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

{“questions”:{“nvky1”:{“id”:”nvky1″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Christopher Busack MD, Chinwe Unegbu MD, and Daniela Perez-Velasco DO \u2013 Children\u2019s National Hospital \r\n\r\nA 6-month-old male with Hypoplastic Left Heart Syndrome (HLHS) s\/p Norwood\/Sano modification receives an ABO-incompatible heart transplant secondary to severe tricuspid regurgitation and right ventricular dysfunction. After separation from cardiopulmonary bypass, the patient received 10 mL\/kg each of platelets, cryoprecipitate, fresh frozen plasma, and packed red blood cells. Which of the administered blood products carries the HIGHEST RISK of transmitting viral pathogens?\r\n\r\n”,”desc”:”EXPLANATION \r\n\r\nTransfusion of blood products has significant risks and benefits which must be carefully balanced. A recent publication by Faraoni et al. provides evidence-based guidelines for the use of blood products in the perioperative period for children undergoing cardiac surgery. \r\nIn the US, to facilitate more targeted transfusion of deficient blood components, whole blood is typically separated into various components. Fresh frozen plasma (FFP) is frozen within 8 hours of collection. Cryoprecipitate, also known as antihemophilic factor, is then prepared from FFP in the following manner. FFP is slowly thawed to 1-6 ^{o <\/sup>C followed by centrifugation. High-molecular-weight plasma proteins form a precipitate during centrifugation known as cryoprecipitate, which is then is collected, resuspended in a small amount of plasma (10-15 mL) and refrozen. Cryoprecipitate has several important coagulation proteins including fibrinogen, antihemophilic factor (factor VIII), von Willebrand factor, fibrin stabilizing factor (factor XIII), fibronectin, and small amounts of other plasma proteins. Cryoprecipitate has higher concentrations of coagulation proteins due to the smaller volume; making this blood product more appropriate for smaller patients in need of an increased concentration of the aforementioned coagulation proteins. Cryoprecipitate doses of 1-2 units per 10 kg of body weight will increase fibrinogen concentration by 60 to 100 mg\/dL. \r\nIn the 1960\u2019s, cryoprecipitate was used to manage patients with Hemophilia A. The initial preparations of cryoprecipitate had relatively low Factor VIII concentrations. Therefore, Hemophilia A patients required transfusion of multiple bags of cryoprecipitate to achieve an adequate dose of 20-30 IU\/kg. Additionally, cryoprecipitate was pooled from large amounts of plasma obtained from multiple donors. Unfortunately, these specimens were often contaminated with hepatitis viruses. It is estimated that in the 1980\u2019s, HIV was transmitted to 50% of people living with Hemophilia A. In current medical practice, cryoprecipitate is used to correct fibrinogen deficiency due to acute blood loss (secondary to trauma, surgery, etc) or qualitative abnormalities of the fibrinogen protein. Currently, there are no widely available commercial methods of inactivating viral pathogens in FFP or cryoprecipitate. As cryoprecipitate is a pooled product, its administration carries the highest risk of the transmission of viral pathogens (Munlemvo et al). Many Western European countries and Canada have banned the use of cryoprecipitate due to concerns about pathogen transmission. Cryoprecipitate is no longer recommended to treat von Willebrand\u2019s Disease or Hemophilia A unless recombinant or viral inactivated products are not available. The availability of highly purified coagulation factor concentrates and recombinant protein preparations has superseded the use of cryoprecipitate for many coagulopathies. \r\nIn contrast, platelets are associated with the highest risk of bacterial contamination due to the need for storage at room temperature. Transfusion transmitted infections, although rare, can have serious consequences. Nucleic acid testing (NAT) has increased the sensitivity of detecting infectious agents in donor blood. This form of testing directly tests the donated blood for viruses, whereas previous screening was done by testing antibody response to viral infection. NAT has reduced the risk of infectious transmission to less than 1 in 1,000,000. Donor