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

{“questions”:{“p5z3o”:{“id”:”p5z3o”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Clementine Vo, DO \u2013 Texas Children\u2019s Hospital\/Baylor College of Medicine, Houston, TX, USA AND\r\nDestiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR, USA \r\n\r\nA 16-year-old girl with a history of an unbalanced atrioventricular canal palliated with the Fontan procedure presents for cardiac catheterization due to ascites. Magnetic resonance imaging of the liver demonstrates hepatic fibrosis, and laboratory studies reveal hypoalbuminemia and elevated stool anti-trypsin levels. Which of the following diagnoses is the MOST likely cause of these clinical findings? \r\n”,”desc”:”EXPLANATION \r\nThe Fontan physiology creates a state of chronic venous hypertension that can lead to multi-system organ dysfunction, characterized by protein-losing enteropathy (PLE), plastic bronchitis, and Fontan-associated liver disease. PLE and plastic bronchitis are associated with lymphatic dysfunction and the leakage of protein-rich lymph fluid into luminal spaces adjacent to lymphatic vessels. The leakage of proteinaceous fluid into the intestinal lumen results in enteric protein loss, also known as PLE. Leakage of proteinaceous material into the bronchial tree causes plastic bronchitis with the formation of bronchial casts. Symptoms of plastic bronchitis include dyspnea, cough, wheezing, and, in severe cases, respiratory failure due to airway obstruction from the casts. The diagnosis is confirmed by bronchoscopy.\r\n\r\nSigns and symptoms of PLE include progressive central and peripheral edema, ascites, and often diarrhea. PLE affects up to 13% of Fontan patients, typically within ten years of the Fontan procedure. Factors predisposing to PLE include altered intestinal mucosal perfusion, low cardiac output, a selective increase in mesenteric vascular resistance, a proinflammatory state altering gut membrane permeability, and low heparan sulfate proteoglycans in the enterocyte membrane resulting in reduced enteral protein transport and increased luminal protein loss. Protein loss results in severe hypoalbuminemia and low serum oncotic pressure, leading to edema. The gold standard for PLE diagnosis is an elevated 24-hour stool alpha-1 antitrypsin clearance. PLE may also be diagnosed with a single stool sample with an elevated alpha-1 antitrypsin level concurrent with serum hypoalbuminemia and generalized edema with no other cause. Chronic protein loss is associated with a multitude of clinical sequelae, such as poor tissue integrity and impaired wound healing, coagulation factor deficiency resulting in coagulopathy and thromboembolic complications, hypoalbuminemia leading to secondary hypocalcemia, and low immunoglobulin levels resulting in immunodeficiency.\r\n\r\nPLE management focuses on reducing chronic venous hypertension by optimizing Fontan hemodynamics and improving nutritional intake. Medical management includes diuretics to reduce fluid overload, albumin to restore oncotic pressure, steroids to reduce inflammation, aldosterone antagonists and\/or heparin to stabilize the proteoglycan layer of the gut, and pulmonary vasodilators to reduce chronic venous congestion. Lymphatic interventions may reroute the lymphatic flow to provide a measure of symptomatic relief and improvement in quality of life. Heart transplantation is the last resort and appears to be effective for symptom relief. However, patients with PLE are often poor candidates due to chronic protein wasting and poor nutritional status and may not survive the waiting period or the transplantation procedure. The prognosis is grim, with 50% mortality within the first five years after diagnosis. \r\n\r\nThe patient in the stem has elevated stool alpha-antitrypsin, hypoalbuminemia, and ascites, which are diagnostic for PLE. Therefore, PLE is the most likely cause of the clinical presentation described in the stem. Plastic bronchitis is not the correct answer, as it is characterized by respiratory symptoms and the presence of airway casts. Although hypoalbuminemia and ascites are both signs of hepatic dysfunction in Fontan-associated liver disease (FALD), the MRI demonstrates fibrosis rather than cirrhosis, a classic finding in FALD. Additionally, FALD is not usually associated with significant hepatic synthetic impairment.