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

{“questions”:{“h2cqv”:{“id”:”h2cqv”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB ChB, FRCA – Children\u2019s Medical Center, Dallas TX \r\n\r\nA dysmorphic, 3-week-old neonate is referred to pediatric cardiology due to a systolic murmur. Genetic testing revels a karyotype of 45 XO. What is the MOST likely congenital cardiac defect in this patient?”,”desc”:”EXPLANATION \r\nThe karyotype 45 XO is consistent with a diagnosis of Turner Syndrome (TS), which occurs in approximately 1 in 2500 live born females. Characteristic clinical features include a webbed neck, short stature, and lymphedema of the hands and feet. Cardiovascular disease is the major cause of death in TS. Congenital heart defects are found in about 50% of individuals with TS, consisting mostly of left-sided lesions. The most common cardiac abnormality is a bicuspid aortic valve (15% – 30% of cases), followed by coarctation of the aorta (7% – 18%). A recent American Heart Association Scientific Statement (1) has recommended that the presence of a bicuspid aortic valve or a left-sided obstructive lesion in a female patient should prompt a genetic evaluation of TS. Other less common but well recognized congenital cardiac defects associated with TS include partial anomalous pulmonary venous connection and hypoplastic left heart syndrome (HLHS). Coronary abnormalities are also common, particularly absent left main coronary. These are important to identify as they may have surgical implications. \r\n\r\n\r\nTurner Syndrome patients are at increased risk of aortic dissection at six times the rate as compared to the general population. Therefore, they require regular follow-up and serial imaging of the aorta, in addition to monitoring and treatment of hypertension. \r\n\r\n\r\nThe most common cardiac surgical procedures performed in TS patients are coarctation repair and aortic arch reconstruction. Due to lymphatic dysfunction, TS patients have a higher incidence of chylothorax and increased length of stay in the intensive care unit. \r\n\r\n\r\nTS patients with HLHS have a higher incidence of poor outcomes, though they have improved in the most recent era. Due to the increased mortality after the Stage I\/Norwood procedure, some centers are more likely to refer TS patients for primary cardiac transplantation as there is evidence of better clinical outcomes. \r\n\r\n\r\nTruncus arteriosus and other cono-truncal abnormalities are frequently associated with chromosome 22q11 deletion syndrome. \r\n\r\n\r\nSupravalvar aortic stenosis is a feature of Williams syndrome. \r\n\r\n\r\n\r\n \r\nREFERENCES \r\n1.\tSilberbach 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. https:\/\/doi.org\/10.1161\/HCG.0000000000000048 \r\n\r\n2.\tChew JD, Hill KD, Jacobs ML, et al. Congenital heart surgery outcomes in Turner Syndrome: The Society of Thoracic Surgeons Database Analysis. Ann Thorac Surg <\/em>.2019;108:1430-1438. \r\n3.\tPhilip J, Gupta D, Bleiweis MS, Pietra BA, Vyas HV. Hypoplastic left heart in Turner\u2019s syndrome: a primary indication for transplant. Card Young <\/em>. 2018; 28:458-460 \r\n4.\tChew JD, Soslow JH, Hall M, et al. Heart transplantation in children with Turner syndrome: Analysis of a linked dataset. Ped Cardiol . 2018; 39:610-616.\r\n”,”hint”:””,”answers”:{“984q5”:{“id”:”984q5″,”image”:””,”imageId”:””,”title”:”A.\tTruncus arteriosus”},”l2mhk”:{“id”:”l2mhk”,”image”:””,”imageId”:””,”title”:”B.\tBicuspid aortic valve”,”isCorrect”:”1″},”y7c6r”:{“id”:”y7c6r”,”image”:””,”imageId”:””,”title”:”C.\tSupravalvar aortic stenosis”},”5tu8y”:{“id”:”5tu8y”,”image”:””,”imageId”:””,”title”:”D.\tCoarctation of the aorta”}}}}}