blood is now screened for many viruses and bacteria such as the following: HIV, hepatitis B, hepatitis C, HTLV, syphilis, West Nile virus, Chagas disease, Zika virus, and babesiosis. Leukoreduction of blood products is also used to decrease the risk of CMV transmission, which can be fatal in newborns and immunocompromised patients. \r\nTransfusion of blood products carries additional risks occurring with greater frequency than infectious transmission. Recent investigations suggest that blood product transfusion during pediatric cardiac surgery may be associated with adverse outcomes such as renal failure, low cardiac output, vascular thrombosis, and alloimmunization leading to increased time to orthotopic transplantation, prolonged hospital stay, and increased mortality. Guidelines have shifted away from recommending specific hemoglobin targets and instead weighing the risk of complications from blood product transfusion versus the risk of complications from inadequate oxygen-carrying capacity, inadequate hemostasis, and\/or inadequate intravascular volume. \r\nREFERENCES \r\n\r\n1. Faraoni D, Meier J, New HV, Van der Linden PJ, Hunt BJ. Patient Blood Management for Neonates and Children Undergoing Cardiac Surgery: 2019 NATA Guidelines. J Thorac Cardiovasc Anesth.<\/em> 2019; 33:3249-3263. https:\/\/doi.org\/10.1053\/j.jvca.2019.03.036 \r\n\r\n2. Munlemvo DM, Tobias JD, Chenault KM, Naguib A. Prothrombin Complex Concentrates to Treat Coagulation Disturbances: An Overview with a Focus on Use in Infants and Children. Cardiol Res.<\/em> 2022;13(1):18-26. doi:10.14740\/cr1342 \r\n\r\n3. Steinbicker AU, Wittenmeier E, Goobie SM. Pediatric non-red cell blood product transfusion practices: what\u2019s the evidence to guide transfusion of the \u201cyellow\u201d blood products? Curr Opin Anesthesiol. <\/em>2020;33(2):259-267. doi:10.1097\/ACO.0000000000000838 \r\n\r\n4. Busch MP, Bloch EM, Kleinman S. Prevention of transfusion-transmitted infections. Blood.<\/em> 2019;133(17):1854-1864. doi:10.1182\/blood-2018-11-833996 \r\n\r\n5. Sparrow RL, Simpson RJ, Greening DW. Preparation of Cryoprecipitate and Cryo-depleted Plasma for Proteomic Research Analysis. Methods Mol Biol.<\/em> 2023;2628:41-49. doi:10.1007\/978-1-0716-2978-9_\r\n”,”hint”:””,”answers”:{“68u32”:{“id”:”68u32″,”image”:””,”imageId”:””,”title”:”A. Platelets”},”p4irc”:{“id”:”p4irc”,”image”:””,”imageId”:””,”title”:”B. Cryoprecipitate”,”isCorrect”:”1″},”zah5m”:{“id”:”zah5m”,”image”:””,”imageId”:””,”title”:”C. Fresh Frozen Plasma (FFP)”},”5ajdc”:{“id”:”5ajdc”,”image”:””,”imageId”:””,”title”:”D. Packed Red Blood Cells (PRBC)”}}}}}}

{“questions”:{“i1s4t”:{“id”:”i1s4t”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Christopher Busack MD, Chinwe Unegbu MD, and Daniela Perez-Velasco DO \u2013 Children\u2019s National Hospital \r\nA 3-month-old female patient presents with failure to thrive. Echocardiography reveals severe LV dysfunction, severe mitral regurgitation, and an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).Which operative intervention MOST LIKELY reduces the risk of in-hospital death in patients undergoing surgical repair of ALCAPA?\r\n\r\n\r\n”,”desc”:”EXPLANATION \r\nAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) comprises less than 1% of congenital heart defects. There are two clinical phenotypes: (1) infants with an absence of adequate collateral circulation from the right coronary artery (RCA) to the left side of the heart who typically present with severe heart failure symptoms within the first few months of life and (2) patients with adequate collateral supply who remain asymptomatic until adolescence or adulthood. However, patients with collateral supply remain at risk for sudden cardiac death and may present with chest pain or dyspnea on exertion. Without surgical intervention, the mortality exceeds 90% at this time. However, with surgery and current advances, the mortality at the time of ALCAPA repair is less than 10%. Echocardiography is the mainstay of diagnosis but CT angiography or magnetic resonance imaging with 3D reconstruction can be helpful. Angiography is rarely indicated except in special situations. \r\n\r\nAlthough mitral regurgitation (MR) is commonly associated with ALCAPA, there is no consensus as to whether it should be