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

{“questions”:{“lcw7k”:{“id”:”lcw7k”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB ChB, FRCA \u2013 Children\u2019s Medical Center, Dallas\r\n\r\nA 30-year-old patient with a history of extracardiac Fontan palliation has been diagnosed with hepatocellular carcinoma. Which of the following is the MOST<\/em> likely 1-year survival rate for Fontan patients diagnosed with hepatocellular carcinoma?\r\n”,”desc”:””,”hint”:””,”answers”:{“38e0f”:{“id”:”38e0f”,”image”:””,”imageId”:””,”title”:”A.\t20%”},”pyaoq”:{“id”:”pyaoq”,”image”:””,”imageId”:””,”title”:”B.\t50%”,”isCorrect”:”1″},”i8jc0″:{“id”:”i8jc0″,”image”:””,”imageId”:””,”title”:”C.\t70%”},”6i25b”:{“id”:”6i25b”,”image”:””,”imageId”:””,”title”:”D.\t90%”}}}},”results”:{“ytbdd”:{“id”:”ytbdd”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Fontan-associated liver disease (FALD) is a well-recognized complication of single ventricle palliation with the Fontan operation. The exact etiology is unclear but is related to chronically elevated central venous pressures and low cardiac output resulting in liver fibrosis and subsequent cirrhosis. Hepatocellular carcinoma (HCC) is a rare but serious complication of FALD. The reported prevalence of HCC after a Fontan operation is between 1-3% in different series of published studies. In a recent multicenter case series of 54 patients with a Fontan circulation and diagnosed with HCC, the mean age at diagnosis was 30+\/- 9.4 years with the youngest patient being 12 years of age. Additionally, the mean duration from Fontan surgery to HCC diagnosis was 21.6 +\/- 7.4 years and the 1-year survival was 50%. Survival was further decreased if the tumor was symptomatic, more than 4 cm in size, or had metastasized. \r\n\r\nDue to the numerous complications associated with the Fontan circulation, these patients require life-long follow up and regular screening for FALD. Recommendations for surveillance have recently been published which include a clinical assessment, liver function tests, serum biomarkers such as FibroSure and alpha-fetoprotein, imaging with an abdominal ultrasound, abdominal computed tomography scan or abdominal magnetic resonance imaging, and a liver biopsy. The surveillance interval should shorten with longer time elapsed from the Fontan completion. \r\n\r\n\r\nReferences\r\n\r\n\r\n1)\t Possner M, Gordon-Walker T, Egbe AC, et al. Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes. Int J Cardiol<\/em>. 2021; 322: 142-148. \r\n2)\t Rychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with a Fontan circulation: A scientific statement from the American Heart Association. Circulation<\/em>. 2019; 140(6): 234-284. \r\n3)\tGorden-Walker TT, Bove K, Veldtman G. Fontan-associated liver disease: A review. J Cardiol<\/em>. 2019; 74: 223-232.\r\n\r\n\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“7155j”:{“id”:”7155j”,”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\nAn 11-month-old male infant with severe pulmonary valve stenosis and estimated peak systolic gradient of 110 mmHg presents for balloon pulmonary valvuloplasty. Immediately following balloon valvuloplasty, the vital signs are as follows: heart rate 165 beats per minute, blood pressure 53\/21, and oxygen saturation 60%. Which of the following measures is the MOST APPROPRIATE<\/em> next step in his management?\r\n”,”desc”:””,”hint”:””,”answers”:{“8203s”:{“id”:”8203s”,”image”:””,”imageId”:””,”title”:”A.\tEpinephrine bolus”},”e6blr”:{“id”:”e6blr”,”image”:””,”imageId”:””,”title”:”B.\tMilrinone bolus”},”aehh0″:{“id”:”aehh0″,”image”:””,”imageId”:””,”title”:”C.\tEsmolol bolus”,”isCorrect”:”1″},”iwrm6″:{“id”:”iwrm6″,”image”:””,”imageId”:””,”title”:”D.\tInhaled nitric oxide”}}}},”results”:{“7ck9i”:{“id”:”7ck9i”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”This patient has developed a rare but serious complication colloquially known as a \u201csuicide right ventricle\u201d or dynamic right ventricular infundibular obstruction following balloon pulmonary valvuloplasty. Severe pulmonary valve stenosis can result in significant right ventricular hypertrophy and in particular right infundibular hypertrophy. An acute relief of the valvar gradient can suddenly unmask a previously unknown infundibular gradient leading to dynamic right ventricular outflow tract obstruction. The pathophysiology is similar to left ventricular outflow tract obstruction in the setting of hypertrophic cardiomyopathy. The mainstay of treatment is to reduce the heart rate and contractility with a beta blocker such as