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

{“questions”:{“zj75s”:{“id”:”zj75s”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Gokul Thimmarayan, MD – Marshfield Clinic, Marshfield, WI and\r\nDestiny F Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR. \r\n\r\nA 1-day-old neonate with congenital complete heart block is scheduled for a pacemaker placement. Echocardiographic findings reveal a structurally normal heart. What is the MOST COMMON cause of isolated congenital complete heart block?”,”desc”:””,”hint”:””,”answers”:{“wlk9w”:{“id”:”wlk9w”,”image”:””,”imageId”:””,”title”:”A) Maternal metabolic disease”},”tbhj2″:{“id”:”tbhj2″,”image”:””,”imageId”:””,”title”:”B) Maternal autoimmune antibodies”,”isCorrect”:”1″},”eck2s”:{“id”:”eck2s”,”image”:””,”imageId”:””,”title”:”C) Maternal medication exposure during pregnancy”},”s3ht8″:{“id”:”s3ht8″,”image”:””,”imageId”:””,”title”:”D) Maternal viral infection during pregnancy”}}}},”results”:{“7zkfx”:{“id”:”7zkfx”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Congenital complete heart block (CCHB) is a rare disorder that is associated with high morbidity and mortality occurring in approximately 1:15-20,000 patients. CCHB describes atrioventricular block diagnosed in utero or during the first 27 days of life. Of the patients with CCHB, fourteen to forty-two percent of cases are associated with congenital heart disease (CHD). This may include congenitally corrected transposition of the great arteries, complete atrioventricular canal defect, and heterotaxy with left atrial isomerism. Maternal autoimmune antibody transfer is the most common cause (ninety-one percent) of CCHB in the remaining percentage of cases. Maternal factors like diabetes, medication exposure (anticonvulsants and retinoic acid), viral infections, and channelopathies are also associated with increased risk of CCHB in the offspring. \r\n\r\nAutoimmune CCHB is a manifestation arising from neonatal lupus. Passively transferred maternal autoantibodies to the ribonuclear protein RO (SS-A) and LA (SS-B) cause neonatal lupus. Women with positive antibody titers carry approximately a two percent risk of having a baby with neonatal cardiac lupus after a prior unaffected pregnancy and have a thirteen to eighteen percent risk in subsequent pregnancies. The autoantibodies bind to the cardiac conduction tissue causing inflammation and fibrosis leading to conduction defects. The antibodies are also postulated to affect myocardial cells resulting in cardiomyopathy and endocardial fibroelastosis. Slow heart rate in the fetus with no structural anomaly of the heart and positive maternal antibody is diagnostic of autoimmune CCHB. The vast majority of infants present with third-degree or complete heart block although the presentation may vary from first- to third- degree atrioventricular block. Autoimmune CCHB has an approximate mortality rate of nineteen percent in which a majority occur in utero. Risk of fetal mortality is increased with hydrops, diagnosis of CCHB at earlier than twenty weeks of gestation, ventricular escape rate less than fifty-five beats per minute , impaired left ventricular function, myocardial inflammation, and dilated cardiomyopathy. The presence of dilated cardiomyopathy is a poor prognostic factor. Noncardiac manifestations of neonatal lupus typically self-resolve with clearance of the maternal autoantibodies over the first months of life. \r\n\r\nFetal echocardiography with doppler is the standard diagnostic tool in evaluating CCHB. The assessment of cardiac anatomy, heart rate and mechanical function can deduce abnormalities of electrophysiologic conduction and cardiac structure. With early diagnosis, subsequent intervention is based on the severity of the heart block along with close follow-up and monitoring with serial fetal echocardiograms. Maternal corticosteroids, intravenous immunoglobulin therapy, beta-agonist therapy, and hydroxychloroquine have been used with varying degrees of success. Once CCHB occurs, it is mostly likely irreversible, necessitating pace-maker implantation. In fact, more than two-thirds of these patients require a permanent pacemaker, the majority of which are implanted in the neonatal period. \r\n\r\nReferences: \r\nSteinberg L. Congenital heart block. Card Electrophysiol Clin<\/em>. 2021; 13(4): 691-702. \r\n\r\nPruetz JD, Miller JC, Loeb GE, Silka MJ, Bar-Cohen Y, Chmait RH. Prenatal diagnosis and management of congenital complete heart block. Birth Defects Res<\/em>. 2019; 111(8): 380-388. \r\n\r\nFriedman DM, Duncanson LJ, Glickstein J, Buyon JP. A review of congenital heart block. Images Paediatr Cardiol<\/em> 2003; 5(3): 36-48. \r\n\r\nIzmirly PM, Chalumeau CN, Pisoni C, et al. Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent Anti-SSA\/RO associated cardiac manifestation of neonatal lupus. Circulation<\/em>. 2012; 126(1): 76-82.\r\n”,”redirect_url”:””}}}

