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

{“questions”:{“1df20”:{“id”:”1df20″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Krupa Desai, MD \u2013 Children\u2019s Hospital of Philadelphia; Chinwe Unegbu, MD \u2013 Children\u2019s National Hospital\r\n\r\nA 14 year old male with Pulmonary Arterial Hypertension secondary to pulmonary venoocclusive disease managed with treprostinil, ambrisentan, and milrinone presents with increasing dyspnea. A transthoracic echocardiogram (TTE) demonstrates suprasystemic right ventricular (RV) systolic pressures, moderately decreased RV function, severe RV dilation, and moderate tricuspid regurgitation. What is the MOST APPROPRIATE treatment? \r\n”,”desc”:””,”hint”:””,”answers”:{“ei2nd”:{“id”:”ei2nd”,”image”:””,”imageId”:””,”title”:”A. Start epoprostenol”},”mdhsi”:{“id”:”mdhsi”,”image”:””,”imageId”:””,”title”:”B. Perform a balloon atrial septostomy (BAS) “},”hs7bc”:{“id”:”hs7bc”,”image”:””,”imageId”:””,”title”:”C. Placement of a Potts shunt”,”isCorrect”:”1″},”w9rgc”:{“id”:”w9rgc”,”image”:””,”imageId”:””,”title”:”D. Start inhaled nitric oxide”}}}},”results”:{“yid37”:{“id”:”yid37″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Pulmonary arterial hypertension (PAH) is a rare and progressive disease. Despite advances in medical therapy, there is significant morbidity and mortality associated with this disease. Patients with PAH often have elevations in pulmonary vascular resistance (PVR) because of pulmonary vascular bed remodeling. As this disease naturally progresses, the physiologic sequelae are increased PVR followed by right ventricular (RV) hypertrophy, RV dysfunction, and ultimately death. \r\n\r\nPulmonary veno-occlusive disease (PVOD) falls into a rare category of PAH and is caused by remodeling of the pulmonary venules with hallmark obliteration of the small pulmonary veins. The diagnosis of PAH secondary to PVOD carries a poor prognosis. In patients with PVOD, severe\/life-threatening pulmonary edema can occur with the initiation of any class of pulmonary vasodilators. To date, strong evidence of the beneficial effect of pulmonary vasodilator therapy in patients with PVOD is lacking. \r\n\r\n\r\nThe goal of medical therapy in patients with PAH is to dilate the pulmonary vascular bed, reverse vascular remodeling, and restore endothelial function. The common treatment modalities target specific pathways involved in PAH pathogenesis: the prostacyclin, endothelin-1, and nitric oxide pathways to achieve these goals. This patient is on treprostinil, ambrisentan, and milrinone. Treprostinil is a prostacyclin analogue commonly administered to patients with PAH via continuous intravenous infusion. Prostacyclin analogues are important pulmonary and systemic vasodilators and patients with PAH often have decreased production of prostacyclins. Epoprostenol and treprostinil are the two most commonly administered prostacyclin analogues. Treprostinil has a longer half-life and greater stability than epoprostenol. \r\n\r\n\r\nEndothelin (ET) is a peptide produced in the kidney with three isoforms – ET-1, ET-2, and ET-3. ET-1 is the predominant isoform and binds to two G-protein-coupled receptors ET_{A<\/sub> and ETB<\/sub>. ETA<\/sub> is the predominate form on vasculature and mediates vasoconstriction. Ambrisentan is a selective ETA<\/sub> receptor antagonist. Patients with PAH have increased levels of circulating ET-1 that bind to ETA<\/sub> to mediate pulmonary arterial vasoconstriction and promote proliferation of pulmonary vascular smooth muscle. Bosentan is a commonly utilized nonselective ETA<\/sub> and ETB<\/sub> receptor antagonist. The renal vascular endothelium expresses the ETB<\/sub> receptor and here ET-1acts in a manner to increase vasodilator secretion. \r\n\r\n\r\nMilrinone is an additional outpatient medical therapy for this patient. Milrinone is a phosphodiesterase type-3 inhibitor that decreases