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

{“questions”:{“5jebq”:{“id”:”5jebq”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Nicholas Houska, DO – University of Colorado, Children\u2019s Hospital Colorado \r\nA 2-day-old boy born at 31 weeks of gestation with a congenital diaphragmatic hernia develops severe hypoxemia despite utilization of high-frequency oscillatory ventilation. The patient is deemed NOT to be a candidate for extracorporeal membrane oxygenation according to institutional policy. Which of the following adverse outcomes is the MOST likely reason why this patient is not a candidate for extracorporeal membrane oxygenation?\r\n\r\n”,”desc”:”EXPLANATION \r\nThe Extracorporeal Life Support Organization (ELSO) Guidelines for Neonatal Respiratory Failure provide evidence-based guidelines on patient selection, modes of support, and technical considerations for extracorporeal membrane oxygenation (ECMO) in neonates. These 2020 guidelines state that postmenstrual age <34 weeks and weight <2 kg are relative contraindications for ECMO, where postmenstrual age is defined as the sum of gestational age and chronologic age. The reason for this is the historically high rate of intracranial hemorrhage (ICH) (36%) and mortality (62%) in neonates <35 weeks gestational age (GA) versus 12% rate of ICH and 49% mortality in those over 35 weeks gestation.\r\n\r\nWith improvements in mortality trends and the rates of ICH in neonates <34 weeks GA, there is increasing utilization of ECMO in this population. Survival in patients <34 weeks GA who are offered ECMO has increased from 21% to between 48-76%. As survival outcomes continue to improve, certain centers are considering younger and smaller patients as candidates for ECMO. In a 2023 article, Burgos et al suggest that ECMO should be considered for patients at 32 to 33 weeks GA when restricted to high-volume neonatal ECMO centers with close reporting to ELSO, targeted oxygen delivery, and continuous technology development. Despite this, prematurity and extremely low birth weight remain high risk factors for morbidity and mortality with ECMO.\r\n\r\nThe correct answer is B. The risk of intracranial bleeding is the major reason to avoid ECMO in the patient described in the stem. While thromboembolism and infection are both causes of morbidity and mortality in premature infants on ECMO, it is the risk of intracranial hemorrhage due to anticoagulation that is the basis for a relative contraindication to ECMO in patients < 34 weeks postmenstrual age. \r\n\r\n \r\nREFERENCES \r\nWild KT, Rintoul N, Kattan J, Gray B. Extracorporeal Life Support Organization (ELSO): Guidelines for Neonatal Respiratory Failure. ASAIO J<\/em>. 2020;66(5):463-470. \r\n\r\nUpp JR Jr, Bush PE, Zwischenberger JB. Complications of neonatal extracorporeal membrane oxygenation. Perfusion<\/em>. 1994;9(4):241-56.\r\n\r\nBurgos CM, Rintoul N, Broman LM. ECMO for premature neonates- Are we there yet? Seminars in Pediatric Surgery<\/em>. 2023;32(4):151335\r\n\r\n”,”hint”:””,”answers”:{“gczo3”:{“id”:”gczo3″,”image”:””,”imageId”:””,”title”:”A. Thromboembolism “},”2ie8t”:{“id”:”2ie8t”,”image”:””,”imageId”:””,”title”:”B. Intracranial hemorrhage “,”isCorrect”:”1″},”l00qy”:{“id”:”l00qy”,”image”:””,”imageId”:””,”title”:”C. Infection\r\n”}}}}}

{“questions”:{“9xj1q”:{“id”:”9xj1q”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Nicholas Houska, DO – University of Colorado, Children\u2019s Hospital Colorado \r\n\r\nA newborn boy with a prenatal diagnosis of Tetralogy of Fallot develops respiratory distress shortly after birth, subsequently requiring intubation and extracorporeal membrane oxygenation. A portable computed tomography scan of the chest demonstrates severe main pulmonary artery dilation and bronchial compression. Which of the following types of Tetralogy of Fallot is the MOST likely diagnosis in this neonate?