\r\n\r\n\r\n \r\nREFERENCES \r\nRychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with Fontan circulation: A scientific statement from the American Heart Association. Circulation<\/em>. 2019;140(6):e234-e284. \r\n\r\nMackie AS, Veldtman GR, Thorup L, Hjortdal VE, Dori Y. Plastic bronchitis and protein-losing enteropathy in the Fontan patient: Evolving understanding and emerging therapies. Can J Cardiol<\/em>. 2022;38(7):988-1001. doi:10.1016\/j.cjca.2022.03.011\r\n\r\nJohn AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F. Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol<\/em>. 2014;64(1):54-62. doi: 10.1016\/j.jacc.2014.04.025. PMID: 24998129.\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. 2019.14;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”,”hint”:””,”answers”:{“1jyk6”:{“id”:”1jyk6″,”image”:””,”imageId”:””,”title”:”A.\tPlastic bronchitis”},”bedeu”:{“id”:”bedeu”,”image”:””,”imageId”:””,”title”:”B.\tProtein-losing enteropathy”,”isCorrect”:”1″},”rz5a2″:{“id”:”rz5a2″,”image”:””,”imageId”:””,”title”:”C.\tFontan-associated liver disease”}}}}}

{“questions”:{“8r6p9”:{“id”:”8r6p9″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Meera Gangadharan, MBBS, FAAP, FASA – Children\u2019s Memorial Hermann Hospital, McGovern Medical School, Houston, TX AND Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR, USA \r\n\r\nA 10-month-old girl with low cardiac output syndrome after cardiac surgery is on a levosimendan infusion in addition to epinephrine and milrinone. Which of the following BEST describes the mechanism of action of levosimendan?\r\n”,”desc”:”EXPLANATION \r\nLevosimendan is an inodilator, first gaining European approval in the year 2000 to treat acutely decompensated chronic heart failure. Currently, it is not FDA-approved in the United States. The inotropic effects of levosimendan result from the binding of troponin C, thus stabilizing calcium-induced conformational changes and prolonging the interaction between actin and myosin filaments during systole. The resultant increase in contractility is not associated with significantly increased myocardial oxygen consumption. Levosimendan also produces vasodilation by opening ATP-sensitive potassium channels in vascular smooth muscle, which may have a cardioprotective effect by reducing excessive calcium during ischemic reperfusion injury. \r\n\r\nPatients with heart failure receiving levosimendan demonstrate improved hemodynamics and improved symptomatology without the development of drug tolerance. Since its introduction, levosimendan has been used clinically to treat cardiogenic shock, acute stress-induced cardiomyopathy, depressed right ventricular function, and pulmonary hypertension in the post-cardiac surgical and medical patient populations. The most commonly reported adverse events are hypotension, headache, atrial fibrillation, hypokalemia, and tachycardia. \r\n\r\nAlthough levosimendan is used in the pediatric population in locations outside of the United States, the literature supporting its clinical efficacy is rather limited. Lapere et al. conducted a systematic review and meta-analysis of nine randomized controlled trials involving 539 patients who were 18 years and younger that assessed the safety and efficacy of perioperative levosimendan use after cardiac surgery as compared to dobutamine, milrinone, or placebo. The authors concluded that levosimendan reduced the incidence of low cardiac output syndrome and increased cardiac index but did not have any effect on mortality, ICU length of stay (LOS), hospital LOS, duration of mechanical ventilation, serum lactate, central venous oxygen saturation, serum creatine levels, or the incidence of acute kidney injury. Pilia et al. conducted a systematic review of 48 studies, including 1,271 patients investigating the safety profile of levosimendan. Hypotension occurred in 28.9% of patients, and arrhythmias, predominantly tachycardias, occurred in 12.3% of patients. Of note, the reported dosing of levosimendan typically included a loading dose of 12 mcg\/kg over 10 to 15 minutes, followed by an infusion of 0.05- 0.2 mcg\/kg\/min. \r\n\r\nThe correct answer is A. The inotropic effects of levosimendan are mediated by increasing the sensitivity of cardiac myocytes to calcium. It also promotes vasodilation by opening ATP-sensitive potassium channels in vascular smooth muscle. Catecholamines such as epinephrine and dobutamine act predominantly by beta-receptor activation, while milrinone is a phosphodiesterase-3 inhibitor.