surgically corrected at the time of primary repair. Obviously, correction would add ischemia time to an already severely compromised ventricle. In most cases, MR improves without intervention after surgery to correct ALCAPA alone due to improved ventricular function and reduced end-diastolic volume. However, MR persists in some cases and may require intervention later. Several recent studies indicate that repair of severe MR at the time of initial surgery can be achieved with low risk, improved outcomes, and a reduced need for reoperation. These data support a selective surgical approach for significant MR at the time of ALCAPA surgery, perhaps focusing on cases with structural defects unlikely to change with improvement in myocardial function or regression of ventricular dilation. Neither prophylactic ventricular assist device insertion nor pulmonary arterioplasty at the time of ALCAPA repair reduce the risk of in-hospital death. \r\nREFERENCES \r\n\r\n1.\tThomas AS, Chan A, Alsoufi B, Vinocur JM, Kochilas L. Long-term Outcomes of Children Operated on for Anomalous Left Coronary Artery From the Pulmonary Artery. Ann Thorac Surg. <\/em>2022;113(4):1223-1230. doi: 10.1016\/j.athoracsur.2021.07.053. \r\n\r\n2.\tYu J, Ren Q, Liu X, et al. Anomalous left coronary artery from the pulmonary artery: Outcomes and management of mitral valve. Front Cardiovasc Med. <\/em>2022;9:953420. doi: 10.3389\/fcvm.2022.953420. \r\n\r\n3.\tWeixler VHM, Zurakowski D, Baird CW, et al. Do patients with anomalous origin of the left coronary artery benefit from an early repair of the mitral valve? Eur J Cardiothorac Surg.<\/em> 2020;57(1):72-77. doi: 10.1093\/ejcts\/ezz158.\r\n\r\n”,”hint”:””,”answers”:{“25awu”:{“id”:”25awu”,”image”:””,”imageId”:””,”title”:”A. Prophylactic ventricular assist device insertion at time of ALCAPA repair”},”kkhq0″:{“id”:”kkhq0″,”image”:””,”imageId”:””,”title”:”B. Concomitant mitral valve surgery at time of ALCAPA repair”,”isCorrect”:”1″},”v9zmu”:{“id”:”v9zmu”,”image”:””,”imageId”:””,”title”:”C. Pulmonary arterioplasty at the time of ALCAPA repair”}}}}}

{“questions”:{“ixofz”:{“id”:”ixofz”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Christopher Busack MD, Chinwe Unegbu MD, and Daniela Perez-Velasco DO \u2013 Children\u2019s National Hospital \r\n\r\nA 4-year-old male patient with an atrial septal defect presents for surgical repair with cardiopulmonary bypass. The surgeon plans on using a clear pump prime with a hyperpolarized cardioplegic arrest. Which cardioplegia solution produces a hyperpolarized cardiac arrest?\r\n\r\n “,”desc”:”EXPLANATION \r\nThe ability to induce cardiac arrest and facilitate open-heart surgery by infusing a high potassium-containing solution into the coronaries was first demonstrated by Melrose et al. in 1955 in a canine experimental model. Different cardioplegia solutions today consist of varying amounts of electrolytes (potassium, sodium, calcium and magnesium), buffers and medications. Cardioplegia is administered after cross clamp of the aorta either in an antegrade fashion via the coronary arteries or retrograde via the coronary sinus. Cardioplegic solutions cause diastolic arrest, decrease cardiac metabolic demand, and improve myocardial tolerance to ischemia. \r\n\r\nThe most common method for achieving cardiac arrest is by providing a high concentration of potassium (K^{+ <\/sup>) ions into the extracellular space. Extracellular cardioplegic solutions often contain high concentrations of sodium (Na+ <\/sup>), calcium (Ca2+ <\/sup>), potassium (K+ <\/sup>), magnesium (Mg2+ <\/sup>), and bicarbonate and cause cardiac arrest by depolarizing the myocardial membrane. The Buckberg and St. Thomas\u2019 Hospital cardioplegia solutions both contain high K+ <\/sup>content and are delivered with blood. A major drawback with both of these solutions is that they require frequent re-dosing. \r\n\r\nA concern with depolarizing arrest is Ca2+ <\/sup> accumulation in the myocytes, thereby preventing myocardial relaxation. To mitigate this effect, contemporary depolarizing cardioplegia solutions contain lidocaine and magnesium. These agents repolarize the cell membrane to some degree and prevent Na+ <\/sup> and Ca2+ <\/sup> accumulation within the cell. Del Nido cardioplegia includes lidocaine and magnesium and