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

{“questions”:{“fuz4p”:{“id”:”fuz4p”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2025\/09\/CCAS-QOW-9-11-25-Pic.png”,”imageId”:”9064″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Anila B Elliott, MD – University of Michigan, C.S. Mott Children\u2019s Hospital \r\n\r\nA 10-year-old with bicuspid aortic valve presents for a Ross procedure. They are consented for a deep parasternal intercostal plane (DPIP) block. What is the correct order of anatomical structures in the ultrasound image below, from superficial to deep (1 to 6)?”,”desc”:”EXPLANATION \r\nThe deep parasternal intercostal plane (DPIP) block is a regional technique used to provide analgesia to the anterior chest wall. This block targets the anterior branches of the intercostal nerves (T2-T6) as they course anterior to the internal mammary vasculature. Local anesthetic is deposited into the fascial layer between the internal intercostal muscle and the transversus thoracic muscle^{1,2<\/sup>. As local anesthetic is injected, these layers are separated, as illustrated in Figure 1.\r\n\r\nFigure 1: Image A: The needle is seen traversing the tissue layers prior to injection of local anesthetic. Note the location of the pleura (arrow). Image B: Following injection, the anesthetic spreads and separates the tissue layers, displacing the pleura inferiorly. \r\n \r\n \r\n\r\nUtilization of the DPIP block has been shown to decrease post-operative pain scores and reduce opioid requirements following pediatric cardiac surgery3<\/sup>. Cadaveric studies have shown that the local anesthetic spread of the DPIP block covers a broader area than the superficial parasternal muscle plane (SPIP) block, offering enhanced pain control4<\/sup>. However, the DPIP is considered a more advanced block as it carries a potential risk of vascular injury, as the needle tip may come within millimeters of the internal mammary artery 4,5<\/sup>. \r\n\r\nOther regional blocks of the anterior chest wall include the PECS I and PECS II blocks. The PECS I block traverses the pectoralis major and deposits local anesthetic between the pectoralis major and pectoralis minor, targeting the pectoral nerves. The PECS II block traverses the pectoralis major and pectoralis minor muscles to deposit local anesthetic between the pectoralis minor and the serratus anterior muscle, targeting the lateral cutaneous intercostal nerves (T2-T6)6<\/sup>. \r\n\r\nThe correct answer is A \u2013 the layers depicted in the picture from superficial to deep are the pectoralis major, external intercostal muscle, internal intercostal muscle, transversus thoracic muscle, internal mammary artery, and the pleura.\r\n\r\n \r\nREFERENCES \r\n1.\tCakmak M, Isik O. Transversus Thoracic Muscle Plane Block for Analgesia After Pediatric Cardiac Surgery. J Cardiothorac Vasc Anesth<\/em>. 2021;35(1):130-136. doi:10.1053\/j.jvca.2020.07.053 \r\n2.\tAydin ME, Ahiskalioglu A, Ates I, et al. Efficacy of Ultrasound-Guided Transversus Thoracic Muscle Plane Block on Postoperative Opioid Consumption After Cardiac Surgery: A Prospective, Randomized, Double-Blind Study. J Cardiothorac Vasc Anesth<\/em>. 2020;34(11):2996-3003. doi:10.1053\/j.jvca.2020.06.044 \r\n3.\tCui YY, Xu ZQ, Hou HJ, Zhang J, Xue JJ. Transversus Thoracic Muscle Plane Block For Postoperative Pain in Pediatric Cardiac Surgery: A Systematic Review And Meta-Analysis of Randomized And Observational Studies. J Cardiothorac Vasc Anesth<\/em>. 2024;38(5):1228-1238. doi:10.1053\/j.jvca.2024.02.016 \r\n4.\tDouglas RN, Kattil P, Lachman N, et al. Superficial versus deep parasternal intercostal plane blocks: cadaveric evaluation of injectate spread. Br J Anaesth<\/em>. 2024;132(5):1153-1159. doi:10.1016\/j.bja.2023.08.014 \r\n5.