heart, the ventricles are normally related and the morphologic right ventricle is right-handed relative to morphologic left ventricle which is left-handed. L-looping of the ventricles is also known as inverted ventricles (morphologic right ventricle is left-handed and morphologic left ventricle is right-handed). The types of ventricular situs (loop or topology) per the Van Praagh system include: solitus <\/em>or D-loop ventricles (D), inverted <\/em>or L-loop ventricles (L); and ambiguous or X-loop ventricles (X). \r\n\r\n\r\nThe great arterial situs is described by the spatial relations between great arteries and the semilunar valves (anterior-posterior and right-left position). In patients with normally related great arteries, the main pulmonary artery is anterior to the aorta and then courses leftward. The aorta is posterior to the main pulmonary artery and courses to the right. The pulmonary valve is anterior to and to the left of the aortic valve. The Van Praagh system describes the types of great arterial situs as follows: 1) solitus <\/em>(aortic valve posterior and to the right of the pulmonary valve)- normally related great arteries (S) or D-transposition (aorta is anterior and to the right of the pulmonary trunk); 2) inversus <\/em>(aortic valve to left of the pulmonary valve)- inverted, normally related great arteries (I), or L-transposition\/malposition (L); 3) ambiguous (right-left location of the aortic valve directly anterior to pulmonary valve is neither a right nor left location- A-transposition\/malposition (A). \r\n\r\n\r\nIn this case scenario, this patient is reported to have situs solitus, levocardia, and {S,D,D} segmental anatomy. The Van Praagh segmental system denotes {S,D,D} as \”S\” for situs solitus or normal atrial arrangement, \”D\” for D-looped or normally related ventricles (morphologic right ventricle is right-handed and the morphologic left ventricle is left-handed), and \”D\” for D-malposed or transposed great arteries, with the aorta to the right of the pulmonary trunk rather than posterior to the main pulmonary artery. \r\n\r\nREFERENCES \r\n \r\n\r\nVan Praagh R. Terminology of congenital heart disease. Glossary and commentary. Circulation.<\/em> 1977;56(2):139-143. \r\n\r\n\r\nAnderson RH, Shirali G. Sequential segmental analysis. Ann Pediatr Cardiol. <\/em>2009; 2: 24-35. \r\n\r\n\r\nJacobs JP, Anderson RH, Weinberg PM, et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young.<\/em> 2007;17 Suppl 2:1-28. 07001138\r\n\r\n\r\nKussman BD, Miller-Hance WC. Development of the cardiovascular system and nomenclature for congenital heart disease. In: Andropoulos DB, ed. Anesthesia for Congenital Heart Disease. 3rd ed. Hoboken, NJ; Wiley-Blackwell. 2015: 42-82. \r\n\r\n\r\nEdwards WD, Maleszewski JJ. Classification and terminology of cardiovascular anomalies. In: Allen HD, Driscoll DJ, Shaddy RE and Feltes, TF, eds. Moss and Adams’ heart disease in infants, children and adolescents, including the fetus and young adult. 8th ed. Philadelphia, PA; Lippincott Williams & Wilkins. 2013: 32-51.\r\n\r\n”,”hint”:””,”answers”:{“sy3cc”:{“id”:”sy3cc”,”image”:””,”imageId”:””,”title”:”A.\tInverted atria, normally related ventricles, aorta to the right of the pulmonary trunk”},”3vv34″:{“id”:”3vv34″,”image”:””,”imageId”:””,”title”:”B.\tNormal atria, inverted ventricles, aorta to the left of the pulmonary trunk”},”8dxzi”:{“id”:”8dxzi”,”image”:””,”imageId”:””,”title”:”C.\tNormal atria, normally related ventricles, aorta to the right of the pulmonary trunk”,”isCorrect”:”1″},”d2fxj”:{“id”:”d2fxj”,”image”:””,”imageId”:””,”title”:”D.\tInverted atria, indeterminate ventricles, aorta anterior to the pulmonary trunk”}}}}}

