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

{“questions”:{“kadbs”:{“id”:”kadbs”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2024\/05\/CCAS-Graphic-522024.jpg”,”imageId”:”7313″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: JE Elliott, MD, Dept of Anesthesiology, Critical Care and Pain Medicine, University of Texas Health Science Center at Houston\/McGovern Medical School, Houston, TX AND\r\nM Gangadharan, MBBS, FAAP, FASA, Dept of Anesthesiology, Critical Care and Pain Medicine, University of Texas Health Science Center at Houston\/McGovern Medical School, Houston, TX \r\n\r\nA three-day-old boy with significant cardiomegaly has a transthoracic echocardiogram that demonstrates a globular appearing left ventricle with severely depressed systolic function. Several transthoracic echocardiographic images are illustrated below. Which of the following types of cardiomyopathies is MOST likely present in this patient? \r\n\r\n”,”desc”:”EXPLANATION \r\nNon-compaction cardiomyopathy was considered a rare condition but is being increasingly diagnosed because of heightened awareness. The left ventricular myocardium of patients with non-compaction cardiomyopathy is characterized by prominent trabeculations and deep intertrabecular recesses which communicate with the ventricular cavity. Non-compaction cardiomyopathy results from a defect in normal myocardial development. The primitive myocardium is a trabecular network of sponge-like muscle fibers in the mid-late embryo, and receives its blood supply from intertrabecular spaces that communicate with the cardiac chambers. During normal development, these trabeculations disappear after epicardial coronary arterial supply has been established. Subsequently, the spongy myocardium is transformed into the compact normal myocardium. Non-compaction results when this transformation from spongy to compact myocardium fails to occur, resulting in an inner prominent spongy layer with a thin outer compact epicardial layer. \r\n\r\nClinically, non-compaction cardiomyopathy is characterized by heart failure, arrhythmias and thromboembolic events that may present at any age. There are several subtypes of left ventricular noncompaction cardiomyopathy (LVNC) and these different phenotypes have prognostic implications. The subtypes are: (1) Isolated non-compaction with normal cardiac function; (2) non-compaction with hypertrophic cardiomyopathy (HCM), and, (3) Non-compaction with dilated cardiomyopathy (DCM). A 2015 study by Jeffries et al, based on the Pediatric Cardiomyopathy Registry, investigated time to death or transplantation in patients with different phenotypes of LVNC. They observed that children with isolated LVNC had the best outcomes followed by LVNC with HCM. LVNC with DCM resulted in the worst patient outcomes. LVNC may be also associated with a variety of congenital heart defects including anomalous coronary arteries, atrial and ventricular septal defects, Ebstein\u2019s anomaly, transposition of the great arteries, absent pulmonary valve, pulmonary stenosis, hypoplastic left heart syndrome, and anomalous pulmonary veins. Reversible forms of LVNC have been reported to occur when the left\/systemic ventricle is subjected to abnormal loading conditions such as during pregnancy and remodeling of the right ventricle in corrected transposition of the great vessels.\r\n\r\nAlthough echocardiography is the most common modality used to confirm diagnosis, there are not widely accepted criteria that constitute a gold standard for the diagnosis of LVNC. However, there is general agreement that LVNC is defined by presence of the following characteristics: (1) prominent trabeculations in the left ventricle; (2) deep recesses between the trabeculations and the presence of a thin compacted layer; and (3) a ratio > 2:1 of the noncompacted to compacted myocardial layers at end-systole. Cardiac magnetic resonance (CMR) is also being increasingly utilized to delineate the non-compacted muscle layer and to better visualize the cardiac apex, in addition to measuring ejection fraction. Cardiac fibrosis can also be evaluated with the use of late gadolinium enhancement during CMR.