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

{“questions”:{“9fe0e”:{“id”:”9fe0e”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Melissa Colizza MD – CHU Saint-Justine, Montreal, Quebec \r\n\r\nA 2-year-old boy with Shone\u2019s complex, mild hypoplasia of the left ventricle, moderate mitral stenosis and moderate aortic regurgitation status-post balloon valvuloplasty of severe aortic valve stenosis is undergoing an elective Ross-Konno procedure and mitral valve repair. Which of the following factors is MOST likely associated with increased morbidity and mortality after biventricular repair of a borderline left heart disease?”,”desc”:”EXPLANATION \r\nBorderline left heart disease falls along the spectrum of hypoplastic left heart syndrome. It is characterized by the size of left-sided heart structures (aortic valve, mitral valve, LV end-diastolic volume) with z-scores of -2 to -5, as well as the presence of endocardial fibroelastosis (EFE).\r\nThe single ventricle pathway continues to have substantial mortality and morbidity including Fontan circulation failure with cyanosis and\/or ventricular dysfunction as well as end-organ dysfunction, such as Fontan-associated liver disease, protein-losing enteropathy, plastic bronchitis and lymphatic dysfunction. In the past two decades, there has been increasing interest and development of surgical techniques to establish a two-ventricle circulation in patients who traditionally would have been palliated via the single-ventricle pathway. Currently, there is no consensus whether a \u201cbad two-ventricle\u201d repair is better than a \u201cgood single-ventricle\u201d circulation, as long-term comparison data is unavailable. \r\nIn recent years, there have been increasing efforts to establish objective anatomic, physiologic, and hemodynamic criteria that could predict which patients with borderline left heart disease would be amenable to recruitment procedures to increase blood flow to the ventricle in order to stimulate growth and then allow for biventricular repair. Strategies to increase blood flow to the left ventricle include creating a fenestrated atrial septal defect, adding an additional source of pulmonary blood flow via a shunt, and resection of EFE tissue. Subsequent surgical decision making on whether to attempt biventricular repair depends on a comprehensive assessment based on multiple characteristics of the left heart structures and function including the following: 1) the size and structure of the mitral valve and its apparatus, 2) the size and function of the aortic valve, 3) the end-diastolic volume and pressure of the LV, 4) the apex-forming status of the LV, 5) the presence of EFE, and 6) the underlying anatomy of the congenital heart defect. \r\nAlthough no single factor has been shown to predict the success or failure of biventricular conversion, there are some data that can inform the decision making process. A retrospective single-institution study assessing outcomes of 51 patients undergoing biventricular repair over a period of thirteen years from 2003-2015 by Herrin et al concluded that a pre-operative left ventricular end-diastolic pressure (LVEDP) \u2265 13mmHg and post-operative right ventricular systolic pressure (RVSP) \u2265 three-quarters of the systemic systolic pressure, as well as the presence of \u2265 moderate EFE, were associated with an increased likelihood of death, transplant or conversion to single ventricle physiology. Data from cardiac magneting resonance (CMR) imaging studies can also be of prognostic value. A CMR study of 32 patients with single ventricles undergoing biventricular repair concluded that a left ventricular end diastolic volume \u2265 45ml\/m^{2 <\/sup>in hypoplastic left heart variants or \u226530ml\/m2 <\/sup> in patients with double outlet right ventricle or atrioventricular canal defect were associated with a greater likelihood of success (Banka et al). These elements illustrate that, while size matters, it is the overall capacity of the left-sided circulation to accommodate a full cardiac output at low filling pressures that allows for a successful biventricular repair. In the event of a failed repair that results in diastolic dysfunction, left atrial hypertension and subsequent pulmonary hypertension, patients could become ineligible for conversion to single-ventricle physiology or for heart transplantation. \r\n\r\n \r\nREFERENCES \r\nDavies SJ, DiNardo JA, Emani SM, Brown ML. A Review of Biventricular Repair for the Congenital Cardiac Anesthesiologist. Semin Cardiothorac Vasc Anesth<\/em>. 