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

{“questions”:{“ikzep”:{“id”:”ikzep”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2021\/06\/QOW-Pic-PNG.png”,”imageId”:”4775″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Sana Ullah, MB ChB, FRCA – Children\u2019s Medical Center, Dallas\r\n\r\nA 16-year-old girl had a syncopal episode at home while watching television. She had a concurrent viral illness with a high fever. There was no previous history of syncope or palpitations and no family history of sudden death. Vital signs after the event were the following:\r\nHeart rate: 80 beats per minute\r\nBlood pressure: 120\/70\r\nPulse oximetry 97% on room air\r\nA 12-lead ECG was completed \u2013 see image below. An echocardiogram revealed no structural cardiac abnormalities. What is the MOST APPROPRIATE next step in the management of this patient?\r\n”,”desc”:””,”hint”:””,”answers”:{“nroyn”:{“id”:”nroyn”,”image”:””,”imageId”:””,”title”:”A.\tCT scan of the brain”},”ah6ee”:{“id”:”ah6ee”,”image”:””,”imageId”:””,”title”:”B.\tWatchful waiting & follow-up in an outpatient clinic”},”wxapu”:{“id”:”wxapu”,”image”:””,”imageId”:””,”title”:”C.\tCardiac MRI”},”ptyi8″:{“id”:”ptyi8″,”image”:””,”imageId”:””,”title”:”D.\tICD placement”,”isCorrect”:”1″}}}},”results”:{“mis1h”:{“id”:”mis1h”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”The ECG is consistent with a diagnosis of Brugada Syndrome (BrS). First described in 1992, BrS is an inherited channelopathy of the cardiac sodium channels. It is associated with a characteristic ECG pattern of \u201ccoved-type\u201d ST-elevation in leads V1-V3 and a right bundle branch pattern, which predisposes to a high risk of sudden death due to ventricular tachycardia or ventricular fibrillation [1, 2]. Sudden death frequently occurs at rest or during sleep. It has frequently been seen in young Asian males where it has been termed \u201csudden unexplained nocturnal death syndrome.\u201d Fever is a well-recognized precipitating factor, although the precise mechanism is still debated. In approximately 20% of patients, it is associated with a loss of function mutation in the SCN5A gene, which is also found in Long QT3 syndrome. In a multi-institution study of 30 children with BrS, the two most common etiologies were family history of BrS and unexplained syncope that was precipitated by fever in half of those children [3]. Diagnosis is usually based on the clinical history and the pathognomonic ECG abnormalities. However, the ECG abnormalities may be intermittent, and in these cases, a specific Brugada ECG protocol may be necessary with the use of sodium channel blocking drugs such as ajmaline, procainamide or flecainide. An ECG during a febrile episode may also be helpful. \r\n\r\n\r\nTreatment of BrS includes general measures such as aggressively treating a febrile illness with antipyretic medication and avoidance of certain medications, which can be found on a website \u2013 www.brugadadrugs.org. \r\n\r\n\r\nFor adult patients with a diagnostic ECG, history of syncope or a family history of sudden death, the definitive treatment is ICD placement. If an ICD is contraindicated, quinidine has been used for treatment. \r\n\r\n \r\nIn children, the use of ICDs is less studied in comparison with adults and is a much more difficult clinical decision. Although ICDs can prevent sudden death, there is a significant incidence of device complications and inappropriate shocks [4]. \r\n\r\n\r\nIn this patient, a brain CT is unlikely to show any abnormality in the absence of a neurological deficit. Watchful waiting is not appropriate based on the history and diagnostic ECG. A cardiac MRI is unlikely to be helpful in the presence of a structurally normal heart as seen on echocardiography. However, a cardiac MRI does have utility if a structural cardiomyopathy is suspected as a cause of aborted sudden death. \r\n\r\n\r\nReferences\r\n\r\n\r\n1.\tBrugada J, Campuzano O, Arbelo E et al. Present status of Brugada syndrome. J Am Coll Cardiol<\/em>. 2018; 72: 1046-1059. \r\n\r\n2.