{“questions”:{“ioram”:{“id”:”ioram”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB ChB, FRCA \u2013 Children\u2019s Medical Center, Dallas TX \r\n\r\nA 4-year-old previously healthy girl is admitted with fever, shortness of breath and abdominal pain. A chest x-ray demonstrates cardiomegaly and a transthoracic echocardiogram (TTE) reveals a large pericardial effusion. Vital signs are as follows: HR 150, BP 65\/45, RR 35. Which of the following echocardiographic findings is MOST diagnostic for cardiac tamponade?”,”desc”:”EXPLANATION \r\nCardiac tamponade is both a clinical diagnosis based on hypotension, tachycardia, and jugular venous distension and one based on echocardiographic findings. The decision to perform a pericardiocentesis may be assisted with echocardiography in determining the severity of tamponade and the potential for cardiovascular compromise during pericardiocentesis. Due to the fixed volume of the pericardial space, changes in intrapericardial pressure are transmitted to the cardiac chambers and the major systemic veins leading to core findings on echocardiogram including a pericardial effusion, diastolic right ventricle (RV) collapse, systolic right atrium (RA) collapse, plethoric IVC with minimal respiratory variation, and exaggerated respiratory changes in mitral valve (MV) and tricuspid valve (TV) inflow velocities. \r\n\r\nCollapse of the RA is seen at early ventricular systole when the RA is relaxed with low blood volume. Brief RA collapse can be seen without tamponade, but the presence of this sign for more than one third of the cardiac cycle is more specific for tamponade. The sensitivity of RA collapse during ventricular systole is higher ranging from 50% in early tamponade to 100% with worsening tamponade physiology. \r\n\r\nCollapse of the right ventricle (RV) is seen during early diastole when RV pressure is at its lowest. Diastolic collapse of the RV is highly specific with lower sensitivity for cardiac tamponade. It indicates very high intrapericardial pressures impeding RV filling. Longer duration of RV collapse correlates with increasing severity of tamponade. \r\n\r\nIncreased intrapericardial pressure impedes systemic venous return and this can be assessed by imaging the inferior vena cava (IVC) at its entrance into the RA. During normal spontaneous inspiration, the IVC diameter decreases by 50% or more. In tamponade physiology, the IVC remains distended (plethoric), and its diameter decreases by less than 50% during inspiration. However, even if there is minimal respiratory variation of IVC diameter with respiration, tamponade is very unlikely in the absence of chamber collapse. \r\n\r\nPulsus paradoxus is a normal physiologic phenomenon whereby the systolic blood pressure decreases by 10% or less during inspiration. Increased venous return to the RV during normal spontaneous inspiration occurs due to increasing negative intrathoracic pressure. This briefly shifts the ventricular septum into the left ventricle resulting in lower LV output and blood pressure. This phenomenon is exaggerated in tamponade physiology. These changes in systemic and pulmonary venous return can be characterized by measuring tricuspid and mitral inflow velocities and their changes with respiration. Under normal circumstances, these variations of inflow velocities during respiration do not exceed 20%-25%. However, during tamponade physiology, mitral inflow decreases<\/em> by 25% or more and tricuspid inflow velocity increases<\/em> by 40% or more. In the absence of any chamber collapse, these changes are not sensitive or specific for tamponade. \r\n\r\nREFERENCES \r\n1.\tAlerhand S, Carter JM. What echocardiographic findings suggest a pericardial effusion is causing tamponade?Am J Emerg Med <\/em>. 2019; 37:321-326. https:\/\/doi.org\/10.1016\/j.ajem.2018.11.004 \r\n2.\tHoit BD. Cardiac tamponade. In Uptodate; 2023. Accessed 1\/22\/2023. https:\/\/www.uptodate.com\/contents\/cardiac-tamponade#H22 \r\n3.\tKlein AL, Abbara S, Agler DA et al. American Society of Echocardiography Clinical Recommendations for Multimodality Cardiovascular Imaging of Patients with Pericardial Disease.J Am Soc Echocardiogr<\/em>. 2013; 26:96501012. http:\/\/dx.doi.org\/10.1016\/j.echo.2013.06.023 \r\n4.\tGuntheroth WG. Sensitivity and specificity of echocardiographic evidence of tamponade: implications for ventricular interdependence and pulsus paradoxus.Pediatr Cardiol <\/em>2007;28(5):358\u201362.\r\n\r\n”,”hint”:””,”answers”:{“rg6si”:{“id”:”rg6si”,”image”:””,”imageId”:””,”title”:”A.\tCollapse of the right atrium during ventricular diastole”},”p4rzx”:{“id”:”p4rzx”,”image”:””,”imageId”:””,”title”:”B.\tCollapse of the right ventricle during ventricular diastole”,”isCorrect”:”1″},”dt4rk”:{“id”:”dt4rk”,”image”:””,”imageId”:””,”title”:”C.\tDilated IVC with 50% reduction in diameter during inspiration”},”f3s41″:{“id”:”f3s41″,”image”:””,”imageId”:””,”title”:”D.\t15% decrease in mitral valve inflow velocity during inspiration”}}}}}

