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

{“questions”:{“6w771”:{“id”:”6w771″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Greesha S. Pednekar, MD – Children\u2019s Memorial Hermann Hospital, University of Texas Health Science Center at Houston, TX and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 2-year-old girl with a history of dilated cardiomyopathy and subsequent left ventricular assist device implantation has persistent right ventricular failure supported with high doses of inotropic medications and inhaled nitric oxide. According to the Pedimacs registry, which of the following percentages MOST accurately reflects the approximate rate of right ventricular failure in the first month after left ventricular assist device placement?\r\n”,”desc”:”EXPLANATION \r\nPatients undergoing left ventricular assist device (LVAD) placement are at risk of right ventricular (RV) failure. Persistent RV failure is associated with increased morbidity and mortality, including multi-organ dysfunction, which may jeopardize transplant candidacy. Anticipation, prevention, and early detection of RV failure is imperative in LVAD planning and management.\r\n\r\nAn analysis of the largest pediatric VAD registry, called the Pedimacs registry, by Simpson et al demonstrated that 55% of children had RV failure during a time frame between one week to one month after LVAD implantation. An additional 25% of pediatric patients had RV failure between one and three months after LVAD placement. Factors associated with RV failure were younger age, lower weight, INTERMACS profile 1, chemical paralysis, and a pulsatile flow device. In addition, RV failure was associated with an increased risk of death during device support at time points of greater than one month (hazard ratio 3.2, 95% CI 1.4-7.7, p = 0.007) and greater than three months after LVAD insertion (hazard ratio 6.9, 95% CI 2-23.1, p = 0.002). In this analysis, RV failure was defined as prolonged inotrope use or subsequent need for right VAD. \r\n\r\nDeterioration of RV function after LVAD implantation can occur due to a number of reasons, including the following:1) Increased cardiac output results in increased systemic venous return and acute increases in RV preload, myofibril stretch, wall tension and myocardial oxygen demand, which may not be well tolerated under circumstances of depressed RV function; 2) LV unloading causes a leftward shift of the interventricular septum, thereby distorting RV geometry and interfering with the RV free wall contractility and worsening tricuspid regurgitation; 3) Apical LVAD implantation can impede the normal twisting motion of the RV, thereby impeding RV contractility and; 4) Intraoperative myocardial ischemia may further worsen RV function. \r\n\r\nAfter LVAD placement, anticipation of and preparation for RV failure during separation from cardiopulmonary bypass is critically important. Support of the RV includes initiation of inhaled nitric oxide, inotropic support, and avoidance of RV distension by excessive volume administration, along with avoidance of hypoxia and hypercarbia. Transesophageal echocardiography may be utilized to assess ventricular function, ventricular volume, and interventricular septal position after VAD placement. \r\n\r\n\r\n \r\nREFERENCES \r\nSimpson KE, Kirklin JK, Cantor RS et al. Right heart failure with left ventricular assist device implantation in children: An analysis of the Pedimacs registry database. J Heart Lung Transplant<\/em>. 2020;39(3):231-240. doi: 10.1016\/j.healun.2019.11.012. \r\n\r\nLaw SP, Morales DLS, Si MS, et al. Right heart failure considerations in pediatric ventricular assist devices. Pediatr Transplant<\/em>. 2021;25(3):e13990. doi:10.1111\/petr.13990\r\n\r\nHorton S, Skiner A, Bacigalupo A, Adachi I, Stayer S, Motta P. Mechanical Circulatory Support. In: Andropoulos D, Mossad E, Gottlieb E, eds. Anesthesia for Congenital Heart Disease<\/em>. 4th Edition. Hoboken, New Jersey: John Wiley & Sons, Inc.; 2023: 996-1025.\r\n\r\n”,”hint”:””,”answers”:{“pllyn”:{“id”:”pllyn”,”image”:””,”imageId”:””,”title”:”A.\t75%”},”j960n”:{“id”:”j960n”,”image”:””,”imageId”:””,”title”:”B.\t50%”,”isCorrect”:”1″},”o2nvy”:{“id”:”o2nvy”,”image”:””,”imageId”:””,”title”:”C.\t25%”}}}}}