\tSepolvere G, Togn\u00f9 A, Tedesco M, Coppolino F, Cristiano L. Avoiding the Internal Mammary Artery During Parasternal Blocks: Ultrasound Identification and Technique Considerations. J Cardiothorac Vasc Anesth<\/em>. 2021;35(6):1594-1602. doi:10.1053\/j.jvca.2020.11.007 \r\n6.\tDost B, De Cassai A, Amaral S, et al. Regional anesthesia for pediatric cardiac surgery: a review. BMC Anesthesiol<\/em>. 2025;25(1):77. Published 2025 Feb 15. doi:10.1186\/s12871-025-02960-z \r\n”,”hint”:””,”answers”:{“5zpwr”:{“id”:”5zpwr”,”image”:””,”imageId”:””,”title”:”A.\tPectoralis major — external intercostal — internal intercostal — transversus thoracic muscle — internal mammary artery — pleura\r\n”,”isCorrect”:”1″},”wpy46″:{“id”:”wpy46″,”image”:””,”imageId”:””,”title”:”B.\tPectoralis major — pectoralis minor — intercostal — transversus thoracic muscle — internal mammary artery — pleura”},”x7jb3″:{“id”:”x7jb3″,”image”:””,”imageId”:””,”title”:”C.\tPectoralis major — serratus anterior — intercostal –transversus thoracic muscle — internal mammary artery — pleura\r\n\r\n”}}}}}}

{“questions”:{“2p83i”:{“id”:”2p83i”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Kanwarpal Singh Bakshi, MD – Children\u2019s Hospital Los Angeles \r\nA 3\u202fkg neonate undergoes a Norwood-mBTT shunt procedure for hypoplastic left heart syndrome (HLHS). Following a prolonged cardiopulmonary bypass run, the patient is placed on veno-arterial (VA) ECMO postoperatively. After several days of support, a clamp trial is performed to assess readiness for decannulation. The patient is transitioned to full mechanical ventilation and restarted on inotropic support. The patient immediately demonstrates signs of hemodynamic instability and inadequate gas exchange, and the trial is aborted. Upon reinstitution of VA ECMO with adequate circuit flow, the patient exhibits persistently low saturations. The ECMO circuit appears intact with no signs of clot or flow disturbance. Which of the following is the MOST LIKELY cause of the desaturation?\r\n”,”desc”:”EXPLANATION \r\nExtracorporeal membrane oxygenation (ECMO) is a form of temporary mechanical support for patients with severe, life-threatening cardiac or respiratory failure. In neonates and infants with congenital heart disease, particularly following complex surgical interventions such as the Norwood procedure for hypoplastic left heart syndrome (HLHS), ECMO provides a vital bridge to recovery or further surgical intervention.^{\u00b9<\/sup> In veno-arterial (VA) ECMO, deoxygenated blood is drained from the venous system\u2014typically via the right atrium or internal jugular vein\u2014and pumped through a membrane oxygenator where gas exchange occurs. The oxygenated blood is then returned to the arterial system, usually via the aorta or carotid artery, to support systemic circulation and oxygen delivery.\u00b2,\u00b3<\/sup> \r\nVA ECMO differs from veno-venous (VV) ECMO in that it supports both the heart and lungs. This makes it the preferred mode in patients with significant myocardial dysfunction, such as those who have undergone a Norwood procedure. Typical VA ECMO parameters include flow rates of 100\u2013150 mL\/kg\/min, careful monitoring of circuit pressures, and regular assessment of gas exchange and end-organ perfusion.\u00b9,\u00b3<\/sup> \r\nKey circuit components include a centrifugal pump, a membrane oxygenator, a sweep gas system, and a heat exchanger. The sweep gas, typically 100% oxygen or a blended gas, drives the removal of carbon dioxide and supports oxygenation across the membrane. Without active sweep flow, the membrane oxygenator cannot perform gas exchange, rendering the circuit ineffective for oxygen delivery. \r\nWhen there is evidence of cardiac and pulmonary recovery, clinicians may attempt aclamp trial<\/em>.