pulmonary vascular resistance via agonism of the nitric oxide\u2013cyclic guanosine monophosphate (NO\u2013cGMP) pathway. In addition to causing pulmonary and systemic vasodilation, milrinone can potentially augment myocardial contractility and lusitropy. \r\n\r\n\r\nUnfortunately despite maximal medical therapy, some patients progress to end-stage PAH and are quite symptomatic. Bilateral lung transplantation is the only definitive therapy that offers the possibility of long term survival, but survival to lung transplantation is low. The Potts shunt and balloon atrial septostomy (BAS) are two palliative procedures that can be used to manage medically refractory PAH. The patient in this scenario is failing medical therapy and is in need of a palliative procedure to reduce afterload on the right ventricle (RV). RV function is an important prognostic indicator in patients with PAH. Many medical therapies aim to reduce right ventricular afterload as a means to improve right ventricular function. \r\n\r\n\r\nOf the options listed, the most appropriate treatment is placement of a Potts shunt, which is a connection from the left pulmonary artery to the descending thoracic aorta. This surgical technique was first described in 1946 by Dr. Willis Potts. It was originally intended to be used in children with Tetralogy of Fallot to provide pulmonary blood flow. In 2004, it was utilized to manage two children with refractory suprasystemic PAH. \r\n\r\n\r\nA Potts shunt should be considered in patients with end stage, medically refractory, suprasystemic pulmonary hypertension. A Potts shunt would serve as a pop off for the right ventricle to reduce RV afterload thereby improving RV systolic function and possibly serving as a bridge to lung transplantation. Current literature demonstrates that the Potts shunt improves hemodynamics, functional status, and transplant-free survival in children with severe PAH. The Potts shunt is also more durable in teenagers and decompresses the right ventricle without causing upper body cyanosis. This shunt sends deoxygenated blood to the lower half of the body while preserving the highest oxygenated blood for the upper half of the body and the brain. \r\n\r\n\r\nA single center retrospective review performed from 2016 to 2019 at Columbia University Medical Center-New York Presbyterian demonstrated 100% survival at 33 months after Potts shunt in 5 pediatric patients with supra-systemic pulmonary arterial hypertension. Further studies are needed to assess long-term survival in patients with a Potts shunt. It is critical to remember that a Potts shunt, in theory, is an unrestrictive communication between the pulmonary and systemic circulations; therefore, its effectiveness lies in reducing right ventricular pressures from suprasystemic to systemic levels. Thus, a shunt placed in a child whose right ventricle cannot generate systemic pressure will prove ineffective. A valved Potts shunt is a modification that allows for unidirectional flow from pulmonary artery to the descending thoracic aorta. This prevents any reversal of flow from the aorta to the pulmonary circulation during diastole. The valved Potts shunt may serve to benefit the child who has systemic right ventricular pressures at rest but suprasystemic pressures with exertion. \r\n\r\n\r\nBalloon atrial septostomy (BAS) is an interventional procedure in which an interatrial orifice is created by needle puncture and dilated with a balloon catheter. Possible contraindications for BAS include: severe RV failure, mean right atrial pressure greater than 20 mmHg, pulmonary vascular resistance index greater than 55U\/m^{2<\/sup>, baseline oxygen saturation less than 90%, and LVEDP greater than 18mmHg. However, these criteria vary based on the center surveyed. Unlike the Potts shunt, which leads to lower extremity deoxygenation, the atrial septostomy leads to global deoxygenation. BAS is