\r\n\r\nManagement should follow evidence-based guidelines for the specific phenotype of LVNC. Heart failure is managed according to standard guidelines. These patients are at increased risk for thromboembolic events such as stroke and may require anticoagulation. Typically, most patients are started on oral aspirin therapy but may require additional medications such as warfarin if there are other risk factors such as reduced ejection fraction or atrial fibrillation. Ventricular arrhythmias are common, and these patients may need placement of a defibrillator to prevent sudden death. First-degree relatives should be screened as one study demonstrated that 30% of the relatives screened by echocardiography were found to have LVNC.\r\n\r\nNeonatal non-compaction cardiomyopathy appears to be associated with particularly poor outcomes. In a small retrospective analysis by Rodrigues-Fanjul et al, the mortality rate of 14 neonates with non-compaction cardiomyopathy was 42.8% (6) over a median follow-up period of 34 months. Five of the six deaths were from ventricular failure. The presence of biventricular involvement and poor ventricular function were associated with a higher risk of death. \r\n\r\nEchocardiographic features of hypertrophic cardiomyopathy (HCM) include wall thickness > 15mm, asymmetrical hypertrophy, systolic anterior motion of the mitral valve (SAM), and a small LV cavity. Echocardiographic features of dilated cardiomyopathy (DCM) include left ventricular (LV) dilation (>112%, corrected for age and body surface area) and systolic dysfunction (LV ejection fraction < 45%) with impaired global contractility (fractional shortening < 25%). Although subsets of noncompaction cardiomyopathy include dilated and hypertrophic forms, the presence of trabeculations in both ventricles make biventricular noncompaction cardiomyopathy the most likely diagnosis in the patient presented in the stem. \r\n\r\n\r\n \r\nREFERENCES \r\nIchida F. Left ventricular noncompaction – Risk stratification and genetic consideration. J Cardiol<\/em>. 2020;75(1):1-9. doi:10.1016\/j.jjcc.2019.09.011\r\n\r\nJeffries JJ, Wilkinson JD, Sleeper LA et al. Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: Results from the Pediatric Cardiomyopathy Registry. J Cardiac Fail<\/em>. 2015; 21:877-884.\r\n\r\nCE Bennett and R Freudenberger. The Current Approach to Diagnosis and Management of Left Ventricular Noncompaction Cardiomyopathy: Review of the Literature. Cardiology Research and Practice<\/em>. Volume 2016, Article ID 5172308, 1-7. \r\n\r\nJ Rodriguez-Fanjul, S Tubio-Comez, JMC Bellon, C Bautista-Rodriguez, and J Sanchez-de-Toledo. Neonatal Non-compacted Cardiomyopathy: Predictors of Poor Outcome. Pediatric Cardiology<\/em>. 2020; 41:175-180.\r\n\r\nMaron BJ, Desai MY, Nishimura RA, et al. Diagnosis and Evaluation of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol<\/em> 2022;79(4):372-389. doi:10.1016\/j.jacc.2021.12.002\r\n\r\nMathew T, Williams L, Navaratnam G, et al. Diagnosis and assessment of dilated cardiomyopathy: a guideline protocol from the British Society of Echocardiography. Echo Res Pract. 2<\/em>017;4(2):G1-G13. doi:10.1530\/ERP-16-0037\r\n\r\n”,”hint”:””,”answers”:{“7mu8b”:{“id”:”7mu8b”,”image”:””,”imageId”:””,”title”:”A.\tDilated Cardiomyopathy”},”e3vfc”:{“id”:”e3vfc”,”image”:””,”imageId”:””,”title”:”B.\tNon-compaction cardiomyopathy”,”isCorrect”:”1″},”hx25y”:{“id”:”hx25y”,”image”:””,”imageId”:””,”title”:”C.\tHypertrophic Cardiomyopathy\r\n\r\n”}}}}}

{“questions”:{“og24v”:{“id”:”og24v”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Fernando Cuadrado, MD – Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, OH and\r\nDestiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 7-year-old boy with a history of pulmonary arterial hypertension treated with sildenafil and ambrisentan presents for cardiac catheterization. After induction of anesthesia, the blood pressure decreases from 98\/50 to 60\/40 mmHg along with a decrease in oxygen saturation from 97% from 88%. Which of following medications is the MOST appropriate treatment for hypotension in this patient?