\u2074<\/sup> Prior to clamping off, the ECMO flows are gradually weaned and the patient is transitioned to full ventilator and inotropic support. Echocardiography is performed during weaning to assess myocardial recovery, assessing LV\/RV contractility, ventricular unloading, atrioventricular valve competency, and neo\/aortic valve opening. If everything looks favorable, the clamp trial begins as ECMO flow is stopped by clamping both the venous and arterial cannulae. \r\nIt is standard practice to discontinue the sweep gas to the oxygenator during the clamp trial.\u2074<\/sup> Although ECMO flow to the patient is halted, residual blood remains in the ECMO circuit, and low-flow recirculation through a bridge between the venous and arterial limbs is typically maintained to prevent blood stasis and reduce the risk of thrombosis. Leaving the sweep gas on allows continued gas exchange in the oxygenator, which can strip CO_{\u2082<\/sub> from the blood. As a result, the blood in the circuit becomes progressively hypocarbic. When the trial ends and full ECMO support is reinitiated, this hypocarbic blood is reinfused into the patient, potentially leading to abrupt systemic hypocarbia. Such sudden changes in PaCO\u2082<\/sub> can cause cerebral vasoconstriction, reducing cerebral blood flow and increasing the risk of neurologic complications, especially in neonates who have immature autoregulatory mechanisms. To avoid this, the sweep gas will be turned off during a clamp trial to prevent alterations in blood gas composition within the idle circuit. \r\nIf the patient fails to tolerate the clamp trial, as demonstrated by persistently low mixed venous oxygen saturations, rising lactate, worsening acidosis, or hypotension, ECMO must be urgently reinitiated.\u00b2,\u2074<\/sup> As part of this reinitiation, it is essential to ensure that sweep gas is turned back on and adjusted appropriately. Failure to do so can result in continued inadequate oxygenation and CO\u2082<\/sub> clearance, further worsening the patient\u2019s condition. Additionally, ventilator and inotropic support must be carefully titrated before, during, and after the trial to optimize conditions for both native and mechanical support.\u2074<\/sup> \r\nIn neonates with single-ventricle physiology, particularly after the Norwood procedure, clamp trials can fail for several reasons. These patients rely on a delicate balance between systemic and pulmonary blood flow (Qp:Qs), and myocardial recovery may be incomplete following a prolonged cardiopulmonary bypass run. Additionally, pulmonary vascular resistance may still be elevated, or the lungs may have residual injury or edema.\u00b3<\/sup> Even with restored ventilation and inotropes, the circulation may not be capable of supporting end-organ perfusion without extracorporeal assistance. Finally, given the technical nature of the Norwood procedure, the patient may need to return to surgery to revise the shunt or Damus\u2013Kaye\u2013Stansel (DKS) anastomosis. \r\nAn alternative to the traditional clamp-off trial is the pump-controlled retrograde trial off <\/em>(PCRTO) protocol, which allows for a more gradual assessment of the patient’s readiness for decannulation.\u2075<\/sup> In this method, the centrifugal pump speed is reduced to allow retrograde flow through the arterial cannula. This setup minimizes ECMO support while preserving circuit patency, enabling continued low-flow circulation and reducing the risk of thrombus formation. Unlike the abrupt cessation seen in clamp trials, PCRTO enables a more physiologic transition to native circulation, mitigating hemodynamic instability.