{“questions”:{“tmpk8”:{“id”:”tmpk8″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Daniela Perez-Velasco, DO; Chinwe Unegbu, MD; and Christopher Busack, MD \u2013 Children\u2019s National Hospital \r\n\r\nThe proposed renaming of the Blalock-Taussig (BT) shunt represents an attempt to acknowledge the tremendous contributions of which researcher, surgical assistant, and instructor?\r\n”,”desc”:”EXPLANATION \r\nVivien Theodore Thomas was born in New Iberia, Louisiana on August 29, 1910. He and his family later moved to Nashville, Tennessee where his father worked as a master carpenter. In 1929, after working to raise money for college and with plans to study medicine, he enrolled at Tennessee Agricultural and Industrial College. While Thomas dreamed of becoming a doctor, circumstances led to a different fate. His college savings were depleted during the Great Depression, forcing him to withdraw from college. \r\nOn February 10, 1930, Thomas walked into the animal lab of a young doctor named Dr. Alfred Blalock to interview for the job of laboratory assistant. In a matter of days, Thomas was administering anesthesia and performing arterial punctures on laboratory dogs. Over the many years at Vanderbilt, Blalock and Thomas worked to compile a massive amount of data pertaining to hemorrhagic and traumatic shock. \r\nAfter four years as a laboratory assistant, Thomas was promoted to the role of a senior research fellow. Unfortunately, Vanderbilt University classified him only as a janitor which was reflected in his salary. In 1937, Blalock then received an offer for the chairmanship and surgeon-in-chief at Henry Ford Hospital in Detroit. However, the hospital\u2019s policy against hiring blacks would result in rejection of the offer. In 1940, Blalock was offered the surgeon-in-chief position at his alma mater, Johns Hopkins Hospital. Blalock ensured that Thomas was also offered a position. They arrived in Baltimore in 1941, a time when the hospital had segregated restrooms and entrances to the hospital. \r\nDr. Helen Taussig soon approached Dr. Blalock, inquiring about a surgical remedy for the \u201cblue babies\u201d under her care. She wondered if there was a way to \u201cchange the pipes around\u201d and deliver more blood to the lungs. Blalock and Thomas had already done previous work to reroute arterial blood to the lungs. Thomas worked diligently to first reproduce the physiology of Tetralogy of Fallot (TOF) in the canine heart. Once Thomas had created the dog model of hypoxic circulation, he contributed to the concept and methodology of diverting blood from the subclavian artery to the pulmonary artery. Thomas single-handedly performed this procedure on nearly 200 dogs and taught Blalock the delicate vascular procedure. During a time that pre-dated vascular and microvascular surgery, many of the instruments and sutures were designed by Thomas. As Dr. Denton Cooley, who was trained by Thomas stated, \u201cThere wasn\u2019t a false move, not a wasted motion, when he operated.