\r\n”,”desc”:”EXPLANATION \r\nPulmonary arterial hypertension (PAH) is defined as mean pulmonary artery pressure (mPAP) greater than 20 mmHg and a pulmonary vascular resistance (PVR) greater than 3 Woods units (WU) in adults and children older than three months of age. Chronically elevated PAP and PVR can significantly impair the right ventricle\u2019s (RV) function over time due to long-standing pressure overload and RV hypertrophy with subsequent diastolic dysfunction and impaired ventricular filling. This may lead to downstream adverse effects on left ventricular (LV) filling and systemic cardiac output as RV function deteriorates. LV function and systemic cardiac output is further compromised with leftward shift or bowing of the interventricular septum into the LV cavity. Due to interventricular dependence, this leftward shift of the ventricular septum negatively affects LV geometry leading to further impaired function. Additionally, RV hypertrophy significantly increases wall tension of the myocardium resulting in increased myocardial oxygen demand and the mean arterial pressure required to maintain adequate coronary perfusion pressure. \r\n\r\nSystemic hypotension and thus reduced coronary perfusion pressure can easily lead to myocardial ischemia and further reduced myocardial function, resulting in a vicious cycle if not treated expeditiously. A hypertrophied RV has very little reserve for coronary hypoperfusion and promptly fails. Rapid treatment of hypotension is essential to preserve coronary perfusion pressure (CPP) and maintain oxygen delivery to the RV at risk for acute decompensation. Pulmonary hypertensive (PH) crisis is a sudden increase in PVR leading to RV failure with subsequent decreased LV preload, cardiac output and coronary perfusion. Cardiac arrest will ensue very quickly if PH crisis is not treated aggressively and rapidly. \r\n\r\nAs mentioned previously, prompt treatment of hypotension is necessary to prevent further deterioration in RV function and RV failure in patients with PH. Several vasoactive medications can be used to increase SVR and maintain coronary perfusion pressure. Vasopressin is the optimal choice to increase SVR, spare PVR, and thereby raise MAP and CPP without increasing heart rate and myocardial oxygen consumption. At moderate to high doses, epinephrine increases SVR, MAP, heart rate and myocardial contractility, as well as PVR. Likewise, dopamine, when used at the higher end of its dosing range, increases SVR, heart rate, MAP, myocardial contractility, and PVR. In addition to their undesirable effects on PVR, both epinephrine and dopamine increase heart rate, thereby shortening diastolic filling time and increasing myocardial oxygen demand, which can be highly detrimental for an RV at high risk of failure. Phenylephrine is a more readily available and commonly used vasoactive medication that increases SVR and MAP leading to a reflex decrease in heart rate. It may be used in this clinical scenario as well. However, it is a less optimal choice compared to vasopressin due to an undesirable effect of increasing PVR. \r\n\r\n\r\nOf the three answer choices in the stem, vasopressin is the ideal drug for treating hypotension in patients with pulmonary hypertension due to minimal effects on PVR. Although other vasoactive agents may be used, they may be associated with detrimental increases in PVR and undesired tachycardia with resultant increased myocardial oxygen demand and shortened diastolic filling time. \r\n\r\n\r\n \r\nREFERENCES \r\nWadia RS, Bernier ML, Diaz-Rodriguez NM, Goswami DK, Nyhan SM, Steppan J. Update on perioperative pediatric pulmonary hypertension management. J Cardiothorac Vasc Anesth<\/em>. 2022;36(3):667-676.\r\n\r\nTriposkiadis F, Giamouzis G, Boudoulas KD, et al. Left ventricular geometry as a major determinant of left ventricular ejection fraction: physiological considerations and clinical implications. Eur J Heart Fail<\/em>. 2018;20(3):436-444.\r\n\r\nKaestner M, Schranz D, Warnecke G, Apitz C, Hansmann G, Miera O. Pulmonary hypertension in the intensive care unit. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart<\/em>. 2016;102 Suppl 2:ii57-ii66.\r\n”,”hint”:””,”answers”:{“j9ub1”:{“id”:”j9ub1″,”image”:””,”imageId”:””,”title”:”A.\tEpinephrine “},”cs074”:{“id”:”cs074″,”image”:””,”imageId”:””,”title”:”B.\tVasopressin”,”isCorrect”:”1″},”ohjcn”:{“id”:”ohjcn”,”image”:””,”imageId”:””,”title”:”C.\tDopamine”}}}}}