\u2075<\/sup> This method is especially valuable in neonates and critically ill patients with marginal myocardial recovery, as it provides a safety mechanism for immediate reinitiation of full support if the patient fails the trial. Additionally, it allows for longer evaluation periods than clamp-off trials, facilitating a more accurate assessment of cardiopulmonary readiness for decannulation.\u2075<\/sup> \r\nOther answer choices are less consistent with the clinical presentation. Air entrainment (A) into the arterial cannula would result in immediate hemodynamic collapse or neurologic injury due to arterial embolism. Oxygenator failure (C) usually presents gradually, with rising transmembrane pressure gradients, worsening oxygenation over hours, and sometimes hemolysis\u2014not an abrupt desaturation event after reinitiating ECMO. \r\n \r\nREFERENCES \r\n1. Valencia E, Nasr VG. Updates in Pediatric Extracorporeal Membrane Oxygenation. J Cardiothorac Vasc Anesth<\/em>. 2020;34(5):1309\u20131323. doi:10.1053\/j.jvca.2019.09.006. \r\n2. Peek GJ, Harvey C. Weaning and decannulation in neonatal respiratory failure. In: MacLaren G, Brodie D, Lorusso R, Peek G, Thiagarajan R, Vercaemst L. Extracorporeal Life Support: The ELSO Red Book. 6th ed. Extracorporeal Life Support Organization; 157-164. \r\n3. Nadkarni AS, Delany DR, Schramm J, Shin YR, Hoskote A, Bembea MM. ECMO considerations in the pediatric cardiac population. Curr Pediatr Rep<\/em>. 2023;11(2):86\u201395. doi:10.1007\/s40124-023-00292-5. E McCartney SL, Krishnan S. \r\n4. Krishnan S, Schmidt GA. ECMO Weaning and Decannulation. In: Schmidt GA, ed. Extracorporeal Membrane Oxygenation for Adults. 3rd ed. Springer; 2022:265\u2013275. \r\n5. Stukov Y, Dibert TT, Narasimhulu SS, et al. Pump-controlled retrograde trial off extracorporeal membrane oxygenation. Multimedia Manual of Cardiothoracic Surgery<\/em>. 2025. Available from: https:\/\/mmcts.org\/tutorial\/1972\r\n\r\n”,”hint”:””,”answers”:{“m3uu4”:{“id”:”m3uu4″,”image”:””,”imageId”:””,”title”:”A. Air entrainment in the arterial cannula”},”b9nnj”:{“id”:”b9nnj”,”image”:””,”imageId”:””,”title”:”B. Sweep gas flow was not restored”,”isCorrect”:”1″},”bim84″:{“id”:”bim84″,”image”:””,”imageId”:””,”title”:”C. Oxygenator membrane failure”}}}}}}}

{“questions”:{“ckp55”:{“id”:”ckp55″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Anila B. Elliott, MD – University of Michigan, C.S. Mott Children\u2019s Hospital \r\nA 3-day-old male with total anomalous pulmonary venous return (TAPVR) is transferred from an outside hospital. He is hemodynamically stable on no vasoactive medications and supported on nasal cannula at 2L\/min with an FiO2 of 0.21. Transthoracic echocardiography shows unobstructed flow of pulmonary venous blood to systemic venous circulation with right to left shunting through a moderate-sized secundum atrial septal defect. Which of the following is a risk factor for increased morbidity and mortality?\r\n\r\n”,”desc”:”EXPLANATION \r\nTotal anomalous pulmonary venous return (TAPVR) is a rare congenital cardiac lesion, occurring in 1-3% of the congenital heart disease population^{1<\/sup>. There are four main types of TAPVR: supracardiac (most common), cardiac, infracardiac and mixed. Mixed TAPVR is the least common and the most challenging to surgically repair as there is often no pulmonary confluence and multiple pulmonary venous connections both above and below the diaphragm2<\/sup>. \r\n\r\n\r\nSince the introduction of prostaglandins to maintain ductal patency in many congenital cardiac lesions, obstructed TAPVR remains one of the few true congenital cardiac surgical emergencies. \r\nTAPVR can be associated with other anomalies, including heterotaxy, hypoplasia of left-sided structures, or other lesions requiring palliation down the single-ventricle