esmolol, labetalol, or propranolol. The decrease in contractility allows for improved ventricular filling during diastole. Beta blockade with oral propranolol may be necessary for a period of time after balloon valvuloplasty to allow for ventricular remodeling. In this case, esmolol is the appropriate treatment due to the fast onset of action given severe oxygen desaturation and hypotension. \r\n\r\nIn this case, an epinephrine bolus would not be helpful because the oxygen desaturation and hypotension are due to right ventricular infundibular obstruction rather than ventricular dysfunction. An epinephrine bolus would increase contractility and likely worsen the signs of infundibular obstruction. \r\n\r\nAlthough this patient likely has right ventricular diastolic dysfunction, a milrinone bolus would acutely produce systemic vasodilation and thereby worsen hypotension. \r\n\r\nInhaled nitric oxide would not be indicated in the treatment of right ventricular infundibular obstruction as it would reduce pulmonary arterial pressure rather than relieve infundibular obstruction. \r\n\r\nReferences: \r\n1)\tKhambatta H, Velado M, Gaffney J, Schechter W, Casta A. Management of right ventricular tract reactivity following pulmonary valve dilation after general anesthesia: experience of a medical mission. Pediatric Anesthesia. <\/em>2006; 16(10): 1087-1089. \r\n\r\n2)\tBen-Shachar G, Cohen M, Sivakoff M, Portman M, Riemenschneider T, Van Heeckeren D. Development of infundibular obstruction after percutaneous pulmonary balloon valvuloplasty. J Am Coll Cardiol. <\/em>1985; 5(3): 754-756. \r\n\r\n3)\tTharpar MK, Rao PS. Significance of infundibular obstruction following balloon valvuloplasty for valvar pulmonic stenosis. Am Heart J. <\/em>1989; 118: 99-103. \r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“kd7st”:{“id”:”kd7st”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2021\/09\/QOW-9-30-2021.jpg”,”imageId”:”4948″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: David J. Krodel, MD, MS, FASA<\/strong> \u2013 Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine \r\nMichael A. Evans, MD<\/strong> \u2013 Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine\r\n\r\nA three-month-old male infant is status post aortic coarctation repair. The following image was documented during a regional block performed for postoperative pain. Which of the following regional anesthetic techniques was MOST LIKELY<\/em> performed based on the image?\r\n\r\n\r\n”,”desc”:””,”hint”:””,”answers”:{“c19ms”:{“id”:”c19ms”,”image”:””,”imageId”:””,”title”:”A. Erector spinae plane block”,”isCorrect”:”1″},”f9gzy”:{“id”:”f9gzy”,”image”:””,”imageId”:””,”title”:”B. Paravertebral block”},”fvu77″:{“id”:”fvu77″,”image”:””,”imageId”:””,”title”:”C. Serratus anterior plane block”},”9jwvm”:{“id”:”9jwvm”,”image”:””,”imageId”:””,”title”:”D. Caudal block”}}}},”results”:{“rbo06”:{“id”:”rbo06″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:””,”redirect_url”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2021\/09\/final-AC-and-SG-edits-Krodel.Evans_.September.QOW4-002-1.pdf.pdf”}}}

{“questions”:{“s8uup”:{“id”:”s8uup”,”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 7kg 11-month-old infant with a history of double outlet right ventricle, pulmonary atresia, and palliation to a bidirectional Glenn presents for orthotopic heart transplantation due to severe ventricular dysfunction. On exam, the patient is noted to be small for chronologic age, has broad thumbs and great toes, microcephaly, a prominent nose with nasal septum extending below the alae nasi, and a high-arched palate. Which of the following syndromes is MOST<\/em> likely to be diagnosed in this patient?\r\n”,”desc”:””,”hint”:””,”answers”:{“42k3a”:{“id”:”42k3a”,”image”:””,”imageId”:””,”title”:”A. DiGeorge Syndrome “},”goiud”:{“id”:”goiud”,”image”:””,”imageId”:””,”title”:”B. Down Syndrome “},”v2yaw”:{“id”:”v2yaw”,”image”:””,”imageId”:””,”title”:”C. Rubinstein-Taybi Syndrome”,”isCorrect”:”1″},”4lqjl”:{“id”:”4lqjl”,”image”:””,”imageId”:””,”title”:”D. Noonan Syndrome “}}}},”results”:{“pjzl7”:{“id”:”pjzl7″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Rubinstein-Taybi Syndrome (RTS or RSTS) or Broad Thumb-Hallux Syndrome is a syndrome characterized by short stature, intellectual disability, characteristic facial appearance, broad thumbs, and broad great toes. Approximately 33% of patients with RST have congenital heart disease (CHD) with average age at diagnosis of 6.4 months. RTS is most commonly caused by a disruption of the CREBBP gene on chromosome 16, which encodes