\r\n”,”desc”:”EXPLANATION \r\nTetralogy of Fallot (TOF) with absent pulmonary valve (TOF\/APV) is a rare type of TOF (3%) associated with high fetal and neonatal morbidity and mortality. In this defect, severe pulmonary insufficiency due to the lack of pulmonary valve cusps leads to massive main and proximal pulmonary artery dilation and thus extrinsic airway compression. Volume and pressure overload of the right ventricle (RV) can lead to dysfunction, and tricuspid regurgitation with associated left ventricular dysfunction. Chromosomal anomalies are found in 46% of TOF\/APV patients who undergo genetic testing, with 35% having the 22q11.2 deletion. The clinical presentation of TOF\/APV lies on a spectrum, ranging from fetal hydrops or severe postnatal respiratory distress to mild cardiopulmonary symptoms during infancy. The necessity for postnatal respiratory or cardiovascular support is common, and many patients do not survive to surgical repair. \r\n\r\nGiven the increased utilization and diagnostic capabilities of fetal imaging and the poor prognosis of TOF\/APV, pregnancy termination is common after prenatal diagnosis. A 2021 study found that in patients with intention to treat after birth, 9% died in utero and 27% died postnatally. Right ventricular dysfunction independently predicted overall mortality, while pulmonary artery z-scores did NOT correlate with outcomes. Management of TOF\/APV includes respiratory support based on symptoms. Patients with mild obstructive symptoms may be supported with medical management, oxygen therapy, or non-invasive positive pressure ventilation until surgical repair. These patients are often prone to frequent respiratory infections, which may require hospitalization. Conversely, patients born with more severe respiratory distress may require prone positioning to alleviate compression and\/or intubation and mechanical ventilation. Patients with the most severe respiratory failure or cardiovascular dysfunction may require extracorporeal membrane oxygenation and early surgical repair. Surgical repair includes addressing the ventricular septal defect, the right ventricular outflow tract with a valved or non-valved conduit, and pulmonary arterioplasty. There have also been case reports of combining cardiac repair of TOF\/APV with external bronchial stenting to relieve airway compression. \r\n\r\nTetralogy of Fallot with Pulmonary Stenosis and Tetralogy of Fallot with Pulmonary Atresia are associated with normal, small, or discontinuous main and\/or branch pulmonary arteries. The patient in the stem has a presentation most consistent with TOF\/APV.\r\n\r\n\r\n \r\nREFERENCES \r\nNuri H, Virgone A. Tetralogy of Fallot and absent pulmonary valve syndrome. Multimed Man Cardiothorac Surg<\/em>. 2022 Nov 8;2022. doi: 10.1510\/mmcts.2022.071\r\n\r\nPinsky WW, Nihill MR, Mullins CE, Harrison G, McNamara DG. The absent pulmonary valve syndrome. Considerations of management. Circulation<\/em>. 1978;57(1):159-162. doi: 10.1161\/01.cir.57.1.159. PMID: 618384.\r\n\r\nChelliah A, Moon-Grady AJ, Peyvandi S, et al. Contemporary outcomes in Tetralogy of Fallot with absent pulmonary valve after fetal diagnosis. J Am Heart Assoc<\/em>. 2021;10(12):e019713.\r\n\r\nSakamoto T, Nagase Y, Hasegawa H, Shin’oka T, Tomimatsu H, Kurosawa H. One-stage intracardiac repair in combination with external stenting of the trachea and right bronchus for tetralogy of Fallot with an absent pulmonary valve and tracheobronchomalacia. J Thorac Cardiovasc Surg<\/em>. 2005;130(6):1717-1718.\r\n\r\n”,”hint”:””,”answers”:{“oz08i”:{“id”:”oz08i”,”image”:””,”imageId”:””,”title”:”A. Tetralogy of Fallot with Pulmonary Stenosis”},”ws0tw”:{“id”:”ws0tw”,”image”:””,”imageId”:””,”title”:”B. Tetralogy of Fallot with Absent Pulmonary Valve”,”isCorrect”:”1″},”969aa”:{“id”:”969aa”,”image”:””,”imageId”:””,”title”:”C. Tetralogy of Fallot with Pulmonary Atresia”}}}}}

{“questions”:{“6u76f”:{“id”:”6u76f”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Nicholas Houska, DO – University of Colorado, Children\u2019s Hospital Colorado \r\nA five-month-old boy has just undergone repair of a perimembranous ventricular septal defect utilizing cardiopulmonary bypass. Shortly after aortic cross clamp removal, the ECG exhibits ST segment elevation in leads II, III, and AVF. Administration of which of the following drugs is the MOST appropriate treatment?”,”desc”:”EXPLANATION \r\nCongenital cardiac surgery often poses the risk for air to enter the left sided cardiac structures, which can subsequently be embolized to the systemic circulation. Due to its anterior location, the right coronary artery is a common anatomical site for air to travel after release of the aortic cross clamp. This typically presents as ST segment elevation on the ECG and a variable degree of right ventricular dysfunction or arrythmias. Given the spectrum of congenital cardiac lesions, air embolism to the left coronary artery should also be considered in situations where it is anatomically located in a non-dependent area, and if clinical signs are suggestive. Management