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OBJECTIVES The hands-on surgical training course utilizes 3-dimensional (3D)-printed heart models to simulate complex congenital heart operations. This study aimed to validate a model and assessment tool in the simulation of 2 techniques of the Norwood operation and investigate whether technical performance improves following rehearsal with or without proctor presence. METHODS Five ‘experienced’ and 5 ‘junior’ surgeons performed 2 techniques of the Norwood operation on 3D-printed models of hypoplastic left heart syndrome. Performances were retrospectively assessed by 10 raters with varying experience in congenital heart surgery (CHS) (Medical Doctorate versus non-Medical Doctorate). Assessments were made with the procedure-specific Hands-On Surgical Training-CHS tool. Results were analysed for technical performance and rater consistency. Following validation, 30 surgeons (24 with proctor guidance and 6 with training videos and objective feedback only) simulated the Norwood operation twice. RESULTS Performance scores were consistently higher for experienced surgeons and raters discriminated clearly between the experienced and junior surgeons (P ≤ 0.001). The hands-on surgical training-CHS tool showed high inter-rater (0.86) and intra-rater (0.80) reliability among all raters. Scores for both experienced and junior surgeons were highly consistent across all raters, with no statistically significant difference (P = 0.50). All surgeons successfully performed the Norwood operation. Sixty attempts were scored in total. Eighty-seven percentage (26/30) of surgeons’ scores (mean: attempt 1 = 92, attempt 2 = 104) and times [mean: attempt 1 = 1:22:00, attempt 2 = 1:08:00 (h:mm:ss)] improved between the 2 attempts by 9% and 15% respectively (P ≤ 0.001). Total scores of all surgeons in the non-proctored subgroup (6) improved by 15% on average (mean: attempt 1 = 86, attempt 2 = 105, P = 0.002). CONCLUSIONS Procedure-specific assessment tools can be developed to evaluate technical performance for complex CHS simulation and be performed reliably by non-expert raters. Rehearsal both under supervision and independently leads to technical skill improvement further supporting its value in CHS training.

Objective Data supporting the use of hands-on simulation in congenital heart surgery are promising but primarily qualitative. This study aimed to demonstrate if there was an objective improvement in time and technical performance of the arterial switch procedure on 3-dimensional printed heart models by surgeons using a validated assessment method. Methods A total of 30 surgeons of varying experience performed the arterial switch procedure twice on 3-dimensional printed models with transposition of the great arteries during the Hands-on Surgical Training courses. Surgeons' performances were recorded and retrospectively assessed for both time and performance using the Hands-on Surgical Training-Congenital Heart Surgery tool, a validated procedure-specific assessment tool for the arterial switch. Results A total of 60 videos were scored. Eighty percent of surgeons (24/30) had improved from their first attempt. The mean total score of the first attempt performance compared with the second was 103 and 120, respectively, with a mean difference in score of 17 (95% confidence interval, 10-24). All surgeons were statistically significantly quicker in their second attempt. The mean time for the first attempt compared with the second was 1 hour, 28 minutes, 4 seconds and 1 hour, 5 minutes, and 45 seconds, respectively, with a mean difference of 0 hours, 22 minutes, 19 seconds (95% confidence interval, 0 hours, 15 minutes, 22 seconds to 0 hours, 25 minutes, 34 seconds). Conclusions This is the first study to demonstrate an objective improvement in time and technical performance of the arterial switch procedure on 3-dimensional printed heart models. This supports the evidence that simulation in the form of deliberate practice with constructive, objective feedback is fundamental in the training of future congenital heart surgeons. These simulations and assessments should be incorporated to create structured, standardized training curricula within congenital heart surgery.

Background Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. Objectives This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission). Methods The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression. Results Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index. Conclusions In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.