{“questions”:{“hc55h”:{“id”:”hc55h”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Gokul Thimmarayan, MD – Marshfield Clinic, Marshfield, WI and\r\nDestiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR. \r\n\r\nA 1-week-old neonate with pulmonary atresia and ventricular septal defect undergoes a complete surgical repair. Postoperatively, the patient has persistent hypocalcemia and prolonged mechanical ventilation due to pneumonia. Subsequent workup demonstrates immunodeficiency. Which of the following syndromes is MOST LIKELY associated with this constellation of findings?\r\n”,”desc”:””,”hint”:””,”answers”:{“8gytg”:{“id”:”8gytg”,”image”:””,”imageId”:””,”title”:”A) 22q11.2 deletion syndrome”,”isCorrect”:”1″},”6n0bu”:{“id”:”6n0bu”,”image”:””,”imageId”:””,”title”:” B) CHARGE syndrome”},”qc38e”:{“id”:”qc38e”,”image”:””,”imageId”:””,”title”:”C) Williams syndrome”},”aj4h6″:{“id”:”aj4h6″,”image”:””,”imageId”:””,”title”:”D) Alagille syndrome”}}}},”results”:{“s50zx”:{“id”:”s50zx”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”22q11.2 deletion syndrome is the most common chromosomal microdeletion disorder affecting 1:2000 to 1:6000 live births. The microdeletion leads to the maldevelopment of structures derived from the third and fourth pharyngeal arches. It is associated with parathyroid aplasia or hypoplasia resulting in hypocalcemia; hypoplasia of the thymus leading to immunodeficiency; and conotruncal anomalies such as tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, interrupted aortic arch, and ventricular septal defect. It is also associated with palatal anomalies, facial dysmorphism, renal anomalies, speech and learning disabilities, and psychiatric illness. 22q11.2 deletion syndrome is a multisystem disorder with a heterogeneous presentation also known as DiGeorge syndrome, Velocardiofacial syndrome, and Conotruncal anomaly face syndrome. \r\n\r\nCongenital heart disease (CHD) is the most common cause of mortality in 22q11.2 deletion syndrome. Patients often require multiple cardiac surgeries due to complex congenital heart disease. The postoperative course may be complicated by prolonged mechanical ventilation, inotropic support, and prolonged intensive care unit and hospital stays. Hypocalcemia from parathyroid hypoplasia can be associated with perioperative hemodynamic instability and postoperative seizures. The incidence of hypocalcemia is higher in those patients with CHD than those without CHD. Thymic hypoplasia is associated with T-cell deficiency resulting in an increased risk of recurrent and severe infections. Immunoglobulin (IgG, IgM, IgA) deficiency is seen in 10% of the patients with 22q11.2 deletion syndrome. Transfusion of blood products in IgA deficient patients could lead to a severe anaphylactic reaction. Irradiated and leukocyte-depleted blood products should be used to reduce the risk of infection and prevent transfusion-associated graft vs host disease due to the immunodeficient state. Craniofacial abnormalities such as micrognathia, retrognathia, and cleft palate could cause difficulty during endotracheal intubation. Of the syndromes listed in the answer choices, this patient\u2019s findings of a conotruncal cardiac lesion, persistent hypocalcemia, and immunodeficiency are most consistent with 22q11.2 deletion syndrome. The other syndromes are briefly described below. \r\nCHARGE syndrome is an acronym for the multisystem genetic conditions: Coloboma, Heart defects, Atresia of choanae, Retardation of growth and mental development, Genitourinary anomalies and Ear malformations\/hearing loss. It occurs with an approximate frequency of 1:10,000 births. It is diagnosed clinically and confirmed by detection of a genetic mutation in the CHD7 gene on chromosome 8q12. Cardiac malformations are present in 75-85% of the patients. Conotruncal defects, atrioventriculoseptal defects, and aortic arch abnormalities are seen commonly in CHARGE syndrome. \r\nWilliams syndrome or Williams-Beuren syndrome (WS) is a multisystem disorder caused by the deletion of multiple genes in chromosome 7 including the elastin gene. Williams syndrome occurs in 1:7,500 to 10,000 births. Patients with WS have characteristic facial findings including flat nasal bridge, short upturned nose, periorbital puffiness, long philtrum and delicate chin. Smooth muscle cells in patients with WS produce a decreased amount of normal elastin resulting in an arterial media with many hypertrophied smooth muscle cells, thickening of the media of large arteries, and ultimately obstructive lesions. The most common lesion is supravalvular aortic stenosis that characteristically develops at the sinotubular junction. Stenosis