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

{“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”}}}}}

{“questions”:{“sb62h”:{“id”:”sb62h”,”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 8-day-old boy born with skeletal abnormalities, congenital heart disease, hypotonia, and facial dysmorphisms presents for cardiac surgery. Genetic testing reveals a pathogenic variant in the KMT2D gene consistent with a diagnosis of Kabuki syndrome. Which of the following types of congenital heart disease is MOST likely to be present?\r\n\r\n”,”desc”:”EXPLANATION \r\nFirst described in 1981, Kabuki syndrome is a heterogeneous disorder associated with multiple congenital defects including heart disease, developmental delay, hypotonia, renal malformations, skeletal anomalies, and distinct facial anomalies (laterally sparse and arched eyebrows, long palpebral fissures, large and everted ears, eversion of the lateral eyebrows, and pillowed lower lip). \r\n\r\n\r\nPrior to 2010, the diagnosis of Kabuki syndrome was based on the phenotypic manifestations above. However, in 2010, the first and most common causative (55-80%) gene, KMT2D<\/em>, was identified. Since then, three additional genes have been identified as pathogenic variants in a minority of Kubuki patients. \r\n\r\n\r\nRetrospective studies describe the presence of congenital heart disease (CHD) in 58-70% of patients with Kabuki syndrome. In patients with Kabuki syndrome and CHD, the most common diagnoses are left-sided obstructive lesions (35-47%). The most common left-sided lesions include coarctation of the aorta (17.1%) and hypoplastic left heart syndrome (10.5%). Other left sided-obstructive lesions include aortic stenosis, mitral stenosis, and Shone\u2019s complex. Septal defects are the next most common heart defects, either as a primary diagnosis or in conjunction with the above obstructive lesions. The remainder of children with Kabuki syndrome and CHD exhibit a heterogenous spectrum of cardiac lesions including conotruncal defects, atrioventricular canal defects, and right sided obstructive lesions. Of the identified genetic mutations associated with Kabuki syndrome, the KMT2D (MLL2) gene has been found to be most frequently associated with CHD. \r\n\r\n\r\nAs indicated above, left-sided obstructive lesions, such as coarctation of the aorta and Hypoplastic Left Heart Syndrome, are most commonly observed in patients with Kabuki syndrome versus Tetralogy of Fallot or Transposition of the Great Arteries. \r\n\r\n\r\n\r\n \r\nREFERENCES \r\n\r\nYuan SM. Congenital heart defects in Kabuki syndrome. Cardiol J<\/em>. 2013;20(2):121-4. doi: 10.5603\/CJ.2013.0023. PMID: 23558868.\r\n\r\n\r\nDigilio MC, Marino B, Toscano A, Giannotti A, Dallapiccola B. Congenital heart defects in Kabuki syndrome. Am J Med Genet<\/em>. 2001 May 15;100(4):269-74. doi: 10.1002\/ajmg.1265. PMID: 11343317.\r\n\r\n\r\nDigilio MC, Gnazzo M, Lepri F et al.Congenital heart defects in molecularly proven Kabuki syndrome patients. Am J Med Genet A<\/em>. 2017 Nov;173(11):2912-2922. doi: 10.1002\/ajmg.a.38417. Epub 2017 Sep 8. PMID: 28884922.\r\n”,”hint”:””,”answers”:{“q7e8o”:{“id”:”q7e8o”,”image”:””,”imageId”:””,”title”:”A.\tTetralogy of Fallot”},”9bkq9″:{“id”:”9bkq9″,”image”:””,”imageId”:””,”title”:”B.\tHypoplastic left heart syndrome (HLHS)”,”isCorrect”:”1″},”gxlic”:{“id”:”gxlic”,”image”:””,”imageId”:””,”title”:”C.\tTransposition of the great arteries”}}}}}

{“questions”:{“phvb5”:{“id”:”phvb5″,”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\nA six-week-old infant with a history of congenital central hypoventilation syndrome (CCHS) presents for an exam under anesthesia and rectal biopsy for suspected Hirschsprung\u2019s disease. Which of the following cardiac arrhythmias is MOST likely to be coexistent in this patient?\r\n”,”desc”:”EXPLANATION \r\nCongenital central hypoventilation syndrome (CCHS), or \u201cOndines curse\u201d, is an abnormality in the autonomic regulation of ventilation by the central nervous system not explained by any pulmonary, neurological, or muscular disorders. This results in a reduced or absent ventilatory response to hypercapnia and hypoxia, as well as other autonomic disorders.