not the most appropriate intervention for this patient as studies have shown that BAS does not provide a lasting reduction of pulmonary artery pressure to preserve right ventricular function as does the Potts shunt. A patient with PAH who improves with BAS is often one in whom right ventricular failure is relatively advanced such that the right ventricular end diastolic pressure must be abnormally elevated to generate right-to-left atrial flow. Literature does support an improvement in symptoms after BAS in pediatric patients with subsystemic right ventricular pressure who had experienced syncope. The overall procedure-related mortality is high (16%) for BAS with refractory hypoxemia being the most common cause of death. The procedure may also need to be repeated due to spontaneous closure of the atrial septal defect. \r\n\r\n\r\nInitiation of epoprostenol, a prostacyclin analogue, will unlikely provide additional benefit since the patient is already on treprostinil, which is in the same category of medications. Initiation of inhaled nitric oxide will likely not provide additional benefit in a patient with pulmonary veno-occlusive disease, which is a fixed obstruction. Placement of a Potts shunt is the most appropriate treatment in a patient with medically refractory, suprasystemic pulmonary arterial hypertension in order to decrease the RV afterload. \r\n\r\n\r\nReferences \r\n\r\n1.\t van Loon RL, Roofthooft MT, Hillege HL, et al. Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation<\/em>. 2011; 124(16): 1755-1764. \r\n\r\n\r\n2.\t Montani D, Lau EM, Dorfm\u00fcller P, et al. Pulmonary veno-occlusive disease. Eur Respir J<\/em>. 2016; 47(5): 1518-1534. doi:10.1183\/13993003.00026-2016 \r\n\r\n\r\n\r\n3.\t Tuder RM, Cool CD, Geraci MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension.\u202f Am J Respir Crit Care Med<\/em>.\u202f1999; 159(6): 1925-1932. \r\n\r\n\r\n4.\t Cacoub P, Dorent R, Nataf P, Carayon A. Endothelin-1 in pulmonary hypertension. N Engl J Med<\/em>.\u202f1993; 329(26): 1967-1968. \r\n\r\n\r\n5.\t Maguire JJ, Davenport AP. Endothelin receptors and their antagonists. Semin Nephrol<\/em>. 2015; 35(2): 125-136. \r\n\r\n\r\n6.\t Aggarwal M, Grady RM, Choudhry S, Anwar S, Eghtesady P, Singh GK. Potts Shunt Improves Right Ventricular Function and Coupling With Pulmonary Circulation in Children With Suprasystemic Pulmonary Arterial Hypertension. Circ Cardiovasc Imaging<\/em>. 2018; 11(12): e007964. doi:10.1161\/CIRCIMAGING.118.007964 \r\n\r\n\r\n7.\t Garekar S, Meeran T, Dhake S, Malankar D. Valved reverse Potts shunt in a case of pulmonary hypertension due to pulmonary veno-occlusive disease. Indian J Thorac Cardiovasc Surg<\/em>. 2021; 37(1): 89-92. doi:10.1007\/s12055-020-00993-2 \r\n\r\n\r\n8.\tGrady RM. Beyond transplant: Roles of atrial septostomy and Potts shunt in pediatric pulmonary hypertension. Pediatr Pulmonol<\/em>. 2021; 56(3): 656-660. doi:10.1002\/ppul.25049 \r\n\r\n\r\n9.\tKeogh AM, Mayer E, Benza RL, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol<\/em>. 2009; 54(1 Suppl): S67-S77. doi:10.1016\/j.jacc.2009.04.016 \r\n\r\n\r\n10.\tKim SH, Jang WS, Lim HG, Kim YJ. Potts shunt in patients with primary pulmonary hypertension. Korean J Thorac Cardiovasc Surg<\/em>. 2015; 48(1): 52-54. doi:10.5090\/kjtcs.2015.48.1.52 \r\n\r\n\r\n11.\tRosenzweig EB, Ankola A, Krishnan U, Middlesworth W, Bacha E, Bacchetta M. A novel unidirectional-valved shunt approach for end-stage pulmonary arterial hypertension: Early experience in adolescents and adults. J Thorac Cardiovasc Surg<\/em>. 2021; 161(4): 1438-1446.e2. doi: 10.1016\/j.jtcvs.2019.10.149. Epub 2019 Nov 14. PMID: 31839227. \r\n”,”redirect_url”:””}}}}}