is categorized as a modified extracellular depolarizing solution. A 2016 retrospective review by Buel et al. demonstrated a six-fold decrease in the rate of defibrillation post cross-clamp with del Nido cardioplegia compared to the St. Thomas Hospital solution. The del Nido cardioplegia solution was developed for pediatric cardiac surgery due to the specific needs of an immature and developing myocardium. The immature myocardium has a higher sensitivity to intracellular Ca2+ <\/sup> since the sarcoplasmic reticulum is underdeveloped with a reduced capacity to store Ca2+ <\/sup>. Studies in adults have also shown benefit of the del Nido solution and it is used in many adult centers. \r\n\r\nIntracellular cardioplegia solutions have low levels of Na+ <\/sup>and Ca2+ <\/sup> mimicking the intracellular electrolyte concentration. These solutions induce a hyperpolarizing arrest of the myocardium which decreases energy consumption and intracellular accumulation of Ca2+ <\/sup>. Histidine\u2013tryptophan\u2013ketoglutarate (HTK) solution (Custodiol HTK\u00ae\/Bretschneider solution) is an intracellular cardioplegic solution that was introduced in the 1970s. Histidine buffers ischemia-induced acidosis; tryptophan is an effective cell membrane stabilizer; ketoglutarate enhances energy production and recovery following reperfusion; and mannitol minimizes cellular edema by maintaining the osmolality of the cellular environment and functions as a free radical scavenger. HTK solution is particularly useful for long complex repairs as it reliably produces cardiac arrest for up to 120 minutes without redosing.\r\n\r\nThere is no consensus regarding the \u201cbest\u201d cardioplegia solution. Numerous studies have demonstrated that multiple cardioplegia options safely achieve myocardial protection. However, a recent 2018 study by Panigrahi et al suggests that the del Nido solution may offer some additional benefits including quicker resumption of normal cardiac rhythm and decreased inotropic support compared to conventional blood cardioplegia. A 2019 randomized controlled trial by Talwar et al. compared HTK solution and del Nido solution. The del Nido group demonstrated a better cardiac index, less mechanical ventilation days, and shorter ICU stays. Electron microscopy also showed less edema of the myocardium and better myofibrillar architecture with del Nido solution. The higher cellular edema seen with the HTK solution may be related to its very low sodium content. Nonetheless, there is more research to be done in this area.\r\n\r\n\r\n \r\nREFERENCES \r\n1.\tMelrose DG, Dreyer B, Bentall HH, Baker JB. Elective cardiac arrest. Lancet <\/em>. 1955;269(6879):21-2. doi: 10.1016\/s0140-6736(55)93381-x \r\n\r\n2.\tTalwar S, Chatterjee S, Sreenivas V, et al. Comparison of del Nido and histidine-tryptophan-ketoglutarate cardioplegia solutions in pediatric patients undergoing open heart surgery: A prospective randomized clinical trial. J Thorac Cardiovasc Surg <\/em>. 2019;157(3):1182-1192.e1. doi: 10.1016\/j.jtcvs.2018.09.140 \r\n\r\n3.\tWatanabe M, Egi K, Shimizu M, et al. Non-depolarizing cardioplegia activates Ca2+-ATPase in sarcoplasmic reticulum after reperfusion. Eur J Cardiothorac Surg<\/em>. 2002;22(6):951-6. doi: 10.1016\/s1010-7940(02)00582-1. \r\n\r\n4.\tPanigrahi D, Roychowdhury S, Guhabiswas R, Rupert E, Das M, Narayan P. Myocardial protection following del Nido cardioplegia in pediatric cardiac surgery. Asian Cardiovasc Thorac Ann<\/em> 2018;26(4):267-272. doi: 10.1177\/0218492318773589 \r\n\r\n5.\tBibevski S, Mendoza L, Ruzmetov M, et al. Custodiol cardioplegia solution compared to cold blood cardioplegia in pediatric cardiac surgery: a single-institution experience. Perfusion <\/em>.2020;35(4):316-322. doi: 10.1177\/0267659119878006 \r\n\r\n6.\tMatte GS, del Nido PJ. History and use of del Nido cardioplegia solution at Boston Children’s Hospital [published correction appears in J Extra Corpor Technol. 2013;45(4):262]. J Extra Corpor Technol <\/em>. 2012;44(3):98-103.\r\n\r\n7.\tDamiano RJ Jr, Cohen NM. Hyperpolarized arrest attenuates myocardial stunning following global surgical ischemia: an alternative to traditional hyperkalemic cardioplegia? J Card Surg <\/em>. 1994;9(3 Suppl):517-25. doi: 10.1111\/jocs.1994.9.3s.517 \r\n\r\n8.