\u201d Dr. Blalock and Mr. Thomas were a remarkable combination. Thomas translated Blalock\u2019s concepts into reality, creating entire surgical procedures where none had existed previously. On November 29, 1944, the first blue baby operation was performed by Blalock. Vivien Thomas, high school graduate, son of a carpenter, and grandson of enslaved ancestors, guided one of the greatest surgeons of the 20th century, Dr. Alfred Blalock, through the revolutionary surgical procedure referred to at the time as the Blalock-Taussig shunt (BT shunt). Blalock insisted that Thomas stand by him on a step stool in order that he could guide Blalock through the surgery for he had only performed the procedure once as Thomas\u2019 assistant. It became widely known that while Blalock was operating, the space behind his right shoulder was reserved for Thomas. In 1946, Thomas went on to conceptualize and perform the atrial septectomy to improve circulation in patients with transposed great vessels. \r\nThe BT shunt ushered in a new era of pediatric cardiovascular surgery. It was the first pediatric cardiac surgical procedure performed in the United States. The Blalock-Taussig shunt was hailed as a miracle operation and established Johns Hopkins Hospital as the hospital to treat \u201cblue babies\u201d with TOF. When the work was reported in the press and published in the 1945 JAMA article entitled \u201cThe Surgical Treatment of the Malformations of the Heart: In Which There is Pulmonary Stenosis or Pulmonary Atresia\u201d by Blalock and Taussig, the name Vivien Thomas was not mentioned anywhere. Thomas\u2019 work was not recognized for the following 30 years. After 37 years at Johns Hopkins, Thomas was finally recognized as a Clinical Instructor and Faculty of the School of Medicine in 1976. Since an Honorary Doctorate of Medicine was not permitted at that time, he was presented with an Honorary Doctorate of Law. \r\nThe alliance between Thomas and Blalock was dramatized in the 2004 movie \u201cSomething the Lord Made\u201d, which was based on the autobiography of Thomas entitled \u201cPartners of the Heart\u201d. Thomas made a place for himself as a teacher to surgeons at a time when he could not become one himself. He was a surgical pioneer before Johns Hopkins opened its doors to the first African American surgical resident 30 years later, Dr. Levi Watkins. Thomas retired in 1979, battled pancreatic cancer, and was put to rest in 1985. \r\nIn operating rooms all over the world, a modified version of the BT shunt is still performed today. While Thomas\u2019 portrait hangs alongside other titans of medicine at Johns Hopkins Hospital, there has been a growing campaign to solidify his contributions with an official name change. In 2003, a proposal to rename the Blalock-Taussig shunt to the Blalock-Thomas-Taussig shunt was published in the journal Pediatric Critical Care Medicine and in 2009, a similar proposal was published in the journal Cardiology in the Young. The idea was again revisited in 2020 at the Society of Thoracic Surgeons Inaugural Vivien T. Thomas Lecture and in a 2022 opinion article published in the JAMA Surgery. \r\nChoice C is the correct answer because Vivien Thomas helped create the Blalock-Taussig shunt. Choice A is not correct. Dr. Daniel Hale Williams was one of the first African American physicians in the Chicago area and one of the first physicians to perform open heart surgery. Choice B is not correct. John Beauregard Johnson was an African American cardiologist and head of the Department of Medicine at Howard University. He pioneered the use of angiography and cardiac catheterization as diagnostic tools. Choice D is not correct as Dr. Levi Watkins Jr. was the first African American cardiac surgery resident at Johns Hopkins Hospital and performed the first implantation of an automatic implantable cardioverter defibrillator. \r\n\r\nREFERENCES \r\n \r\n\r\nThe Remarkable Story of Vivien Thomas, the Black Man Who Helped Invent Heart Surgery. Washingtonian<\/em>. 2020. https:\/\/www.washingtonian.com\/2020\/06\/19\/the-remarkable-story-of-vivien-thomas-the-black-man-who-helped-invent-heart-surgery. Accessed November 11, 2022. \r\nBlake K, Yancy CW. Change the Name of the Blalock-Taussig Shunt to Blalock-Thomas-Taussig Shunt. JAMA Surg.<\/em> 2022;157(4):287\u2013288. doi:10.1001\/jamasurg.2021.5611\r\nBlalock A., Taussig HB. The Surgical Treatment of Malformations of the Heart: In which There is Pulmonary Stenosis or Pulmonary Atresia. JAMA. <\/em> 1945;128(3):189-202. doi:10.100\/jama.1945.02860200029009\r\nKiran U, Aggarwal S, Choudhary A, Uma B, Kapoor PM. The Blalock and Taussig Shunt Revisited. Ann Card Anaesth<\/em>. 