of coronary air embolism involves increasing the coronary perfusion pressure by increasing the aortic diastolic blood pressure. This often requires co-management between the anesthesiologist, surgeon, and perfusionist to decide the best method to increase diastolic blood pressure. Often a combination of increasing cardiopulmonary bypass flow and administration of vasoactive medications is usually successful. Myocardial dysfunction and ECG changes are typically transient, though this may be a cause of difficulty in separating from cardiopulmonary bypass due to arrythmia or ventricular dysfunction. \r\n\r\nThe correct answer in the scenario is A, administration of phenylephrine to increase the coronary perfusion pressure. Other treatments include increasing the cardiopulmonary bypass pump flow or administration of other vasoactive agents, such as norepinephrine, epinephrine or vasopressin. These treatment options typically provide sufficient pressure to force the air bubbles through the coronary circulation. Administration of nitroglycerin is unlikely to be of benefit as the problem is related to a mechanical obstruction with air rather than coronary vasospasm. Esmolol is not the best option in this clinical scenario, though prevention of tachycardia is helpful to decrease myocardial oxygen demand. \r\n\r\n \r\nREFERENCES \r\nMonaco F, Di Prima AL, Kim JH et al. Management of Challenging Cardiopulmonary Bypass Separation. J Cardiothorac Vasc Anesth<\/em>. 2020;34(6):1622-1635. doi: 10.1053\/j.jvca.2020.02.038. \r\n\r\nSarkar M, Prabhu V. Basics of cardiopulmonary bypass. Indian J Anaesth<\/em>. 2017;61(9):760-767. doi: 10.4103\/ija.IJA_379_17.\r\n”,”hint”:””,”answers”:{“4s6r1”:{“id”:”4s6r1″,”image”:””,”imageId”:””,”title”:”A. Phenylephrine “,”isCorrect”:”1″},”lfmru”:{“id”:”lfmru”,”image”:””,”imageId”:””,”title”:”B. Esmolol”},”kx80v”:{“id”:”kx80v”,”image”:””,”imageId”:””,”title”:”C. Nitroglycerin \r\n\r\n”}}}}}

{“questions”:{“6tspy”:{“id”:”6tspy”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Nicholas Houska, DO – University of Colorado, Children\u2019s Hospital Colorado \r\n\r\nA one-month-old infant born to Hispanic parents in Southern Colorado presents with facial abnormalities, seizure disorder, congenital heart disease, and developmental delay. He is diagnosed with recombinant chromosome 8 syndrome (San Luis Valley Syndrome). Which of the following congenital heart defects is MOST likely to be associated with this syndrome?”,”desc”:”EXPLANATION \r\nFirst described by Fujimoto in 1975, Recombinant 8 syndrome, also known as San Luis Valley syndrome, is a rare genetic disorder most frequently encountered in children of Hispanic descent in the southwestern United States. This syndrome is presumed to have originated from a single founder who emigrated from Spain to Colorado or New Mexico. There is a 6.2 % risk for a parental carrier of the gene to have a child with the phenotype, with males and female offspring equally affected. The hallmarks of the disease are dysmorphic features, congenital heart defects, and developmental delay. Diagnosis is made through chromosomal analysis in children with clinical features and parental risk factors. Genetic counseling for families is recommended due to the carrier rate.\r\n\r\nThe dysmorphic features of children with San Luis Valley syndrome most commonly feature hypertelorism, wide face, thin upper lip, anteverted nares, abnormal hair whorl and infraorbital creases. These children also present with musculoskeletal abnormalities including abnormal tone, clinodactyly, deep plantar furrows, scoliosis, and pectus excavatum. Urogenital abnormalities are also common. Congenital heart disease is found in nearly all children with San Luis Valley syndrome, most commonly atrial septal defects and ventricular septal defects (50-69%), pulmonary stenosis (25-29%), Tetralogy of Fallot (16-40.5%), persistent left superior vena cava (14%) and double outlet right ventricle (8%). Case series have differed on whether these children have adverse surgical outcomes as compared to the general pediatric population, with recent studies (Pickler et al) suggesting no difference. Management includes cardiac, orthopedic, and urological surgery, along with neurodevelopmental support and rehabilitation. The most common cause of death in these children is related to congenital heart disease. \r\n\r\nSan Luis Valley Syndrome is associated with atrial septal and ventricular septal defects, and conotruncal defects such as Tetralogy of Fallot. It is not known to be associated with coarctation of the aorta or hypoplastic left heart syndrome. \r\n\r\n \r\nREFERENCES \r\nGelb BD, Towbin JA, McCabe ER, Sujansky E. San Luis Valley recombinant chromosome 8 and tetralogy of Fallot: a review of chromosome 8 anomalies and congenital heart disease. Am J Med Genet<\/em>. 