\r\n\r\n\r\nThe main presenting symptom of CCHS is acute or chronic hypoventilation. Depending on the phenotype, this may present as a neonate, infant, or less commonly later in life. Other conditions associated with CCHS include: Hirschsprung disease, neural crest tumors, esophageal dysmotility, and cardiac arrythmias. \r\n\r\n\r\nIn 2003, mutations in the PHOX2B gene were identified as the major genetic cause of CCHS. These mutations have further been classified as polyalanine repeat mutations (PARMs), or less commonly, non-PARMs. These genotypes have been found to exhibit different phenotypes of CCHS, each with varying onset, degrees of hypoventilation, and other autonomic disorders as mentioned in the previous paragraph. Transmission of the PHOX2B mutation is autosomal dominant, with a 50% risk of transmission to offspring. \r\n\r\n\r\nDiagnosis is based on clinical signs and symptoms, exclusion of other causes of hypoventilation, and polysomnography which typically shows hypoventilation, worse while sleeping than awake, and most severe during non-rapid eye movement sleep. Concern for signs, symptoms, and other known manifestations of CCHS should prompt genetic testing for mutations of the PHOXB gene. \r\n\r\n\r\nThere is no pharmacologic management for the ventilatory symptoms of CCHS, and lifelong ventilatory support will be required for these patients. Depending on phenotype, this ventilatory support may be required only while asleep or 24 hours a day. Ventilation strategies may be non-invasive or invasive ventilation via tracheostomy, particularly in children. Support options include positive pressure ventilation, mask ventilation, and diaphragm pacing via phrenic nerve stimulation. The anesthesiologist caring for these children should be aware that even patients requiring nighttime ventilatory support only will often exhibit increased hypoventilation due to the effects of anesthesia. Preparation should be made for extended monitoring and ventilatory support in the perioperative period. \r\n\r\n\r\nPatients with CCHS often exhibit cardiac arrythmias, typically sinus node dysfunction, sinus pauses, bradycardia, and prolonged R-R intervals. Manifestations of this autonomic dysfunction include syncope, postural hypotension, nocturnal hypertension, and increased risk of sudden death. Patients should be monitored with an annual ECG Holter monitor. Syncope should warrant extended Holter monitoring and\/or consideration of pacemaker implantation. The current recommendation for CCHS patients is to follow the ACC\/AHA guidelines for pacemaker placement. These guidelines recommend pacemaker placement for patients with R-R interval >3 seconds and recurrent syncopal episodes. Typically, a single chamber atrial pacemaker is sufficient. However, a dual-chamber device may be preferable due to the subsequent possibility of developing atrioventricular block. Patients with phrenic nerve pacers require special attention during pacemaker implantation to not cause cross interference between devices. In these patients, the cardiac pacemaker lead should be bipolar to avoid interference with the phenic nerve pacer.\r\n\r\n\r\nFor the patient in the stem, the most likely arrhythmia is sick sinus syndrome due to a known association of CCHS with sinus node dysfunction. Supraventricular tachycardias and ectopic atrial tachycardia are not commonly associated with CCHS.\r\n\r\n \r\nREFERENCES \r\n\r\nTrang H, Samuels M, Ceccherini I, et al. Guidelines for diagnosis and management of congenital central hypoventilation syndrome.Orphanet J Rare Dis<\/em>. 2020;15(1):252.\r\n\r\n\r\nGronli JO, Santucci BA, Leurgans SE, Berry-Kravis EM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B<\/em> genotype determines risk for sudden death. Pediatr Pulmonol<\/em>. 2008; 43(1): 77-86. \r\n\r\n\r\nWeese-Mayer DE, Rand CM, Khaytin I, et al. Congenital Central Hypoventilation Syndrome. 2004 Jan 28 [Updated 2021 Jan 28]. In: Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews\u00ae [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024. Available from: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1427\/\r\n”,”hint”:””,”answers”:{“dbbdo”:{“id”:”dbbdo”,”image”:””,”imageId”:””,”title”:”A. Supraventricular tachycardia “},”53kdl”:{“id”:”53kdl”,”image”:””,”imageId”:””,”title”:”B. Sick sinus syndrome”,”isCorrect”:”1″},”ujv5y”:{“id”:”ujv5y”,”image”:””,”imageId”:””,”title”:”C. Ectopic atrial tachycardia”}}}}}