Congenital heart surgery is a technically demanding specialty resulting in a prolonged training period. With the growing expectation of perfect patient outcomes, there is a need for improved training methods by implementing simulation. We assess the utilization of simulation in the training of congenital heart surgeons and discuss its future implications. A keyword-based PubMed literature search was conducted for hands-on surgical simulation in congenital heart surgery. The abstracts/titles of the search were reviewed and papers using simulation specific to congenital cardiac surgery were selected. Studies that did not include surgeons operating on the simulator, or did not incorporate assessment methods were excluded. Analysis included the problem addressed, simulator-type, methodology, assessment methods, results, benefits/limitations, and reproducibility. Five papers fulfilled our selection criteria of hands-on surgical simulation in congenital heart surgery with an assessment of the simulator or procedural performance. One simulation used animal models and 4 utilized 3D-printed models. Simulators covered either single or multiple complex procedures. All studies highlight usefulness of simulation; however, only 1 study has been replicated with >10 participants. The studies demonstrate how hands-on surgical simulation is possible within congenital heart surgery. Although primarily proof of concept studies, the next step would involve using a greater number of participants and demonstrate how repetition and deliberate practice will improve outcomes. Congenital heart surgery is one of the most technically demanding surgical specialties; therefore, we should lead the way in utilizing simulation to complement the training of our surgeons as we face the challenges ahead.

No area of congenital heart disease has undergone greater change and innovation than single ventricle management over the past 20 years. Surgical and catheterisation laboratory interventions have transformed outcomes such that in some subgroups more than 80% of the patients can survive into adulthood. Driven by parallel development in diagnostic imaging and cardiac intensive care, surgical management is focused on the neonatal period as the key time to creating a balanced circulation and limiting pulmonary blood flow. Different configurations of the circulation, including new types of surgical shunts, and the role of "hybrid" circulations provide greater options and better physiology. This overview focuses on these changes in surgical management and timing, but also looks at the exciting areas of regenerative therapies to improve ventricular function, and the concept of ventricular rehabilitation to achieve biventricular circulations in certain groups of patients. The importance of early (neonatal) intervention and multidisciplinary approach to management is emphasised, as well as looking beyond simply survival to also improving neurodevelopmental outcomes.

The recent demonstration of normal development of preterm sheep in an artificial extrauterine environment has renewed interest in artificial placenta (AP) systems as a potential treatment strategy for extremely preterm human infants. However, the feasibility of translating this technology to the human preterm infant remains unknown. Here we report the support of 13 preterm fetal pigs delivered at 102±4 days (d) gestation, weighing 616±139g with a circuit consisting of an oxygenator and a centrifugal pump, comparing these results with our previously reported pumpless circuit (n=12; 98±4d; 743±350g). The umbilical vessels were cannulated, and fetuses were supported for 46.4±46.8 hours using the pumped AP versus 11±13 hours on the pumpless AP circuit. Upon initiation of AP support on the pumped system, we observed supraphysiologic circuit flows, tachycardia, and hypertension, while animals maintained on a pumpless AP circuit exhibited subphysiologic flows. On the pumped AP circuit, there was a progressive decline in UV flow and oxygen delivery. We conclude that the addition of a centrifugal pump to the AP circuit improves survival of preterm pigs by augmenting UV flow through the reduction of right ventricular afterload. However, we continued to observe the development of heart failure within a matter of days.

Background COVID-19 and some anti-SARS-CoV-2 vaccines trigger a humoral autoimmune response against a broad range of endogenous components, which may affect recipients' prognosis in predisposed individuals. Autoantibodies directed against apolipoprotein A-1 (AAA1 IgG) the major protein fraction of High Density Lipoprotein have been shown to be raised in COVID-19 and in rheumatoid arthritis (RA) patients and other populations where they have been associated with poorer outcomes. We wanted to assess the impact of anti-SARS-CoV-2 mRNA-based vaccination on AAA1 autoimmune biomarkers in RA patients. Methods 20 healthy controls and 77 RA mRNA-based vaccinated patients were collected at baseline, 3 weeks after the first vaccination, 2 and 8 weeks after the second vaccination. AAA1 and SARS-CoV-2 serologies were measured by immunoassays. Systemic and local symptoms occurring during the vaccination protocol were recorded. Results mRNA-based vaccination induced a significant increase in median AAA1 IgG levels in both healthy controls and RA patients overtime. However, in both populations, these medians trend did not translate into significant increase in AAA1 IgG seropositivity rates despite evolving from 5 to 10% in healthy controls, and from 9 to 12.9% in RA patients. No associations were retrieved between AAA1 IgG and symptoms of any kind during the vaccination protocol. Conclusions mRNA-based vaccination seems to induce a light AAA1 IgG response in immunocompetent individuals within 2 months after the last injection. Although we did not observe any warning signs, the formal demonstration of the harmlessness of such biological warrants further studies.