pathway2<\/sup>. \r\n\r\n\r\nA pure right-to-left shunt across the atrial septum on echocardiogram is usually indicative of TAPVR1<\/sup>. A key factor in peri-operative management is whether there is obstruction to pulmonary venous flow. On echocardiography, flow acceleration in pulmonary veins of \u2265 2.0 m\/sec indicates significant obstruction2<\/sup>. Clinical presentation of obstructed TAPVR includes severe pulmonary edema, pulmonary hypertension, cyanosis, metabolic acidosis and cardiogenic shock3<\/sup>. Management involves maintaining forward flow and avoiding pulmonary vasodilation, which can lead to increased flow in the already congested pulmonary circulation. In those with unobstructed pulmonary veins, as long as there is adequate mixing, they may present with mild cyanosis, signs of pulmonary over circulation, and potentially right-sided dilation\/hypertrophy depending on age1<\/sup>.\r\n\r\n\r\nSupracardiac TAPVR is usually unobstructed but may become obstructed if the vertical vein passes between the bronchus and the left pulmonary artery2<\/sup>. Infracardiac TAPVR usually presents as (or is considered to be) obstructed due to the long course of the vasculature to return to the atrium, with the potential for narrowing and obstruction at several points, especially in the intra-hepatic region3<\/sup>. Cardiac TAPVR is less likely to be obstructed1<\/sup>. \r\n\r\n\r\nThe most common post-operative complication includes recurrence of pulmonary venous obstruction and has been reported to occur in 8-54% of cases2<\/sup>. Other risk factors for increased morbidity and mortality include single ventricle multidistributive circulation, obstructed veins pre-operatively, pre-operative invasive ventilation, and pulmonary hypertension1,3<\/sup>. \r\n\r\n\r\nThe correct answer is choice C: perioperative complications (including mortality) are increased2,3<\/sup> in patients with persistent pulmonary hypertension. Pulmonary veins draining into the coronary sinus describe cardiac TAPVR, which is less likely to be obstructed1<\/sup> and a patient with a vertical vein and unobstructed flow is less likely to present in extremis with pulmonary hypertension or end-organ dysfunction3<\/sup>. \r\n\r\n\r\n \r\nREFERENCES \r\n\r\n1.\tHancock Friesen, CL,, Zurakowski, D., Thiagarajan, RR., et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg<\/em>. 2005; 79(2): 596-606 \r\n2.\tKaramlou, T., Gurofsky, R., Al Sukhni, E., et al. Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation<\/em>. 2007; 115:1591-1598 \r\n3.\tSchulz, A., Wu, DM., Ishigami, S., et al. Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival. JTCVS Open<\/em>. 2022; 12: 335-343\r\n”,”hint”:””,”answers”:{“sroch”:{“id”:”sroch”,”image”:””,”imageId”:””,”title”:”A.\tPulmonary veins draining into the coronary sinus”},”7n5kz”:{“id”:”7n5kz”,”image”:””,”imageId”:””,”title”:”B.\tVertical vein with unobstructed flow”},”un2q4″:{“id”:”un2q4″,”image”:””,”imageId”:””,”title”:”C.\tPersistent pulmonary hypertension”,”isCorrect”:”1″}}}}}}

{“questions”:{“xxwqj”:{“id”:”xxwqj”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Anila B. Elliott, MD – University of Michigan, C.S. Mott Children\u2019s Hospital \r\nA 5-month-old male with a history of Tetralogy of Fallot (TOF) underwent complete repair with transannular patch and VSD closure. On arrival to the intensive care unit, the ECG shows narrow complex tachycardia with AV dissociation and heart rates between 170-210 beats per minute (bpm), with a systolic blood pressure in the 60s. Axillary temperature is 35.4 degrees celsius and most recent labs are within normal limits, with a potassium of 4.2 and magnesium 2.7. An amiodarone infusion is started after a loading dose. Which of the following