\r\n\r\n\r\n \r\nREFERENCES \r\nNieminen MS, Fruhwald S, Heunks LM, et al. Levosimendan: current data, clinical use, and future development. Heart Lung Vessel<\/em>. 2013;5(4):227-245.\r\n\r\nPapp Z, Agostoni P, Alvarez J, et al. Levosimendan efficacy and safety: 20 years of SIMDAX in clinical use. Card Fail Rev<\/em>. 2020;6:e19. doi:10.15420\/cfr.2020.03\r\n\r\nLapere M, Rega F, Rex S. Levosimendan in pediatric cardiac anaesthesiology: A systematic review and meta-analysis. Eur J Anaesthesiol<\/em>. 2022;39(8):646-655. doi:10.1097\/EJA.0000000000001711\r\n\r\nPilia E, Silvetti S, Bohane SM, Pusceddu E, Belletti A; Safety of levosimendan in pediatric patients: an up-to-date systematic review. J Cardiothorac Vasc Anesth<\/em>. 2024;38(3):820-828. doi:10.1053\/j.jvca.2023.11.020\r\n\r\n”,”hint”:””,”answers”:{“6zedp”:{“id”:”6zedp”,”image”:””,”imageId”:””,”title”:”A.\tIncreased myocyte sensitivity to calcium “,”isCorrect”:”1″},”g555o”:{“id”:”g555o”,”image”:””,”imageId”:””,”title”:”B.\tActivation of \u03b2-1 receptors”},”attie”:{“id”:”attie”,”image”:””,”imageId”:””,”title”:”C.\tInhibition of phosphodiesterase-3\r\n\r\n”}}}}}

{“questions”:{“mlbh2”:{“id”:”mlbh2″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Anuradha Dev, MD and Marc Atwell MD – Georgetown Public Health Corporation (GPHC), Georgetown, Guyana AND Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR, USA \r\nA 10-year-old boy presents with shortness of breath and declining exercise capacity. Transthoracic echocardiography demonstrates a double chamber right ventricle. Which of the following percentages reflects the approximate number of patients with double chamber right ventricle with a concurrent ventricular septal defect? \r\n\r\n”,”desc”:”EXPLANATION \r\nDouble chamber right ventricle (DCRV) is a rare cardiac diagnosis reported in up to 2.6% of patients with congenital heart disease. It typically presents in childhood with few cases reported in adults. DCRV is described by a sub-infundibular tissue substrate within the body of the right ventricle (RV) that leads to progressive RV outflow obstruction. The obstructing tissue has been identified as hypertrophied trabecular muscle bands and\/or atypical moderator bands that divide the RV into a proximal high-pressure inlet chamber located close to the apical region of the RV and a distal low-pressure outlet chamber located near the infundibular region. There is typically an increased pressure gradient greater than 20 mmHg across the two chambers (see Figure below). \r\n\r\nIt is hypothesized that the anatomical substrate for DCRV is often present at birth. For example, congenital cardiac defects that create flow turbulence may trigger tissue hypertrophy leading to obstruction within the RV cavity. Although over 75% of DCRVs are associated with ventricular septal defect (VSD), it is also associated with tetralogy of Fallot (TOF), double outlet right ventricle, and Ebstein anomaly. The VSD of DCRV is usually small and perimembranous. Although it may be located in any location along the interventricular septum, the VSD usually connects with the proximal high-pressure inlet chamber. The resulting pathophysiology is due to shunt flow characteristics as determined by the location of the VSD in relation to the obstruction within the RV. When the VSD is distal to the obstructing muscle bundle, the resulting physiology is akin to an isolated VSD, but when the VSD connects to the RV proximal to the muscle bundle, the physiology is akin to tetralogy of Fallot. \r\n\t\r\n\r\n\r\n \r\n\r\nFigure: Two-dimensional TTE with color-flow Doppler. Parasternal short-axis view at the aortic valve level in end systole demonstrates muscular septation of the RV into a high-pressure proximal chamber and low-pressure distal subvalvular chamber. Arrows indicate RV muscular bundles causing subvalvular obstruction. AV= aortic valve, PV=pulmonic valve, TV=tricuspid valve, RA=right atrium, pRV=proximal RV, dRV=distal RV. \r\nFrom Malone RJ, Henderson ER, Wilson ZR, et al. Double-chambered right ventricle in adulthood: a case series. CASE (Phila). 