{“questions”:{“7q7ak”:{“id”:”7q7ak”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB, ChB, FRCA \u2013 Children\u2019s Medical Center, Dallas\r\n\r\nWhat is the MOST COMMON indication for surgical or catheter-based interventions following an arterial switch operation?\r\n\r\n”,”desc”:””,”hint”:””,”answers”:{“jbj6u”:{“id”:”jbj6u”,”image”:””,”imageId”:””,”title”:”A.\tCoronary stenosis”},”9kdwv”:{“id”:”9kdwv”,”image”:””,”imageId”:””,”title”:”B.\tNeoaortic root dilatation”},”1x38l”:{“id”:”1x38l”,”image”:””,”imageId”:””,”title”:”C.\tNeoaortic valve dilatation”},”ih5e9″:{“id”:”ih5e9″,”image”:””,”imageId”:””,”title”:”D.\tRight ventricular outflow tract obstruction”,”isCorrect”:”1″}}}},”results”:{“q2zi8”:{“id”:”q2zi8″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”The arterial switch operation (ASO) was introduced in 1975 and is now the standard surgical procedure for simple and complex forms of Transposition of the Great Arteries (TGA). Long-term survival is over 95% at 10 and 25 years with an excellent quality of life. Two recent single-center studies investigating the long term complications up to 40 years after an ASO have confirmed previously published findings and reveal new insights, emphasizing the need for life-long follow-up. \r\n\r\n\r\nThe most common indication for surgical or catheter-based interventions after an ASO is right ventricular outflow tract obstruction (RVOTO). RVOTO is found most frequently at the level of the branch pulmonary arteries (PAs) but also at the pulmonary valve and main pulmonary artery. Balloon dilation is effective but has a high recurrence rate of re-stenosis which then necessitates stent placement. The onset of pulmonary stenosis can be as early as 30 days or as late as 10 years after the initial operation. There are several reasons hypothesized as the cause of RVOTO. One potential cause is the LeCompte maneuver which when performed by the surgeon relocates the pulmonary trunk and branches over the aortic root. If the aortic root or ascending aorta becomes dilated, there may be resultant PA compression. Other potential contributing factors to RVOTO include extent of mobilization of the PAs, inadequate dissection of the branch PAs, age at the time of operation, and size mismatch of the switched great vessels.. In one recent study, surgical correction of RVOTO was required in 50% of patients and catheter-based interventions in 76% of patients who had previously undergone an ASO. \r\n\r\n\r\nThe second most common indication for reoperation or reintervention is neoaortic valve regurgitation with neoaortic root dilatation. Approximately 15%-18% of patients require surgical intervention with neoaortic valve replacement or repair and\/or ascending aorta replacement, such as a Bentall procedure. \r\n\r\n\r\nCoronary stenosis and coronary ischemia necessitating revascularization are the most common causes of early mortality, but occur rarely, in approximately 2-6% of patients, as long-term complications after an ASO. \r\n \r\n\r\nReferences\r\n\r\n\r\n(1)\tVan der Palen RLF, Blom NA, Kuipers IM, et al. Long-term outcome after the arterial switch operation: 43 years of experience. Eur J Cardiothorac Surg<\/em>. 2021; 59(5): 968-977. \r\n\r\n\r\n(2)\tFricke TA, Buratto E, Weintraub RG, et al. Long-term outcomes of the arterial switch operation. [Published online ahead of print February 11 2021]. J Thorac Cardiovas Surg<\/em>. 2021. doi:10.1016\/j.jtcvs.2021.01.134. \r\n”,”redirect_url”:””}}}

{“questions”:{“sjmix”:{“id”:”sjmix”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB ChB, FRCA – Children\u2019s Medical Center, Dallas\r\n\r\n\r\nA 14-year-old boy with a history of Wolff-Parkinson-White (WPW) Syndrome is admitted to the hospital with acute appendicitis. He is brought to the operating room for an appendectomy and 30 seconds after intubation he develops atrial fibrillation. The blood pressure and heart rate are stable. Which of the following interventions is the MOST APPROPRIATE to treat the arrhythmia?\r\n”,”desc”:””,”hint”:””,”answers”:{“vz2ov”:{“id”:”vz2ov”,”image”:””,”imageId”:””,”title”:”A.\tAdenosine”},”t9kj1″:{“id”:”t9kj1″,”image”:””,”imageId”:””,”title”:”B.