is the BEST NEXT course of action?”,”desc”:”EXPLANATION \r\nJunctional ectopic tachycardia (JET) is a rare arrhythmia often seen in patients with congenital cardiac disease, originating from the atrioventricular (AV) junction or bundle of His^{1<\/sup>. JET is characterized by abnormal automaticity rather than reentrant mechanisms. It presents as a narrow complex tachycardia with AV dissociation, often at rates of 200-250bpm1<\/sup>. ECG findings include gradual acceleration and deceleration, distinguishing it from other tachyarrhythmias. Hemodynamic compromise is common due to rapid ventricular rates and loss of synchronized atrial contraction, resulting in decreased cardiac output. \r\n\r\nThere are two types of JET: congenital and post-operative. Congenital JET presents without a history of cardiac surgery with etiologies ranging from genetic predisposition, intrinsic abnormalities of the conduction system, ion channel dysfunction or histopathologic changes such as AV nodal fibrosis. Congenital JET tends to be refractory to treatment, increasing the risk of heart failure and sudden cardiac death. Mortality has been reported as high as 35% in untreated or refractory cases of congenital JET. Post-operative JET typically occurs within the first 72 hours following congenital cardiac surgery3<\/sup>. Mechanisms for post-op JET include ischemia, mechanical stretching or trauma to the AV conduction system. Certain anatomical features and surgical procedures increase the risk, with those that involve structures near the AV node having the highest risk. Although post-operative JET is self-limited and will resolve spontaneously within 5-7 days, it can lead to significant morbidity and mortality3<\/sup>.\r\n\r\nA summary of risk factors that increase the risk of post-operative JET1<\/sup>: \r\n1.\tType of procedure (Repair of TOF, double-outlet right ventricle (DORV), ventricular septal defect (VSD), atrioventricular septal defect (AVSD), transposition of the great vessels) \r\n2.\tAge < 6 months \r\n3.\tHigher post-operative core temperature \r\n4.\tUse of inotropes (epinephrine, milrinone, dopamine) \r\n5.\tElectrolyte abnormalities (hypokalemia, hypomagnesemia) \r\n6.\tProlonged cardiopulmonary bypass times (\u2265 75 minutes)\r\n\r\nThe three main goals of managing JET are: rate control, restoring AV synchrony, and treatment of any underlying causes. Treatment focuses on correcting electrolyte abnormalities (specifically potassium, magnesium and calcium) administering antiarrhythmics, sedation, and cooling3<\/sup>. In refractory cases, catheter-directed ablation can be utilized. Although adenosine will not terminate JET, it may slow down the rhythm enough to confirm the absence of p waves, helping to distinguish it from other tachyarrhythmias. \r\n\r\nTreatment of JET includes the following1-4<\/sup>: \r\n1.\tReplace potassium, magnesium, and calcium as needed \r\n2.\tCorrect acid-base status (especially metabolic acidosis) \r\n3.\tDecrease inotropic agents if appropriate \r\n4.\tAvoid\/treat fever and consider instituting mild therapeutic hypothermia (34-35 degrees)\r\n5.\tAmiodarone (loading dose: 5mg\/kg over 30 minutes or slower, infusion 10-20mg\/kg\/day)\r\n6.\tConsider sedation (i.e. dexmedetomidine) to reduce sympathetic tone \r\n7.