\t Behere SP, Weindling SN. Brugada syndrome in children \u2013 Stepping into unchartered territory. Ann Pediat Card<\/em>. 2017; 10: 248-258. \r\n\r\n3.\t Probst V, Denjoy I, Meregalli PG et al. Clinical aspects and prognosis of Brugada Syndrome in children. Circulation<\/em>. 2007; 115: 2042-2048. \r\n\r\n4.\t Corcia MCG, Sieira J, Pappaert G et al. Implantable cardioverter-defibrillators in children and adolescents with Brugada syndrome. J Am Coll Cardiol<\/em>. 2018; 71: 148-157.\r\n\r\n\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“089do”:{“id”:”089do”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Sana Ullah, MB ChB, FRCA – Children\u2019s Medical Center, Dallas\r\n\r\nA 15-year-old female with a history of orthotopic heart transplantation 2 months prior presents with intermittent fevers, abdominal pain, diarrhea and enlarged lymph nodes in the neck. Post-transplant lymphoproliferative disease (PTLD) is confirmed by lymph node biopsy and a PET-CT scan of her body. Which of the following risk factors in the recipient at the time of heart transplantation is MOST LIKELY associated with the development of PTLD? \r\n”,”desc”:””,”hint”:””,”answers”:{“em9dq”:{“id”:”em9dq”,”image”:””,”imageId”:””,”title”:”A.\tPositive cytomegalovirus (CMV) serology.”},”2hp62″:{“id”:”2hp62″,”image”:””,”imageId”:””,”title”:”B.\tPositive Epstein-Barr virus (EBV) serology. “},”ubqcf”:{“id”:”ubqcf”,”image”:””,”imageId”:””,”title”:”C.\tNegative EBV serology.”,”isCorrect”:”1″},”hyo9q”:{“id”:”hyo9q”,”image”:””,”imageId”:””,”title”:”D.\tHigh panel reactive antibody (PRA).”}}}},”results”:{“9gsnt”:{“id”:”9gsnt”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”While immunosuppressant therapy is critical for preventing organ rejection after transplantation, one of the serious risks is the development of various malignancies in the recipient as the body\u2019s immune surveillance systems are compromised. Many of these malignancies are caused by viruses, in particular Epstein-Barr virus (EBV) and Human Papilloma Virus. A study from 2006 showed the incidence of post-transplant lymphoproliferative disease (PTLD) is approximately 5% in children after heart transplant. The majority of these cases were of B-cell origin and of these, 87% were EBV positive. The probability of survival after diagnosis was 75% at 1 year and 68% at 3 years (1). The most common sites for PTLD are the upper airway (hypertrophy of tonsils and adenoids) and the gastrointestinal tract. \r\n\r\nRisk factors for PTLD include the following: (1) Negative EBV recipient and positive EBV donor serology, (2) younger age, as younger patients are more likely to be EBV-na\u00efve (EBV infections occur more frequently in older children and adults), and (3) multiple rejection episodes, as these require higher and more frequent doses of immunosuppressant medications. \r\n\r\nClinical features vary and include fever, weight loss, night sweats, gastrointestinal disturbances, hypertrophy of tonsils and adenoids, and lymph node enlargement in the neck, axilla and groin. Diagnostic evaluation includes clinical examination, chest x-ray, ultrasound of the abdomen and lymph nodes, computed tomography and magnetic resonance imaging, and lymph node biopsy. More recently, positron emission tomography-computed tomography has become the imaging modality of choice (2,3). \r\n\r\nTreatment depends on the type and severity of the disease but usually starts with a reduction of immunosuppressive drug therapy to allow the patient to mount an immune response to EBV. The obvious risk with this approach is acute rejection. Targeted therapy with rituximab, a chimeric anti-CD20 monoclonal antibody, can be used for CD20 positive B-cell lymphomas. \r\n\r\nCytomegalovirus (CMV) is the most common viral infection after transplantation and is associated with an increased risk of rejection, coronary allograft vasculopathy, and graft failure (4). \r\n\r\nA high panel reactive antibody (PRA) titer is associated with an increased risk of rejection. \r\n\r\nReferences\r\n\r\n1)\tWebber SA, Naftel DC, Fricker FJ, et al. Pediatric Heart Transplant Study. Lymphoproliferative disorders after paediatric heart transplantation: a multi-institutional study. Lancet<\/em>. 