{“questions”:{“un5e3”:{“id”:”un5e3″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR and Lawrence Greiten, MD \u2013 Pediatric Cardiothoracic Surgery, Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 14-month-old, 10 kg male toddler with complex multilevel left ventricular outflow tract obstruction and aortic insufficiency is status post the Ross-Konno procedure during which the perfusionist uses del Nido cardioplegia. Which of the following additives is unique to del Nido cardioplegia?”,”desc”:”EXPLANATION \r\nAdequate myocardial protection during periods of cardiac arrest and myocardial ischemia while on cardiopulmonary bypass (CPB) is of paramount importance for good outcomes after cardiac surgery. Poor myocardial protection can lead to irreversible myocardial damage from ischemia and has been is associated with increased postoperative morbidity and mortality. \r\n\r\nThe principles of myocardial protection center on maximally reducing metabolic rate, supporting aerobic and anaerobic metabolism, preserving intracellular pH, and minimizing intracellular metabolic injury due to sodium, calcium, and oxygen free-radical accumulation. Visible end-points of myocardial protection are mechanical cardiac arrest, lack of electrical activity on the ECG and hypothermic core body temperature. Cardioplegia solutions for myocardial protection have been researched for over 40 years. Currently, there are a number of cardioplegia solutions with variable compositions, ranging from commercial to customized institutional solutions. They can be broadly classified as crystalloid cardioplegia or blood- cardioplegia depending upon the addition of blood to the cardioplegia solution. Additionally, techniques and protocols for the administration of cardioplegia vary widely depending on the patient population, surgical procedure, surgeon preference and institutional factors. Cardioplegia dose is dependent upon the particular cardioplegia formulation and the expected time of myocardial arrest. Limiting the total number and frequency of repeated cardioplegia doses is desired to reduce overall manipulation of the heart, reduce the aortic cross-clamp time and prevent myocardial edema. \r\n\r\n\r\nA common characteristic of cardioplegia solutions is high potassium concentration in order to induce contractile arrest through membrane hyperpolarization. Components such as magnesium, mannitol, sodium bicarbonate, and lidocaine are present in cardioplegia for a number of reasons. Lidocaine is a sodium channel blocker and antiarrhythmic. Sodium channel blockage increases myocyte refractory time. Additionally, sodium channel blockage helps to counteract the negative impact of hyperkalemic arrest by polarizing the cell membrane and preventing intracellular sodium and calcium accumulation. Magnesium is a natural calcium channel blocker and reduces the accumulation of intracellular calcium. Low intracellular calcium concentrations have been associated with reduced myocardial injury. Sodium bicarbonate is a buffer which scavenges excess hydrogen ions and assists with maintaining intracellular pH. Hyperosmotic mannitol scavenges free radicals and reduces myocardial swelling. The addition of red blood cells to cardioplegia has been shown to preserve myocardial metabolism and function, resulting in less metabolic ischemic stress and reperfusion injury when compared to crystalloid-cardioplegia. In addition, red blood cells contain carbonic anhydrase, an enzyme that facilitates the scavenging of hydrogen ions to generate carbon dioxide and water. \r\n\r\n\r\nA survey of the utilization of cardioplegia solutions by congenital cardiac surgical programs in North America found that blood-based cardioplegia formulations are predominantly used by 86% of respondents. Use of the del Nido solution was reported most commonly in 38% of respondents, followed by customized solutions in 32%. \r\n\r\n\r\nOriginally, the same cardioplegia formulations used in