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

{“questions”:{“z49yi”:{“id”:”z49yi”,”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\nWhat is the MOST COMMON syndrome associated with pulmonary arteriovenous malformations ?\r\n\r\n”,”desc”:””,”hint”:””,”answers”:{“cye57”:{“id”:”cye57″,”image”:””,”imageId”:””,”title”:”A.\tScimitar syndrome”},”imdso”:{“id”:”imdso”,”image”:””,”imageId”:””,”title”:”B.\tOsler-Weber-Rendu syndrome”,”isCorrect”:”1″},”vlz1s”:{“id”:”vlz1s”,”image”:””,”imageId”:””,”title”:”C.\tKartagener\u2019s syndrome”},”vd63t”:{“id”:”vd63t”,”image”:””,”imageId”:””,”title”:”D.\tAlagille syndrome”}}}},”results”:{“9wcta”:{“id”:”9wcta”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:”Pulmonary arteriovenous malformations (AVMs) are structurally abnormal blood vessels which form direct communications between the pulmonary arterial and pulmonary venous circulations, producing a right-to-left shunt bypassing the alveolar gas exchange regions and the normal filtering functions of the lungs. The most common cause of pulmonary AVMs is Osler-Weber-Rendu syndrome, which is also known as Hereditary Hemorrhagic Telangiectasia (HHT). Osler-Weber-Rendu is inherited in an autosomal dominant manner and affects approximately 1 in 5,000 to 8,000 people. In addition to AVMs, smaller telangiectatic vessels that are prone to bleeding are frequently found in nasal, mucocutaneous, hepatic, gastrointestinal and cerebral vascular beds. \r\n\r\nPulmonary AVMs can also develop after surgical palliation with the bidirectional cavopulmonary shunt and produce significant systemic desaturation. The purported mechanism is due to the lack of a \u201chepatic factor\u201d that bypasses the pulmonary circulation after the bidirectional cavopulmonary shunt. Once the hepatic venous return is redirected back into the pulmonary circulation after Fontan completion, the pulmonary AVMs generally regress over a period of weeks to months. \r\n\r\n\r\nThe major clinical manifestations of pulmonary AVMs are recurrent bleeding manifesting as hemoptysis or hemothorax, systemic desaturation due to right-to-left shunting, ischemic strokes due to paradoxical thromboembolism, and cerebral abscesses resulting from bacteria in the blood that bypasses the filtering mechanism of the lungs. There is also an increased risk of pregnancy-related deaths in pregnant women with pulmonary AVMs. A rare but interesting phenomenon as a result of pulmonary AVMs is platypnea-orthodeoxia, which is best described as systemic desaturation and increased shortness of breath on standing up but improvement on lying flat. This is because most pulmonary AVMs are in the basal regions of the lungs thereby increasing the right-to-left shunt due to increased blood flow on assuming the upright posture. \r\n\r\n\r\nComputed tomography of the chest is the gold-standard for diagnosis of pulmonary AVMs and offers better resolution than MRI. Contrast echocardiography using agitated saline injected into an arm vein and imaging the left side of the heart can also be used, but it lacks specificity even though it is highly sensitive. Transcatheter embolization is recommended for treatment of all pulmonary AVMs that are amenable to vessel access. \r\n\r\n\r\nScimitar syndrome is a rare variant of partial anomalous pulmonary venous return of a portion or the entirety of the right lung to the inferior vena cava. The abnormal venous channel forms a characteristic curved shadow on chest x-ray along the right heart border which resembles a sword known as a scimitar. Associated abnormalities include hypoplasia of the right lung, secondary dextroposition of the heart, and pulmonary sequestration of portions of the right. The sequestered lung does not take part in gas exchange and is prone to recurrent bleeding and infection. Management of Scimitar Syndrome is largely determined by the degree of volume overload to the heart and associated cardiac anomalies. Lung segments affected by sequestration may need to be resected. \r\n\r\n\r\nKartagener\u2019s syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesis resulting in a triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. \r\n\r\n\r\nAlagille syndrome is an autosomal dominant disorder consisting of bile duct paucity and cholestasis, characteristic triangular facies, widespread vascular anomalies and congenital heart disease. The congenital heart disease often manifests as peripheral pulmonary arterial stenosis or hypoplasia, pulmonary valve stenosis, and\/or Tetralogy of Fallot. Treatment of pulmonary arterial stenosis often requires a combination of surgical and transcatheter based techniques. Approximately 15% of patients eventually develop liver failure requiring transplantation. Many of these patients harbor a mutation in the JAG1 gene. \r\n\r\n\r\n\r\nReferences\r\n\r\n\r\n1.\t Shovlin CL. Pulmonary Arteriovenous Malformations. Am J Respir Crit Care Med<\/em>. 2014; 190(11): 1217-1228. \r\n\r\n2.\t Vida VL, Guariento A. A sword threatening the heart: The scimitar syndrome. JCTVS Techniques<\/em>. 2020; 1: 75-80. \r\n\r\n3.\t Tretter JT, McElhinney DB. Cardiac, Aortic, and Pulmonary Vascular Involvement in Alagille Syndrome. 2018. In: Kamath B., Loomes K. (eds) Alagille Syndrome. Springer, Cham. https:\/\/doi.org\/10.1007\/978-3-319-94571-2_6\r\n\r\n4.\t Kamath BM, Spinner NB, Emerick KM, et al. Vascular anomalies in Alagille syndrome: A significant cause of morbidity and mortality. Circulation<\/em>. 2004; 109:1354-1358.\r\n”,”redirect_url”:””}}}

{“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”:””}}}