\tEsmolol”},”2690y”:{“id”:”2690y”,”image”:””,”imageId”:””,”title”:”C.\tDC cardioversion”},”vm4oq”:{“id”:”vm4oq”,”image”:””,”imageId”:””,”title”:”D.\tProcainamide”,”isCorrect”:”1″}}}},”results”:{“a6tpd”:{“id”:”a6tpd”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Wolff-Parkinson-White (WPW) syndrome is a form of pre-excitation with characteristic abnormalities on the ECG and an increased predisposition to tachyarrhythmias involving an accessory pathway. It results from the presence of one or more accessory pathways (AP) of conduction between the atria and the ventricles. The AP conducts electrical impulses faster resulting in a shorter PR interval in the surface ECG and has a shorter refractory period than the atrioventricular node (AVN). The typical findings of AP function in sinus rhythm are preexcitation, in which depolarization of the ventricles occurs in part or fully through the accessory pathway that is separate from the AVN and earlier than expected after atrial depolarization. This results in shortening of the PR interval and a delta wave followed by a prolonged or widened QRS complex. The short refractory period leads to more rapid transmission of atrial impulses, which can result in supraventricular tachycardia. The typical supraventricular tachycardia associated with WPW syndrome is atrioventricular reentrant or reciprocating tachycardia (AVRT). AVRT is further classified into orthodromic AVRT and antidromic AVRT. Pre-excited atrial fibrillation or atrial flutter with rapid ventricular response may also occur. \r\n\r\n\r\nThe perioperative period is a particularly high-risk time period in which arrhythmias are more likely to occur due to an imbalance in parasympathetic and sympathetic tone. For example, the administration of neostigmine which causes slowing of the heart rate due to decreased atrioventricular nodal conduction can divert conduction to the accessory pathway. Similarly, sympathetic stimulation due to pain, laryngoscopy or emergence from anesthesia can cause tachycardia with a resultant increased number of impulses being transmitted via the AP. \r\n\r\n\r\nAcute treatment of arrhythmias in the setting of WPW can be challenging. Patients are typically treated for symptomatic arrhythmias or if certain high-risk features are present in asymptomatic patients. Hemodynamically significant arrhythmias require immediate direct current (DC) cardioversion. Catheter ablation is almost always preferred for long-term prevention of recurrent arrhythmias involving an accessory pathway. In situations of stable hemodynamics and acute onset, pharmacological treatment is usually effective. If time allows or if there is any doubt about the diagnosis, expert cardiology consultation is warranted. The presence of an accessory pathway influences the choice of correct pharmacologic treatment. \r\n\r\n\r\nA stable narrow complex tachycardia typically results from orthodromic AVRT with antegrade conduction via the AVN followed by retrograde conduction along the AP. Orthodromic AVRT occurs in 90 to 95 percent of reentrant tachycardias linked with WPW syndrome. AV nodal blocking drugs are the first-line therapy. Adenosine is usually effective. Verapamil is also effective, but caution is advised in the setting of hypotension or diminished ventricular function. A short-acting beta-blocker such as esmolol is another option. \r\n\r\n\r\nA stable wide-complex tachycardia results from antidromic AVRT with antegrade conduction over the accessory pathway and onto the ventricles followed by retrograde conduction back to the atria via the AVN. Wide complex tachycardia may also result from orthodromic AVRT with aberrant QRS conduction resulting in a wide QRS complex. Stable wide complex tachycardia may also be ventricular tachycardia. In the case of antidromic AVRT, the best option is procainamide, which is classified as a sodium-channel