Introduction: Favorable survivorship after pediatric extracorporeal cardiopulmonary resuscitation (ECPR) may be limited by prolonged resuscitations. Surgical cannulation metrics for pediatric ECPR have not been widely reported by centers that use time interval benchmarks with a cardiovascular service responding to different hospital locations. Hypothesis: We hypothesize that survival is associated with resuscitation duration, and cannulation duration differs between peripheral and central approaches. Methods: This was a single-center retrospective study of patients 0-18 years with in-hospital ECPR between January 2015 and December 2020. Primary outcome was survival to hospital discharge. Secondary outcomes were odds of favorable neurologic outcome (dichotomized pediatric cerebral performance category), total resuscitation duration defined as cardiac arrest start to ECMO flow start (CA-ECMO), and cannulation duration. Non-parametric and regression methods were used. Results: Of the 92 events that met ECPR criteria, median weight and age were 4 months (IQR 1 month, 16 years) and 4.4 kg (range 1.9-133 kg). Cannulation occurred in the cardiac intensive care unit (ICU) (66%, 61 of 92), followed by operating room (13%, 12 of 92), pediatric ICU (12%, 11 of 92), and catheterization lab (9%, 8 of 92). Central cannulation was performed in 43% (40 of 92), and 21% (19 of 92) had open chests at the time of the event. Median duration of CA-ECMO was 35 min (IQR 26, 45 min); cannulation duration was 11 min (IQR 5, 16.5 min) for central compared to 18.5 min (IQR 12, 23 min) for peripheral approaches (P=0.01). Survival was 40% (37 of 92), and favorable neurologic outcome occurred in 38% (35 of 92). Survival (adjusted OR, 0.94; 95% CI 0.91-0.99, P=0.018) and favorable neurologic outcome (adjusted OR, 0.95; 95% CI 0.917-1.000, P=0.053) were associated with CA-ECMO duration after adjusting for cannulation approach, location, difficulty, shockable rhythm, and weight. Conclusion: In pediatric in-hospital ECPR, total CA-ECMO duration remains a key metric associated with patient outcomes. Central cannulation is faster than peripheral approaches. Since cannulation strategy alters CPR maneuvers, CPR effectiveness with each approach needs further study.

Introduction: Guidelines recommend dosing Epinephrine (Epi) at regular intervals during pediatric cardiac arrest, including patients requiring extracorporeal membrane oxygenation (ECMO). The impact of Epi-induced vasoconstriction on systemic afterload and veno-arterial ECMO support is poorly understood. Hypothesis: Higher total dose of Epi and shorter interval between Epi dose and ECMO flow during cardiac arrest will increase systemic afterload and interfere with ECMO support. Methods: This is an ancillary study to a single-center, retrospective observational study of patients 0-18 years old who required ECMO cannulation during resuscitation over a six-year period. Patients were excluded if ECMO was initiated prior to arrest or if the resuscitation record was incomplete. The primary exposure was time from last dose of Epi to initiation of ECMO flows; secondary exposures included cumulative Epi dose delivered and indexed to arrest time. Mean arterial pressure (MAP) and systemic vasodilator therapy were used as surrogates for systemic afterload; ECMO pump speed and vasoactive-inotrope score (VIS) were used as measures of ECMO support. Results: A total 92 events in 87 patients analyzed. The patient cohort was 53% female with median (IQR) age of 122 (30-478) days, weight 4.4 (3.3 - 8.7) kg, and 43% single ventricle physiology. On average, Epi was given 7 (4 - 10) times during a 35 (27 - 44) min arrest, for a total dose of 65 (37 - 101) mcg/kg; the last dose was given 6 (2 -16) min prior to the initiation of ECMO flows. In the 6 hours following initiation of ECMO, MAP increased from 42 (36 - 56) mmHg to 57 (47 - 70) mmHg, (p Conclusion: There is limited evidence to support that regular dosing of Epi throughout a cardiac arrest is associated with clinically significant increases in afterload after ECMO cannulation. Additional studies are needed to validate findings against ECMO flows and clinically relevant outcomes.