1991; 40(4): 471-476. doi: 10.1002\/ajmg.1320400420. \r\n\r\nPickler L, Wilson R, Tsai AC. Revisiting recombinant 8 syndrome. Am J Med Genet A<\/em>. 2011; 155A(8): 1923-1929. doi: 10.1002\/ajmg.a.34104. \r\n\r\nHabhab W, Mau-Holzmann U, Singer S, Rie\u00df A, Kagan KO, Gerbig I, Sch\u00e4ferhoff K, Dufke A, Kehrer M. Pre- and postnatal findings in a patient with a recombinant chromosome rec(8)(qter\u2192q21.11:p23.3\u2192qter) due to a paternal pericentric inversion inv(8)(p23.3q21.11) and review of the literature. Am J Med Genet A<\/em>. 2020; 182(11): 2680-2684. doi: 10.1002\/ajmg.a.61804. .\r\n\r\nFujimoto A, Wilson MG, Towner JW. Familial inversion of chromosome No. 8: an affected child and a carrier fetus. Humangenetik<\/em>. 1975; 27(1): 67-73.\r\n\r\n”,”hint”:””,”answers”:{“ewip3”:{“id”:”ewip3″,”image”:””,”imageId”:””,”title”:”A. Tetralogy of Fallot”,”isCorrect”:”1″},”nj0e1″:{“id”:”nj0e1″,”image”:””,”imageId”:””,”title”:”B. Coarctation of the Aorta”},”0rku7″:{“id”:”0rku7″,”image”:””,”imageId”:””,”title”:”C. Hypoplastic Left Heart Syndrome”}}}}}

{“questions”:{“mup28”:{“id”:”mup28″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Melissa Colizza, MD – Stollery Children\u2019s Hospital, Edmonton Canada \r\n\r\nA 3-week-old girl presents with acute onset of shortness of breath and diaphoresis during feeds. A transthoracic echocardiogram demonstrates severe left ventricular dysfunction, moderate mitral regurgitation, and poor visualization of the left coronary ostium. Which of the following diagnoses is the MOST likely cause of severe left ventricular dysfunction?”,”desc”:”EXPLANATION \r\nAn anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare lesion, with an incidence of 1 per 300,000 births. It is also known as Bland-White-Garland syndrome, named after the first clinical description in 1933. Normally, left ventricular (LV) perfusion occurs during ventricular diastole as ventricular systole limits coronary blood flow. In patients with ALCAPA, left ventricular perfusion occurs with mixed venous blood originating from the pulmonary artery due to elevated pulmonary vascular resistance (PVR) over the first weeks of life. However, once PVR and pulmonary artery (PA) pressure begin to decrease over the first several weeks of life, myocardial perfusion also decreases as coronary perfusion pressure and coronary blood flow decrease. This may lead to a reversal in blood flow in the ALCAPA, leading to a \u201ccoronary steal phenomenon\u201d and further increasing the risk of LV ischemia and dysfunction. The right ventricle (RV) remains somewhat protected from ischemia as myocardial perfusion occurs in both systole and diastole. Progression of LV dysfunction will eventually lead to LV dilation and impaired RV filling from interventricular septal shift. Mitral valve regurgitation may also ensue, secondary to annular dilation or papillary muscle ischemia. Mitral regurgitation increases volume load to the LV, thus exacerbating dysfunction and pulmonary congestion. Like other dilated or ischemic cardiomyopathies, patients with ALCAPA are at high risk for ventricular arrhythmias. \r\n\r\nThe presentation of ALCAPA will vary amongst individuals in terms of symptoms and timing. This is mostly due to variations in coronary artery anatomy. A dominant right coronary artery (RCA) will supply a greater proportion of myocardial blood flow and is more likely to result in a later onset of symptoms with less ventricular dysfunction. Stenosis of the ALCAPA might also be beneficial as it reduces coronary steal and favors collateralization from the RCA to the LCA territory, which may minimize myocardial ischemia. A study by Straka et al compared the presentation and outcome of patients with ALCAPA diagnosed in infancy to those diagnosed later in childhood. Most patients who presented in infancy were between two to six months of age, and 62.5% had heart failure as the primary symptom. As expected, most patients had LV dysfunction, LV dilation, and moderate or severe mitral regurgitation. Patients who presented later were more likely to be asymptomatic (62.2%) or have chest pain (20.8%) with preserved LV function. One patient in that group presented with cardiac arrest.