Repair Of Ventricular Septal Defect Research Articles

Successful Repair of Congenital Morgagni Hernia and Ventricular Septal Defect via Median Sternotomy in a Patient with Down Syndrome, Anal Atresia, Pectus Carinatum and Congenital Hypothyroidism

Successful Repair of Congenital Morgagni Hernia and Ventricular Septal Defect via Median Sternotomy in a Patient with Down Syndrome, Anal Atresia, Pectus Carinatum and Congenital Hypothyroidism

Evaluating variation in pre-operative evaluation and planning for children undergoing atrial or ventricular septal defect repair.

CHD care is resource-intensive. Unwarranted variation in care may increase cost and result in poorer health outcomes. We hypothesise that process variation exists within the pre-operative evaluation and planning process for children undergoing repair of atrial septal defect or ventricular septal defect and that substantial variation occurs in a small number of care points. From interviews with staff of an integrated congenital heart centre, an initial process map was constructed. A retrospective chart review of patients with isolated surgical atrial septal defect and ventricular septal defect repair from 7/1/2018 through 11/1/2020 informed revisions of the process map. The map was assessed for points of consistency and variability. Thirty-two surgical atrial septal defect/ventricular septal defect repair patients were identified. Ten (31%) were reviewed by interventional cardiology before surgical review. Of these, 6(60%) had a failed catheter-based closure and 4 (40%) were deemed inappropriate for catheter-based closure. Thirty (94%) were reviewed in case conference, all attended surgical clinic, and none were admitted prior to surgery. The process map from interviews alone identified surgery rescheduling as a point of major variability; however, chart review revealed this was not as prominent a source of variability as pre-operative interventional cardiology review. Significant variation in the pre-operative evaluation and planning process for surgical atrial septal defect/ventricular septal defect patients was identified. If such process variation is widespread through CHD care, it may contribute to variations in outcome and cost previously documented within CHD surgery. Future research will focus on determining whether the variation is warranted or unwarranted, associated health outcomes and cost variation attributed to these variations in care processes.

Postmyocardial Infarction Ventricular Septal Defect Repair With Perioperative Impella 5.5 Support

Postmyocardial Infarction Ventricular Septal Defect Repair With Perioperative Impella 5.5 Support

Staged definitive repair for pulmonary atresia and ventricular septal defect 40 years after palliative surgery: a case report

We present a very rare case of pulmonary atresia and ventricular septal defect with staged definitive repair more than 40 years after palliative surgery. The patient, a 43-year-old male, had undergone a Waterston operation at the age of one and had been untreated since then. Two years ago, he underwent an urgent surgery for impending rupture of a huge pulmonary artery aneurysm. Then, after evaluation of cardiac and pulmonary functions, a definitive repair was performed concomitantly with aortic root replacement for the dilated aortic root. He was discharged uneventfully and received ambulatory care 5 years after surgery.

Reduced length of stay after implementation of a clinical pathway following repair of ventricular septal defect.

There is variation in care and hospital length of stay following surgical repair of ventricular septal defects. The use of clinical pathways in a variety of paediatric care settings has been shown to reduce practice variability and overall length of stay without increasing the rate of adverse events. A clinical pathway was created and used to guide care following surgical repair of ventricular septal defects. A retrospective review was done to compare patients two years prior and three years after the pathway was implemented. There were 23 pre-pathway patients and 25 pathway patients. Demographic characteristics were similar between groups. Univariate analysis demonstrated a significantly shorter time to initiation of enteral intake in the pathway patients (median time to first enteral intake after cardiac ICU admission was 360 minutes in pre-pathway patients and 180 minutes in pathway patients, p < 0.01). Multivariate regression analyses demonstrated that the pathway use was independently associated with a decrease in time to first enteral intake (-203 minutes), hospital length of stay (-23.1 hours), and cardiac ICU length of stay (-20.5 hours). No adverse events were associated with the use of the pathway, including mortality, reintubation rate, acute kidney injury, increased bleeding from chest tube, or readmissions. The use of the clinical pathway improved time to initiation of enteral intake and decreased length of hospital stay. Surgery-specific pathways may decrease variability in care while also improving quality metrics.