{“questions”:{“3q03h”:{“id”:”3q03h”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Melissa Colizza – Stollery Children\u2019s Hospital – Edmonton, Canada \r\nAccording to a retrospective analysis by Saengsin et al. from the November 2023 issue of Anesthesia and Analgesia, that compared patients who received milrinone to matched cohorts who did not receive milrinone after Tetralogy of Fallot repair, which of the following outcomes was MOST likely to be observed during the first 72 hours after surgery in patients who received milrinone? \r\n”,”desc”:”EXPLANATION \r\nLow cardiac output syndrome (LCOS) is a common occurrence in the pediatric cardiac surgical population, with an incidence of 25%-60% reported in the literature. LCOS is a clinical syndrome in which there is an imbalance of oxygen supply and demand to the tissues. Prevention and management often necessitate the use of both pharmacologic and nonpharmacologic strategies to restore oxygen balance. The etiology of post-surgical LCOS is multifactorial, including myocardial ischemia during aortic cross clamping, the effects of cardioplegia, activation of the inflammatory and complement cascades and changes in pulmonary and\/or systemic vascular resistance. It is associated with a decrease in cardiac index, accompanied by an increase in systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR). Risk factors include younger age (especially neonates), complex procedures with prolonged cardiopulmonary bypass times, use of large patch materials for repair, and ventriculotomies.\r\n\r\n\r\nMilrinone is a phosphodiesterase-3 (PDE-3) inhibitor with vasodilatory and inotropic properties. The Prophylactic Intravenous use of Milrinone After Cardiac Operation in Pediatrics \r\n(PRIMACORP) study by Hoffman et al (2003) is a landmark study, as it highlighted the importance of LCOS prevention. The study compared mortality and the development of LCOS (requiring additional pharmacological or other support) within in the first 36 hours after treatment in three groups of patients under six years old following biventricular repair. Patients were randomly assigned to three groups, a high-dose milrinone (75mcg\/kg loading dose with an infusion at 0.75 mcg\/kg\/min), a low-dose milrinone (25mcg\/kg loading dose with an infusion at 0.25 mcg\/kg\/min), or placebo treatment group. LCOS was defined as clinical signs or symptoms of hypoperfusion, with or without a widened arterio-venous oxygen saturation (AVO_{2<\/sub>) difference or metabolic acidosis. The trial showed a relative risk reduction of 64% for LCOS in the high dose milrinone group when compared to placebo. It also revealed a 5-9% decrease in systolic blood pressure after milrinone loading dose administration. Notably, this trial advanced the use of milrinone as an alternative or adjuvant to other vasoactive agents and has led to its widespread use after pediatric cardiac surgery. \r\n\r\n\r\nDespite the results of the PRIMACORP study, strategies to prevent and treat LCOS vary widely. While many centers start milrinone intra-operatively and continue post-operatively, dosage and timing of administration differs greatly. Interestingly, milrinone dosage is often lower than that used in the PRIMACORP study, and it is frequently co-administered with other vasoactive agents. A 2015 Cochrane review by Burkhardt et al examined five randomized-controlled trials comparing milrinone to either placebo, levosimendan, or dobutamine. The authors concluded that milrinone could reduce incidence of LCOS versus placebo in the short-term (up to 36 hours), but that there is insufficient evidence to suggest its effectiveness in preventing mortality or LCOS in the pediatric cardiac surgical population, especially when compared to other agents. \r\n\r\n\r\n\r\nA retrospective study from Boston Children\u2019s Hospital by Mills et al (2016) investigated the use of milrinone to reduce SVR in neonates undergoing Stage 1 palliation for hypoplastic left heart syndrome (HLHS). Patients were administered a loading dose of milrinone (25 to 100 mcg\/kg titrated to goal SVR at a cardiac index of 2 L\/min m^{2<\/sup>) prior to weaning from cardiopulmonary bypass and an infusion of milrinone (0.25 to 1 mcg\/kg\/min) upon separation from cardiopulmonary bypass (CPB) which was continued in the cardiac intensive care unit (CICU). Epinephrine was used as the main inotropic agent. The study demonstrated that the milrinone-treated group, after correction for shorter CPB times, had lower SVR, decreased AV-O2<\/sub> difference and lower lactate levels. This study highlights the physiologic impact of high SVR on the parallel, single-ventricle circulation by demonstrating that lower SVR in conjunction with adequate inotropy leads to improved oxygen delivery (DO2<\/sub>) and is beneficial in this patient population. \r\n\r\n\r\nMore recently, Saengsin et al investigated milrinone use in children less than one year of age undergoing complete Tetralogy of Fallot (TOF) repair. The