\tPacing to restore AV synchrony \r\n\r\nStudies have found other common agents used in anesthetic practice may be helpful in management, such as dexmedetomidine. In a recent study, prophylactic dexmedetomidine significantly lowered the rate of postoperative JET compared to placebo (3.3% versus 16.7%)4<\/sup>. Pacing can restore AV synchrony, suppressing the junctional automaticity, allowing for improvement in hemodynamics1<\/sup>. \r\n\r\nAcross major pediatric cardiac critical care centers, both medication and pacing are the most commonly utilized treatments. It is important to note that there may be significant variation in institutional practices for JET management. For example, although amiodarone is widely considered the first-line drug5<\/sup>, other medications, such as procainamide, are also used successfully. Cooling was used less frequently and typically in conjunction with other therapies, rarely as the sole management strategy4<\/sup>. \r\n\r\nThe correct answer is choice A, AV sequential pacing. The serum magnesium is within normal limits, and other treatment modalities are warranted prior to considering catheter-directed ablation for post-operative JET. \r\n\r\n \r\nREFERENCES \r\n\r\n1.\tHoffman, TM., Bush, DM., Wernovsky, G., et al. Postoperative junctional ectopic tachycardia in children: incidence, risk factors, and treatment. Ann Thorac Surg<\/em> 2002; 74(5): 1607-1611 \r\n2.\tSasikumar, N., Kumar, RK., Balaji, S. Diagnosis and management of junctional ectopic tachycardia in children. Ann of Pediatr Cardiol<\/em>. 2021; 14: 372-381\r\n3.\tKim, ME., Baskar, S., Janson, CM., et al. Epidemiology of postoperative junctional ectopic tachycardia in infants undergoing cardiac surgery. Ann Thorac Surg<\/em>. 2024; 117: 1178-1186 \r\n4.\tEl Amrousy, DM., Elshmaa, NS., El-Kashlan, M., et al. Efficacy of prophylactic dexmedetomidine in preventing postoperative junctional ectopic tachycardia after pediatric cardiac surgery. J Am Heart Assoc<\/em>. 2017; 6(3): e004780 \r\n5.\tEntenmann A, Michel M, Herberg U, et al. Management of postoperative junctional ectopic tachycardia in pediatric patients: a survey of 30 centers in Germany, Austria, and Switzerland. Eur J Pediatr<\/em>. 2017;176(9):1217-1226. doi:10.1007\/s00431-017-2969-x\r\n”,”hint”:””,”answers”:{“srx8p”:{“id”:”srx8p”,”image”:””,”imageId”:””,”title”:”A.\tAtrioventricular sequential pacing”,”isCorrect”:”1″},”q0x42″:{“id”:”q0x42″,”image”:””,”imageId”:””,”title”:”B.\t30mg\/kg IV magnesium sulfate”},”vtbhr”:{“id”:”vtbhr”,”image”:””,”imageId”:””,”title”:”C.\tConsult electrophysiology (EP) team for catheter ablation”}}}}}}

{“questions”:{“0bt44”:{“id”:”0bt44″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Amy Babb, MD AND Amanpreet Kalsi, MBBS, FRCA – Vanderbilt University Medical Center – Monroe Carell Jr. Children’s Hospital at Vanderbilt \r\nA 1-year-old patient is diagnosed with congenitally corrected transposition of the great arteries (cc-TGA) with intact ventricular septum and no pulmonary stenosis. Which of the following is the MOST appropriate initial surgical strategy with the end-goal of complete anatomic correction?”,”desc”:”EXPLANATION \r\nCongenitally corrected transposition of the great arteries (cc-TGA) or L-TGA is defined by the presence of both atrioventricular and ventriculoarterial discordance secondary to abnormal looping of the ventricles during embryologic development. The abnormal looping results in normally positioned atria, with the morphologic left ventricle (LV) in the subpulmonary position and the morphologic right ventricle (RV) in the subaortic position. The great vessels are L-looped with the aorta anterior and to the left of the pulmonary artery. Although this results in a physiologically \u201cnormal\u201d or \u201ccorrected\u201d circulation, it is associated with long-term morbidity and mortality due to failure of the morphologic right ventricle as the systemic ventricle and the development of tricuspid regurgitation.\r\n\r\n\r\nL-TGA is commonly associated with other cardiac lesions including a ventricular septal defect (VSD), subpulmonary stenosis, an \u201cEbstein-like\u201d tricuspid valve and conduction system anomalies. Roughly 75% of patients with cc-TGA will have a VSD and require surgical repair. A common approach involves restoring the morphologic LV as the systemic ventricle with a Rastelli approach and an atrial switch.