{“questions”:{“6w771”:{“id”:”6w771″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Greesha S. Pednekar, MD – Children\u2019s Memorial Hermann Hospital, University of Texas Health Science Center at Houston, TX and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 2-year-old girl with a history of dilated cardiomyopathy and subsequent left ventricular assist device implantation has persistent right ventricular failure supported with high doses of inotropic medications and inhaled nitric oxide. According to the Pedimacs registry, which of the following percentages MOST accurately reflects the approximate rate of right ventricular failure in the first month after left ventricular assist device placement?\r\n”,”desc”:”EXPLANATION \r\nPatients undergoing left ventricular assist device (LVAD) placement are at risk of right ventricular (RV) failure. Persistent RV failure is associated with increased morbidity and mortality, including multi-organ dysfunction, which may jeopardize transplant candidacy. Anticipation, prevention, and early detection of RV failure is imperative in LVAD planning and management.\r\n\r\nAn analysis of the largest pediatric VAD registry, called the Pedimacs registry, by Simpson et al demonstrated that 55% of children had RV failure during a time frame between one week to one month after LVAD implantation. An additional 25% of pediatric patients had RV failure between one and three months after LVAD placement. Factors associated with RV failure were younger age, lower weight, INTERMACS profile 1, chemical paralysis, and a pulsatile flow device. In addition, RV failure was associated with an increased risk of death during device support at time points of greater than one month (hazard ratio 3.2, 95% CI 1.4-7.7, p = 0.007) and greater than three months after LVAD insertion (hazard ratio 6.9, 95% CI 2-23.1, p = 0.002). In this analysis, RV failure was defined as prolonged inotrope use or subsequent need for right VAD. \r\n\r\nDeterioration of RV function after LVAD implantation can occur due to a number of reasons, including the following:1) Increased cardiac output results in increased systemic venous return and acute increases in RV preload, myofibril stretch, wall tension and myocardial oxygen demand, which may not be well tolerated under circumstances of depressed RV function; 2) LV unloading causes a leftward shift of the interventricular septum, thereby distorting RV geometry and interfering with the RV free wall contractility and worsening tricuspid regurgitation; 3) Apical LVAD implantation can impede the normal twisting motion of the RV, thereby impeding RV contractility and; 4) Intraoperative myocardial ischemia may further worsen RV function. \r\n\r\nAfter LVAD placement, anticipation of and preparation for RV failure during separation from cardiopulmonary bypass is critically important. Support of the RV includes initiation of inhaled nitric oxide, inotropic support, and avoidance of RV distension by excessive volume administration, along with avoidance of hypoxia and hypercarbia. Transesophageal echocardiography may be utilized to assess ventricular function, ventricular volume, and interventricular septal position after VAD placement. \r\n\r\n\r\n \r\nREFERENCES \r\nSimpson KE, Kirklin JK, Cantor RS et al. Right heart failure with left ventricular assist device implantation in children: An analysis of the Pedimacs registry database. J Heart Lung Transplant<\/em>. 2020;39(3):231-240. doi: 10.1016\/j.healun.2019.11.012. \r\n\r\nLaw SP, Morales DLS, Si MS, et al. Right heart failure considerations in pediatric ventricular assist devices. Pediatr Transplant<\/em>. 2021;25(3):e13990. doi:10.1111\/petr.13990\r\n\r\nHorton S, Skiner A, Bacigalupo A, Adachi I, Stayer S, Motta P. Mechanical Circulatory Support. In: Andropoulos D, Mossad E, Gottlieb E, eds. Anesthesia for Congenital Heart Disease<\/em>. 4th Edition. Hoboken, New Jersey: John Wiley & Sons, Inc.; 2023: 996-1025.\r\n\r\n”,”hint”:””,”answers”:{“pllyn”:{“id”:”pllyn”,”image”:””,”imageId”:””,”title”:”A.\t75%”},”j960n”:{“id”:”j960n”,”image”:””,”imageId”:””,”title”:”B.\t50%”,”isCorrect”:”1″},”o2nvy”:{“id”:”o2nvy”,”image”:””,”imageId”:””,”title”:”C.\t25%”}}}}}