study included patients with classic TOF or TOF with pulmonary atresia (without major aortopulmonary collateral arteries) between September 2011 and January 2020. Propensity scoring was used to match 212 patients based on anatomical and surgical risks (106 milrinone-treated versus 106 non milrinone-treated). The primary outcome measure was the need for administration of volume (blood products and 5% albumin) during the first 72 hours after surgery. Secondary outcomes included vital signs (heart rate and blood pressure) and indices of cardiac output and oxygen delivery. The dose and timing of milrinone administration were not protocolized. Milrinone-treated patients tended to be younger with lower weights and smaller tricuspid valves, pulmonary valves and main pulmonary arteries, and were more frequently repaired with transannular patches. The study demonstrated that patients treated with milrinone received more fluid boluses than those who did not (66% vs 52%; confidence interval 1%-27%, P=0.036). In addition, the total volume administered during the first 72 postoperative hours was significantly associated with the total amount of milrinone administered. There was no difference in perfusion indices, such as AVO2<\/sub> difference or serum lactates, nor in ICU or hospital lengths of stay. This study has several limitations. In addition to being a single-center, retrospective study, the dosing of milrinone was not protocolized, and surgeries occurred over a nine-year period when perioperative management strategies may have changed. However, it does provide some physiological insights into the altered physiology after surgical repair of TOF. The right ventricle (RV) in TOF is restrictive due to hypertrophy, use of patch material, a ventriculotomy incision and myocardial edema, which limit its capacitance due to diastolic dysfunction. In turn, this limits RV preload and cardiac output. The restrictive RV requires higher filling pressures to maintain an adequate stroke volume. The vasodilatory properties of milrinone may be counterproductive in this setting, and thus, may lead to an increased need for fluid administration. \r\n\r\n\r\nThe available evidence suggests that neonates undergoing the Stage I palliation are most likely to benefit from milrinone, especially if used with an additional inotrope to target appropriate perfusion indices. Milrinone should be used with caution in patients undergoing TOF repair due to the risk of reducing RV preload or stroke volume and thus cardiac output. There are advantages and drawbacks to each vasoactive agent and usage should be individualized to each patient\u2019s unique physiology with the goal of adequate end organ perfusion.\r\n\r\n\r\nIn summary, based on a recent retrospective study of milrinone use after TOF repair, these patients may be more likely to require increased fluid administration in the early post-operative period. However, milrinone did not appear to improve indices of cardiac output or reduce the length of stay in the intensive care unit.\r\n\r\n\r\n\r\n \r\nREFERENCES \r\nHoffman TM, Wernovsky G, Atz AM, et al. Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation<\/em>. 2003;107(7):996-1002. doi: 10.1161\/01.cir.0000051365.81920.28\r\n\r\nBurkhardt BE, R\u00fccker G, Stiller B. Prophylactic milrinone for the prevention of low cardiac output syndrome and mortality in children undergoing surgery for congenital heart disease. Cochrane Database Syst Rev<\/em>. 2015;(3):CD009515. doi:10.1002\/14651858.CD009515.pub2\r\n\r\nMills KI, Kaza AK, Walsh BK, et al. Phosphodiesterase Inhibitor-Based Vasodilation Improves Oxygen Delivery and Clinical Outcomes Following Stage 1 Palliation. J Am Heart Assoc<\/em>. 2016;5(11): e003554. doi: 10.1161\/JAHA.116.003554\r\n\r\nSaengsin K, Sperotto F, Lu M, et al. Administration of Milrinone Following Tetralogy of Fallot Repair Increases Postoperative Volume Administration Without Improving Cardiac Output. Anesth Analg<\/em>. 2023;137(5):1056-1065. doi: 10.1213\/ANE.0000000000006662\r\n\r\nBojan M, Pouard P. Hemodynamic Management. In Andropoulos DB. Anesthesia for Congenital Heart Disease<\/em>. 4th ed. USA: Wiley-Blackwell; 2023: 494-526.\r\n\r\n”,”hint”:””,”answers”:{“ba9uw”:{“id”:”ba9uw”,”image”:””,”imageId”:””,”title”:”A. Improved indices of cardiac output”},”qrjiw”:{“id”:”qrjiw”,”image”:””,”imageId”:””,”title”:”B. Decreased intensive care unit length of stay”},”5itwm”:{“id”:”5itwm”,”image”:””,”imageId”:””,”title”:”C. Increased need for fluid resuscitation”,”isCorrect”:”1″}}}}}}}