the CREB-Binding Protein, and is estimated to affect 1 in 300,000 live births. \r\n\r\nThere are no standard diagnostic criteria for RTS due to the wide variability in phenotypes for patients and as such, RTS is most often a clinical diagnosis. Concurrent failure to thrive, microcephaly, dysmorphic facial features, broad thumbs and great toes lead to a presumed diagnosis until there is confirmatory genetic testing. The distinct thumb and toe abnormalities are unique to this syndrome. \r\n\r\nDown syndrome or Trisomy 21, is a genetic disorder caused by a third copy of chromosome 21. Similar to RTS, it is a syndrome that is characterized by growth delays, intellectual disability, and characteristic facies. Forty percent of children diagnosed with Trisomy 21 have CHD. The characteristic facies of Trisomy 21 include up-slanting palpebral fissures, epicanthic folds, a flat nasal bridge, and macroglossia. Many patients with Trisomy 21 do possess a gap between the first and second toes. \r\n\r\nDiGeorge syndrome, also known as 22q11.2 Deletion Syndrome, occurs due to a microdeletion on the long arm of chromosome 22. It has an estimated prevalence of 1 in 4000 live births and is the most common microdeletion syndrome. Patients with DiGeorge Syndrome also have characteristic facial features, cognitive impairment, CHD (particularly conotruncal defects), and palate abnormalities. The characteristic facial features include retro- or micrognathia, high and broad nasal bridge, small teeth with downturned mouth, short philtrum, low-set ears, and hypertelorism. \r\n\r\nNoonan syndrome is an autosomal dominant disease that involves multiple organ systems. It is the second most common syndromic cause of CHD after Trisomy 21. Cardiac manifestations vary widely, but pulmonary stenosis, hypertrophic cardiomyopathy (HCM), and atrial septal defect (ASD) are the most common findings. The syndrome is also associated with distinctive facies (coarse features with tall forehead and low posterior hairline in infancy), developmental delay, and short stature. \r\n\r\nReferences \r\n1. Wiley S, Swayne S, Rubinstein JH, Lanphear NE, Stevens CA. Rubinstein-Taybi syndrome medical guidelines. Am J Med Genet A<\/em>. 2003;119A(2):101-110. doi:10.1002\/ajmg.a.10009 \r\n2. Stevens CA, Bhakta MG. Cardiac abnormalities in the Rubinstein-Taybi syndrome. Am J Med Genet<\/em>. 1995;59(3):346-348. doi:10.1002\/ajmg.1320590313 \r\n3. Rubinstein JH, Taybi H. Broad thumbs and toes and facial abnormalities. A possible mental retardation syndrome. Am J Dis Child<\/em>. 1963;105:588-608. doi:10.1001\/archpedi.1963.02080040590010 \r\n4. Rubinstein JH. Broad thumb-hallux (Rubinstein-Taybi) syndrome 1957-1988. Am J Med Genet Suppl<\/em>. 1990;6:3-16. doi:10.1002\/ajmg.1320370603 \r\n5. Loomba RS, Geddes G. Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome. Ann Pediatr Cardiol<\/em>. 2015;8(2):157-160. doi:10.4103\/0974-2069.154151 \r\n6. Roberts AE, Allanson JE, Tartaglia M, Gelb BD. Noonan syndrome. Lancet<\/em>. 2013;381(9863):333-342. doi:10.1016\/S0140-6736(12)61023-X \r\n7. Bassett AS, McDonald-McGinn DM, Devriendt K, et al. Practical guidelines for managing patients with 22q11.2 deletion syndrome. J Pediatr<\/em>. 2011;159(2):332-339. doi:10.1016\/j.jpeds.2011.02.039\r\n\r\n”,”redirect_url”:””}}}

{“questions”:{“2jfs5”:{“id”:”2jfs5″,”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 17-year-old adolescent male with a history of Shone\u2019s complex, depressed ejection fraction of 20% and severe mitral regurgitation is awaiting heart transplantation as an inpatient due to vasoactive dependence and worsening end-organ dysfunction. Over the last two days, he has had increased abdominal pain with emesis and escalation to high flow nasal cannula but remains hemodynamically stable on unchanging doses of epinephrine and milrinone. Which of the following is the MOST<\/em> appropriate Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) classification for this patient?