2023;27(1):51-63. doi: 10.1177\/10892532221143880\r\n\r\nHerrin MA, Zurakowski D, Baird CW, et al. Hemodynamic parameters predict adverse outcomes following biventricular conversion with single-ventricle palliation takedown. J Thorac Cardiovasc Surg<\/em>. 2017;154(2):572-582. 10.1016\/j.jtcvs.2017.02.070 \r\n \r\nAndersen ND, Scherba JC, Turek JW. Biventricular Conversion in the Borderline Hypoplastic Heart. Curr Cardiol Rep<\/em>. 2020;22(10):115. doi: 10.1007\/s11886-020-01363-5\r\n\r\nChiu P, Emani S. Left Ventricular Recruitment in Patients With Hypoplastic Left Heart Syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu<\/em>. 2021;24:30-36. doi: 10.1053\/j.pcsu.2021.03.001\r\n\r\nBanka P, Schaetzle B, Komarlu R, Emani S, Geva T, Powell AJ. Cardiovascular magnetic imaging parameters associated with early transplant-free survival in children with small left hearts following conversion from a univentricular to biventricular circulation. J Cardiovasc Magn Reson <\/em>.2014; 16:73. doi: 10.1186\/s12968-014-0073-1\r\n\r\n”,”hint”:””,”answers”:{“nneh6”:{“id”:”nneh6″,”image”:””,”imageId”:””,”title”:”A.\tPre-operative left ventricular end-diastolic pressure > 13mmHg”,”isCorrect”:”1″},”xtnke”:{“id”:”xtnke”,”image”:””,”imageId”:””,”title”:”B.\tLeft ventricular end -diastolic volume z-score of -2 to -5″},”7ot74″:{“id”:”7ot74″,”image”:””,”imageId”:””,”title”:”C.\tParachute mitral valve”}}}}}}

{“questions”:{“lz52i”:{“id”:”lz52i”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Destiny F. Chau, MD – Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 19-year-old female with a history of Turner Syndrome, chronic hypertension and aortic coarctation repair in infancy presents for a surveillance cardiac MRI. She is recovering from a viral upper respiratory tract illness four weeks prior. On preoperative evaluation, she complains of new onset headache and sharp chest pain radiating to her upper back and stomach that started an hour ago, which is unchanged by position or deep breathing. Current vital signs are: HR 94, 179\/79, SpO2 97%, RR 22, Temp 36.8^{o<\/sup>C. Cardiac imaging six months prior demonstrated a bicuspic aortic valve with minimal gradient across the valve, repaired aortic coarctation, and an indexed aortic size of 2.5 cm\/m2<\/sup>. Physical exam reveals a webbed neck, mild micrognathia and Mallampati Class II airway. Which of the following is MOST likely etiology of chest pain? \r\n”,”desc”:”EXPLANATION \r\nTurner syndrome is a chromosomal abnormality associated with the haploinsufficiency of the X chromosome, whereby one copy is absent or deficient, and the remaining X chromosome is not adequate to produce the needed genetic product to preserve normal function. The diagnosis of Turner syndrome requires the individual to be phenotypically female, and it occurs with a frequency of 1:2,500 live female births. Clinical manifestations of this syndrome include cardiac and renal abnormalities, webbed neck, lymphedema, short stature, premature ovarian failure and abnormalities in other organ systems. The majority of patients with Turner syndrome have normal intelligence, although they may have associated learning disabilities. \r\n\r\nPatients with Turner syndrome have an increased risk of aortopathy and other congenital cardiovascular abnormalities, which are found in up to 50% of live born girls. Bicuspid aortic valve (~30%) and coarctation of the aorta (~18%) are