adults were also used in pediatric patients. However, in the 1990\u2019s, the del Nido cardioplegia solution was created for myocardial protection of the immature myocardium of neonates and pediatric patients. At the present time, it is increasingly being utilized for myocardial preservation during adult cardiac surgery. \r\n\r\n\r\nAlthough the current del Nido cardioplegia solution has evolved from the original cardioplegia formulations, it is unique in that it contains lidocaine. It contains a base solution of Plasma-Lyte A, with an electrolyte composition resembling extracellular fluid. A one-liter formulation of del Nido cardioplegia includes the following: Plasmalyte-A (1L), magnesium (2 g, 4 mL), potassium chloride (26 mEq, 13 mL), mannitol (3.26 g, 16 mL), sodium bicarbonate (13 mEq, 13 mL) and lidocaine (130 mg, 13 mL). Calcium is not added, although a small amount of calcium is derived from the addition of whole blood. This formulation is deemed to arrest the heart in diastole. The del Nido solution is generally used as a single-dose of 20 mL\/kg. Larger doses may be needed for patients with a thickened myocardium, aortic insufficiency or other conditions where myocardial protection is more challenging. Compared to other formulations, del Nido cardioplegia has been reported to provide comparable myocardial protection, longer cardiac arrest time from a single dose, and lower total volumes of cardioplegia solution with less hemodilution. \r\n\r\n\r\nIt is important for cardiac anesthesiologist to be familiar with cardioplegia solution types used during cardiac surgeries. Multiple dosing with high total volume for complex surgeries leads to hemodilution. Although the accumulation of cardioplegia components such as potassium and lidocaine are usually not clinically significant, awareness and perspective on this information is relevant. \r\nREFERENCES \r\nSinha P. Myocardial Protection. In: Jonas RA, ed. Comprehensive Surgical Management of Congenital Heart Disease.<\/em> 2nd Edition. Boca Raton, Florida: CRC Press, Taylor and Francis Group, LLC; 2014: 214-216. \r\n\r\n\r\nWaterford SD, Ad N. Del Nido cardioplegia: Questions and (some) answers. J Thorac Cardiovasc Surg.<\/em> 2021;S0022-5223(21)01676-7. \r\n\r\n\r\nMatte GS, del Nido PJ. History and use of del Nido cardioplegia solution at Boston Children’s Hospital [published correction appears in J Extra Corpor Technol. 2013 Dec;45(4):262]. J Extra Corpor Technol.<\/em> 2012;44(3):98-103. \r\n\r\n\r\nGinther RM Jr, Gorney R, Forbess JM. Use of del Nido cardioplegia solution and a low-prime recirculating cardioplegia circuit in pediatrics. J Extra Corpor Technol.<\/em> 2013;45(1):46-50. \r\n\r\n\r\nKotani Y, Tweddell J, Gruber P, et al. Current cardioplegia practice in pediatric cardiac surgery: a North American multi-institutional survey. Ann Thorac Surg.<\/em> 2013;96(3):923-929. \r\n\r\n\r\nHaranal M, Chin HC, Sivalingam S, et al. Safety and Effectiveness of Del Nido Cardioplegia in Comparison to Blood-Based St. Thomas Cardioplegia in Congenital Heart Surgeries: A Prospective Randomized Controlled Study. World J Pediatr Congenit Heart Surg.<\/em> 2020;11(6):720-726. \r\n\r\n”,”hint”:””,”answers”:{“9ilh7”:{“id”:”9ilh7″,”image”:””,”imageId”:””,”title”:”A.\tPotassium “},”rliok”:{“id”:”rliok”,”image”:””,”imageId”:””,”title”:”B.\tMagnesium”},”fc7ht”:{“id”:”fc7ht”,”image”:””,”imageId”:””,”title”:”C.\tMannitol”},”pxye6″:{“id”:”pxye6″,”image”:””,”imageId”:””,”title”:”D.\tLidocaine”,”isCorrect”:”1″}}}}}