blocker that slows conduction in both the AVN and the AP. If the exact mechanism of wide complex is not certain, the presumptive diagnosis should be ventricular tachycardia and treated accordingly. \r\n\r\n\r\nAtrial fibrillation (AF) can be very dangerous in the setting of WPW, as conduction of atrial impulses at rates of up to 500 beats per minute can result in ventricular tachycardia or ventricular fibrillation. In this setting, AV nodal blocking drugs such as adenosine, verapamil, digoxin and esmolol are contraindicated as their use will divert atrial impulses to the AP. The recommended first-line treatment is procainamide to restore sinus rhythm. For young children, the dose recommended is 10-15 milligrams per kilogram over 15 to 30 minutes followed by an infusion of 20 to 80 micrograms per kg per minute. If ineffective, an intravenous loading dose of amiodarone followed by an infusion can be attempted. However, amiodarone use should be carried out with caution as its av-nodal blocking properties may increase conduction via an accessory pathway. Practitioners should be prepared to treat degradation of atrial fibrillation to ventricular fibrillation with immediate defibrillation. Pharmacologic cardioversion of atrial fibrillation may be slow. While under anesthesia, DC cardioversion is still an appropriate option for treatment, even in the setting of a stable rhythm if resolution with pharmacologic agents does not occur within a short period of time. \r\n\r\n\r\nReferences\r\n\r\n\r\n(1)\tDi Biase, L, Walsh EP. Treatment of symptomatic arrhythmias associated with Wolff-Parkinson-White Syndrome. In UpToDate, Levy S, Knight BP (Eds), UpToDate<\/em>, Waltham, MA. (Accessed on June 17, 2021.) \r\n\r\n\r\n(2)\tDi Biase, L, Walsh EP. Wolff-Parkinson syndrome: Anatomy, epidemiology, clinical manifestations, and diagnosis. In UpToDate<\/em>, Levy S, Knight BP (Eds), UpToDate, Waltham, MA. (Accessed on June 17, 2021.) \r\n\r\n\r\n(3)\tDi Biase, L, Walsh EP.Wolff-Parkinson syndrome: Atrioventricular reentrant tachycardia (AVRT) associated with an accessory pathway. In UpToDate<\/em>, Levy S, Knight BP (Eds), UpToDate, Waltham, MA. (Accessed on June 17, 2021.) \r\n\r\n\r\n(4)\tJanuary CT, Wann LS, Alpert JS et al. ACC\/AHA Task Force Members. 2014 AHA\/ACC\/HRS Guideline for the management of patients with atrial fibrillation: executive summary: a report of the American College of Cardiology\/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation. 2014; 130(23): 2071-2104. \r\n\r\n\r\n(5)\tBengali R, Wellens HJJ, Jiang Y. Perioperative Management of the Wolff-Parkinson-White Syndrome. J Cardiothorac Vasc Anesth<\/em>. 2014; 28(5): 1375-1386. doi: 10.1053\/j.jvca.2014.02.003. Epub 2014 Jul 11. \r\n\r\n\r\n\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“ikzep”:{“id”:”ikzep”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2021\/06\/QOW-Pic-PNG.png”,”imageId”:”4775″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Sana Ullah, MB ChB, FRCA – Children\u2019s Medical Center, Dallas\r\n\r\nA 16-year-old girl had a syncopal episode at home while watching television. She had a concurrent viral illness with a high fever. There was no previous history of syncope or palpitations and no family history of sudden death. Vital signs after the event were the following:\r\nHeart rate: 80 beats per minute\r\nBlood pressure: 120\/70\r\nPulse oximetry 97% on room air\r\nA 12-lead ECG was completed \u2013 see image below. An echocardiogram revealed no structural cardiac abnormalities. What is the MOST APPROPRIATE next step in the management of this patient?\r\n”,”desc”:””,”hint”:””,”answers”:{“nroyn”:{“id”:”nroyn”,”image”:””,”imageId”:””,”title”:”A.\tCT scan of the brain”},”ah6ee”:{“id”:”ah6ee”,”image”:””,”imageId”:””,”title”:”B.\tWatchful waiting & follow-up in an outpatient clinic”},”wxapu”:{“id”:”wxapu”,”image”:””,”imageId”:””,”title”:”C.