can also occur in the pulmonary arteries, coronary arteries, aortic arch, descending aorta, renal arteries, and mesenteric arteries. Patients with Williams syndrome are known to have increased risk of sudden cardiac death, especially in the setting of sedation and anesthesia. This is mostly attributed to the presence of coronary artery stenosis and biventricular outflow tract obstruction. Hypertension, hypercalcemia, impaired growth and impaired cognition are other associated findings. \r\n\r\nAlagille syndrome is a rare autosomal dominant, multisystem disorder occurring in approximately 1:70,000 births. It is related to mutations in the JAG1 gene or the NOTCH2 gene. It is associated with a paucity of intrahepatic bile ducts leading to cholestasis and potential liver failure. Other abnormalities include cardiac anomalies such as peripheral pulmonary artery stenosis and tetralogy of Fallot; butterfly vertebrae; typical facial features such as prominent forehead, deep-set eyes with moderate hypertelorism, pointed chin, and straight nose with a bulbous tip; and vascular and renal anomalies. \r\n\r\nReferences: \r\nYeoh TY, Scavonetto F, Hamlin RJ, Burkhart HM, Sprung J, Weingarten TN. Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery. J Cardiothorac Vasc Anesth.<\/em> 2014; 28(4): 983-989. \r\n\r\nRayannavar A, Levitt Katz LE, Crowley TB, et al. Association of hypocalcemia with congenital heart disease in 22q11.2 deletion syndrome. Am J Med Genet A.<\/em> 2018; 176(10): 2099-2103. \r\n\r\nDavies EG. Immunodeficiency in DiGeorge Syndrome and Options for Treating Cases with Complete Athymia. Front Immunol.<\/em> 2013; 4: 322. \r\n\r\nNational Institute of Health. National Center for Advancing Translational Sciences. Genetic and Rare diseases Information Center. 22q11.2 deletion syndrome. Last updated 2017 https:\/\/rarediseases.info.nih.gov\/diseases\/10299\/22q112-deletion-syndrome Accessed October 25, 2021. \r\n\r\nHsu P, Ma A, Wilson M, et al. CHARGE syndrome: a review. J Paediatr Child Health.<\/em> 2014; 50(7): 504-511. \r\n\r\nPober BR. Williams-Beuren syndrome. N Engl J Med.<\/em> 2010; 362(3): 239-252. \r\n\r\nBurch TM, McGowan FX, Kussman, BD, Powell AJ, DiNardo JA. Congenital Supravalvar Aortic Stenosis and Sudden Death Associated with Anesthesia: What\u2019s the Mystery? Anesth Analg. 2008;107(6); 1848-1854. \r\n\r\nCollins Ii RT, Collins MG, Schmitz ML, Hamrick JT. Peri-procedural risk stratification and management of patients with Williams syndrome. Congenit Heart Dis.<\/em> 2017; 12(2): 133. \r\n\r\nSaleh M, Kamath BM, Chitayat D. Alagille syndrome: clinical perspectives. Appl Clin Genet.<\/em> 2016; 9: 75-82. \r\n\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“02yzs”:{“id”:”02yzs”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Anna Hartzog MD and Chinwe Unegbu MD \u2013 Children\u2019s National Hospital \r\n\r\nA 2-month-old infant with dilated cardiomyopathy presents for Left Ventricular Assist Device (LVAD) insertion. The surgeon implants a Levitronix CentriMag pump with Berlin Heart Excor cannulas. What is the MOST LIKELY benefit of initial placement of the CentriMag pump over initial insertion of the complete Berlin Excor system? \r\n\r\n”,”desc”:””,”hint”:””,”answers”:{“7nnsc”:{“id”:”7nnsc”,”image”:””,”imageId”:””,”title”:”A. Anticipated need for long-term VAD support”},”ljm8f”:{“id”:”ljm8f”,”image”:””,”imageId”:””,”title”:”B. No anticoagulation needed with the CentriMag pump “},”gxo1n”:{“id”:”gxo1n”,”image”:””,”imageId”:””,”title”:”C. Reduced hospital costs in the immediate perioperative period”,”isCorrect”:”1″},”g9xok”:{“id”:”g9xok”,”image”:””,”imageId”:””,”title”:”D. Improved chances of myocardial recovery “}}}},”results”:{“j3qmg”:{“id”:”j3qmg”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Approximately 20% to 30% of pediatric patients awaiting heart transplantation are bridged with a Ventricular Assist Device (VAD). This has resulted in a 50% reduction in the waitlist mortality. Candidacy for VAD implantation is often determined by serial monitoring for end-organ dysfunction (ie hepatic, renal, gastrointestinal dysfunction), nutritional status, level of inotropic support, and degree of respiratory compromise. Generally, VAD therapy should occur prior to the onset of severe end-organ dysfunction as this is an independent risk factor of mortality.