the most common cardiac abnormalities that are observed in Turner syndrome patients. Other less frequent cardiac lesions include partial anomalous pulmonary venous return, ventricular septal defect, mitral valve anomalies and hypoplastic left heart syndrome. Patients with Turner syndrome are prone to the early development of systemic hypertension and aortic dilatation. Additionally, these patients are also pre-disposed to coronary artery disease, dyslipidemia, obesity, diabetes mellitus and stroke. Congenital and acquired cardiovascular disease is the major cause of death. Aortic dissection in Turner syndrome occurs earlier, at smaller aortic dilatation dimensions, and at approximately seven times the frequency of the general population. A recent scientific statement by the American Heart Association (Silberbach et al) states that Turner syndrome patients who are older than 15 years with bicuspid aortic valve, aortic coartation and\/or hypertension, and an indexed aortic size greater than 2.3 cm\/m2<\/sup> are considered to be at high cardiovascular risk and should undergo cardiac evaluation every 6 -12 months. The statement also highlights the importance of prompt recognition of the presenting signs and symptoms of aortic dissection, an often-fatal complication. \r\n\r\nPerioperative considerations for patients with Turner syndrome include a potential difficult airway due to the presence of short neck and micrognathia and other comordities involving the cardiovascular, renal and endocrine systems. As Turner syndrome patients advance in age, there is an increase in cardiovascular risk, portending to greater perioperative morbidity and mortality. The patient in the stem is at high cardiovascular risk and presents with the signs and symptoms of an aortic dissection. Though the patient is at risk for pericarditis given a recent viral upper respiratory tract infection, chest pain related to pericarditis is typically worsened by recumbent position or deep breathing. Chest pain may also be indicative of myocardial infarction, however the location and quality of chest pain described in the stem is more concerning for aortic dissection and rupture. The patient\u2019s blood pressure is elevated on presentation and should prompt timely blood pressure control but pales in comparison to potential aortic dissection. Effective communication with the cardiac and surgical teams for further evaluation and medical management of the blood pressure is imperative to minimize morbidity and mortality. \r\n\r\n\r\n \r\n\r\nREFERENCES \r\nSilberbach M, Roos-Hesselink JW, Andersen NH, et al. Cardiovascular health in Turner syndrome: A scientific statement from the American Heart Association. Circ Genom Precis Med <\/em>.2018;11(10):e000048. doi:10.1161\/HCG.0000000000000048\r\n\r\n\r\nMashour GA, Sunder N, Acquadro MA. Anesthetic management of Turner syndrome: a systematic approach. J Clin Anesth<\/em>. 2005;17(2):128-130. doi:10.1016\/j.jclinane.2004.06.010\r\n\r\n\r\nHuang AC, Olson SB, Maslen CL. A review of recent developments in Turner syndrome research. J Cardiovasc Dev Dis<\/em>. 2021;8(11):138. doi:10.3390\/jcdd8110138\r\n\r\n\r\nBondy CA; Turner Syndrome Study Group. Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab<\/em>. 2007;92(1):10-25. doi:10.1210\/jc.2006-1374\r\n\r\n”,”hint”:””,”answers”:{“psh4k”:{“id”:”psh4k”,”image”:””,”imageId”:””,”title”:”A.\tMyocardial infarction “},”g4r9f”:{“id”:”g4r9f”,”image”:””,”imageId”:””,”title”:”B.\tAortic dissection “,”isCorrect”:”1″},”uw37a”:{“id”:”uw37a”,”image”:””,”imageId”:””,”title”:”C.\tPericarditis”}}}}}}