2024;8(3Part A):202-209. doi:10.1016\/j.case.2023.12.012. Creative Commons Licensing 4.0. \r\n\r\nPatients with severe RVOT obstruction typically present with dyspnea on exertion and limited exercise capacity. Echocardiography is a good first-line screening tool for many cardiac defects, but the identification of DCRV may often be overlooked unless there is a high index of suspicion. Other imaging modalities such as magnetic resonance imaging or cardiac catheterization can confirm the diagnosis of DCRV. Surgery involves transatrial or transventricular resection of the obstructing muscle bundles and a VSD closure. Survival rates in the current era are excellent. However, there is the potential for a right bundle branch block or complete heart block. \r\n\r\nThe correct answer is C, over 75% of patients with DCRV also have an associated VSD.\r\n\r\n\r\n \r\nREFERENCES \r\nLoukas M, Housman B, Blaak C, Kralovic S, Tubbs RS, Anderson RH. Double-chambered right ventricle: a review. Cardiovasc Pathol<\/em>. 2013;22(6):417-423. doi:10.1016\/j.carpath.2013.03.004\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. 2019.14;73(18):2361]. J Am Coll Cardiol<\/em>. 2019;73(12):1494-1563. doi:10.1016\/j.jacc.2018.08.1028\r\n\r\nSaid SM, Burkhart HM, Dearani JA, O’Leary PW, Ammash NM, Schaff HV. Outcomes of surgical repair of double-chambered right ventricle. Ann Thorac Surg<\/em>. 2012;93(1):197-200. doi:10.1016\/j.athoracsur.2011.08.043\r\n\r\nKahr PC, Alonso-Gonzalez R, Kempny A, et al. Long-term natural history and postoperative outcome of double-chambered right ventricle–experience from two tertiary adult congenital heart centres and review of the literature. Int J Cardiol<\/em>. 2014;174(3):662-668. doi:10.1016\/j.ijcard.2014.04.177\r\n\r\nHubail ZJ, Ramaciotti C. Spatial relationship between the ventricular septal defect and the anomalous muscle bundle in a double-chambered right ventricle. Congenit Heart Dis<\/em>. 2007; 2:421-423. \r\n\r\n\r\n\r\n”,”hint”:””,”answers”:{“842tz”:{“id”:”842tz”,”image”:””,”imageId”:””,”title”:”A.\t25%”},”c3amd”:{“id”:”c3amd”,”image”:””,”imageId”:””,”title”:”B.\t50%”},”qjgeb”:{“id”:”qjgeb”,”image”:””,”imageId”:””,”title”:”C.\t75%”,”isCorrect”:”1″}}}}}

{“questions”:{“cl5v4”:{“id”:”cl5v4″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Philicia Findlay-Hardyal, MD and Arturo Marrero, MD- Georgetown Public Health Corporation – Georgetown, Guyana AND Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR, USA \r\n\r\nA 3-month-old infant with a suspected vascular ring is undergoing rigid bronchoscopy, which demonstrates the presence of complete tracheal rings. Which of the following types of vascular rings is MOST likely present in this patient? “,”desc”:”EXPLANATION \r\nVascular rings describe congenital anomalies whereby the aortic arch and its primary branches form a circle around the trachea, the esophagus, or both, potentially resulting in tracheal and\/or esophageal obstruction and obstructive symptoms. The incidence of isolated vascular rings has been reported as seven in 10,000 live births (0.07%). As vascular rings are rare, diagnosis requires a high index of clinical suspicion. Patients may be misdiagnosed with refractory asthma and\/or gastrointestinal pathology, thus deferring further workup until the vascular ring is finally diagnosed. \r\n\r\n\r\nThe embryologic mechanism for vascular rings originates from the interruption at different stages of the normal regression of the six pharyngeal arches that are connected to the dorsal aortae during the embryologic development of the aortic arch and its main branches. This results in the persistence of vascular structures or ligamentous remnants that may compress the trachea or esophagus.\r\n\r\n\r\nThe majority of vascular ring malformations can be classified into the following grouping system described by Backer and Mavroudis: \r\n\r\n\r\nA.\tDouble aortic arch: the left and right aortic arches encircle the trachea and esophagus. \r\nB.\tRight aortic arch with left ligamentum: the aortic arch is to the right of the trachea, with the ligamentum connecting the main pulmonary artery to the descending aorta. \r\nC.\tInnominate artery compression syndrome: the innominate artery compresses the anterior trachea by over 80%. \r\nD.\tPulmonary artery sling: the left pulmonary artery originates from the right pulmonary artery traveling posteriorly, between the trachea and esophagus.