\tGiordano R, Arcieri L, Cantinotti M, et al. Custodiol Solution and Cold Blood Cardioplegia in Arterial Switch Operation: Retrospective Analysis in a Single Center. Thorac Cardiovasc Surg<\/em>. 2016;64(1):53-8. doi: 10.1055\/s-0035-1566235 \r\n\r\n9.\tGhiragosian C, Harpa M, Stoica A, et al. Theoretical and Practical Aspects in the Use of Bretschneider Cardioplegia. J Cardiovasc Dev Dis<\/em>. 2022;9(6):178. doi: 10.3390\/jcdd9060178 \r\n\r\n10.\tTurkoz R. Myocardial protection in pediatric cardiac surgery. Artif Organs<\/em>. 2013;37(1):16-20. doi: 10.1111\/aor.12029\r\n\r\n”,”hint”:””,”answers”:{“dn2p6”:{“id”:”dn2p6″,”image”:””,”imageId”:””,”title”:”A. Buckberg solution”},”ozwpt”:{“id”:”ozwpt”,”image”:””,”imageId”:””,”title”:”B. St. Thomas solution”},”m090t”:{“id”:”m090t”,”image”:””,”imageId”:””,”title”:”C. Histidine\u2013tryptophan\u2013ketoglutarate (HTK) solution”,”isCorrect”:”1″},”3fcsw”:{“id”:”3fcsw”,”image”:””,”imageId”:””,”title”:”D. del Nido solution\r\n\r\n”}}}}}}

{“questions”:{“n0imx”:{“id”:”n0imx”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2023\/03\/CCAS-Graphic-JPEG.jpg”,”imageId”:”6384″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB, ChB, FRCA – Children\u2019s Medical Center, Dallas \r\nA 3-year-old female toddler with a large atrial septal defect (ASD) in addition to dysplastic mitral and tricuspid valves (both with severe regurgitation) presents for repair of both valves and ASD closure. After intubation, vital signs are as follows: HR 120, BP 90\/50, SpO2 92%. During the pre-bypass transesophageal echo (TEE), agitated saline contrast is injected into a left arm peripheral IV with two of the TEE images demonstrated below. What is the MOST likely diagnosis? \r\n\r\n”,”desc”:”EXPLANATION \r\nIn the TEE images above, contrast is clearly seen entering the left atrium initially and then into the left ventricle, confirming the presence of a left superior vena cava (LSVC) draining into an unroofed coronary sinus. A persistent LSVC is found in approximately 4% of patients with congenital heart disease compared with 0.5% in the general population. In 80-90% of cases, it drains into the right atrium via the coronary sinus. In 10-20% of cases, the LSVC drains into the left atrium due to partial or complete absence of the \u201croof\u201d of the coronary sinus. It can be diagnosed with echocardiography by injecting agitated saline contrast into an IV placed in the left arm. \r\n\r\nAn unroofed coronary sinus has several important clinical implications including: (1) It is a source of right to left shunting (often called a coronary sinus ASD) leading to mild cyanosis; (2) It can lead to systemic thromboembolism, potentially resulting in an ischemic stroke or cerebral abscess; (3) It can impact repair of an ostium primum ASD and usually requires baffling into the right atrium. \r\n\r\n\r\nA persistent LSVC also has important clinical implications: (1) It may require additional venous drainage cannulation during cardiopulmonary bypass cases, depending on the presence of a bridging vein; (2) An unrecognized LSVC can be a source of systemic desaturation in a bidirectional cavopulmonary anastomosis or a Fontan; (3) A heart transplant recipient with a LSVC will require modification to the venous drainage anastomoses; (3) It may render retrograde cardioplegia ineffective; (4) It can complicate procedures such as central venous cannulation or pacemaker implantation. \r\n\r\n\r\nPulmonary arteriovenous fistulas are congenital or acquired intrapulmonary right-to-left shunts. The diagnosis can be confirmed with echocardiography by showing a shortened transpulmonary transit time from the right heart to the left atrium during injection of contrast into a peripheral IV. \r\n\r\n\r\nA sinus venosus defect will show contrast entering the right atrium first, and therefore, answer C is incorrect. \r\n\r\nREFERENCES \r\n \r\n\r\n1.\tAzizova A, Onder O, Arslan S, Ardali S, Hazirolan T. Persistent left superior vena cava: clinical importance and differential diagnoses. Insights into Imaging. <\/em>2020. 11:110. https:\/\/doi.org\/10.1186\/s13244-020-00906-2 \r\n2.\tOotaki Y, Yamaguchi M, Yoshimura N et al. Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J Thorac Cardiovasc Surg. <\/em> 2003; 126:1655-6. doi:10.1067\/S0022-5223(03)01019-5\r\n\r\n”,”hint”:””,”answers”:{“e61zy”:{“id”:”e61zy”,”image”:””,”imageId”:””,”title”:”A.\tLeft superior vena cava (LSVC) with an unroofed coronary sinus”,”isCorrect”:”1″},”vcajs”:{“id”:”vcajs”,”image”:””,”imageId”:””,”title”:”B.\tPulmonary arteriovenous fistula”},”0hy3h”:{“id”:”0hy3h”,”image”:””,”imageId”:””,”title”:”C.\tSinus venosus atrial septal defect”}}}}}