{“questions”:{“uwca7”:{“id”:”uwca7″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Destiny F. Chau MD – Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences, Little Rock, AR.\r\n\r\nA 6-month-old infant is undergoing a full repair for Tetralogy of Fallot. The patient separates from cardiopulmonary bypass (CPB) on milrinone 0.05 mcg\/kg\/min, dexmedetomidine 0.5 mcg\/kg\/h and morphine 0.025 mg\/kg\/h. The surgeon measures a right ventricular (RV)-to-aortic pressure ratio of 0.5, and right ventricular outflow tract (RVOT) gradient of 30 mmHg. Ten minutes later the vital signs are: BP 50\/38, HR 167 bpm in sinus rhythm, SpO2 100%, with adequate anesthetic depth. The transesophageal echocardiogram (TEE) is now showing hyperdynamic right ventricular systolic function with subvalvar obstruction. In addition to volume administration, what is the recommended next step in management? \r\n”,”desc”:”EXPLANATION \r\n\r\nTetralogy of Fallot is the most common type of cyanotic congenital heart disease, representing approximately 10% of all congenital heart defects with an estimated incidence of 1 in 2,500\u2013 3,000 live births. It was first comprehensively described by Etienne Fallot in 1888 as a group of four characteristic features: ventricular septal defect (VSD), overriding aorta, right ventricular outflow tract obstruction (RVOTO) and right ventricular hypertrophy. In 1924, Maude Abbott coined this cardiac abnormality as \u201ctetralogy of Fallot\u201d. \r\n\r\nTetralogy of Fallot was the cardiac anomaly for which the first Blalock-Taussig-Thomas shunt (BTTs) was performed to augment pulmonary blood flow in 1945. This successful operation launched the modern era of congenital cardiac repair. Although the timing, techniques and approaches of surgical repair, and overall medical management of TOF have evolved in the decades since the BTTs, the surgical goals remain the same: closure of the VSD and establishment of pulmonary blood flow. \r\n\r\n\r\nA 2022 expert consensus from the American Association for Thoracic Surgery by Miller et al published recommendations for the management of pediatric patients with tetralogy of Fallot. For assessing adequacy of surgical repair, the panel recommends direct pressure measurements to objectively evaluate for residual obstruction and guide any need for reintervention, and recommends to aim for RV-to-aortic pressure ratio of 0.5 or less with an RVOT gradient less than 30 to 40 mm Hg. These parameters must be considered in the context of the patient\u2019s condition and specific anatomic limitations to surgical resection. After relief of RVOTO , dynamic outflow obstruction associated with a hypertrophied RV is not uncommon, especially in the setting of relative hypovolemia and a hypercontractile right ventricle. Anticipatory management for set hemodynamic goals is indicated, which includes optimization of RV preload with adequate volume administration, minimization of hypercontractility, and maintaining adequate diastolic filling time via heart rate control. Hyperdynamic obstruction improves over time as the hypertrophied RV remodels once the primary obstruction has been reduced. \r\n\r\n\r\nIn the setting of adequate surgical results which in this case is supported by the initial RVOT gradient and RV-to-aorta pressure ratio, the hemodynamic decline in this patient is most likely due to hyperdynamic obstruction from the pre-existing hypertrophied RV. Management for this condition would be to maintain filling pressures with volume administration, minimization of inotropic agents such as calcium and epinephrine, and slowing of the heart rate to allow for increased diastolic filling time. An esmolol drip is helpful for controlling the tachycardia. If these medical interventions fail to restore hemodynamic stability, other etiologies should be sought. The surgeon should be notified and the cardiac status reevaluated via TEE and repeat pressure measurements as necessary. A return to CPB may be needed if evidence suggests that surgical reintervention is necessary. \r\n\r\n\r\n\r\n \r\n\r\nREFERECES \r\nSchmitz ML, Chau DF, Das RR, Thompson LL, Ullah S. Anesthesia for right-sided obstructive lesions. In: Andropoulos DB, Mossad EB, Gottlieb EA, eds. Anesthesia for Congenital Heart Disease <\/em>.4th ed. Hoboken, NJ; Wiley-Blackwell. 