\r\n\r\nTreatment consists of re-establishing aortic perfusion to the ALCAPA. It is usually done by reimplantation of the coronary artery to the aorta. The Takeuchi procedure, an alternative surgical technique which can be used in the setting of a short ALCAPA segment, creates an aorto-pulmonary window and an intra-pulmonary baffle from the aorta to the ALCAPA. The study by Stratka and colleagues demonstrated that the early group had surgery between one and three days after diagnosis and that 31.1% required mitral valve repair for more than moderate regurgitation. \r\n\r\nAnesthetic considerations in the pre-bypass phase include maintaining systemic blood flow in the face of potentially severe LV dysfunction and adequate coronary perfusion pressure to the ALCAPA, while avoiding excessive tachycardia to minimize myocardial oxygen consumption. Induction is high risk and should be done in a slow, titrated manner. These patients are also at high risk for arrhythmias such as ventricular fibrillation due to myocardial ischemia, such that defibrillation or rapid institution of cardiopulmonary bypass may be required. Finally, maintaining a relatively elevated PVR will promote perfusion to the ALCAPA. Therefore, maintaining a moderately elevated PaCO2 and limiting the inspired oxygen concentration will achieve an elevated PVR. After repair, myocardial recovery is not immediate, and patients are likely to require high doses of vasoactive medications or mechanical circulatory support. \r\n\r\nSurgical outcomes are generally excellent but are less favorable in those who present at a younger age. The study by Straka et al demonstrated that only one child in the <1 year of age group died, four (8.9%) required extracorporeal membrane oxygenation, and 29 (64.4%) had delayed sternal closure. However, there was no mortality, no need for mechanical support, and no need for delayed sternal closure in the late onset group. Moreover, hospital length-of-stay was significantly shorter in the late onset group. Interestingly, while the early onset group had more than moderate LV dilation and mitral regurgitation at discharge, these parameters were no longer significant at the one-year follow-up. An additional retrospective analysis of surgical outcomes by El-Louali et al that compared patients with an early onset to a late onset of symptoms demonstrated that early onset patients had a significantly more severe clinical presentation and LV dysfunction, higher incidence of delayed sternal closure, longer duration of mechanical ventilation and longer ICU lengths of stay.\r\n\r\nALCAPA and dilated cardiomyopathy have very similar clinical presentations in the infant population. However, the echocardiographic findings described in the stem are highly suggestive of ALCAPA. Dilated cardiomyopathy is more likely to present with echocardiographic findings of biventricular enlargement and clearly delineated coronary ostia. Kawasaki disease may give rise to coronary anomalies in the form of aneurysms and may lead to myocardial ischemia, but the absence of a viral prodrome and typical rash along with the lack of coronary aneurysms make this diagnosis less likely. \r\n\r\n\r\nREFERENCES \r\n \r\n\r\nMcKenzie I, Zestos MK, Stayer SA, Kaminski E, Davies P, Andropoulos DB. Anesthesia for Miscellaneous Cardiac Lesions. In: Andropoulos DB, Mossad EB, Gottlieb EA, eds. Anesthesia for Congenital Heart Disease<\/em>. Fourth edition. John Wiley & Sons, Inc.; 2023: 795-800.\r\n\r\nStraka N, Gauvreau K, Huang Y, et al. Analysis of Perioperative and Long-Term Outcomes Among Presentations of Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed Beyond Infancy Versus During Infancy. Pediatr Cardiol<\/em>. Published online December 15, 2023. doi:10.1007\/s00246-023-03344-1\r\n\r\n\r\nEl-Louali F, Lenoir M, Gran C, Allary C, Fouilloux V, Ovaert C. Early Presentation of Patients with Abnormal Origin of Left Coronary Artery from the Pulmonary Artery is a Predictor of Poor Mid-term Outcomes. Pediatr Cardiol<\/em>. 2022;43(4):719-725. doi:10.1007\/s00246-021-02777-w\r\n”,”hint”:””,”answers”:{“ece6u”:{“id”:”ece6u”,”image”:””,”imageId”:””,”title”:”A)\tAnomalous Left Coronary Artery from the Pulmonary Artery”,”isCorrect”:”1″},”v5gs2″:{“id”:”v5gs2″,”image”:””,”imageId”:””,”title”:”B)\tDilated Cardiomyopathy”},”zpak9″:{“id”:”zpak9″,”image”:””,”imageId”:””,”title”:”C)\tKawasaki disease\r\n\r\n”}}}}}