\tCardiac MRI”},”ptyi8″:{“id”:”ptyi8″,”image”:””,”imageId”:””,”title”:”D.\tICD placement”,”isCorrect”:”1″}}}},”results”:{“mis1h”:{“id”:”mis1h”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”The ECG is consistent with a diagnosis of Brugada Syndrome (BrS). First described in 1992, BrS is an inherited channelopathy of the cardiac sodium channels. It is associated with a characteristic ECG pattern of \u201ccoved-type\u201d ST-elevation in leads V1-V3 and a right bundle branch pattern, which predisposes to a high risk of sudden death due to ventricular tachycardia or ventricular fibrillation [1, 2]. Sudden death frequently occurs at rest or during sleep. It has frequently been seen in young Asian males where it has been termed \u201csudden unexplained nocturnal death syndrome.\u201d Fever is a well-recognized precipitating factor, although the precise mechanism is still debated. In approximately 20% of patients, it is associated with a loss of function mutation in the SCN5A gene, which is also found in Long QT3 syndrome. In a multi-institution study of 30 children with BrS, the two most common etiologies were family history of BrS and unexplained syncope that was precipitated by fever in half of those children [3]. Diagnosis is usually based on the clinical history and the pathognomonic ECG abnormalities. However, the ECG abnormalities may be intermittent, and in these cases, a specific Brugada ECG protocol may be necessary with the use of sodium channel blocking drugs such as ajmaline, procainamide or flecainide. An ECG during a febrile episode may also be helpful. \r\n\r\n\r\nTreatment of BrS includes general measures such as aggressively treating a febrile illness with antipyretic medication and avoidance of certain medications, which can be found on a website \u2013 www.brugadadrugs.org. \r\n\r\n\r\nFor adult patients with a diagnostic ECG, history of syncope or a family history of sudden death, the definitive treatment is ICD placement. If an ICD is contraindicated, quinidine has been used for treatment. \r\n\r\n \r\nIn children, the use of ICDs is less studied in comparison with adults and is a much more difficult clinical decision. Although ICDs can prevent sudden death, there is a significant incidence of device complications and inappropriate shocks [4]. \r\n\r\n\r\nIn this patient, a brain CT is unlikely to show any abnormality in the absence of a neurological deficit. Watchful waiting is not appropriate based on the history and diagnostic ECG. A cardiac MRI is unlikely to be helpful in the presence of a structurally normal heart as seen on echocardiography. However, a cardiac MRI does have utility if a structural cardiomyopathy is suspected as a cause of aborted sudden death. \r\n\r\n\r\nReferences\r\n\r\n\r\n1.\tBrugada J, Campuzano O, Arbelo E et al. Present status of Brugada syndrome. J Am Coll Cardiol<\/em>. 2018; 72: 1046-1059. \r\n\r\n2.\t Behere SP, Weindling SN. Brugada syndrome in children \u2013 Stepping into unchartered territory. Ann Pediat Card<\/em>. 2017; 10: 248-258. \r\n\r\n3.\t Probst V, Denjoy I, Meregalli PG et al. Clinical aspects and prognosis of Brugada Syndrome in children. Circulation<\/em>. 2007; 115: 2042-2048. \r\n\r\n4.\t Corcia MCG, Sieira J, Pappaert G et al. Implantable cardioverter-defibrillators in children and adolescents with Brugada syndrome. J Am Coll Cardiol<\/em>. 2018; 71: 148-157.\r\n\r\n\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“089do”:{“id”:”089do”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Sana Ullah, MB ChB, FRCA – Children\u2019s Medical Center, Dallas\r\n\r\nA 15-year-old female with a history of orthotopic heart transplantation 2 months prior presents with intermittent fevers, abdominal pain, diarrhea and enlarged lymph nodes in the neck. Post-transplant lymphoproliferative disease (PTLD) is confirmed by lymph node biopsy and a PET-CT scan of her body. Which of the following risk factors in the recipient at the time of heart transplantation is MOST LIKELY associated with the development of PTLD? \r\n”,”desc”:””,”hint”:””,”answers”:{“em9dq”:{“id”:”em9dq”,”image”:””,”imageId”:””,”title”:”A.