\r\n\r\n\r\nSmall children and infants have limited device options. The majority of children who require a VAD are supported with either a pulsatile paracorporeal device or a continuous flow intracorporeal device. The Berlin Heart Excor VAD (Berlin Heart GmbH, Berlin, Germany) is a pulsatile paracorporeal device that is situated externally. The main continuous flow intracorporeal devices are the HeartWare HVAD (Medtronic, Minneapolis, MN) and the HeartMate 3 (Abbott Corporation, Abbott Park, IL) which have pumps that are implanted internally. The HeartWare HVAD and HeartMate 3 are currently US Food and Drug Administration-approved for the therapy of advanced heart failure in adults. However, both devices have been safely and successfully used in patients as small as twenty kilograms and body surface area of 0.6 m^{2<\/sup>. Continuous flow VADs have a 92% survival rate at 6 months, which is superior to the survival rates in pediatric patients with pulsatile devices.\r\n\r\n\r\nThe Berlin Heart Excor VAD is a pneumatically-driven, pulsatile pump with a fixed volume chamber ranging from 10 to 80 milliliters. During systole the pump moves compressed air into the pneumatic chamber causing ejection. In diastole negative pressure is applied to the pneumatic chamber to aid filling. Cardiac output is dependent on the size of the chamber and the pump rate. Unfortunately, complications such as stroke, bleeding, and infection are more common with the Berlin Heart EXCOR in comparison to continuous flow devices. \r\n\r\n\r\nIn addition to durable long-term VADs, temporary or short-term VADs also exist. Temporary circulatory support devices are traditionally defined as those providing support for two to four weeks as a \u201cbridge to recovery\u201d or \u201cbridge to decision\u201d in patients with an acute process. Temporary circulatory support devices now account for 19% of VAD implants. In 2018 Lorts et al used data from the Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) to investigate outcomes in patients temporarily supported by VADs. The authors found that 71% had a positive outcome (ie bridge to recovery, bridge to transplant, bridge to a durable device, or alive with a device). Additionally, the authors also discovered that \u201ctemporary\u201d devices were utilized for a wide variety of indications and for durations of time greater than six weeks.\r\n\r\n\r\nThe CentriMag\/PediMag (Levitronix LLC, Waltham, MA) is a third generation paracorporeal continuous flow device that is being used with increasing frequency for temporary circulatory support. The CentriMag is driven with a bearingless motor, enabling the spinning component within the pump to be magnetically levitated and rotated without contact or wear. By eliminating the bearings, shafts, and seals associated with a conventional centrifugal pump, the incidence of thrombus accumulation and the degree of hemolysis are greatly reduced, which decreases the risk of adverse events. As there is low risk of thromboembolic events, many centers use minimal anticoagulation. This is advantageous after VAD implantation as a significant percentage of patients require re-exploration due to bleeding. In contrast, the Berlin Heart Excor system requires early initiation of anticoagulation therapy. \r\n\r\n\r\nThe need for minimal anticoagulation and lower hospital-related costs have made the CentriMag pump in combination with the Berlin Heart Excor cannulas a logical choice in some centers. This combination allows the CentriMag pump to stabilize the patient over a period of several weeks. Then once stabilized, the Berlin Heart Excor pump and drive unit can replace the CentriMag. The pump exchange can be accomplished in one to two minutes. The CentriMag pump is less expensive with a cost of $10,000 to $13,000 per pump. In comparison, the Berlin Heart Excor pump costs $39,000 and the driver monthly rental is approximately $10,000. Immediately following VAD implantation, there is considerable inflammation leading to fibrin and clot build up that may necessitate several pump exchanges. Pump exchange is quite expensive, especially with use of the Berlin Excor system. The combined use of both systems can be viewed as a cost effective \u201cbridge to a bridge\u201d.\r\n\r\n\r\nChoice C is the correct answer as perioperative costs are reduced with the CentriMag. Choice A, B and D are incorrect as the Centrimag is not intended for long-term support, requires some form of anticoagulation, and is not associated with improved myocardial recovery.\r\n\r\n\r\nReferences\r\n\r\n1.\tNavaratnam M, Maeda K, Hollander SA. Pediatric ventricular assist devices: Bridge to a new era of perioperative care. Pediatric Anesthesia<\/em>. 2019; 29: 506\u2013518. doi:10.1111\/pan.13609.\r\n\r\n\r\n2.\tVanderPluym CJ, Adachi I, Niebler R, et al. Outcomes of children supported with an intracorporeal continuous-flow left ventricular assist system. J Heart Lung Transplant<\/em>. 2019; 38(4): 385-393.\r\n\r\n3.\tChatterjee A, Feldmann C, Hanke JS, et al. The momentum of HeartMate 3: a novel active magnetically levitated centrifugal left ventricular assist device (LVAD). J Thorac Dis<\/em>. 2018; 10(Suppl 15): S1790-S1793. \r\n\r\n4.