2017;20(3):323-330. doi:10.4103\/aca.ACA_80_17\r\nThomas VT. Partners of the Heart : Vivien Thomas and His Work with Alfred Blalock : An Autobiography<\/em>. Univ. Of Pennsylvania Press; 1998. \r\n\r\nJust One More-Vivien Thomas: Remembering a Pioneering Legend. Cardiology Magazine<\/em>. 2021. https:\/\/www.acc.org\/latest-in-cardiology\/articles\/2021\/02\/01\/01\/42\/just-one-more-vivien-thomas-remembering-a-pioneering-legend. Accessed September 20, 2022. \r\n\r\nBrogan TV, Alfieris GM. Has the Time Come to Rename the Blalock-Taussig Shunt? Pediatr Crit Care Med<\/em>. 2003;4(4):450-453. doi:10.1097\/01.PCC.0000090014.95775. \r\nEvans WN. The Blalock-Taussig shunt: The Social History of an Eponym. Cardiol Young<\/em>. 2009;19(2):119-128. doi:10.1017\/S1047951109003631.”,”hint”:””,”answers”:{“qf6f3”:{“id”:”qf6f3″,”image”:””,”imageId”:””,”title”:”A. Daniel Hale Williams”},”caeez”:{“id”:”caeez”,”image”:””,”imageId”:””,”title”:”B. John Beauregard Johnson”},”3pc0o”:{“id”:”3pc0o”,”image”:””,”imageId”:””,”title”:”C. Vivien Thomas”,”isCorrect”:”1″},”omq6n”:{“id”:”omq6n”,”image”:””,”imageId”:””,”title”:”D. Levi Watkins Jr.”}}}}}

{“questions”:{“u22op”:{“id”:”u22op”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Michael A. Evans, MD; Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine \r\n\r\nA 25-year-old woman with a history of hypoplastic left heart syndrome status-post Fontan palliation presents at 35-weeks gestation in active labor after spontaneous rupture of membranes. Which of the following complications is MOST LIKELY to occur in a parturient with single ventricle physiology intrapartum or postpartum?”,”desc”:”EXPLANATION \r\nMaternal congenital heart disease (CHD) poses a major risk to mother and child. There is a higher risk of spontaneous abortions and fetal demise in mothers with CHD when compared to the general population, a higher preterm birth rate (10-12% for all CHD), a higher frequency of adverse neonatal events (small for gestational age, respiratory distress syndrome, intraventricular hemorrhage), and a higher perinatal mortality rate (>4x higher than the general population). \r\n\r\nWhen pregnancies in mothers with Fontan physiology are examined, the adverse event rates throughout pregnancy, delivery, and postpartum are all increased when compared to other forms of CHD and in mothers without CHD. The most commonly observed complications in Fontan pregnancies and parturients include preterm delivery (59-82%), thromboembolic events (2-15%), postpartum hemorrhage or bleeding events (14-50%), fetal growth restriction (46-70%), small for gestational age (20-57%), pregnancy loss (27-69%), arrhythmia (0-29%), ventricular dysfunction or heart failure (4-14%), and neonatal death (2-7%). \r\n\r\nThe risk of preterm delivery (59-82% of Fontan pregnancies) is consistently significantly higher than any of the listed other observed complications, thus Answer D is correct. Although arrhythmias (Answer A, 0-29% of Fontan pregnancies) are more common in single ventricle mothers compared to the general population, they are not more common than preterm \r\ndeliveries. The same is true of postpartum hemorrhage (Answer B, 14-50%) and thromboembolic events (Answer C, 2-15%), which are both clinically important and contribute significantly to maternal morbidity in Fontan pregnancies. \r\n\r\nA summary of selected literature examining mothers with Fontan physiology and perinatal outcomes can be found below: \r\n\r\n\r\nREFERENCES \r\nClark DE, Staudt G, Byrne RD, et al. Anesthetic Management in Parturients With Fontan Physiology. J Cardiothorac Vasc Anesth. <\/em>2023;37(1):167-176. doi:10.1053\/j.jvca.2022.07.009 \r\n\r\nGirnius A, Zentner D, Valente AM, et al. Bleeding and thrombotic risk in pregnant women with Fontan physiology. Heart.