2023: 674-710. \r\n\r\n\r\nExpert Consensus Panel:, Miller JR, Stephens EH, et al. The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot. J Thorac Cardiovasc Surg<\/em>. 2023;165(1):221-250. doi:10.1016\/j.jtcvs.2022.07.025 \r\n\r\n\r\nWise-Faberowski L, Asija R, McElhinney DB. Tetralogy of Fallot: Everything you wanted to know but were afraid to ask. Paediatr Anaesth <\/em>.2019;29(5):475-482. doi:10.1111\/pan.13569 \r\n\r\n\r\nJonas A. Tetralogy of Fallot with pulmonary stenosis. In: Jonas A, ed. Comprehensive Surgical Management of Congenital Heart Disease<\/em>. 2nd Edition. Boca Raton, Florida: Taylor & Francis Group, LLC; 2014: 351-361.\r\n\r\n\r\n”,”hint”:””,”answers”:{“iiogb”:{“id”:”iiogb”,”image”:””,”imageId”:””,”title”:”A.\tReturn to CPB for reintervention”},”zej6b”:{“id”:”zej6b”,”image”:””,”imageId”:””,”title”:”B.\tAdminister calcium”},”q32hb”:{“id”:”q32hb”,”image”:””,”imageId”:””,”title”:”C.\tBolus esmolol and begin an infusion\r\n\r\n”,”isCorrect”:”1″}}}}}

{“questions”:{“sz7qf”:{“id”:”sz7qf”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Arturo Marrero MD, Georgetown Public Hospital Corporation, Georgetown, Guyana and\r\nDestiny F. Chau MD, Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences, Little Rock, AR, USA \r\n\r\nUn beb\u00e9 de 4 meses se presenta para una reparaci\u00f3n quir\u00fargica completa de tetralog\u00eda de Fallot. El ecocardiograma transtor\u00e1cico preoperatorio demuestra una arteria coronaria descendente anterior izquierda que surge de la arteria coronaria derecha. Despu\u00e9s de la esternotom\u00eda media, el cirujano nota que una arteria coronaria cruza el tracto de salida del ventr\u00edculo derecho y planifica hacer un conducto del ventr\u00edculo derecho a la arteria pulmonar. \u00bfQu\u00e9 porcentaje de pacientes con tetralog\u00eda de Fallot tienen arterias coronarias que cruzan el tracto de salida del ventr\u00edculo derecho? \r\nA 4-month-old infant presents for complete surgical repair of Tetralogy of Fallot (TOF). The preoperative transthoracic echocardiogram demonstrated a left anterior descending coronary artery arising from the right coronary artery. After median sternotomy, the surgeon notes a coronary crossing the right ventricular outflow tract (RVOT) and plans for a right ventricle to pulmonary artery conduit. What percentage of patients with TOF have coronary arteries crossing the RVOT? “,”desc”:”Explicaci\u00f3n\/Explanation \r\n\r\nLa tetralog\u00eda de Fallot es el tipo m\u00e1s com\u00fan de cardiopat\u00eda cong\u00e9nita cian\u00f3tica y representa aproximadamente el 5 a 10% de todos los defectos card\u00edacos cong\u00e9nitos. Se describe como un grupo de cuatro anomal\u00edas caracter\u00edsticas: defecto del tabique ventricular o comunicaci\u00f3n interventricular (CIV), aorta superpuesta, obstrucci\u00f3n del tracto de salida del ventr\u00edculo derecho e hipertrofia del ventr\u00edculo derecho. \r\n\r\nLos objetivos quir\u00fargicos para la reparaci\u00f3n completa de la tetralog\u00eda de Fallot son el cierre de la CIV y la restauraci\u00f3n del flujo sangu\u00edneo pulmonar normal a trav\u00e9s del alivio de la obstrucci\u00f3n del tracto de salida del ventr\u00edculo derecho. La presencia de anomal\u00edas coronarias espec\u00edficas puede requerir modificaciones en la t\u00e9cnica quir\u00fargica. Para evitar el da\u00f1o iatrog\u00e9nico a las arterias coronarias, la delineaci\u00f3n preoperatoria precisa de la distribuci\u00f3n de las arterias coronarias es esencial, especialmente si se planea una ventriculotom\u00eda como parte de la operaci\u00f3n. \r\n\r\nLos pacientes con tetralog\u00eda de Fallot tienen una mayor incidencia de anomal\u00edas arteriales coronarias en comparaci\u00f3n con la poblaci\u00f3n general. Sin embargo, no todas las formas de anomal\u00eda de la arteria coronaria son quir\u00fargicamente importantes. Las de particular inter\u00e9s son aquellos vasos coronarios que cruzan el tracto de salida del ventr\u00edculo derecho, ya que puede ser necesaria una incisi\u00f3n a trav\u00e9s de esta \u00e1rea durante