\tO\u2019Connor MJ, Lorts A, Davies RR, et al. Early experience with the HeartMate 3 continuous flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis. J Heart Lung Transplant<\/em>. 2020; 39: 573-579.\r\n\r\n5.\tAlmond CS, Morales DL, Blackstone EH, et al. Berlin heart EXCOR pediatric ventricular assist device for bridge to heart transplantation in US children. Circulation<\/em>. 2013; 127: 1702\u20101711. doi: 10.1161\/CIRCULATIONAHA.112.000685. \r\n\r\n6.\tBlume ED, VanderPluym C, Lorts A, et al. Second annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) report: Pre\u2010implant characteristics and outcomes. J Heart Lung Transplant<\/em>. 2018; 37(1): 38\u201045. doi: 10.1016\/j.healun.2017.06.017.\r\n\r\n\r\n7.\tLorts A, Eghtesady P, Mehegan M, et al. Outcomes of children supported with devices labeled as \u201ctemporary\u201d or short term: A report from the Pediatric Interagency Registry For Mechanical Circulatory Support. J Heart Lung Transplant<\/em>. 2018; 37(1): 54\u201060. doi:10.1016\/j.healun.2017.10.023. \r\n\r\n8.\tStiller B, Lemmer J, Schubert S, et al. Management of pediatric patients after implantation of the Berlin Heart EXCOR ventricular assist device. ASAIO J<\/em>. 2006; 52(5): 497-500. PMID: 16966844.\r\n\r\n\r\n9.\tDe Rita F, Hasan A, Haynes S, et al. Outcome of mechanical cardiac support in children using more than one modality as a bridge to heart transplantation. Eur J Cardiothorac Surg<\/em>. 2015; 48: 917-922. doi:10.1093\/ejcts\/ezu544.\r\n\r\n10.\tConway J, Al-Aklabi M, Granoski D, et al. Supporting pediatric patients with short-term continuous-flow devices. J Heart Lung Transplant<\/em>. 2016; 35: 603-609. doi:10.1016\/j.healun.2016.01.1224. \r\n\r\n11.\tMaat A.P, van Thiel R.J, Dalinqhaus M, Bogers A.J. Connecting the CentriMag Levitronix pump to Berlin Heart Excor cannula. J Heart Lung Transplant<\/em>. 2008; 27: 112-115. doi: 10.1016\/j.healun.2007.10.010 \r\n\r\n\r\n12.\t Loforte A, Potapov E, Krabatsch T, et al. Levitronix CentriMag to Berlin Heart Excor: A \u201cBridge to Bridge\u201d Solution in Refractory Cardiogenic Shock. ASAIO J<\/em>. 2009; 55(5): 465-468. doi: 10.1097\/MAT.0b013e3181b58c50 \r\n\r\n13.\tJohn R, Long J, Massey T, et al. Outcomes of a multicenter trial of the Levitronix CentriMag ventricular assist system for short-term circulatory support. Mechanical Circulatory Support<\/em>. 2008; 27(1): 112-115. doi:https:\/\/doi.org\/10.1016\/j.jtcvs.2010.03.046 \r\n\r\n\r\n”,”redirect_url”:””}}}}

{“questions”:{“yv9hs”:{“id”:”yv9hs”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Anna Hartzog MD and Chinwe Unegbu MD \u2013 Children\u2019s National Hospital \r\n\r\nA 1-day old neonate with critical aortic stenosis and decreased left ventricular function is intubated due to respiratory failure, pulmonary edema and increasing left atrial pressures. The patient presents to the operating room for surgical repair. What is the GREATEST benefit of a surgical aortic valve repair over aortic balloon valvuloplasty? \r\n”,”desc”:””,”hint”:””,”answers”:{“m2h7l”:{“id”:”m2h7l”,”image”:””,”imageId”:””,”title”:”A. Decreased rate of aortic valve replacement”},”olcn2″:{“id”:”olcn2″,”image”:””,”imageId”:””,”title”:”B. Increased survival rate”},”vt433″:{“id”:”vt433″,”image”:””,”imageId”:””,”title”:”C. Decreased long-term aortic insufficiency “,”isCorrect”:”1″},”8eogh”:{“id”:”8eogh”,”image”:””,”imageId”:””,”title”:”D. Decreased length of hospital stay”}}}},”results”:{“wqsr7”:{“id”:”wqsr7″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Congenital aortic stenosis occurs in approximately 6% of patients with congenital heart disease. While valvar aortic stenosis is most common, stenosis can occur at the subvalvar, valvar, or supravalvar level. Valvar aortic stenosis is more common in males, and is often associated with a bicuspid aortic valve, coarctation of the aorta, patent ductus arteriosus, and ventricular septal defect. Unicuspid aortic valve is most often associated with critical aortic stenosis. If not recognized early, neonates with severe aortic stenosis can present with congestive heart failure, arrhythmias, lactic acidosis, cardiogenic shock, and even sudden death. \r\n\r\nThe size and function of the left ventricle is the main determinant for appropriateness of a single-ventricle versus a biventricular repair. Measurements that predict a favorable two-ventricle repair include aortic annulus \u2265 3.0 cm\/m^{2<\/sup>, aortic root \u2265 3.5 cm\/m2<\/sup>, mitral valve area \u2265 4.75 cm2<\/sup>\/m2<\/sup>, ratio of long axis of the left ventricle to the heart \u22650.8, and left ventricular cross-sectional area \u2265 2.0 cm2<\/sup>. The most common interventions which preserve biventricular anatomy include balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV). The goal of