{“questions”:{“h0uon”:{“id”:”h0uon”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB ChB, FRCA. Children\u2019s Medical Center, Dallas \r\n\r\nA 17-year-old male with a history of hypertrophic cardiomyopathy presents to cardiology clinic for routine follow-up. During transthoracic echocardiography, which of the following maneuvers is MOST likely to reveal a latent gradient across the left ventricular outflow tract?”,”desc”:”EXPLANATION \r\nHypertrophic cardiomyopathy (HCM) is the most common cause of sudden death in competitive athletes. The most common etiology is ventricular arrhythmia secondary to myocardial fibrosis, microvascular coronary disease, and structural disarray of cardiomyocytes. A comprehensive evaluation for HCM includes a detailed family history as well as a thorough clinical history and physical exam with the following diagnostic work up: (1) 12-lead and Holter ECG; (2) transthoracic echocardiography (TTE) to assess wall thickness, left ventricular outflow tract (LVOT) gradient, and degree of mitral regurgitation; (3) cardiac MRI to detect late gadolinium enhancement (LGE) as a marker for fibrosis; and (4) genetic testing. \r\n\r\nAn LVOT gradient of > 50 mmHg is a hemodynamically significant gradient. If less than 50 mmHg, provocative measures may be used to assess the dynamic component of the LVOT gradient. Maneuvers that increase LV afterload, such as isometric hand grip, squatting, and phenylephrine infusion, will reduce the gradient. Factors that decrease afterload and\/or increase contractility will increase the gradient and can be useful in detecting a latent gradient. TTE after stress with exercise is the preferred method to detect LVOT gradient versus pharmacologic stress. The effect of various maneuvers on the LVOT gradient are summarized in the table. \r\n\r\nAssessment of risk for sudden cardiac death (SCD) is a key component to managing patients with HCM. Any HCM patient surviving cardiac arrest is a candidate for secondary prevention with an implantable cardioverter-defibrillator (ICD) device. Guidelines for primary prevention have recently been published by the American Heart Association and the American College of Cardiology (1,2). Accurate risk stratification for primary prevention is important as ICDs are associated with significant morbidity. This includes inappropriate defibrillation in approximately 30% of patients as well as negative psychological repercussions and the imposition of limitations on participation in certain sports. Major factors under recommendation for risk stratification include: (1) Family history of sudden death; (2) extreme LV hypertrophy; (3) unexplained syncope; (4) non-sustained ventricular tachycardia; (5) LGE on cardiac MRI; (6) end-stage HCM, and (7) LV apical aneurysm. Family history of sudden death, extreme LV hypertrophy, and unexplained syncope are the most important factors for risk stratification of children and adolescents. Patients with one or more of these factors are candidates for an ICD device. \r\n\r\nREFERENCES \r\n1.\tOmmen SR, Mital S, Burke MA et al. 2020 AHA\/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy. J Am Coll Cardiol <\/em>. 2020;76:e159-e240. https:\/\/doi.org\/10.1016\/j.jacc.2020.08.045 \r\n\r\n2.\tMaron BJ, Desai MY, Nishimura RA et al. Management of hypertrophic cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol <\/em>.2022; 79:390-414. https:\/\/doi.org\/10.1016\/j.jacc.2021.11.021\r\n\r\n3.\tMaron 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; 372-389. https:\/\/doi.org\/10.1016\/j.jacc.2021.12.002\r\n\r\n”,”hint”:””,”answers”:{“kc7q5”:{“id”:”kc7q5″,”image”:””,”imageId”:””,”title”:”A.\tIsometric hand grip”},”lq4f2″:{“id”:”lq4f2″,”image”:””,”imageId”:””,”title”:”B.\tExercise”,”isCorrect”:”1″},”nydnn”:{“id”:”nydnn”,”image”:””,”imageId”:””,”title”:”C.\tPhenylephrine infusion”},”2b5ro”:{“id”:”2b5ro”,”image”:””,”imageId”:””,”title”:”D.\tSquatting”}}}}}