2006; 367(9506): 233-239. \r\n\r\n2)\tSchubert S. Lymhoproliferative disorders in pediatric heart transplant recipients. In: Canter C, Everitt MD, Burch M et al. (Eds). ISHLT Monograph Series Vol 13. Pediatric Heart Transplantation<\/em>. UAB Printing; 2019. \r\n\r\n3)\tAddonizio LJ, Boyle GJ. Post-transplant malignancy: Risk factors, incidence, diagnosis, treatment. In: Canter C, Kirklin JK (Eds). ISHLT Monograph Series Vol. 2. Pediatric Heart Transplantation<\/em>. Elsevier; 2007. \r\n\r\n4)\tSchowengerdt KO, Azeka E. Infection following pediatric heart transplantation. In: Canter C, Kirklin JK (Eds). ISHLT Monograph Series Vol. 2. Pediatric Heart Transplantation<\/em>. Elsevier; 2007.\r\n\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“xbdu6”:{“id”:”xbdu6″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Michael A. Evans, MD \u2013 Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine\r\n\r\nA 12-month-old boy presents for planned myringotomy and tympanostomy tube placement for otitis media. A mid-systolic murmur is auscultated at the left lower sternal border that becomes louder when the child bears down. Based on this child\u2019s murmur, which of the following echocardiographic findings is MOST LIKELY to be found?\r\n”,”desc”:””,”hint”:””,”answers”:{“cwk5i”:{“id”:”cwk5i”,”image”:””,”imageId”:””,”title”:”A. Patent ductus arteriosus”},”10jnp”:{“id”:”10jnp”,”image”:””,”imageId”:””,”title”:”B. Aortic Stenosis”},”ddkgs”:{“id”:”ddkgs”,”image”:””,”imageId”:””,”title”:”C. Asymmetric septal hypertrophy”,”isCorrect”:”1″},”iyqh1″:{“id”:”iyqh1″,”image”:””,”imageId”:””,”title”:”D. Normal echocardiogram”}}}},”results”:{“4aqbt”:{“id”:”4aqbt”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”The murmur present in patients with hypertrophic cardiomyopathy (HCM) can easily be confused with the benign Still\u2019s murmur as both are vibratory mid-systolic murmurs that are loudest at the apex or left lower sternal border. However, the murmur of HCM becomes louder with Valsalva, and a Still\u2019s murmur lessens or even disappears with Valsalva. The Valsalva maneuver results in an acute increase in intrathoracic and intraabdominal pressure that results in decreased venous return to the heart with subsequently decreased preload. The decrease in preload leads to a decreased left ventricular end-diastolic volume (LVEDV). This decreased LVEDV worsens left ventricular outflow tract obstruction (LVOTO) in patients with obstructive HCM, which increases the severity of the murmur. Patients with asymmetric septal hypertrophy due to hypertrophic obstructive cardiomyopathy will have a murmur similar to the patient in the question. \r\n\r\nStill\u2019s murmur refers to a benign murmur that is thought to be the result of elevated flow across the aortic valve in high cardiac output or high contractility states. Still\u2019s murmur is frequently found in young children but often disappears in adolescents. It is often described as \u201cmusical\u201d or \u201cvibratory\u201d in quality. \r\n\r\nThe murmur of aortic stenosis is a systolic ejection murmur that is best auscultated at the right upper sternal border with radiation to the carotid arteries. The intensity decreases with Valsalva as the decreased LVEDV results in decreased transvalvular flow, which decreases the severity of the murmur. Additionally, the murmur of HCM does not radiate to the carotids. \r\n\r\nA patent ductus arteriosus produces a machine-like murmur heard continuously throughout systole and diastole at the left upper sternal border. It is often described as a crescendo-decrescendo murmur with a crescendo in systole and decrescendo into diastole. \r\n\r\n\r\nReferences \r\n1. Sumski CA, Goot BH. Evaluating Chest Pain and Heart Murmurs in Pediatric and Adolescent Patients. Pediatr Clin North Am<\/em>. 2020; 67(5): 783-799. \r\ndoi:10.1016\/j.pcl.2020.05.003 \r\n\r\n2. Biancaniello T. Innocent murmurs. Circulation<\/em>. 2005; 111(3): e20-22. \r\ndoi: 10.1161\/01.CIR.0000153388.41229.CB. PMID: 15668345. \r\n\r\n3. Salazar SA, Borrero JL, Harris DM. On systolic murmurs and cardiovascular physiological maneuvers. Adv Physiol Educ<\/em>. 2012; 36(4): 251-256. doi:10.1152\/advan.00128.2011 \r\n\r\n4. Frank JE, Jacobe KM. Evaluation and management of heart murmurs in children. Am Fam Physician<\/em>. 2011; 84(7): 793-800. \r\n\r\n5. Stein PD, Sabbah HN. Aortic origin of innocent murmurs. Am J Cardiol<\/em>. 1977; 39(5): 665-671. doi:10.1016\/s0002-9149(77)80126-4 \r\n”,”redirect_url”:””}}}