la reparaci\u00f3n quir\u00fargica. Esto tambi\u00e9n incluye variantes normales que cruzan el tracto de salida del ventr\u00edculo derecho, como una gran rama de la arteria conal. La presencia de arterias coronarias que cruzan el tracto de salida del ventr\u00edculo derecho proh\u00edbe el aumento con parche transanular. \r\n\r\nUn metan\u00e1lisis reciente de anomal\u00edas de las arterias coronarias en tetralog\u00eda de Fallot realizado por Koppel et al encontr\u00f3 que entre 6956 pacientes con tetralog\u00eda de Fallot en 28 estudios, el 6 % de los pacientes tienen arterias coronarias an\u00f3malas. De aquellos con arterias coronarias an\u00f3malas, el 72 % cruzaron el tracto de salida del ventr\u00edculo derecho ( 4 % de todos los pacientes con tetralog\u00eda de Fallot) y el resto sigui\u00f3 con mayor frecuencia un curso retroa\u00f3rtico. La arteria m\u00e1s com\u00fan que cruzaba el tracto de salida del ventr\u00edculo derecho es la arteria coronaria descendente anterior izquierda que surge de la arteria coronaria derecha o seno de Valsalva derecho. Adem\u00e1s, se encontr\u00f3 que el 6 % de los pacientes ten\u00eda una arteria conal grande que tambi\u00e9n cruza esta \u00e1rea. Por lo tanto, con una frecuencia combinada del 10%, es probable que uno de cada diez pacientes con tetralog\u00eda de Fallot tenga una arteria coronaria que cruce el tracto de salida del ventr\u00edculo derecho. \r\n\r\nDurante la separaci\u00f3n del bypass cardiopulmonar, la manifestaci\u00f3n de baja funci\u00f3n card\u00edaca y signos continuos de isquemia mioc\u00e1rdica son fuerte indicativos de la transecci\u00f3n de una arteria coronaria durante la reparaci\u00f3n de tetralog\u00eda de Fallot en la que se realiz\u00f3 una ventriculotom\u00eda derecha. En esta situaci\u00f3n, se necesita un bypass de arteria coronaria emergente para restaurar la circulaci\u00f3n coronaria. \r\n\r\n————————————————–\r\n\r\nTetralogy of Fallot is the most common type of cyanotic congenital heart disease, representing approximately 5-10% of all congenital heart defects. It consists of four characteristic abnormalities: ventricular septal defect (VSD), overriding aorta, right ventricular outflow tract obstruction (RVOTO) and right ventricular hypertrophy. \r\n\r\nThe surgical goals of complete repair of TOF are VSD closure, relief of RVOTO, and restoration of normal pulmonary blood flow. The presence of specific coronary abnormalities may require modifications to the surgical technique. To avoid coronary artery injury, accurate delineation of coronary artery distribution is essential, especially if a ventriculotomy is planned as a part of the repair. \r\n\r\nPatients with TOF have a higher incidence of coronary artery anomalies compared to the general population. Those of particular surgical importance are coronary arteries crossing the RVOT, as an incision across the RVOT may be required during the surgical repair. This includes normal variants crossing the RVOT such as a large conal branch. A coronary vessel crossing the RVOT precludes transannular patch augmentation. \r\n\r\nA recent meta-analysis of coronary artery anomalies in TOF by Koppel et al demonstrated that amongst 6956 patients with TOF across 28 studies, 6% of patients have anomalous coronary arteries. Of those with anomalous coronary arteries, 72% crossed the RVOT (4% overall in TOF) and the remaining most commonly followed a retro-aortic course. The most common artery crossing the RVOT was the left anterior descending artery (LAD) originating from the right coronary artery (RCA) or right sinus of Valsalva. Additionally, 6% of patients were found to have a large conal branch crossing the RVOT. Therefore, with a combined frequency of 10%, one in ten patients with TOF is likely to have a coronary artery crossing the RVOT. \r\n\r\nDuring separation from cardiopulmonary bypass, evidence of poor cardiac function and ongoing signs of myocardial ischemia are strongly indicative of coronary artery injury during repair in which a right ventriculotomy was performed. In this situation, an emergent coronary artery bypass is needed to restore the coronary circulation. \r\n\r\n\r\nReferencias\/References \r\nMiller JR, Stephens EH, Goldstone AB et al. The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot. J Thorac Cardiovasc Surg <\/em>. 