\r\n”,”desc”:””,”hint”:””,”answers”:{“pvqh4”:{“id”:”pvqh4″,”image”:””,”imageId”:””,”title”:”A. Pedimacs 1″},”qyea5″:{“id”:”qyea5″,”image”:””,”imageId”:””,”title”:”B. Pedimacs 2″,”isCorrect”:”1″},”vfgpa”:{“id”:”vfgpa”,”image”:””,”imageId”:””,”title”:”C. Pedimacs 3″},”6da98″:{“id”:”6da98″,”image”:””,”imageId”:””,”title”:”D. Pedimacs 4″}}}},”results”:{“je07f”:{“id”:”je07f”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”This patient has shown the \u201cprogressive decline\u201d that is characteristic of the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) 2 classification, as evidenced by worsening end-organ dysfunction (abdominal pain) and by increased respiratory support (high flow nasal cannula) while remaining on two vasoactive agents. \r\n\r\nThe Pedimacs patient profiles provide a general description of patients that require mechanical circulatory support (MCS) with Ventricular Assist Device(s) (VADs). The patient profile classification is recorded into a registry at the time of VAD implantation as a means of tracking outcomes. Pedimacs classification categories are analogous to Intermacs (Interagency Registry for Mechanically Assisted Circulatory Support) categories in the adult population. The numeric classes correlate between the classification systems. In both instances, patient scoring at time of initiation of MCS is useful to predict prognosis. \r\n\r\nPatients classified into the Pedimacs 1 category are in \u201ccritical cardiogenic shock\u201d by definition with rapid clinical deterioration. These patients may have life-threatening hypotension or arrhythmias and require escalating doses of vasoactive agents. This patient population often suffers from worsening lactic acidosis. Pedimacs 1 patients require emergent intervention. \r\n\r\nPatients classified into the Pedimacs 2 category, such as the patient above, demonstrate a progressive clinical decline in the setting of vasoactive dependence. The decline may be manifested by any type of end-organ dysfunction – such as renal, hepatic, respiratory, cardiac (new arrhythmias), or gastrointestinal dysfunction. \r\n\r\nPatients classified into the Pedimacs 3 category are \u201cstable but inotrope dependent\u201d by definition. Patients with temporary MCS devices in place but with failure to wean from these devices are also classified as Pedimacs 3. \r\n\r\nPatients classified into the Pedimacs 4 category have \u201cresting symptoms.\u201d Often, a patient may be at home on an oral heart failure regimen and still experience heart failure symptoms at rest, including orthopnea, shortness of breath while performing activities of daily living, gastrointestinal symptoms, or severe edema or ascites. \r\n\r\nPatients classified into the Pedimacs 5 category are \u201cexertion intolerant\u201d but comfortable at rest while Pedimacs 6 patients are \u201cexertion limited.\u201d Finally, patients classified into the Pedimacs 7 category are considered to be in the \u201cAdvanced NYHA Class 3\u201d classification. They have reasonable comfort with activity at baseline but have experienced prior episodes of decompensated heart failure. \r\n\r\nA \u201ccurrent device strategy at time of implant\u201d is also recorded in the registry in order to illustrate reasoning and medical decision-making at the time of VAD implantation. VADs may be placed as a \u201cBridge to Recovery\u201d when recovery of cardiac function is anticipated. They may also be used as a \u201cRescue Therapy\u201d when an acute event has occurred in a patient without prior cardiac dysfunction. VADs are also used as a \u201cBridge to Transplant\u201d when a patient whom has previously been listed for heart transplantation requires mechanical support. Additionally, VADs are used as a \u201cBridge to Decision\u201d when a potentially-transplant-eligible patient requires MCS but has not been evaluated for transplant eligibility or as a \u201cDestination Therapy\u201d when a patient is not eligible for transplant. A wealth of additional information is recorded in the Pedimacs registry at the time of implantation and can be found in the Society of Thoracic Surgeons Pedimacs Users\u2019 Guide. \r\n\r\nBased on the most recent Pedimacs data published in 2020, the most common diagnosis leading to MCS was cardiomyopathy in 58.1% of patients (93.8% had dilated cardiomyopathy). At the time of implantation, 30% of patients were Pedimacs profile 1, 55% were profile 2, 13% were profile 3, and 2% were profiles 4 to 7. Device strategy at time of VAD implantation was a bridge to transplantation in 49.3% of patients, bridge to decision in 38%, bridge to recovery in 7.6%, and destination therapy in 1.3%. Survival of patients on VAD support at 6 months was 74%.\r\n\r\nReferences\r\n1. Version 5.0 STS Pedimacs Users\u2019 Guide Version Date 03\/26\/2018. Accessed 8\/9\/2021. https:\/\/www.sts.org\/sites\/default\/files\/Puf%20documents\/Pedimacs_Users_Guide_v5_0_2018_03_26.docx\r\n2. Morales DLS, Adachi I, Peng DM, et al. Fourth Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report. Ann Thorac Surg<\/em>. 2020;110(6):1819-1831. doi:10.1016\/j.athoracsur.2020.09.003\r\n”,”redirect_url”:””}}}