{“questions”:{“di0y4”:{“id”:”di0y4″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2024\/04\/CCAS-Combined-Graphic-jpeg.jpg”,”imageId”:”7258″,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Pedro Solorzano, MD and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nA 12-year-old boy with a history of Wolff-Parkinson-White syndrome and intermittent supraventricular tachycardia is scheduled for an electrophysiologic study. The following electrocardiographic (ECG) rhythm is noted prior to induction of anesthesia. Which of the following cardiac rhythms is MOST likely illustrated below?”,”desc”:”EXPLANATION \r\nSinus arrhythmia is a variant of normal sinus rhythm (NSR) that is commonly seen in children and young adults. The heart rate increases with inspiration and decreases with expiration. This form of \u201crespiratory sinus arrhythmia\u201d characteristically presents with P waves of normal morphology consistent with originating from the sinus node. Each P wave is followed by a normal QRS complex, and the P-P intervals vary by more than 120 milliseconds or more than 10% of the shortest P-P interval. If the variation in rate is severe (i.e. a 100% change resulting in a heart rate of half), this is indicative of a more serious medical problem. One mechanism implicated in sinus arrhythmia involves the respiratory cycle and inherent cardiorespiratory interactions. It is postulated that inspiration inhibits vagal tone, thereby increasing the heart rate, and expiration restores the vagal tone to its previous state, such that the heart rate declines. \r\n\r\n\r\nIt is more noticeable in younger children as they have faster heart rates, and it has been seen in all children at some point during a 24-hour monitoring. Thus, in young, otherwise healthy patients without any other symptoms, sinus arrhythmia is considered a benign finding and further evaluation of sinus arrhythmia is usually unnecessary. However, severe sinus arrhythmia in older patients, often termed \u201cnon-respiratory sinus arrhythmia\u201d in which the heart rate does not vary with the phases of respiration, may indicate underlying cardiac disease, although it is not considered a marker for structural heart disease (Issa et al). \r\n\r\n\r\nThis patient\u2019s ECG rhythm strip shows monomorphic P waves that vary more than 10% of the shortest P-P interval. Each P-wave is followed by the patient\u2019s baseline QRS complex (which also shows a delta wave consistent with Wolff-Parkinson-White syndrome). This is consistent with a benign form of sinus arrhythmia which requires no treatment. Atrial fibrillation typically demonstrates an irregularly irregular rhythm and P waves are not visualized. Mobitz type II is a form of second-degree heart block characterized by a constant P-P interval without lengthening of the PR interval prior to a non-conducted P wave. This type of heart block is not a normal variant and is always considered pathologic. Common causes in children include post-cardiac surgery, myocarditis, orthotopic heart transplant rejection, and post-transplant coronary artery disease.\r\n\r\n\r\n \r\nREFERENCES \r\nCannon BC, Snyder CS. Disorders of cardiac rhythm and conduction. In: Shaddy R, Penny D, Feltes T, Cetta F and Mital S, eds. Moss and Adams\u2019 Heart Disease in Infants, Children and Adolescents including the Fetus and Young Adult<\/em>. 