\r\n\r\n\r\nThe figure below illustrates reconstructed computed tomography of the different types of vascular rings.\r\n\r\n\r\n\r\n \r\n\r\nFigure: Vascular rings reconstructed from cardiac computed tomography. A: double aortic arch; two aortic arches (white bold arrows) comprise the complete ring in this patient. B: right aortic arch with persistent left ligamentum arteriosum; Kommerell’s diverticulum (arrowhead) and fibrotic band (white dotted arrows) comprise the complete ring in this patient. C: right aortic arch with aberrant left subclavian artery (red bold arrow). D: pulmonary sling; left pulmonary artery originates from the right pulmonary artery (red dotted arrow), compressing the bronchus. \r\nSource: Suh YJ, Kim GB, Kwon BS, et al. The clinical course of vascular rings and risk factors associated with mortality. Korean Circ J. 2012;42(4):252-258. doi:10.4070\/kcj.2012.42.4.252- Creative Commons License\r\n\r\n\r\n\r\nOnce clinical suspicion arises, confirmatory diagnosis is usually made by cardiac computed tomography or magnetic resonance imaging. Echocardiography is unreliable for definitive diagnosis but does aid in the detection of associated cardiac anomalies occurring in approximately 12% of patients. Bronchoscopy allows for the delineation of tracheal abnormalities, such as tracheobronchomalacia, and for the detection of complete tracheal rings.\r\n\r\n\r\nExcept for pulmonary artery slings, vascular ring repair is usually performed through a thoracotomy for surgical division of the ring. Surgical repair of a pulmonary artery sling is performed through a median sternotomy approach and under cardiopulmonary bypass, as the left pulmonary artery needs to be translocated. Additionally, 60-80% of patients with pulmonary artery slings also have tracheal stenosis secondary to complete cartilaginous tracheal rings, which are repaired during the same procedure. Tracheal reconstruction is usually recommended after the vascular ring repair to avoid contamination of the surgical field from secretions within the trachea. The most common repair for complete tracheal rings is a slide tracheoplasty for long-segment stenosis. Resection of a short, narrowed segment of complete tracheal rings may be repaired with an end-to-end anastomosis. Although survival rates are excellent, patients often require repeat airway evaluation and intervention for persistent tracheomalacia, granuloma formation, or balloon dilation. Patients also require regular follow-up to evaluate for the development of left pulmonary artery stenosis.\r\n\r\n\r\nIn this infant, the presence of the complete tracheal rings indicates a moderate to high probability of a pulmonary artery sling. Although double aortic arch and right aortic arch\/left ligamentum are much more common than pulmonary artery slings, they are not typically associated with complete tracheal rings. \r\n\r\n\r\n \r\nREFERENCES \r\n\r\nWadle M, Joffe D, Backer C, Ross F. Perioperative and anesthetic considerations in vascular rings and slings. Semin Cardiothorac Vasc Anesth<\/em>. 2024;28(3):152-164. doi:10.1177\/10892532241234404\r\n\r\n\r\nWorhunsky DJ, Levy BE, Stephens EH, Backer CL. Vascular rings. Semin Pediatr Surg<\/em>. 2021;30(6):151128. doi:10.1016\/j.sempedsurg.2021.151128\r\n\r\n\r\nMcKenzie I, Markakis Zestos M, Stayer S, Kaminski E, Davies P, Andropoulos D. Anesthesia for miscellaneous lesions. In: Andropoulos D, Mossad E, Gottlieb E, eds. Anesthesia for Congenital Heart Disease<\/em>. 4th Edition. New Jersey: John Wiley & Sons, Inc.; 2023: 816-820.\r\n\r\n\r\nSuh YJ, Kim GB, Kwon BS, et al. Clinical course of vascular rings and risk factors associated with mortality. Korean Circ J. 2012;42(4):252-258. doi:10.4070\/kcj.2012.42.4.252\r\n”,”hint”:””,”answers”:{“etcn7”:{“id”:”etcn7″,”image”:””,”imageId”:””,”title”:”A.\tDouble aortic arch”},”keig0″:{“id”:”keig0″,”image”:””,”imageId”:””,”title”:”B.\tPulmonary artery sling”,”isCorrect”:”1″},”tfkzq”:{“id”:”tfkzq”,”image”:””,”imageId”:””,”title”:”C.\tRight aortic arch with left ligamentum\r\n\r\n”}}}}}