either intervention is to reduce the aortic valve gradient and prevent aortic regurgitation. \r\n\r\nBAV is performed in the cardiac catheterization lab. The aortic valve can be approached via the femoral, umbilical, or carotid arteries. It is recommended to use a balloon with a balloon to aortic valve annulus ratio of 0.8 to 1.0 to avoid over-dilation, which can result in significant aortic insufficiency (AI). A unicuspid aortic valve morphology is not always amenable to BAV due to increased risk of AI. After BAV, an immediate reduction in the peak pressure gradient across the valve is typically seen. The degree of immediate post-valvuloplasty insufficiency is predictive of late onset AI and suggests the likelihood for re-intervention. \r\n\r\nSAV is performed in the operating room with cardiopulmonary bypass, cardioplegia, and hypothermic myocardial protection. Early surgical techniques for SAV were associated with roughly 50% mortality. However, advancements in surgical technique have improved mortality to a 100% 10-year survival. If not amenable to SAV, a valve replacement with a prosthetic or allograft valve may be performed. Alternatively, the Ross procedure may be performed in which the patient\u2019s pulmonary valve replaces the aortic valve. Prerequisites to the Ross procedure include a normal right ventricular outflow tract and pulmonary valve. The disadvantage of the the Ross procedure is the risk of future aortic and pulmonary valve disease. \r\n\r\nEarly studies demonstrated equivocal outcomes in terms of survival and the need for re-intervention when comparing SAV and BAV. Advancements in surgical technique have demonstrated improved outcomes after SAV in more recent studies. In a study by Siddiqui et al, freedom from re-intervention at five years was 65% after SAV compared to 27% after BAV. Similar results were demonstrated in a meta-analysis by Hill et al in which BAV resulted in reduced gradient reduction, increased AI post-procedure, and greater need for subsequent intervention compared to SAV. Additionally, there was no difference in long-term survival and freedom from aortic valve replacement between the two groups, thus answer A is incorrect. The study also demonstrated a greater rate of additional intervention in the BAV group. \r\n\r\nA study by Hermann et al demonstrated that SAV resulted in greater gradient reduction, reduced long-term AI, and a lower re-intervention rate at 10 years compared to BAV. However, SAV has been associated with higher morbidity and longer hospital stay compared to BAV. Studies have demonstrated no difference in mortality between the two groups. As a result, in this question the correct answer is C because there is less long-term aortic insufficiency in the SAV group. There is no difference in survival or rate of valve replacement between the two groups. Patients undergoing SAV require a longer hospital stay. \r\n\r\nReferences: \r\n\r\nSpaeth JP and Loepke AW. Anesthesia for Left-sided Obstructive lesions. In: Andropoulos, D, Stayer S, Mossad EB, Miller-Hance WC. Anesthesia for Congenital Heart Disease<\/em>. Third Edition. Hoboken, New Jersey: John Wiley & Sons, Inc. 2015, 497-515. \r\n\r\nSingh, G. Congenital Aortic Valve Stenosis. Children (Basel)<\/em>. 2019; 6(5): 69. doi: 10.3390\/children6050069 \r\n\r\nHerrmann JH, Clark AJ, Colgate C, et al. Surgical valvuloplasty versus balloon dilation for congenital aortic stenosis in pediatric patients. World J Pediatr Congenit Heart Surg<\/em>. 2020; 11(4): 444-451. doi:10.1177\/2150135120918774. \r\n\r\nMcCrindle B, Blackson EH, Williams WG, et al. Are outcomes of surgical versus transcatheter balloon valvotomy equivalent in neonatal critical aortic stenosis? Circulation<\/em>. 2001; 104: I152-158. \r\n\r\nSiddiqui J, Brizard CP, Galati JC, et al. Surgical valvotomy and repair for neonatal and infant congenital aortic stenosis achieves better results than interventional catheterization. J Am Coll Cardiol<\/em>. 2013; 62(22): 2134-2140. doi:10.1016\/j.jacc.2013.07.052. \r\n\r\nHill G, Ginde S, Rios R, Frommelt PC, Hill KD. Surgical valvotomy versus balloon valvuloplasty for congenital aortic valve stenosis: A systematic review and meta-analysis. J Am Heart Assoc<\/em>. 2016; 5(8): e003931. doi: 10.1161\/JAHA.116.003931. \r\n\r\nBrown J, Rodefeld MD, Ruzmetov M, Eltayeb O, Yurdakok, Turrentine MW. Surgical valvuloplasty versus balloon aortic dilation for congenital aortic stenosis: Are evidence-based outcomes relevant? Ann Thorac Surg<\/em>. 2012; 94(1): 146-153. doi:10.1016\/j.athoracsur.2012.02.054. \r\n\r\n”,”redirect_url”:””}}}}