10th Edition. Philadelphia: Wolters Kluwer; 2022. 586-616.\r\n\r\n\r\nIssa ZF, Miller JM, Zipes DP. Sinus Node Dysfunction. In: Issa ZF, Miller JM, Zipes DP, Eds. Clinical Arrhythmology and Electrophysiology<\/em>. 3rd Edition. Elsevier; 2019: 238-254.\r\n”,”hint”:””,”answers”:{“crehu”:{“id”:”crehu”,”image”:””,”imageId”:””,”title”:”A. Atrial fibrillation”},”e2ood”:{“id”:”e2ood”,”image”:””,”imageId”:””,”title”:”B. Sinus arrhythmia”,”isCorrect”:”1″},”zqqus”:{“id”:”zqqus”,”image”:””,”imageId”:””,”title”:”C. Mobitz type II heart block”}}}}}

{“questions”:{“pe13n”:{“id”:”pe13n”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: David Fitzgerald, MD and Destiny F. Chau MD – Arkansas Children\u2019s Hospital \/University of Arkansas for Medical Sciences – Little Rock, AR \r\n\r\nAn 18-year-old woman with a history of exertional dyspnea and frequent lower respiratory tract infections undergoes bronchoscopy that demonstrates abnormal right lower lobe bronchial architecture. A chest x-ray demonstrates a nonspecific irregularity over the right lower lung field with dextroposition of the heart, and an echocardiogram reveals right heart dilation. Which of the following congenital heart defects is the MOST likely diagnosis?\r\n”,”desc”:”EXPLANATION \r\nScimitar syndrome is a subtype of partial anomalous pulmonary venous return (PAPVR) in which the right pulmonary vein drains abnormally to the inferior vena cava (IVC), typically near the level of the diaphragm. It is associated with varying degrees of right lung hypoplasia and cardiac dextroposition. The name, Scimitar syndrome, is owed to the crescent shape of the anomalous right pulmonary vein resembling the curved blade of a scimitar sword. The incidence is approximately one to three per 100,000 live births, and accounts for 0.5 to 1% of all congenital heart disease. \r\n\r\n\r\nScimitar syndrome can present in infancy or early adulthood. In infants, the median age at diagnosis is estimated to be seven months old. Among neonates diagnosed with Scimitar syndrome, up to 75% have associated anomalies, which include right lung hypoplasia, dextrocardia, right pulmonary artery hypoplasia, collateral blood vessels from infra-diaphragmatic aorta to the right lower lobe of the lung, secundum atrial septal defect (ASD), and diaphragmatic hernia. More severe cases are typically diagnosed within the first two months of life due to the presence of pulmonary hypertension, heart failure, and recurrent pulmonary infections. The adult form is typically milder in clinical presentation with the Scimitar vein often found incidentally in otherwise healthy patients. Symptomatic young adults usually present with recurrent pulmonary infections and exertional dyspnea from pulmonary sequestration. The characteristic Scimitar vein is often demonstrated on chest x-ray (CXR) (see Figure 1). \r\n\r\n\r\n\r\n\r\n\r\n\r\nFigure 1. Chest radiograph demonstrating Scimitar vein. https:\/\/commons.wikimedia.org\/wiki\/File:Scimitar_syndrome_chest_xray.jpg, licensed under the Creative Commons Attribution-Share Alike 4.0 International license.\r\n\r\nThe Scimitar vein produces a left to right shunt leading to excessive pulmonary blood flow and right heart dilation. Medical management centers on reducing excessive pulmonary blood flow to slow the progression to pulmonary hypertension and heart failure. Patients with the infantile form who exhibit signs of pulmonary hypertension require surgical correction. Other indications for surgical correction include a Q_{p<\/sub>:Qs<\/sub> greater than 1.5 in an asymptomatic patient, recurrent pneumonia, and heart failure. \r\n\r\n\r\nAny preoperative anesthetic evaluation should include a review of cardiac imaging and functional studies given the high association