^{1<\/sup> When a VSD is present, anatomic correction and VSD closure is predictably tolerated because the LV has been exposed to systemic pressures via the VSD. \r\n\r\n\r\nConversely, approximately 25% of patients with cc-TGA have an intact ventricular septum with a variable clinical course, ranging from minimal heart failure symptoms to significant RV dysfunction, tricuspid regurgitation and early death without surgical intervention.2<\/sup> Unfortunately, it remains difficult to predict which patients will develop heart failure, leading some experts to advocate for early surgical intervention to restore \u201cnormal cardiac anatomy\u201d.3<\/sup> The double switch operation describes one such approach where both an atrial baffle (Senning\/Mustard procedures) and arterial switch result in the morphologic left ventricle as the systemic ventricle.1<\/sup> \r\n\r\n\r\nFor the double switch operation to be successful, the morphologic left ventricle must be able to support a full cardiac output with normal systemic vascular resistance. However, the LV of patients born with cc-TGA and intact ventricular septum involutes within a few weeks of life due to the rapidly falling pulmonary vascular resistance, and may become unable to pump against a higher afterload if acutely challenged.3<\/sup> As a result, the first surgical procedure for these patients should be pulmonary artery (PA) band placement to increase morphologic LV afterload and stimulate muscle growth prior to pursuing a double switch procedure.1-4<\/sup> Some patients will require multiple PA band titrations before the LV is considered adequate for a double switch procedure.3<\/sup> Older patients have a lower likelihood of successful LV re-training, but some experts still advocate for PA banding in an effort to preserve tricuspid valve competence,2,4<\/sup> as the increased afterload to the subpulmonic ventricle creates septal shift, improving the morphological RV geometry. \r\n\r\n\r\nThe double switch operation would not be a successful option in a 1 year-old with cc-TGA and intact ventricular septum due to potential LV \u201cdetraining\u201d and a central shunt is not appropriate in a child with biventricular circulation without outflow stenoses or septation defects. \r\n\r\n\r\n \r\nREFERENCES \r\n1.\tMainwaring R, Hanley F. Double Switch With Hemi-Mustard and Bidirectional Glenn Procedure for \u201cAnatomical\u201d Repair of Corrected Transposition. Operative Techniques in Thoracic and Cardiovascular Surgery<\/em>. 2013; 18(3): 171 \u2013 189. \r\n\r\n2.\tCui H, Hage A, Piekarski B, Marx G, et al. Management of Congenitally Corrected Transposition of the Great Arteries with Intact Ventricular Septum: Anatomic Repair or Palliative Treatment?Circulation: Cardiovascular Interventions<\/em>. 2021; 14(7): Page e010154. \r\n\r\n3.\tMac Felmly L, Mainwaring RD, Ho DY, Arunamata A, Algaze C, Hanley FL. Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining.World J Pediatr Congenit Heart Surg<\/em>. 2024;15(3):279-286. doi:10.1177\/21501351231224329 \r\n\r\n4.\tMainwaring R, Patrick W, Arunamata A. et al. Left Ventricular Retraining in Corrected Transposition: Relationship between Pressure and Mass. The Journal of Thoracic and Cardiovascular Surgery<\/em>. 2020; 159(6): 2356-66. \r\n\r\n”,”hint”:””,”answers”:{“gf24w”:{“id”:”gf24w”,”image”:””,”imageId”:””,”title”:”A.\tDouble switch operation”},”x9cr9″:{“id”:”x9cr9″,”image”:””,”imageId”:””,”title”:”B.\tCentral shunt placement “},”glhph”:{“id”:”glhph”,”image”:””,”imageId”:””,”title”:”C.\tPulmonary artery banding”,”isCorrect”:”1″}}}}}}