\tPositive cytomegalovirus (CMV) serology.”},”2hp62″:{“id”:”2hp62″,”image”:””,”imageId”:””,”title”:”B.\tPositive Epstein-Barr virus (EBV) serology. “},”ubqcf”:{“id”:”ubqcf”,”image”:””,”imageId”:””,”title”:”C.\tNegative EBV serology.”,”isCorrect”:”1″},”hyo9q”:{“id”:”hyo9q”,”image”:””,”imageId”:””,”title”:”D.\tHigh panel reactive antibody (PRA).”}}}},”results”:{“9gsnt”:{“id”:”9gsnt”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”While immunosuppressant therapy is critical for preventing organ rejection after transplantation, one of the serious risks is the development of various malignancies in the recipient as the body\u2019s immune surveillance systems are compromised. Many of these malignancies are caused by viruses, in particular Epstein-Barr virus (EBV) and Human Papilloma Virus. A study from 2006 showed the incidence of post-transplant lymphoproliferative disease (PTLD) is approximately 5% in children after heart transplant. The majority of these cases were of B-cell origin and of these, 87% were EBV positive. The probability of survival after diagnosis was 75% at 1 year and 68% at 3 years (1). The most common sites for PTLD are the upper airway (hypertrophy of tonsils and adenoids) and the gastrointestinal tract. \r\n\r\nRisk factors for PTLD include the following: (1) Negative EBV recipient and positive EBV donor serology, (2) younger age, as younger patients are more likely to be EBV-na\u00efve (EBV infections occur more frequently in older children and adults), and (3) multiple rejection episodes, as these require higher and more frequent doses of immunosuppressant medications. \r\n\r\nClinical features vary and include fever, weight loss, night sweats, gastrointestinal disturbances, hypertrophy of tonsils and adenoids, and lymph node enlargement in the neck, axilla and groin. Diagnostic evaluation includes clinical examination, chest x-ray, ultrasound of the abdomen and lymph nodes, computed tomography and magnetic resonance imaging, and lymph node biopsy. More recently, positron emission tomography-computed tomography has become the imaging modality of choice (2,3). \r\n\r\nTreatment depends on the type and severity of the disease but usually starts with a reduction of immunosuppressive drug therapy to allow the patient to mount an immune response to EBV. The obvious risk with this approach is acute rejection. Targeted therapy with rituximab, a chimeric anti-CD20 monoclonal antibody, can be used for CD20 positive B-cell lymphomas. \r\n\r\nCytomegalovirus (CMV) is the most common viral infection after transplantation and is associated with an increased risk of rejection, coronary allograft vasculopathy, and graft failure (4). \r\n\r\nA high panel reactive antibody (PRA) titer is associated with an increased risk of rejection. \r\n\r\nReferences\r\n\r\n1)\tWebber SA, Naftel DC, Fricker FJ, et al. Pediatric Heart Transplant Study. Lymphoproliferative disorders after paediatric heart transplantation: a multi-institutional study. Lancet<\/em>. 2006; 367(9506): 233-239. \r\n\r\n2)\tSchubert S. Lymhoproliferative disorders in pediatric heart transplant recipients. In: Canter C, Everitt MD, Burch M et al. (Eds). ISHLT Monograph Series Vol 13. Pediatric Heart Transplantation<\/em>. UAB Printing; 2019. \r\n\r\n3)\tAddonizio LJ, Boyle GJ. Post-transplant malignancy: Risk factors, incidence, diagnosis, treatment. In: Canter C, Kirklin JK (Eds). ISHLT Monograph Series Vol. 2. Pediatric Heart Transplantation<\/em>. Elsevier; 2007. \r\n\r\n4)\tSchowengerdt KO, Azeka E. Infection following pediatric heart transplantation. In: Canter C, Kirklin JK (Eds). ISHLT Monograph Series Vol. 2. Pediatric Heart Transplantation<\/em>. Elsevier; 2007.\r\n\r\n\r\n”,”redirect_url”:””}}}