of Scimitar syndrome with other congenital cardiac defects. Evaluation should also include the presence and severity of pulmonary hypertension, quantification of left to right shunt, baseline cardiopulmonary status, and degree of pulmonary hypoplasia. While the adolescent and adult forms tend to be milder in clinical presentation, the presence of recurrent infections, lung hypoplasia and longstanding pulmonary circulatory overload should be closely evaluated prior to anesthetic administration. \r\n\r\n\r\nThis patient\u2019s history, clinical presentation, CXR, echocardiography and bronchoscopy strongly suggest a diagnosis of Scimitar syndrome. Though both sinus venosus atrial septal defect and Ebstein\u2019s anomaly lead to right heart dilation, neither defect is typically associated with right lung hypoplasia. \r\n\r\n\r\n\r\n \r\nREFERENCES \r\n\r\nMidyat L, Demir E, A\u015fkin M, et al. Eponym. Scimitar syndrome. Eur J Pediatr<\/em>. 2010;169(10): 1171-1177. doi:10.1007\/s00431-010-1152-4. \r\n\r\nBrown D, Geva T. Anomalies of the Pulmonary Veins. In: Shaddy R, Penny D, Feltes T, Cetta F and Mital S, eds. Moss and Adams\u2019 Heart Disease in Infants, Children and Adolescents including the Fetus and Young Adult. 10th Edition. Philadelphia: Wolters Kluwer; 2022: 854-856.\r\n\r\n\r\nChowdhury UK, Anderson RH, Sankhyan LK, et al. Surgical management of the scimitar syndrome. J Card Surg<\/em>. 2021; 36(10):3770-3795. doi:10.1111\/jocs.15857\r\n\r\n\r\nStout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA\/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology\/American Heart Association Task Force on Clinical Practice Guidelines [published correction appears in J Am Coll Cardiol<\/em>. 2019 May 14;73(18):2361]. J Am Coll Cardiol<\/em>. 2019;73(12):1494-1563. doi:10.1016\/j.jacc.2018.08.1028\r\n\r\n\r\n\r\n”,”hint”:””,”answers”:{“x73aq”:{“id”:”x73aq”,”image”:””,”imageId”:””,”title”:”A.\tEbstein\u2019s anomaly”},”62p0m”:{“id”:”62p0m”,”image”:””,”imageId”:””,”title”:”B.\tSinus venosus atrial septal defect”},”5iyl3″:{“id”:”5iyl3″,”image”:””,”imageId”:””,”title”:”C.\tScimitar syndrome\r\n\r\n”,”isCorrect”:”1″}}}}}}

{“questions”:{“1ld82”:{“id”:”1ld82″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Nicholas Houska, DO – University of Colorado – Children\u2019s Hospital Colorado \r\n\r\nAn 11-year-old boy with a family history of desmoplakin cardiomyopathy presents for cardiac magnetic resonance imaging. He was recently found to be a carrier for a DSP gene mutation. Which of the following features is MOST likely to be present in desmoplakin cardiomyopathy as compared to other types arrhythmogenic cardiomyopathy?”,”desc”:”EXPLANATION \r\nDesmoplakin (DSP)<\/em> is a desmosomal protein encoded by the DSP<\/em> gene located on chromosome 6. It is expressed in both cardiomyocytes and skin and plays an important role in linking the cardiac desmosome to intermediate filaments. It is essential for normal force transmission in myocardial tissue. Mutations in the DSP<\/em> gene have been identified in arrhythmogenic right ventricular cardiomyopathy (ARVC) and in familial dilated cardiomyopathy (DCM). However, more recently, mutations in the DSP<\/em> gene have been associated with a distinct form of cardiomyopathy with a high prevalence of left ventricular fibrosis and systolic dysfunction, which differs from ARVC and DCM. Termed desmoplakin (DSP)<\/em> cardiomyopathy, this disease has distinct features as compared to other forms of arrhythmogenic cardiomyopathy. Recently, the Padua criteria has categorized arrhythmogenic cardiomyopathy (ACM) into three phenotypes including arrhythmogenic right ventricular cardiomyopathy (ARVC), arrhythmogenic left ventricular cardiomyopathy (ALVC), and biventricular arrhythmogenic cardiomyopathy. \r\n\r\nThe