The safety of hemispherectomy between staged cardiac procedures is unknown and not previously reported. Retrospective review of a case with drug-resistant epilepsy due to stroke following bidirectional cavopulmonary connection (BDCPC). This report describes the first case of a successful pediatric peri-insular functional hemispherectomy in the setting of a BDCPC. A discussion of the complex preoperative planning from both a cardiac and neurological perspective is presented. Considerations regarding hemispherectomy and its effects on the cardiac physiology, and perioperative considerations are emphasized in clinical decision making. A multidisciplinary approach was critical in this child which led to a successful outcome.

We hypothesized that preoperative patient characteristics and branch pulmonary artery (PA) size might influence the rate of postoperative branch PA reintervention in patients with transposition of the great arteries who undergo the arterial switch operation (ASO).The retrospective single-center study included 262 consecutive (2008-2017) newborns who underwent the ASO. Demographic characteristics, echocardiography, and clinical outcomes were reviewed. Competing risk analysis modeled incidence of branch PA reintervention and cause-specific hazard regression for predictors analyses.Median age and weight were 7 (range, 5-11) days and 3.4 (range, 3.1-3.8) kg, respectively. Various types of early branch PA reinterventions (concomitant revision or reintervention during the intensive care unit stay) were required in 28 (10.7%) patients. These patients had prolonged ventilation (P .001), intensive care unit duration (P .001), worse right ventricular function (P = .043), and high in-hospital mortality (P = .010). Branch PA dimensions significantly decreased immediately after ASO compared with baseline measurements. The median follow-up duration was 20.8 (range, 0.9-44.7) months. Branch PA reintervention was common among survivors without early reinterventions (9.4%), and even more frequent among those with early reinterventions (25%). Subsequent reintervention (all catheter-based) was necessary for more than one-third of patients after initial branch PA reintervention. The multivariable analysis showed preoperative dimension of the left PA (hazard ratio, 0.527 [95% CI, 0.337-0.823]; P = .005), and right PA (hazard ratio, 0.503 [95% CI, 0.318-0.796]; P = .003) were independently associated with late branch PA reinterventions.Branch PA reintervention was common and often required surgical or catheter-based reinterventions after ASO. PA branch diameters became significantly smaller after ASO. Smaller preoperative branch PA predicted late branch PA reintervention, indicating a smaller margin of geometrical tolerance to this effect.

BACKGROUND We aimed to examine the rationale and assess risk of additional cardiopulmonary bypass (CPB) episodes, and specifically, re-institution of CPB for reintervention, in congenital heart surgery. METHODS AND RESULTS We retrospectively reviewed all children undergoing cardiac surgery with CPB at one institution (2014-2016). Tetralogy of Fallot was excluded as the annulus-sparing surgical strategy involves planned intraoperative revision. The final cohort of 922 patients was stratified: single CPB run (CPB=1, n=771); >1CPB without revisions (CPB>1NR, n=77) for reperfusion, bleeding, or intractable arrhythmias; and >1CPB with revision (CPB>1R, n=74). Successful revisions were defined as: those resulting in at most mild residual lesions, did not induce new lesions, did not require postoperative revision, and did not result in mortality prior to discharge. Forced revisions were defined as revisions for unacceptable hemodynamics or failure to wean from CPB. Outcomes of interest were in-hospital mortality, complications, need for postoperative revision, and a composite (death, stroke, ECMO, arrest, and leaving OR with open chest). Predictors of the composite outcome were assessed by logistic regression. Mean age was 2.9±4.5 years, 54% were male and mean RACHS was 2.5±0.8. In CPB>1R, 18 (24%) required 2 or 3 revisions. Revisions were valvular in 34 (46%) and forced in 51 (69%) patients. Success with a single revision occurred in 22 (30%) and by the final attempt in 32 (43%) patients. One patient's lesion worsened as a result of the revision. When comparing CPB=1 vs CPB>1NR vs CPB>1R, outcomes with significant differences included: stroke, arrest, postoperative revision, open chest upon leaving OR, and the composite outcome (Table 1). In-hospital death, re-exploration, need for ECMO, and hospital stay were similar (Table 1). Among CPB>1R patients, 12 (16%) required postoperative revision. Logistic regression identified revision as a predictor of the composite outcome with greatest effect seen in 2 revisions: 1: OR=2.39 (95% CI: 1.35, 4.21) p=0.002, 2: OR=3.68 (95% CI: 1.26, 10.74) p=0.02, 3: OR=1.23 (95% CI: 0.25, 12.23) p=0.8. Additionally, CPB runs=3 was associated with the composite outcome: OR=2.25 (95% CI: 1.00, 5.05) p=0.048. A trend towards CPB runs = 4 was seen while CPB runs=2 was not correlated with the composite outcome. CONCLUSION Revisions are uncommon, and most are forced. Revisions frequently do not eliminate residual lesions, occasionally require additional postoperative revisions, but are not associated with increased early mortality. Revisions and >2 CPB runs were associated with greater risk of the composite outcome. Those for non-forced reasons should likely be considered with caution.