{“questions”:{“nm19a”:{“id”:”nm19a”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Nicholas Houska, DO – University of Colorado – Childrens Hospital Colorado\r\nA 7-year-old boy with a history of acute lymphoblastic leukemia treated with doxorubicin presents with symptoms of heart failure. Which of the following morphological subtypes of cardiomyopathy is MOST likely to be found in this patient? \r\n\r\n”,”desc”:”EXPLANATION \r\nAnthracyclines are a class of cytotoxic drugs extracted from the Streptomyces<\/em> bacterium. They are utilized as chemotherapeutic agents and include doxorubicin (the most commonly used), daunorubicin, and epirubicin. First used in the 1960s, anthracyclines remain one of the most commonly used and effective chemotherapeutics for solid and hematological cancers. Anthracyclines are inhibitors of the DNA topoisomerase II enzyme, leading to DNA double-strand breaks and subsequent upregulation of p53, which results in programmed cell death (apoptosis). \r\n\r\nAnthracyclines are thought to be the primary drugs related to chemotherapy-induced cardiotoxicity. The risk of cardiotoxicity increases as the total cumulative dose of doxorubicin (Dox) increases, with a 3-5% risk at 400 mg\/m^{2<\/sup> and 18-48% at 700 mg\/m2<\/sup>. Apoptosis-mediated loss of cardiomyocytes and oxidative stress are the main culprits of Dox-induced cardiomyopathy. Extremes of age (less than 5 years of age or greater than 65 of age), preexisting cardiac disease or cardiovascular risk factors, and radiation therapy are additional risk factors for Dox-induced cardiomyopathy. \r\n\r\nAnthracycline-induced cardiotoxicity can develop at various time points after the initiation of doxorubicin treatment, leading to a dated classification. Acute cardiotoxicity occurs after a single dose or course with the onset of symptoms within 2 weeks. Early-onset chronic occurs within 1 year and presents as a dilated-hypokinetic cardiomyopathy, with progressive development of heart failure. Late-onset chronic occurs years to decades after completion of doxorubicin therapy. Anthracycline-induced cardiotoxicity is defined as a decrease in left ventricular ejection fraction greater than 10% with a final value less than 53% after exposure. It is typically detected via cardiac imaging such as echocardiography and magnetic resonance imaging, along with monitoring of cardiac biomarkers. \r\n\r\nMost guidelines recommend serial monitoring after anthracycline use. While historically, doxorubicin-induced cardiotoxicity has been thought of as irreversible and associated with high mortality, more recent studies are suggesting that there may be some effectiveness of heart failure therapy. Primary prevention includes lifestyle modification of cardiovascular risk factors, limitations on total dosage, and the use of less cardiotoxic analogs such as epirubicin. Additionally, dexrazoxane, an iron-chelating agent that prevents the accumulation of oxygen-free radicals, is often included in chemotherapy regimens to protect against the cardiotoxic effects of anthracyclines. The mainstays of heart failure therapy for anthracycline-mediated dilated cardiomyopathy are angiotensin-converting enzyme inhibitors and beta blockers. \r\n\r\nAnthracyclines, and specifically doxorubicin-induced cardiotoxicity, are most commonly associated with a dilated hypokinetic cardiomyopathy (answer B). Hypertrophic cardiomyopathy (answer C) is typically associated with genetic disorders. While restrictive cardiomyopathy (answer A) can develop as a result of anthracycline use, it is much less common and presents years or decades after exposure. Restrictive cardiomyopathy is more commonly associated with deposition disorders such as amyloidosis and hemochromatosis. \r\n\r\n\r\n \r\nREFERENCES \r\nCardinale D, Iacopo F, Cipolla CM. Cardiotoxicity of Anthracyclines. Front Cardiovasc Med<\/em>. 2020;7:26. doi: 10.3389\/fcvm.2020.00026. \r\n\r\nRawat PS, Jaiswal A, Khurana A, Bhatti JS, Navik U. Doxorubicin-induced cardiotoxicity: An update on the molecular mechanism and novel therapeutic strategies for effective management. Biomed Pharmacother<\/em>.2021;139:111708. doi: 10.1016\/j.biopha.2021.111708. \r\n\r\nMancilla TR, Iskra B, Aune GJ. Doxorubicin-Induced Cardiomyopathy in Children. Compr Physiol<\/em>. 2019:12;9(3):905-931. doi: 10.1002\/cphy.c180017. \r\n\r\n”,”hint”:””,”answers”:{“kenij”:{“id”:”kenij”,”image”:””,”imageId”:””,”title”:”A.\tRestrictive cardiomyopathy”},”ggit0″:{“id”:”ggit0″,”image”:””,”imageId”:””,”title”:”B.\tDilated cardiomyopathy”,”isCorrect”:”1″},”huyfa”:{“id”:”huyfa”,”image”:””,”imageId”:””,”title”:”C.\tHypertrophic cardiomyopathy\r\n\r\n”}}}}}}