{“questions”:{“di0y4”:{“id”:”di0y4″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2024\/04\/CCAS-Combined-Graphic-jpeg.jpg”,”imageId”:”7258″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Pedro Solorzano, MD and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 12-year-old boy with a history of Wolff-Parkinson-White syndrome and intermittent supraventricular tachycardia is scheduled for an electrophysiologic study. The following electrocardiographic (ECG) rhythm is noted prior to induction of anesthesia. Which of the following cardiac rhythms is MOST likely illustrated below?”,”desc”:”EXPLANATION \r\nSinus arrhythmia is a variant of normal sinus rhythm (NSR) that is commonly seen in children and young adults. The heart rate increases with inspiration and decreases with expiration. This form of \u201crespiratory sinus arrhythmia\u201d characteristically presents with P waves of normal morphology consistent with originating from the sinus node. Each P wave is followed by a normal QRS complex, and the P-P intervals vary by more than 120 milliseconds or more than 10% of the shortest P-P interval. If the variation in rate is severe (i.e. a 100% change resulting in a heart rate of half), this is indicative of a more serious medical problem. One mechanism implicated in sinus arrhythmia involves the respiratory cycle and inherent cardiorespiratory interactions. It is postulated that inspiration inhibits vagal tone, thereby increasing the heart rate, and expiration restores the vagal tone to its previous state, such that the heart rate declines. \r\n\r\n\r\nIt is more noticeable in younger children as they have faster heart rates, and it has been seen in all children at some point during a 24-hour monitoring. Thus, in young, otherwise healthy patients without any other symptoms, sinus arrhythmia is considered a benign finding and further evaluation of sinus arrhythmia is usually unnecessary. However, severe sinus arrhythmia in older patients, often termed \u201cnon-respiratory sinus arrhythmia\u201d in which the heart rate does not vary with the phases of respiration, may indicate underlying cardiac disease, although it is not considered a marker for structural heart disease (Issa et al). \r\n\r\n\r\nThis patient\u2019s ECG rhythm strip shows monomorphic P waves that vary more than 10% of the shortest P-P interval. Each P-wave is followed by the patient\u2019s baseline QRS complex (which also shows a delta wave consistent with Wolff-Parkinson-White syndrome). This is consistent with a benign form of sinus arrhythmia which requires no treatment. Atrial fibrillation typically demonstrates an irregularly irregular rhythm and P waves are not visualized. Mobitz type II is a form of second-degree heart block characterized by a constant P-P interval without lengthening of the PR interval prior to a non-conducted P wave. This type of heart block is not a normal variant and is always considered pathologic. Common causes in children include post-cardiac surgery, myocarditis, orthotopic heart transplant rejection, and post-transplant coronary artery disease.\r\n\r\n\r\n \r\nREFERENCES \r\nCannon BC, Snyder CS. Disorders of cardiac rhythm and conduction. In: Shaddy R, Penny D, Feltes T, Cetta F and Mital S, eds. Moss and Adams\u2019 Heart Disease in Infants, Children and Adolescents including the Fetus and Young Adult<\/em>. 10th Edition. Philadelphia: Wolters Kluwer; 2022. 586-616.\r\n\r\n\r\nIssa ZF, Miller JM, Zipes DP. Sinus Node Dysfunction. In: Issa ZF, Miller JM, Zipes DP, Eds. Clinical Arrhythmology and Electrophysiology<\/em>. 3rd Edition. Elsevier; 2019: 238-254.\r\n”,”hint”:””,”answers”:{“crehu”:{“id”:”crehu”,”image”:””,”imageId”:””,”title”:”A. Atrial fibrillation”},”e2ood”:{“id”:”e2ood”,”image”:””,”imageId”:””,”title”:”B. Sinus arrhythmia”,”isCorrect”:”1″},”zqqus”:{“id”:”zqqus”,”image”:””,”imageId”:””,”title”:”C. Mobitz type II heart block”}}}}}

{“questions”:{“pe13n”:{“id”:”pe13n”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: David Fitzgerald, MD and Destiny F. Chau MD – Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences – Little Rock, AR \r\n\r\nAn 18-year-old woman with a history of exertional dyspnea and frequent lower respiratory tract infections undergoes bronchoscopy that demonstrates abnormal right lower lobe bronchial architecture. A chest x-ray demonstrates a nonspecific irregularity over the right lower lung field with dextroposition of the heart, and an echocardiogram reveals right heart dilation. Which of the following congenital heart defects is the MOST likely diagnosis?