{“questions”:{“p4uw2”:{“id”:”p4uw2″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Ahmed Zaghw, MB.BCH – University of California, Davis, CA and Destiny Chau, MD – Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 4-month-old male infant undergoes full repair for Tetralogy of Fallot. During the first 24 hours postoperatively, the patient develops a hemodynamically significant arrhythmia requiring aggressive medical intervention. What is the MOST COMMON type of arrhythmia occurring after pediatric cardiac surgery?\r\n”,”desc”:””,”hint”:””,”answers”:{“d6xty”:{“id”:”d6xty”,”image”:””,”imageId”:””,”title”:”A.\tComplete heart block”},”y5ah9″:{“id”:”y5ah9″,”image”:””,”imageId”:””,”title”:”B.\tJunctional ectopic tachycardia “,”isCorrect”:”1″},”7zeg0″:{“id”:”7zeg0″,”image”:””,”imageId”:””,”title”:”C.\tAtrial ectopic tachycardia”},”ehc31″:{“id”:”ehc31″,”image”:””,”imageId”:””,”title”:”D.\tSecond degree atrioventricular heart block”}}}},”results”:{“3z56y”:{“id”:”3z56y”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”EXPLANATION \r\nEarly postoperative arrhythmias after congenital cardiac surgery occurs with a reported incidence of 7.5% to 48%. Commonly encountered rhythm derangements include junctional ectopic tachycardia (JET), complete heart block, and supraventricular tachycardia. In the postoperative period, arrhythmias are poorly tolerated and may lead to life-threatening hemodynamic instability due to diminished cardiac output. Arrhythmias are commonly associated with surgery in proximity to the sinus node or the AV node. They may also be related to an atriotomy or a ventriculotomy incision. Additional risk factors for early postoperative arrhythmias include electrolyte abnormalities, myocardial ischemia, myocardial inflammation, increased sympathetic tone, prolonged cardiopulmonary bypass and aortic cross-clamp duration, and the use of arrhythmogenic vasoactive medications. \r\n\r\nBradyarrhythmias diminish cardiac output due to a decrease in heart rate. Furthermore, bradyarrhythmia in combination with non-sinus rhythm may impair cardiac filling and decrease resultant stroke volume due to a disruption in atrioventricular (AV) synchrony. Tachyarrhythmias reduce cardiac output by decreasing stroke volume due to a shorter diastolic filling time, and while occurring simultaneously with non-sinus rhythm may similarly compromise cardiac output due to a loss of AV synchrony. When causing a decrease in cardiac output, both bradyarrhythmias and tachyarrhythmias result in decreased myocardial oxygen supply, thus creating an imbalance in myocardial oxygen supply and demand. Tachyarrhythmias may cause additional disparity in myocardial oxygen supply and demand because of increased myocardial oxygen demand secondary to an elevated heart rate. \r\n\r\nOverall, JET is the most commonly reported rhythm disturbance after pediatric congenital cardiac surgery. It occurs most frequently after repair of Tetralogy of Fallot, ventricular septal defects, atrioventricular septal defects, Transposition of the Great Arteries, and total anomalous pulmonary venous return. The incidence of JET after Tetralogy of Fallot repair is reported to occur in up to 20% of patients. \r\nManagement of JET includes reducing core temperature, atrial pacing, and anti-arrhythmic drugs such as amiodarone. For medically refractory cases, support with extracorporeal membrane oxygenation may be indicated. Cardioversion is generally deemed ineffective for terminating JET. \r\nSusceptibility to a particular rhythm disturbance differs by cardiac lesion and type of cardiac surgery. After orthotopic heart transplantation, atrial fibrillation is the most common arrhythmia in the early postoperative period, occurring in up to 24% of patients. Some reports suggest that the biatrial surgical method is associated with a greater risk of atrial tachyarrhythmias as compared to the bicaval technique. Of note, early cardiac graft rejection may present clinically with atrial tachyarrhythmias. \r\n \r\nComplete heart block is the most common postoperative bradyarrhythmia, with a reported incidence of 1.5% to 17.8%. Temporary pacing is an important treatment modality for the postoperative management of bradyarrhythmia with most cases recovering within 1- 2 weeks after surgery. \r\nIn conclusion, in the early postoperative period after congenital cardiac surgery, JET is the most common arrhythmia overall. Complete heart block is the most common bradyarrhythmia. The majority of arrhythmias are transient and self-limiting with adequate medical management. \r\n\r\nREFERENCES \r\nDelaney JW, Moltedo JM, Dziura JD, et al. Early postoperative arrhythmias after pediatric cardiac surgery. J Thorac Cardiovasc Surg.