pathophysiology of DSP<\/em> cardiomyopathy includes episodic inflammatory myocardial injury that leads to progressive ventricular fibrosis and myocardial scarring, left ventricular systolic dysfunction, and ventricular arrythmias. This clinically manifests as episodic chest pain, heart failure, and ventricular arrythmias. Patients will often exhibit ST segment changes and troponin elevations but will have normal coronary angiography. There is heterogeneity in phenotype regarding single or biventricular systolic dysfunction and arrhythmogenic burden. Ventricular arrythmias may be life-threatening and the initial presentation may include syncope or cardiac arrest. Varying criteria have been established for diagnosis, but typically include left ventricular (LV) or biventricular systolic dysfunction, ECG abnormalities, cardiac magnetic resonance imaging (CMRI) demonstrating late LV gadolinium enhancement due to scar, ventricular arrhythmias, and personal or familial DSP<\/em> mutation. CMRI is the most sensitive test for phenotype in DSP<\/em> cardiomyopathy as extensive fibrosis may be detected prior to echocardiographic or ECG changes. Due to the expression of DSP<\/em> in skin, more than 50% of patients also exhibit palmoplantar keratoderma (callused skin on hands and soles of the feet). \r\n\r\nTherapy includes management of underlying heart failure and arrythmias with prevention of sudden cardiac death (SCD). Recommendations for risk stratification and management specific to DSP<\/em> cardiomyopathy continue to evolve. Studies by Wang and Smith have differed on the prognostic markers, such as male gender and ejection fraction (EF), which predispose patients to an increased risk of arrhythmia and SCD. An EF below 35% has consistently been associated with high risk for malignant ventricular arrhythmias, though some events may also be associated with an EF of 35% to 55%. Without specific guidelines for DSM<\/em> cardiomyopathy, the current recommendations for primary prevention of SCD with an implantable cardioverter-defibrillator (ICD) are to follow guidelines for other ventricular arrhythmias and heart failure. \r\n\r\nIn contrast to other types of arrhythmogenic and dilated cardiomyopathy, DSP<\/em> cardiomyopathy predominantly involves the left ventricle, often without any RV involvement, and has a unique pathophysiology, clinical presentation, and outcome. Episodes of myocardial injury and epicardial fibrosis occur and precede overt systolic dysfunction in DSP<\/em> cardiomyopathy. Ventricular arrhythmias are common to all forms of arrhythmogenic cardiomyopathy. Left ventricular systolic dysfunction rather than biventricular systolic dysfunction is more common in DSP<\/em> cardiomyopathy. Right ventricular dysfunction is more typical of ARVC. \r\n\r\n \r\nREFERENCES \r\nBrand\u00e3o M, Bariani R, Rigato I, Bauce B. Desmoplakin Cardiomyopathy: Comprehensive Review of an Increasingly Recognized Entity. J Clin Med<\/em>. 2023;12(7):2660. doi: 10.3390\/jcm12072660. \r\n\r\nSmith ED, Lakdawala NK, Papoutsidakis N et al. Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy. Circulation<\/em>. 2020;141(23):1872-1884. doi: 10.1161\/CIRCULATIONAHA.119.044934. \r\n\r\nWang W, Murray B, Tichnell C et al. Clinical characteristics and risk stratification of desmoplakin cardiomyopathy.Europace<\/em>. 2022;24(2):268-277. doi: 10.1093\/europace\/euab183.\r\n\r\nDi Lorenzo F, Marchionni E, Ferradini V et al. DSP-Related Cardiomyopathy as a Distinct Clinical Entity? Emerging Evidence from an Italian Cohort.Int J Mol Sci<\/em>. 2023;24(3):2490. doi: 10.3390\/ijms24032490.\r\n\r\nGraziano F, Zorzi A, Cipriani A et al. The 2020 \”Padua Criteria\” for Diagnosis and Phenotype Characterization of Arrhythmogenic Cardiomyopathy in Clinical Practice. J Clin Med<\/em>. 2022;11(1):279. doi: 10.3390\/jcm11010279. \r\n”,”hint”:””,”answers”:{“tqyx6”:{“id”:”tqyx6″,”image”:””,”imageId”:””,”title”:”A. Left ventricular epicardial fibrosis”,”isCorrect”:”1″},”zso0c”:{“id”:”zso0c”,”image”:””,”imageId”:””,”title”:”B. Ventricular arrhythmias”},”yjc44″:{“id”:”yjc44″,”image”:””,”imageId”:””,”title”:”C. Biventricular systolic dysfunction “}}}}}