\r\n”,”desc”:”EXPLANATION \r\nScimitar syndrome is a subtype of partial anomalous pulmonary venous return (PAPVR) in which the right pulmonary vein drains abnormally to the inferior vena cava (IVC), typically near the level of the diaphragm. It is associated with varying degrees of right lung hypoplasia and cardiac dextroposition. The name, Scimitar syndrome, is owed to the crescent shape of the anomalous right pulmonary vein resembling the curved blade of a scimitar sword. The incidence is approximately one to three per 100,000 live births, and accounts for 0.5 to 1% of all congenital heart disease. \r\n\r\n\r\nScimitar syndrome can present in infancy or early adulthood. In infants, the median age at diagnosis is estimated to be seven months old. Among neonates diagnosed with Scimitar syndrome, up to 75% have associated anomalies, which include right lung hypoplasia, dextrocardia, right pulmonary artery hypoplasia, collateral blood vessels from infra-diaphragmatic aorta to the right lower lobe of the lung, secundum atrial septal defect (ASD), and diaphragmatic hernia. More severe cases are typically diagnosed within the first two months of life due to the presence of pulmonary hypertension, heart failure, and recurrent pulmonary infections. The adult form is typically milder in clinical presentation with the Scimitar vein often found incidentally in otherwise healthy patients. Symptomatic young adults usually present with recurrent pulmonary infections and exertional dyspnea from pulmonary sequestration. The characteristic Scimitar vein is often demonstrated on chest x-ray (CXR) (see Figure 1). \r\n\r\n\r\n\r\n\r\n\r\n\r\nFigure 1. Chest radiograph demonstrating Scimitar vein. https:\/\/commons.wikimedia.org\/wiki\/File:Scimitar_syndrome_chest_xray.jpg, licensed under the Creative Commons Attribution-Share Alike 4.0 International license.\r\n\r\nThe Scimitar vein produces a left to right shunt leading to excessive pulmonary blood flow and right heart dilation. Medical management centers on reducing excessive pulmonary blood flow to slow the progression to pulmonary hypertension and heart failure. Patients with the infantile form who exhibit signs of pulmonary hypertension require surgical correction. Other indications for surgical correction include a Q_{p<\/sub>:Qs<\/sub> greater than 1.5 in an asymptomatic patient, recurrent pneumonia, and heart failure. \r\n\r\n\r\nAny preoperative anesthetic evaluation should include a review of cardiac imaging and functional studies given the high association of Scimitar syndrome with other congenital cardiac defects. Evaluation should also include the presence and severity of pulmonary hypertension, quantification of left to right shunt, baseline cardiopulmonary status, and degree of pulmonary hypoplasia. While the adolescent and adult forms tend to be milder in clinical presentation, the presence of recurrent infections, lung hypoplasia and longstanding pulmonary circulatory overload should be closely evaluated prior to anesthetic administration. \r\n\r\n\r\nThis patient\u2019s history, clinical presentation, CXR, echocardiography and bronchoscopy strongly suggest a diagnosis of Scimitar syndrome. Though both sinus venosus atrial septal defect and Ebstein\u2019s anomaly lead to right heart dilation, neither defect is typically associated with right lung hypoplasia. \r\n\r\n\r\n\r\n \r\nREFERENCES \r\n\r\nMidyat L, Demir E, A\u015fkin M, et al. Eponym. Scimitar syndrome. Eur J Pediatr<\/em>. 2010;169(10): 1171-1177. doi:10.1007\/s00431-010-1152-4. \r\n\r\nBrown D, Geva T. Anomalies of the Pulmonary Veins. In: Shaddy R, Penny D, Feltes T, Cetta F and Mital S, eds. Moss and Adams\u2019 Heart Disease in Infants, Children and Adolescents including the Fetus and Young Adult. 10th Edition. Philadelphia: Wolters Kluwer; 2022: 854-856.\r\n\r\n\r\nChowdhury UK, Anderson RH, Sankhyan LK, et al. Surgical management of the scimitar syndrome. J Card Surg<\/em>. 2021; 36(10):3770-3795. doi:10.1111\/jocs.15857\r\n\r\n\r\nStout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA\/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology\/American Heart Association Task Force on Clinical Practice Guidelines [published correction appears in J Am Coll Cardiol<\/em>. 2019 May 14;73(18):2361]. J Am Coll Cardiol<\/em>. 2019;73(12):1494-1563. doi:10.1016\/j.jacc.2018.08.1028\r\n\r\n\r\n\r\n”,”hint”:””,”answers”:{“x73aq”:{“id”:”x73aq”,”image”:””,”imageId”:””,”title”:”A.\tEbstein\u2019s anomaly”},”62p0m”:{“id”:”62p0m”,”image”:””,”imageId”:””,”title”:”B.\tSinus venosus atrial septal defect”},”5iyl3″:{“id”:”5iyl3″,”image”:””,”imageId”:””,”title”:”C.\tScimitar syndrome\r\n\r\n”,”isCorrect”:”1″}}}}}}