<\/em> 2006;131(6):1296-1300. \r\n\r\nTalwar S, Patel K, Juneja R, et al. Early postoperative arrhythmias after pediatric cardiac surgery. Asian Cardiovasc Thorac Ann.<\/em> 2015;23(7):795-801. \r\n\r\nNelson JS, Vanja S, Maul TM, et al. Early arrhythmia burden in pediatric cardiac surgery fast-track candidates: Analysis of incidence and risk factors. Progress in Pediatric Cardiology.<\/em> 2019;(52): 8-12.\r\n\r\nSahu MK, Das A, Siddharth B, et al. Arrhythmias in children in early postoperative period after cardiac surgery. World J Pediatr Congenit Heart Surg.<\/em> 2018;9(1):38-46. \r\n\r\nJoglar JA, Wan EY, Chung MK, et al. Management of arrhythmias after heart transplant: current state and considerations for future research. Circ Arrhythm Electrophysiol. <\/em> 2021;14(3):e007954. \r\n\r\nIshaque S, Akhtar S, Ladak AA, et al. Early postoperative arrhythmias after pediatric congenital heart disease surgery: a 5-year audit from a lower- to middle-income country. Acute Crit Care.<\/em> 2022;37(2):217-223. \r\n\r\nValdes SO, Kim JJ, Miller-Hance WC. Arrhythmias: diagnosis and management. In: Andropoulos DB, ed. Anesthesia for Congenital Heart Disease. 3rd ed. Hoboken, NJ; Wiley-Blackwell. 2015: 404-436.\r\n\r\n\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“u2t8o”:{“id”:”u2t8o”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Bryce Ferry, DO and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 7-year-old male child presents to the emergency room with a history of syncope while playing soccer. He also has a family history of sudden death at a young age. Workup demonstrates a normal electrocardiogram at rest, a structurally normal heart on transthoracic echocardiogram, and a right heart biopsy demonstrating normal myocardium. Genetic testing reveals a mutation in the RYR2 gene. Which of the following conditions is MOST LIKELY in this patient?”,”desc”:””,”hint”:””,”answers”:{“6av5d”:{“id”:”6av5d”,”image”:””,”imageId”:””,”title”:”A. Arrhythmogenic right ventricular cardiomyopathy “},”3gk8b”:{“id”:”3gk8b”,”image”:””,”imageId”:””,”title”:”B. Long QT syndrome”},”bn5hw”:{“id”:”bn5hw”,”image”:””,”imageId”:””,”title”:”C. Catecholaminergic polymorphic ventricular tachycardia “,”isCorrect”:”1″},”8nebs”:{“id”:”8nebs”,”image”:””,”imageId”:””,”title”:”D. Brugada syndrome”}}}},”results”:{“r279t”:{“id”:”r279t”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”EXPLANATION \r\nSyncope due to cardiac disease can be life-threatening. Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare arrhythmogenic disorder characterized by bidirectional or polymorphic ventricular tachycardia (VT) that is triggered by an adrenergic surge secondary to exercise or emotional stress. CPVT may result from mutations in the cardiac ryanodine receptor gene (RYR2), which is inherited in an autosomal dominant pattern or the calsequestrin gene CASQ2, inherited in an autosomal recessive pattern. These genes are likely involved in calcium release from the sarcoplasmic reticulum. To date, other genes have also been implicated in CPVT. \r\nThe mean age of symptom presentation is between 7 and 12 years old with syncope being a common presenting sign. Diagnostic workup often reveals a normal resting baseline electrocardiogram (ECG) and a