{“questions”:{“sl1zl”:{“id”:”sl1zl”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Gibbs Yim, MD and Nicholas Houska, DO – University of Colorado and Children\u2019s Hospital Colorado \r\n\r\nA 13-year-old boy with dilated cardiomyopathy is prescribed dapagliflozin as part of a regimen for decompensated heart failure. Which of the following adverse events is MOST likely associated with dapagliflozin? \r\n”,”desc”:”EXPLANATION \r\nDapagliflozin is an oral sodium-glucose co-transporter (SGLTS) inhibitor used in the treatment of heart failure, diabetes, and chronic kidney disease. SGLT2 is a sodium-glucose co-transporter located in the renal proximal tubules responsible for the reabsorption of glucose. Inhibition of this receptor reduces serum glucose and decreases the renal reabsorption of sodium, inducing natriuresis and diuresis. This medication has been shown to have cardioprotective and renal protective effects in adults and is recommended as a component of medical therapy for heart failure with reduced ejection fraction. More recently dapagliflozin has been trialed in children with heart failure and reduced ejection fraction.\r\n\r\n\r\nIn a single-center, prospective observational study by Newland et al, of 38 nondiabetic pediatric patients with heart failure, dapagliflozin was added to their standard multi-drug heart failure regimen. Sixty-eight percent of patients had dilated cardiomyopathy and 65.8% had a reduced left ventricular ejection fraction of 40% or less. Dapagliflozin was shown to decrease serum B-type natriuretic peptide at 130 days and to improve ejection fraction in the subset of patients with heart failure due to dilated cardiomyopathy. However, the authors point out that this improvement in ejection fraction may not be attributed to dapagliflozin alone, as many of these patients were on a multi-drug regimen therapy for heart failure. \r\n\r\n\r\nAdverse events associated with dapagliflozin use include increased rates of urinary tract infections, which was demonstrated in 16% of patients in the study by Newland. This is likely due to the increased urine glucose concentration because of inhibited renal glucose reuptake. Dapagliflozin is not associated with significant changes in serum electrolytes, hypoglycemia, or hypovolemia. When used in diabetic patients, dapagliflozin has the potential for reducing the degree of hyperglycemia exhibited in diabetic ketoacidosis (DKA). In adult patients on SGLT2 inhibitors, euglycemic DKA has been reported. \r\n\r\n \r\nREFERENCES \r\n\r\nNewland DM, Law YM, Albers EL, Friedland-Little JM, Ahmed H, Kemna MS, Hong BJ. Early Clinical Experience with Dapagliflozin in Children with Heart Failure. Pediatr Cardiol<\/em>. 2023; 44(1):146-152. doi: 10.1007\/s00246-022-02983-0. \r\n\r\n\r\nGrube PM, Beckett RD. Clinical studies of dapagliflozin in pediatric patients: a rapid review. Ann Pediatr Endocrinol Metab<\/em>. 2022; 27(4):265-272. doi: 10.6065\/apem.2244166.083. \r\n\r\n\r\nLoss KL, Shaddy RE, Kantor PF. Recent and Upcoming Drug Therapies for Pediatric Heart Failure. Front Pediatr<\/em>. 2021;9:681224. doi: 10.3389\/fped.2021.681224. \r\n”,”hint”:””,”answers”:{“8t78h”:{“id”:”8t78h”,”image”:””,”imageId”:””,”title”:”A.\tHypoglycemia”},”x2znb”:{“id”:”x2znb”,”image”:””,”imageId”:””,”title”:”B.\tUrinary Tract Infection”,”isCorrect”:”1″},”263me”:{“id”:”263me”,”image”:””,”imageId”:””,”title”:”C.\tHyperkalemia”}}}}}