{“questions”:{“lbzwo”:{“id”:”lbzwo”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Michael A. Evans, MD \u2013 Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine\r\n\r\nA previously healthy 8-year-old male presents with three days of high fever, diarrhea, and abdominal pain. On exam, he exhibits conjunctivitis, cracked lips, and edema of the hands and feet. Both parents had positive SARS-CoV-2 nasopharyngeal swabs. You suspect Multisystem Inflammatory Syndrome in Children (MIS-C). Which of the following common cardiovascular manifestations of MIS-C is MOST LIKELY to be found in this patient? \r\n”,”desc”:””,”hint”:””,”answers”:{“eayn2”:{“id”:”eayn2″,”image”:””,”imageId”:””,”title”:”A. Elevated Troponin > 0.2 ng\/ml “},”8hbuv”:{“id”:”8hbuv”,”image”:””,”imageId”:””,”title”:”B. B-type natriuretic peptide (BNP) > 400 ng\/L “,”isCorrect”:”1″},”put4o”:{“id”:”put4o”,”image”:””,”imageId”:””,”title”:”C. Left Ventricular Ejection Fraction < 55% "},"w8tba":{"id":"w8tba","image":"","imageId":"","title":"D. Coronary Aneurysm > 2.5mm”}}}},”results”:{“w2853”:{“id”:”w2853″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”In the largest case-series of children diagnosed with Multisystem Inflammatory Syndrome in Children (MIS-C) published to date, 80% of patients had cardiovascular involvement. Of those with cardiovascular findings: 73% had B-type natriuretic peptide (BNP) > 400 ng\/L, 50% had elevated troponin, and 8% had coronary aneurysms > 2.5mm on echocardiography. Beyond elevated BNP and troponin, elevations in C-reactive protein (CRP), procalcitonin (PCT), and erythrocyte sedimentation rate (ESR) are typically found in patients with MIS-C.\r\n\r\nMany other smaller case-series of MIS-C patients have been reported in the literature. The incidence of ventricular dysfunction has varied widely from 35-100% of children diagnosed with MIS-C depending on the publication. Six to fourteen percent of these patients did not fully recover their LV function prior to hospital discharge. Forty-eight percent of patients with cardiac involvement require vasoactive support, and 80% of all children diagnosed with MIS-C require intensive care. Two percent of children with MIS-C die.\r\n\r\nIn a report of 186 pediatric patients diagnosed with SARS- CoV-2, the most predominant organ systems involved in MIS-C were gastrointestinal (92%), cardiovascular (80%), hematologic (76%), and mucocutaneous (74%). This was followed by respiratory involvement in 70% of pediatric patients. This differs from the incidences of organ system involvement in adults diagnosed with SARS-CoV-2. \r\n\r\nReferences\r\n1. Feldstein LR, Rose EB, Horwitz SM, et al. Multisystem Inflammatory Syndrome in U.S. Children and Adolescents. N Engl J Med<\/em>. 2020; 383(4): 334-346. doi:10.1056\/\r\n\r\n2. Sperotto F, Friedman KG, Son MBF, VanderPluym CJ, Newburger JW, Dionne A. Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children: a comprehensive review and proposed clinical approach. Eur J Pediatr<\/em>. 2021; 180(2): 307-322.\r\n\r\n3. Riphagen S, Gomez X, Gonzalez-Martinez C, Wilkinson N, Theocharis P. Hyperinflammatory shock in children during COVID-19 pandemic. Lancet<\/em>. 2020; 395(10237): 1607-1608. doi:10.1016\/S0140-6736(20)31094-1\r\n\r\n4. Belhadjer Z, M\u00e9ot M, Bajolle F, et al. Acute Heart Failure in Multisystem Inflammatory Syndrome in Children in the Context of Global SARS-CoV-2 Pandemic. Circulation<\/em>. 