2023; 165:221-50. doi.org\/10.1016\/j.jtcvs.2022.07.025 \r\n\r\n\r\nWise-Faberowski L, Asija R, McElhinney DB. Tetralogy of Fallot: Everything you wanted to know but were afraid to ask. Paediatr Anaesth<\/em>. 2019;29(5):475-482. doi:10.1111\/pan.13569 \r\n\r\nKoppel CJ, Jongbloed MRM, Ki\u00e8s P, et al. Coronary anomalies in tetralogy of Fallot – A meta-analysis. Int J Cardiol<\/em>. 2020;306:78-85. doi:10.1016\/j.ijcard.2020.02.037\r\n\r\nTalwar S, Sengupta S, Marathe S, Vaideeswar P, Airan B, Choudhary SK. Tetralogy of Fallot with coronary crossing the right ventricular outflow tract: A tale of a bridge and the artery [published correction appears in Ann Pediatr Cardiol. 2021 Oct-Dec;14(4):571]. Ann Pediatr Cardiol<\/em>. 2021;14(1):53-62. doi:10.4103\/apc.APC_165_19 \r\n\r\nJonas A. Tetralogy of Fallot with pulmonary stenosis. In: Jonas A, ed. Comprehensive Surgical Management of Congenital Heart Disease<\/em>. 2nd Edition. Boca Raton, Florida: Taylor & Francis Group, LLC; 2014: 351-361.\r\n\r\n\r\n”,”hint”:””,”answers”:{“tnceh”:{“id”:”tnceh”,”image”:””,”imageId”:””,”title”:”A.\t10%”,”isCorrect”:”1″},”o15kt”:{“id”:”o15kt”,”image”:””,”imageId”:””,”title”:”B.\t20%”},”8iuw9″:{“id”:”8iuw9″,”image”:””,”imageId”:””,”title”:”C.\t30%”}}}}}

{“questions”:{“odfaq”:{“id”:”odfaq”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Bryce Ferry, DO and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 3-month-old infant presenting with jaundice and pruritus undergoes liver biopsy which demonstrates multiple branches of the hepatic artery and vein and sparsity of bile ducts suggesting Alagille syndrome. What cardiac defect is MOST commonly associated with this syndrome? “,”desc”:”EXPLANATION \r\nAlagille syndrome is a rare inherited autosomal dominant disorder commonly associated with bile duct paucity, congenital cardiac defects and anomalies of other organs. It is associated with mutations in the JAG1 gene located on chromosome 20 (95%) or NOTCH2 gene located on chromosome 1 (5%). These mutations affect the notch signaling pathway leading to defective organogenesis and resulting in multiple systemic manifestations with a highly variable phenotype. This rare syndrome has an estimated prevalence of 1 in 70,000 newborns. De novo mutations account for half of the cases, with genetic inheritance accounting for the remainder. \r\nThe diagnostic criteria and major multi-system clinical manifestations are summarized in the table below, from Menon et al. (used under Creative Commons License). \r\n\r\n\r\nDiagnostic criteria for Alagille syndrome. From Menon et al. Multidisciplinary management of Alagille syndrome. J Multidiscip Healthc. 2022; 15:353-364. Used under Open Access Creative Commons License from Dove Press. (AS -Alagille Syndrome) \r\n\r\nCongenital cardiac disease remains one of the primary causes of death. Emerick et al. studied 92 patients with Alagille syndrome and found the following causes of mortality: 15% from complex congenital heart disease, 25% from intracranial hemorrhage, and 25% from hepatic disease or transplantation. Patients with structural intracardiac lesions had a 40% predicted probability of being alive at 20 years old as compared to 80% for those without intracardiac defects. As hepatic involvement can worsen over time, interventions to correct and manage cardiac lesions must be promptly addressed to minimize morbidity and mortality due to other systemic diseases. \r\n\r\nPulmonary hemorrhage is common in patients with Alagille syndrome undergoing cardiac catherization procedures. In a retrospective, single-institution study, Adamson et al. reported the outcomes of 87 cardiac catheterizations