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

{“questions”:{“nalqn”:{“id”:”nalqn”,”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 14-year-old girl with a past medical history of obesity and family history of Factor V Leiden presents with chest pain, dyspnea, tachycardia and hypotension. Computed tomography pulmonary angiography demonstrates a pulmonary embolism in the main pulmonary artery with extension into bilateral branch pulmonary arteries. Transthoracic echocardiography reveals interventricular septal flattening, severe tricuspid regurgitation, and severely diminished right ventricular function. Which of the following medication regimens is the MOST appropriate treatment for severe high-risk pulmonary embolism?”,”desc”:”EXPLANATION \r\nThough less common in children than adults, pulmonary embolism (PE) is associated with significant morbidity and mortality. Guidelines for management of acute pulmonary embolism in children are based on those created for adults, due to the more established literature in this population. In most guidelines, risk stratification of pulmonary embolism is the initial step in the diagnostic and treatment algorithm. High risk (massive) pulmonary embolism is typically defined as having signs of severe cardiopulmonary dysfunction or obstructive shock, such as tachycardia, hypotension, and altered mental status. Intermediate risk (sub massive) PE typically lacks signs of obstructive shock but retains the signs of right ventricular (RV) strain as evidenced by echocardiography and electrocardiogram, or elevated cardiac enzymes. \r\n\r\n\r\nSigns and symptoms of PE in children may differ from those in adults. Children often have a delayed presentation due to an increased ability to physiologically compensate as compared to adults, non-specific symptoms, and difficulty in symptom communication. These signs and symptoms include chest pain, dyspnea, hypoxemia, cough, hemoptysis, or more severely, hypotension, tachycardia, and cardiac arrest. There should be a high index of suspicion in children with risk factors for PE, which include use of oral contraceptives, hypercoagulable state, presence of an indwelling central venous catheter, obesity, malignancy, sickle cell anemia, and sepsis. \r\n\r\n\r\nSevere pulmonary embolism causes an acute increase in pulmonary artery pressure based on the degree of obstruction. Acute RV dysfunction or failure can lead to RV dilation, tricuspid regurgitation, left ventricular (LV) dysfunction, and cardiac arrest. The combination of systemic hypotension and increased RV pressure leads to a decrease in myocardial oxygen supply with increased demand that results in a downward spiral of myocardial ischemia followed by further mismatch in myocardial oxygen supply and demand. Useful laboratory values include d-dimer, coagulation studies, and cardiac enzymes. The electrocardiogram will often show signs of right ventricular strain. Echocardiography may demonstrate the pulmonary embolism and indirect signs of acute pulmonary hypertension with tricuspid regurgitation, RV dilation, and RV systolic dysfunction. When risk factors, clinical signs and symptoms, and laboratory values suggest a high probability of PE, computed tomography pulmonary angiography (CTPA) is considered the diagnostic test of choice. \r\n\r\n\r\nIn patients with severe high-risk pulmonary embolism, systemic thrombolysis with thrombolytic agents such as tissue plasminogen activator (tPA) has been shown to improve outcomes. This is reflected in guidelines recommending this treatment, with the greatest benefit being initiation within 24 hours of symptom onset. Surgical embolectomy (SE) or catheter-based embolectomy (with or without catheter directed thrombolytic treatment) may also be considered in severe cases, though evidence on outcomes is less robust. In patients with intermediate risk PE, systemic thrombolysis may be associated with less progression of symptoms but is associated with increased risk for severe intracranial bleeding. Retrospective studies in adults have found equivalent short- and long-term mortality between patients receiving systemic thrombolysis versus surgical embolectomy. These studies have found that the thrombolysis patients experienced more stroke, reintervention, and recurrence, as compared to the SE group, which experienced a higher rate of major bleeding. A small retrospective study in children showed similar mortality rate between thrombolysis and SE but higher rates of non-fatal major hemorrhage in patients undergoing SE. Other practical considerations in choosing management are the timing and availability of a surgical team versus catheterization team, how distal the embolus resides, and if the patient has contraindications to systemic thrombolysis. \r\n\r\n\r\nGiven the severity of the signs and symptoms in this patient consistent with a high-risk massive PE with hemodynamic compromise, thrombolytic intervention with tissue plasminogen activator would be indicated to prevent further decompensation. Unfractionated heparin alone would not be sufficient for a high-risk PE but may appropriate for intermediate risk PE. Warfarin alone would not be an appropriate treatment in a patient with high-risk PE because it requires administration for 5 to 7 days to achieve a therapeutic level and has a transient procoagulant effect during initial administration requiring coadministration with either unfractionated heparin or low molecular-weight heparin. \r\n\r\n\r\n \r\nREFERENCES \r\n\r\nRoss C, Kumar R, Pelland-Marcotte MC et al. Acute Management of High-Risk and Intermediate-Risk Pulmonary Embolism in Children: A Review. Chest<\/em>. 2022 161(3):791-802. doi: 10.1016\/j.chest.2021.09.019. \r\n\r\n\r\nZaidi AU, Hutchins KK, Rajpurkar M. Pulmonary Embolism in Children. Front Pediatr<\/em>. 2017;5:170. doi: 10.3389\/fped.2017.00170. \r\n\r\n\r\nOrtel TL, Neumann I, Ageno W, et al. American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism. Blood Advances<\/em>. 2020;4(19):4693-4738.\r\n\r\n\r\nKonstantinides SV, Meyer G, Becattini C, et al. 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (Ers). Eur Heart J<\/em>. 2020;41(4):543-603.\r\n\r\n\r\nNavanandan N, Stein J, Mistry RD. Pulmonary Embolism in Children. Pediatr Emerg Care<\/em>. 2019:35(2): 143-151.\r\n”,”hint”:””,”answers”:{“yw3nb”:{“id”:”yw3nb”,”image”:””,”imageId”:””,”title”:”A. Unfractionated heparin “},”zgmzc”:{“id”:”zgmzc”,”image”:””,”imageId”:””,”title”:”B. Tissue plasminogen activator”,”isCorrect”:”1″},”rs8ml”:{“id”:”rs8ml”,”image”:””,”imageId”:””,”title”:”C. Warfarin\r\n\r\n”}}}}}

{“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”}}}}}