KEY POINTS The margin of human viability has extended to the extremes of gestational age (

Right ventricular (RV) failure is a commonly encountered problem in patients with congenital heart disease but can also be a consequence of left ventricular disease, primary pulmonary hypertension, or RV-specific cardiomyopathies. Improved survival of the aforementioned pathologies has led to increasing numbers of patients suffering from RV dysfunction, making it a key contributor to morbidity and mortality in this population. Currently available therapies for heart failure were developed for the left ventricle (LV), and there is clear evidence that LV-specific strategies are insufficient or inadequate for the RV. New therapeutic strategies are needed to address this growing clinical problem, and stem cells show significant promise. However, to properly evaluate the prospects of a potential stem cell-based therapy for RV failure, one needs to understand the unique pathophysiology of RV dysfunction and carefully consider available data from animal models and human clinical trials. In this review, we provide a comprehensive overview of the molecular mechanisms involved in RV failure such as hypertrophy, fibrosis, inflammation, changes in energy metabolism, calcium handling, decreasing RV contractility, and apoptosis. We also summarize the available preclinical and clinical experience with RV-specific stem cell therapies, covering the broad spectrum of stem cell sources used to date. We describe two different scientific rationales for stem cell transplantation, one of which seeks to add contractile units to the failing myocardium, while the other aims to augment endogenous repair mechanisms and/or attenuate harmful remodeling. We emphasize the limitations and challenges of regenerative strategies, but also highlight the characteristics of the failing RV myocardium that make it a promising target for stem cell therapy.

The monthly in-house Hands-On Surgical Training (HOST) program was incorporated into the congenital heart surgery (CHS) curriculum for surgical trainees within our institution. This study evaluated whether there was an improvement and retention of technical skills throughout the curriculum via objective assessment methods.Twelve 3-dimensional-printed surgical heart models were included into the year-long curriculum. The monthly sessions were attended by all trainees and staff surgeons. Proctors demonstrated the operation on a model, which was followed by 2 attempts by each trainee. Attempts were recorded for objective assessment. On completion of the curriculum trainees repeated 4 procedures an additional 2 times after a delay to assess skill retention.Twelve sessions were completed by 7 trainees within the curriculum. Objective assessments were performed in 7 sessions. Eighty-one percent of trainees' scores improved between the 2 attempts, with a mean improvement of 13% (attempt 1: HOST-CHS score of 79, attempt 2: HOST-CHS score of 89; P .001). Similarly, 91% of procedural times improved by a mean of 25% (attempt 1, 1:22:00 [hours:minutes:seconds]; attempt 2, 1:01:21; P.001). During individual procedure analysis, statistical significance remained in 3 of 7 procedures (P.05). Four procedures were assessed for skill retention after a delay (2-14 months). Scores decreased by 4% in 47% of trainees during attempt 3 (attempt 2: HOST-CHS score of 94, attempt 3: HOST-CHS score of 91; P = .34) but improved in 79% during attempt 4 (attempt 3: HOST-CHS score of 91, attempt 4: HOST-CHS score of 99; P = .004), matching their previous performance.The monthly HOST course was successfully incorporated into a training curriculum for CHS surgeons using objective assessments to measure technical performance. Trainees demonstrated an improvement across all evaluated procedures and retained their skills when reassessed after a delay highlighting its value in CHS training.