<\/em> 2021;107(17):1390-1397. doi:10.1136\/heartjnl-2020-317397 \r\n\r\nGarcia Ropero A, Baskar S, Roos Hesselink JW, et al. Pregnancy in Women With a Fontan Circulation: A Systematic Review of the Literature. Circ Cardiovasc Qual Outcomes.<\/em> 2018;11(5):e004575. doi:10.1161\/CIRCOUTCOMES.117.004575 \r\n\r\nCauldwell M, Steer PJ, Bonner S, et al. Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair. Heart.<\/em> 2018;104(5):401-406. doi:10.1136\/heartjnl-2017-311763 \r\n\r\nCanobbio MM, Warnes CA, Aboulhosn J, et al. Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association. Circulation.<\/em> 2017;135(8):e50-e87. doi:10.1161\/CIR.0000000000000458 \r\n\r\nBonner SJ, Asghar O, Roberts A, Vause S, Clarke B, Keavney B. Cardiovascular, obstetric and neonatal outcomes in women with previous fontan repair [published correction appears in Eur J Obstet Gynecol Reprod Biol. 2018 Jan 4;:]. Eur J Obstet Gynecol Reprod Biol. <\/em>2017;219:53-56. doi:10.1016\/j.ejogrb.2017.10.013 \r\n\r\nMonteiro RS, Dob DP, Cauldwell MR, Gatzoulis MA. Anaesthetic management of parturients with univentricular congenital heart disease and the Fontan operation. Int J Obstet Anesth. <\/em>2016;28:83-91. doi:10.1016\/j.ijoa.2016.08.004 \r\n\r\nGouton M, Nizard J, Patel M, et al. Maternal and fetal outcomes of pregnancy with Fontan circulation: A multicentric observational study. Int J Cardiol. <\/em>2015;187:84-89. doi:10.1016\/j.ijcard.2015.03.344\r\n”,”hint”:””,”answers”:{“oheyk”:{“id”:”oheyk”,”image”:””,”imageId”:””,”title”:”A. Cardiac Arrhythmia”},”wyzn6″:{“id”:”wyzn6″,”image”:””,”imageId”:””,”title”:”B. Postpartum Hemorrhage”},”r51uh”:{“id”:”r51uh”,”image”:””,”imageId”:””,”title”:”C. Thromboembolic Event”},”3j4cf”:{“id”:”3j4cf”,”image”:””,”imageId”:””,”title”:”D. Preterm Delivery”,”isCorrect”:”1″}}}}}

{“questions”:{“h76h7”:{“id”:”h76h7″,”mediaType”:”video”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2023\/01\/PRE-REPAIR_-Mid-Esophageal-Aortic-Valve-Long-Axis.mp4″,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Kevin Spellman, MD and Michael A. Evans, MD; Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine \r\n\r\nA 3-year-old male toddler with a history of balanced, complete atrioventricular septal defect who is status post a two-patch repair presents to the operating room for resection of a subaortic membrane. Pre- and post-repair intraoperative transesophageal echocardiogram (TEE) images are illustrated below. \r\n\r\nBased on the TEE findings, which of the following is the MOST appropriate course of clinical management? \r\n\r\nTop: Clip 1 Preoperative: Mid Esophageal Aortic Valve Long Axis \r\nBottom: Clip 2 Post-Repair: Mid Esophageal Aortic Valve Long Axis \r\n”,”desc”:””,”hint”:””},”y9atq”:{“id”:”y9atq”,”mediaType”:”video”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2023\/01\/POST-REPAIR_-Mid-Esophageal-Aortic-Valve-Long-Axis.mp4″,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:””,”desc”:”EXPLANATION \r\nA subaortic membrane may occur alone or in combination with other congenital heart defects – such as Shone\u2019s complex, Tetralogy of Fallot, or ventricular septal defect. Discrete membranous subaortic stenosis (DMSS) is the most common type of congenital subvalvar aortic stenosis, which can occur alone or in association with other cardiac anomalies of the aortic and mitral valves. Mitral valve anomalies are present in 48% of patients with DMSS due to the close relationship between the mitral valve and the aortic valve. \r\n\r\nPatients with complete atrioventricular septal defects (AVSDs) are at risk for developing a subaortic membrane throughout life due to turbulent flow that occurs secondary to an elongated and narrowed left ventricular outflow tract (LVOT). Common complications that may occur after surgical resection of a subaortic membrane include residual or recurrent subaortic stenosis, heart block, septal perforation, mitral valve perforation, and mitral valve regurgitation. Often, a septal myotomy or myectomy is performed along with a subaortic membrane resection, which is associated with a decreased risk of subaortic membrane recurrence in patients who have had previous