structurally normal heart on echocardiography. However, an exercise or pharmacologic stress test typically reveals bidirectional or polymorphic VT. In children who cannot perform a stress test, a Holter monitor or an event recorder can aid in detecting an abnormal ECG during periods of adrenergic stress. Typically, when the heart rate goes above a threshold of 100-120 beats per minute (BPM), the ECG will demonstrate premature ventricular complexes (PVCs) followed by short runs of non-sustained VT. With continued stress, the VT can degenerate into ventricular fibrillation (VF). Self-resolution of the arrhythmia can occur when the stress occurs over a short time period. \r\nThe first line treatment of CPVT is nonselective beta blockers, such as nadolol, often at high doses to achieve clinical effectiveness. It may also be clinically indicated to add flecainide or a calcium-channel blocker to beta blocker therapy. With persistent syncope or progression to cardiac arrest, the recommendation is an implantable cardioverter defibrillator. When left untreated, CPVT will result in cardiac arrest in up to 30% and recurrent syncope in up to 80% of affected patients. \r\nAnesthetic management for patients with CPVT centers on preventing periods of adrenergic stress and catecholamine surges. To this point, a patient’s emotional distress and pain should be anticipated and prevented. It is imperative that beta blockers are continued during the perioperative period. Adrenergic drugs are to be avoided and tachycardia should be promptly treated. Prevention of postoperative nausea and vomiting and adequate pain control is important. Patients with the cardiac ryanodine gene (RYR2) do not seem susceptible to malignant hyperthermia, which is linked to the RYR1 receptor. \r\nArrhythmogenic cardiomyopathy, also known as arrhythmogenic right ventricular dysplasia\/cardiomyopathy, is a rare non-ischemic cardiomyopathy in which the right ventricular myocardium is replaced by fibrofatty tissue that may lead to arrythmias, dyspnea, and possible syncopal episodes. Arrhythmogenic cardiomyopathy can develop during childhood, but most cases arise during the third to fourth decade of life. Long QT syndrome can present similarly to CPVT with regards to emotional or physical stress but instead displays prolongation of the QT interval on the resting ECG. Brugada syndrome may also present with syncope, but often has characteristic ECG findings of variable ST segment abnormalities. This patient’s presentation, diagnostic findings, and genetic results are most consistent with catecholaminergic polymorphic ventricular tachycardia. \r\n\r\nREFERENCES \r\nPflaumer A, Davis AM. Guidelines for the diagnosis and management of catecholaminergic polymorphic ventricular tachycardia. Heart Lung Circ.<\/em> 2012;21(2):96-100. \r\n\r\nOmiya K, Mitsui K, Matsukawa T. Anesthetic management of a child with catecholaminergic polymorphic ventricular tachycardia undergoing insertion of implantable cardioverter defibrillator : a case report.JA Clin Rep.<\/em> 2020;6(1):16. \r\n\r\nStaikou C, Chondrogiannis K, Mani A. Perioperative management of hereditary arrhythmogenic syndromes. Br J Anaesth.<\/em> 2012;108(5):730-744. \r\n\r\nBrugada J, Campuzano O, Arbelo E, Sarquella-Brugada G, Brugada R. Present status of Brugada syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol.<\/em> 2018;72(9):1046-1059. \r\n\r\nShah SR, Park K, Alweis R. Long QT Syndrome: A comprehensive review of the literature and current evidence. Curr Probl Cardiol.<\/em> 2019;44(3):92-106. \r\n\r\nGandjbakhch E, Redheuil A, Pousset F, Charron P, Frank R. Clinical diagnosis, imaging, and genetics of arrhythmogenic right ventricular cardiomyopathy\/dysplasia: JACC State-of-the-Art Review. J Am Coll Cardiol.<\/em> 2018;72(7):784-804. \r\n\r\n”,”redirect_url”:””}}}