Objectives: To review and compare the anesthetic management of atrial septal defect (ASD) closures via mini lateral thoracotomy and sternotomy approaches. Design: Retrospective analysis. Setting: Single- center pediatric quaternary care hospital. Participants: Patients aged Intervention: None. Measurements and Main Results: Outcome measures included anesthetic technique, total amount and type of analgesics, pain scores, procedure duration, complications, blood transfusion requirements, and duration of stay. Each group had 15 patients. All patients in the sternotomy group received 0.25% bupivacaine subcutaneous infiltration. Eleven of the 15 thoracotomy patients received a fascial plane block, including serratus anterior and erector spinae blocks, and 3 received subcutaneous infiltration. There was no difference in opioid consumption intraoperatively or in the first 24 hours after surgery (0.28 ± 0.24 mg/kg morphine equivalents in thoracotomy group and 0.21 ± 0.12 mg/kg in sternotomy group). Duration of procedure and cardiopulmonary bypass duration were longer in the thoracotomy group. There was no difference in cross-clamp duration between groups. There was no difference in intensive care unit or hospital stay. Conclusions: The authors reviewed perioperative pain management strategies used in surgical ASD closures. Different fascial plane blocks were used. This study has paved way to design a randomized control trial to compare various regional techniques for cardiac surgeries and identified opportunities for improved pain assessment scoring in children after cardiac surgery.

OBJECTIVES Cerebral protection is a major issue in the treatment of infants with complex congenital heart disease. We tested a new device combining tissue spectrometry and laser Doppler flowmetry for non-invasive determination of cerebral oxygen metabolism following cardiac surgery in infants. METHODS We prospectively measured regional cerebral oxygen saturation cSO 2 and microperfusion (rcFlow) in 43 infants 12-24 h following corrective ( n = 30) or palliative surgery ( n = 13) of congenital heart defects. For comparison, cerebral blood flow (CBF) was determined by colour duplex sonography of the extracranial cerebral arteries. Cerebral fractional tissue oxygen extraction, approximated cerebral metabolic rate of oxygen (aCMRO 2 ) and cerebral metabolic rate of oxygen (CMRO 2 ) were calculated. RESULTS cSO 2 was lower [54.6% (35.7-64.0) vs 59.7% (44.5-81.7); P

Neonates with a systemic-to-pulmonary shunt often experience hemodynamic instability due to pulmonary overcirculation and resultant systemic malperfusion. Herein, we describe a technique of shunt banding, a reproducible method to mechanically limit the blood flow through the systemic-to-pulmonary shunt. A mathematical rationale, the surgical techniques, postoperative management, and our clinical experiences are described.

Graphical abstract, Highlights • PA-MAIVF is a rare condition that is usually asymptomatic. • Symptoms related to PA-MAIVF generally represent complications. • Early identification of this entity is crucial to guide acute management strategies.

The purpose of this review is to survey the contemporary literature surrounding congenital anomalies of origin of the coronary arteries and to identify remaining gaps in knowledge.In recent years, lineage tracing analyses and mechanistic studies in model organisms have enhanced our understanding of the normal embryologic development of the coronary arteries, and how disruption of this intricate process can lead to congenital coronary anomalies. The true incidence of these anomalies remains unknown. Although a majority of cases are believed to be clinically silent, clinical presentation varies widely, from asymptomatic to sudden cardiac death. Cardiac computed tomography angiography and/or magnetic resonance angiography are the mainstay diagnostic modalities. Management of anomalous coronary arteries depends on the morphology and clinical presentation. Surgery is the gold-standard treatment for anomalous left coronary artery arising from the pulmonary artery and anomalous aortic origin of a coronary artery with intramural or interarterial course.Several large multicenter initiatives are currently underway and should help address some of the numerous knowledge gaps surrounding the evaluation and management of anomalous coronary arteries.