{“questions”:{“m0xg1”:{“id”:”m0xg1″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Anna Hartzog MD and Chinwe Unegbu MD \u2013 Children\u2019s National Hospital \r\n\r\nA 4-day-old full term infant is diagnosed with Tetralogy of Fallot (TOF) with severe pulmonary stenosis. What is the MOST LIKELY reason to select a two-stage repair with an initial modified Blalock Taussig shunt (mBTS) instead of a complete repair with a transannular patch? \r\n\r\n”,”desc”:””,”hint”:””,”answers”:{“c7jk9”:{“id”:”c7jk9″,”image”:””,”imageId”:””,”title”:”A. Decreased need for transannular patch “},”utrdp”:{“id”:”utrdp”,”image”:””,”imageId”:””,”title”:”B. Improved long-term outcomes “},”7m71a”:{“id”:”7m71a”,”image”:””,”imageId”:””,”title”:”C. Institutional preference”,”isCorrect”:”1″},”b1wlo”:{“id”:”b1wlo”,”image”:””,”imageId”:””,”title”:”D. Decreased pulmonary artery distortion “}}}},”results”:{“y2qal”:{“id”:”y2qal”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. It is characterized by four distinct anatomic features described by Etienne Fallot in 1888 including the following: 1) anterior malalignment ventricular septal defect, 2) right ventricular outflow tract obstruction, 3) overriding aorta, and 4) right ventricular hypertrophy. The anterior malalignment of the conoventricular septum produces the spectrum of disease which can range in severity from an asymptomatic neonate to one that is severely cyanotic and has ductal-dependent physiology. The severity of the right ventricular outflow tract obstruction (RVOTO) determines the degree of cyanosis. Most commonly, cyanosis is mild at birth and gradually progresses with age as there is an increase in infundibular hypertrophy. By six to twelve months of life, the cyanosis tends to be significant. A smaller percentage of patients with TOF and pulmonary stenosis (PS) have marked cyanosis soon after birth. In this population, the RVOTO is often secondary to a hypoplastic pulmonary valve annulus and the cyanosis is constant because of the fixed nature of the obstruction to pulmonary blood flow. \r\n\r\nTraditionally, there have been two surgical approaches to Tetralogy of Fallot with pulmonary stenosis including primary complete repair versus a two-stage repair with an aortopulmonary shunt followed by a complete repair either with a transannular patch or a valve sparing procedure. At present, the ideal age for complete correction of TOF remains elusive and is highly institution dependent. Historically, the approach to a neonate or infant with TOF has been to wait for surgical repair until symptoms develop or until the infant was older. The morbidity and mortality of the operation was thought to be less in an older patient. \r\n\r\nAn early primary repair restores a normal circulation, which can diminish the deleterious physiologic effects of TOF to the heart, lungs, brain and other organ systems. Studies have shown that normalizing pulmonary arterial flow early optimizes pulmonary angiogenesis and alveolar development. Additionally, due to the unrestrictive anterior malalignment VSD, the right ventricle is exposed to systemic pressure resulting in right ventricular hypertrophy and decreased compliance. There is some evidence that left ventricular function is compromised as well when repair is delayed. There is a lower incidence of ventricular arrhythmias among children repaired at younger ages. An additional benefit of primary repair is the avoidance of aortopulmonary shunt related complications such as pulmonary artery distortion, shunt thrombosis, congestive heart failure, and\/or pulmonary vascular disease. Thus, answer choice B and D are incorrect. At present, there is essentially no contraindication to early primary repair. Historically, indications for delayed repair include an anomalous coronary artery crossing the right ventricular outflow tract, hypoplastic or discontinuous pulmonary arteries, and multiple VSDs. \r\n\r\n\r\nDespite success with early primary surgical repair of TOF, a two-stage repair remains favored at many institutions. Thus, answer choice C is correct. This is primarily the result of institutional culture, experience, and outcomes. An additional reason to delay complete repair in favor of two-stage repair revolves around the physiologic sequelae of a transannular patch over time, which includes increased ventricular dimensions, decreased ventricular function, decreased exercise capacity, arrhythmias, heart failure, and sudden cardiac death. However, there is insufficient evidence to support the idea that a two-stage repair decreases the risk of later pulmonary valve replacement. The largest published series describing late-phase events in adults with repaired TOF demonstrated that the risk of later reoperation is independent of the type of initial repair. Likewise, there is no evidence to suggest that initial palliation with an aortopulmonary shunt results in a decreased incidence of later complete repair with a transannular patch. Thus, answer choice