cardiac surgery. This benefit has not been reproduced in patients with an isolated subaortic membrane. \r\n\r\n\r\nTEE Clip 2, obtained after surgical resection of the subaortic membrane, demonstrates moderate to severe left atrioventricular valve (LAVV) regurgitation. Thus, the correct answer choice is B – resume cardiopulmonary bypass and repair the LAVV. \r\n\r\n\r\nThe close association of the subaortic membrane with the anterior leaflet of the LAVV creates a potential hazard for anterior leaflet perforation or injury when resecting a subaortic membrane. Acute LAVV regurgitation is not tolerated well hemodynamically due to a sudden development of left atrial hypertension, diminished stroke volume due to regurgitant blood flow, and a subsequent reduction in cardiac output. Recognition of acute LAVV regurgitation on a TEE can be delayed when the severity is underestimated due to a low flow state across the valve secondary to acute heart failure. \r\n\r\n\r\nAnswer A (to resect additional subaortic membrane) is incorrect, as the post-repair TEE demonstrates more laminar flow in the LVOT. Answer C (perform aortic valve replacement) is incorrect, as the post-repair echo demonstrates only trivial aortic insufficiency. Aortic insufficiency is commonly seen in patients with a subaortic membrane and typically improves after resection of the membrane. Answer D (administer protamine) is incorrect because the acute LAVV regurgitation should be repaired as it may not be well tolerated hemodynamically and may worsen over time. \r\n\r\n\r\n \r\nREFERENCES \r\nSerraf A, Zoghby J, Lacour-Gayet F, et al. Surgical treatment of subaortic stenosis: a seventeen-year experience. J Thorac Cardiovasc Surg<\/em>. 1999;117(4):669-678. doi:10.1016\/S0022-5223(99)70286-2 \r\n\r\n\r\nOzyuksel A, Yildirim O, Onsel I, Bilal MS. Severe mitral regurgitation due to anterior mitral leaflet perforation after surgical treatment of discrete subaortic stenosis. BMJ Case Rep<\/em>. 2014;2014:bcr2014204463. Published 2014 May 23. doi:10.1136\/bcr-2014-204463 \r\n\r\n\r\nCohen L, Bennani R, Hulin S, et al. Mitral valvar anomalies and discrete subaortic stenosis. Cardiol Young<\/em>. 2002;12(2):138-146. doi:10.1017\/s104795110200029x \r\n\r\n\r\nEdwards H, Mulder DG. Surgical Management of Subaortic Stenosis. Arch Surg<\/em>.1983; 118(1): 79-83. doi:10.1001\/archsurg.1983.01390010055013 \r\n\r\n\r\nFong LS, Betts K, Bell D, et al. Complete atrioventricular septal defect repair in Australia: Results over 25 years. J Thorac Cardiovasc Surg<\/em>. 2020;159(3):1014-1025.e8. doi:10.1016\/j.jtcvs.2019.08.005 \r\n\r\n\r\nPerez Y, Dearani JA, Miranda WR, Stephens EH. Subaortic Stenosis in Adult Patients With Atrioventricular Septal Defect [published online ahead of print, 2022 Aug 17]. Ann Thorac Surg<\/em>. 2022;S0003-4975(22)01115-8. doi:10.1016\/j.athoracsur.2022.08.011 \r\n\r\n\r\nTalwar S, Anand A, Gupta SK, et al. Resection of subaortic membrane for discrete subaortic stenosis. J Card Surg<\/em>. 2017;32(7):430-435. doi:10.1111\/jocs.13160 \r\n\r\n\r\nZoghbi WA, Adams D, Bonow RO, et al. Recommendations for Noninvasive Evaluation of Native Valvular Regurgitation: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance. J Am Soc Echocardiogr<\/em>. 2017;30(4):303-371. doi:10.1016\/j.echo.2017.01.007 \r\n\r\n\r\nHirata Y, Chen JM, Quaegebeur JM, Mosca RS. The role of enucleation with or without septal myectomy for discrete subaortic stenosis. J Thorac Cardiovasc Surg<\/em>. 2009;137(5):1168-1172. doi:10.1016\/j.jtcvs.2008.11.039 \r\n”,”hint”:””,”answers”:{“8kxug”:{“id”:”8kxug”,”image”:””,”imageId”:””,”title”:”A.\tResume cardiopulmonary bypass and resect residual subaortic membrane\r\n”},”oq2a9″:{“id”:”oq2a9″,”image”:””,”imageId”:””,”title”:”B.\tResume cardiopulmonary bypass and repair the left atrioventricular valve “,”isCorrect”:”1″},”bxt2p”:{“id”:”bxt2p”,”image”:””,”imageId”:””,”title”:”C.\tResume cardiopulmonary bypass and perform aortic valve replacement”},”6400w”:{“id”:”6400w”,”image”:””,”imageId”:””,”title”:”D.\tAdminister protamine “}}}}}