2020; 142(5): 429-436. doi:10.1161\/CIRCULATIONAHA.120.048360 \r\n\r\n5. Verdoni L, Mazza A, Gervasoni A, et al. An outbreak of severe Kawasaki-like disease at the Italian epicentre of the SARS-CoV-2 epidemic: an observational cohort study. Lancet<\/em>. 2020; 395(10239): 1771-1778. doi:10.1016\/S0140-6736(20)31103-X\r\n\r\n6. National Center for Immunization and Respiratory Diseases (NCIRD). Health Department-Reported Cases of Multisystem Inflammatory Syndrome in Children (MIS-C) in the United States. Centers for Disease Control and Prevention. https:\/\/www.cdc.gov\/mis-c\/cases\/index.html. Updated March 29, 2021. Accessed April 25, 2021.\r\n”,”redirect_url”:””}}}

{“questions”:{“1hy2x”:{“id”:”1hy2x”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Michael A. Evans, MD \u2013 Ann & Robert H. Lurie Children\u2019s Hospital of Chicago \r\n\r\nA Hispanic male presents to the emergency room with tachypnea and a fever of 40\u00b0 Celsius. A SARS-CoV-2 RT-PCR is positive. Blood tests reveal an elevated C-reactive protein (CRP) and B-type natriuretic peptide (BNP). Which of the following age groups does this patient MOST LIKELY belong to based on the demographics of patients with Multisystem Inflammatory Syndrome in Children (MIS-C)?”,”desc”:””,”hint”:””,”answers”:{“fguqd”:{“id”:”fguqd”,”image”:””,”imageId”:””,”title”:”A. 1 to 4 years of age “},”2zjho”:{“id”:”2zjho”,”image”:””,”imageId”:””,”title”:”B. 5 to 9 years of age”,”isCorrect”:”1″},”30hoh”:{“id”:”30hoh”,”image”:””,”imageId”:””,”title”:”C. 10 to 14 years of age”},”7woxs”:{“id”:”7woxs”,”image”:””,”imageId”:””,”title”:”D. 15 to 20 years of age”}}}},”results”:{“10x4t”:{“id”:”10x4t”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”According to the Centers for Disease Control and Prevention (CDC) as of May 2021, the majority of the 3742 cases of MIS-C have occurred in children and adolescents between the ages of one and fourteen years old with a median of nine years. There have been 35 MIS-C deaths to date. The cases are broken down by age group in the following table. \r\n\r\nAge Range——————————-% of Cases\r\nLess than 1 year of age——————3%\r\n1 to 4 years of age———————–21%\r\n5 to 9 years of age———————–34%\r\n10 to 14 years of age———————27%\r\n15 to 20 years of age———————15%\r\n\r\nMIS-C has a slight predominance in males (60% of cases) and children of Hispanic\/Latino descent (33%) or Black\/Non-Hispanic descent (30%) versus White\/Non-Hispanic descent (28%).\r\n\r\nThe diagnostic criteria for MIS-C are as follows:\r\n\u2022\tPatient aged <21 years with fever or history of fever and laboratory evidence of inflammation [elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), fibrinogen, procalcitonin, d-dimer, ferritin, lactic acid dehydrogenase (LDH), interleukin 6 (IL-6), or neutrophils; reduced lymphocytes, or low albumin], and evidence of clinically severe illness requiring hospitalization with multisystem organ involvement (\u22652 of the following systems: cardiac, respiratory, renal, gastrointestinal, neurologic, hematologic, or dermatologic), AND\r\n\u2022\tCurrent or recent SARS-CoV-2 infection or COVID-19 exposure within 4 weeks of symptom onset, AND\r\n\u2022\tNo alternative plausible diagnoses\r\n\r\nReferences\r\n1. National Center for Immunization and Respiratory Diseases (NCIRD). Health Department-Reported Cases of Multisystem Inflammatory Syndrome in Children (MIS-C) in the United States. Centers for Disease Control and Prevention. https:\/\/www.cdc.gov\/mis-c\/cases\/index.html. Updated May 11, 2021. Accessed May 16, 2021.\r\n\r\n2. Belhadjer Z, Meot M, Bajolle F, et al. Acute Heart Failure in Multisystem Inflammatory Syndrome in Children in the Context of Global SARS-CoV-2 Pandemic. Circulation<\/em>. 2020; 142(4): 429\u2013436.\r\n\r\n3. National Center for Immunization and Respiratory Diseases (NCIRD). Information for Healthcare Providers about Multisystem Inflammatory Syndrome in Children (MIS-C). Centers for Disease Control and Prevention. https:\/\/www.cdc.gov\/MIS-C\/hcp\/. Updated February 21, 2021. Accessed May 16, 2021.\r\n\r\n”,”redirect_url”:””}}}