A is incorrect. \r\n\r\n\r\nAl Habib et al. analyzed contemporary patterns of management of TOF\/PS using The Society of Thoracic Surgeons database and demonstrated that procedure type in neonates was equally divided between primary repair and a two-stage approach. In addition, use of a transannular patch was the most prevalent surgical technique for both primary repair and for a 2-stage repair. The discharge mortality from TOF repair in neonates was not significantly different between palliation (6.2%) and primary repair (7.8%). Thus, answer choice B is incorrect. Kanter et al. examined outcomes in neonates with symptomatic TOF who underwent either a primary repair or a two-stage repair and found equivalent mortality rates. Additionally, the study demonstrated that shunted patients had fewer transannular patch repairs but this has not been a consistent finding across studies. Antegrade pulmonary blood flow is the primary stimulus for growth of right ventricular outflow tract structures and thus a palliative aortopulmonary shunt would perpetuate reduced pulmonary flow. Thus, the expectation would be that the pulmonary valve would become smaller with time in patients with an aortopulmonary shunt and increase the need for a transannular patch. Nonetheless, the debate about the effect of early primary repair with a transannular patch on the growth of the pulmonary valve annulus remains a hot topic. \r\n\r\nReferences \r\n\r\n1.\tBarratt-Boyes BG, Neutze JM. Primary repair of Tetralogy of Fallot in infancy using profound hypothermia with circulatory arrest and limited cardiopulmonary bypass: a comparison with conventional two stage management. Ann Surg<\/em>. 1973; 178: 406\u2013411. \r\n\r\n2.\tCastaneda AR, Freed MD, Williams RG, Norwood WI. Repair of Tetralogy of Fallot in infancy: early and late results. J Thorac Cardiovasc Surg<\/em>. 1977; 74: 372\u2013381. \r\n\r\n3.\tDi Donato RM, Jonas RA, Lang P, Rome JJ, Mayer JE Jr, Castaneda AR. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg<\/em>. 1991; 101(1):126-137. PMID: 1986154. \r\n\r\n4.\tSullivan ID, Presbitero P, Gooch VM, Arura E, Deanfield JE. Is ventricular arrhythmia in repaired tetralogy of Fallot an effect of operation or a consequence of the course of the disease? A prospective study. Br Heart J<\/em>. 1987; 58: 40\u201344.\r\n\r\n5.\tHegerty A, Anderson RH, Deanfield JE. Myocardial fibrosis in tetralogy of Fallot: effect of surgery or part of the natural history? Br Heart J<\/em>. 1988; 59: 123. \r\n\r\n6.\tJonas RA. Early primary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu<\/em>. 2009: 39-47.\r\n\r\n7.\tParry AJ, McElhinney DB, Kung GC, Reddy M, Brook MM, Hanley FL. Elective Primary Repair of Acyanotic Tetralogy of Fallot in Early Infancy: Overall Outcome and Impact on the Pulmonary Valve. J Am Coll Cardiol<\/em>. 2000; 36: 2279\u20132283. \r\n\r\n8.\tKirklin JW, Blackstone EH, Pacifico AD, Brown RN, Bargeron LM Jr. Routine primary repair vs two stage repair of tetralogy of Fallot. Circulation<\/em>. 1979; 60: 373\u2013386. \r\n\r\n9.\tVobecky SJ, Williams WG, Trusler GA, et al. Survival analysis of infants under age 18 months presenting with tetralogy of Fallot. Ann Thorac Surg<\/em>. 1993; 56: 944\u2013949. \r\n\r\n\r\n10.\tVan Arsdell GS, Maharaj GS, Tom J et al. What is the Optimal Age for Repair of Tetralogy of Fallot? Circulation<\/em>. 2000;102:Iii-123\u2013129. \r\n\r\n11.\tGladman G, McCrindle BW, Williams WG, et al. The modified Blalock-Taussig shunt: clinical impact and morbidity in Fallot\u2019s tetralogy in the current era. J Thorac Cardiovasc Surg<\/em>. 1997; 114: 25\u201330. \r\n\r\n12.\tUva MS, Chardigny C, Galetti L, et al. Surgery for tetralogy of Fallot at less than six months of age. Is palliation \”old-fashioned\u201d? Eur J Cardiothorac Surg<\/em>. 1995; 9(8): 453\u2013460. \r\n\r\n13.\tLee CH, Kwak JG, Lee C. Primary repair of symptomatic neonates with tetralogy of Fallot with or without pulmonary atresia. Korean J Pediatr<\/em>. 2014; 57(1): 19-25. doi: 10.3345\/kjp.2014.57.1.19. \r\n\r\n14.\tAl Habib HF, Jacobs JP, Mavroudis C, et al. Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. Ann Thorac Surg<\/em>. 2010; 90(3): 813-819. \r\n\r\n15.\tKanter KR, Kogon BE, Kirshbom PM, Carlock PR. Symptomatic neonatal tetralogy of Fallot: repair or shunt? Ann Thorac Surg<\/em>. 2010; 89(3): 858-863. \r\n\r\n16.\tGuyton RA, Owens JE, Waumett JD, Dooley KJ, Hatcher CR Jr, Williams WH. The Blalock-Taussig shunt. Low risk, effective palliation, and pulmonary artery growth. J Thorac Cardiovasc Surg<\/em>. 1983; 85(6): 917-922. \r\n\r\n17.\tPigula FA, Khalil PN, Mayer JE, del Nido PJ, Jonas RA. Repair of Tetralogy of Fallot in Neonates and Young Infants. Circulation<\/em>. 1999; 100: II-157\u2013161.\r\n\r\n\r\n”,”redirect_url”:””}}}