in 30 children with Alagille syndrome. A total of 37% had interventions on the branch pulmonary arteries and 73% were diagnostic cardiac catheterizations. The study demonstrated that 44% (14 of 32) of interventional and 22% (12 of 55) of diagnostic catheterizations were complicated by pulmonary hemorrhage; the coagulation profiles and liver function tests were similar in those with and without pulmonary hemorrhage. The etiology of pulmonary hemorrhage was unclear. Notably, the JAG1 gene mutation, which is present in the majority of patients with Alagille syndrome, is expressed in both the arterial endothelium and in megakaryocytes. The JAG 1 gene plays an important role in vascular development and normal hemostasis. Therefore, mutation of the JAG1 gene may be a major contributing factor to pulmonary hemorrhage. Other factors associated with pulmonary hemorrhage included diagnosis of Tetralogy of Fallot, greater right ventricular systolic pressure to aortic systolic pressure ratio, and procedural\/surgical interventions on the branch pulmonary arteries. Sixty five percent of pulmonary hemorrhages were mild, 15% were moderate, 19% were severe. The authors recommended a high degree of vigilance for this complication. \r\n\r\nPatients with Alagille syndrome present for a variety of diagnostic and surgical procedures including the Kasai operation, liver biopsy and liver transplantation. Therefore, a thorough preoperative evaluation, with particular attention to the hepatic and cardiovascular systems, is essential for perioperative management. Hepatic disease varies from intractable pruritus to advanced liver dysfunction requiring liver transplantation. Patients may present with hepatomegaly and splenomegaly secondary to portal hypertension. Liver function tests usually show elevated gamma glutamyl transpeptidase, bilirubin and prolonged PT\/PTT. The associated splenomegaly can result in thrombocytopenia, which may increase the risk of bleeding. Anesthetic drugs and neuromuscular blocking agents with minimal hepatic metabolism or excretion should be considered for patients with hepatic dysfunction. As chronic cholestasis can lead to osteopenia and fractures of long bones in this patient population, additional patient positioning precautions during general anesthesia are important. The presence of facial dysmorphism and vertebral anomalies are indicators of potential difficulty with mask ventilation and airway management. Associated cardiac lesions warrant appropriate anesthetic considerations tailored to the specific cardiac defect.\r\n\r\nPeripheral pulmonary artery stenosis is the most common cardiac defect found in patients with Alagille syndrome. Hypoplastic left heart syndrome and common atrioventricular canal are not typically associated with this syndrome. \r\n\r\nREFERENCES \r\nMenon J, Shanmugam N, Vij M, Rammohan A, Rela M. Multidisciplinary management of Alagille syndrome. J Multidiscip Healthc <\/em>. 2022;(15):353-364. doi:10.2147\/JMDH.S295441 \r\nYildiz T, Yumuk N, Baykal D, Solak M, Toker K. Alagille syndrome and anesthesia management. Pediatr Anesth <\/em>. 2007; 87-97 doi:10.1111\/j.1460-9592.2006.02022.x \r\nEmerick KM, Rand EB, Goldmuntz E, Krantz ID, Spinner NB, Piccoli DA. Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology<\/em>. 1999;29(3):822-829. doi:10.1002\/hep.510290331 \r\n\r\nMitchell E, Gilbert M, Loomes KM. Alagille Syndrome. Clin Liver Dis<\/em> .2018;22(4):625-641. doi:10.1016\/j.cld.2018.06.001 \r\n \r\nAdamson GT, Peng LF, Feinstein JA, et al. Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization. Catheter Cardiovasc Interv<\/em>. 2020;95(2):262-269. doi:10.1002\/ccd.28508\r\n\r\n”,”hint”:””,”answers”:{“i8wc4”:{“id”:”i8wc4″,”image”:””,”imageId”:””,”title”:”A. Peripheral pulmonary artery stenosis “,”isCorrect”:”1″},”oqr76″:{“id”:”oqr76″,”image”:””,”imageId”:””,”title”:”B. Common atrioventricular canal”},”i0lnh”:{“id”:”i0lnh”,”image”:””,”imageId”:””,”title”:”C. Hypoplastic left heart syndrome “}}}}}