PO-01-106 ATRIAL ISOCHRONAL LATE ACTIVATION MAPPING KEEPS THE DIAPHRAGM ALIVE

Isochronal late activation mapping (ILAM) is a well-established mapping method for ventricular arrhythmias. However, the role of ILAM mapping during atrial arrhythmias is less clear. This case provides an example of ILAM use during right atrial flutter ablation using the high-density Optrell (Biosense Webster, CARTO) catheter in a patient with congenital heart disease. N/A N/A A 27-year-old female with D-transposition of the great arteries status post Nikaidoh (arterial switch operation with ventricular septal defect repair, pulmonary valve replacement, and typical atrial flutter status post ablation presented with SVT and was referred for catheter ablation. During EPS, conventional activation mapping of fleeting atrial flutter was attempted utilizing the Optrell catheter, which localized the region of interest to a large section of the inferior posterolateral RA, which also had diffuse phrenic nerve capture (figure 1, panel A). However, during periods of sinus rhythm, ILAM and voltage mapping was conducted that demonstrated a distinct, confined, region of isochronal crowding along the mid-posterolateral right atrium, distant from the phrenic nerve (figure 1, panel B). The ILAM site was addressed with catheter ablation rendering the atrial flutter non-inducible with preservation of phrenic nerve function. The patient has remained free of atrial arrhythmias in the 6 months period since the ablation. The application of ILAM is well-established for ventricular tachycardia, however, its role in atrial arrhythmias is less clear. ILAM may be especially useful in adults with congenital heart disease given the complexity of atrial arrhythmia and the benefit of ablation in this unique population. Novel high-density mapping catheters, such as Optrell, allow for rapid and granular voltage data and translate well to ILAM regions within the atrium. Application of ILAM within atrial substrate, particularly for congenital heart disease which predisposes to higher recurrence rates, may improve both recurrence rates and safety, like in this case where the use of this method allowed avoiding ablation on the phrenic nerve.

Ventricular Septal Defect Exposure by Tricuspid Valve Chordal Detachment-A Retrospective Matched Study.

Background: Transatrial approach is the standard method in repairing ventricular septal defects (VSD) in the pediatric population. However, the tricuspid valve (TV) apparatus might obscure the inferior border of the VSD risking the adequacy of repair by leaving residual VSD or heart block. Detachment of the TV chordae has been described as an alternative technique to TV leaflet detachment. The aim of this study is to investigate the safety of such a technique. Methods: Retrospective review of patients who underwent VSD repair between 2015 and 2018. Group A (n = 25) had VSD repair with TV chordae detachment were matched for age and weight to group B (n = 25) without tricuspid chordal or leaflet detachment. Electrocardiogram (ECG) and echocardiogram at discharge and at 3 years of follow-up were reviewed to identify new ECG changes, residual VSD, and TV regurgitation. Results: Median ages in groups A and B were 6.13 (IQR 4.33-7.91) and 6.33 (4.77-7.2) months. New onset right bundle branch block (RBBB) was diagnosed at discharge in 28% (n = 7) of group A versus 56% (n = 14) in group B (P = .044), while the incidence dropped to 16% (n = 4) in group A versus 40% (n = 10) in group B (P = .059) in the 3 years follow-up ECG. Echocardiogram at discharge showed moderate tricuspid regurgitation in 16% (n = 4) in group A and 12% (n = 3) in group B (P = .867). Three years of follow-up echocardiography revealed no moderate or severe tricuspid regurgitation and no significant residual VSD in either group. Conclusion: No significant difference in operative time was observed between the two techniques. TV chordal detachment technique reduces the incidence of postoperative RBBB without increasing the incidence of TV regurgitation at discharge.

Evaluation of health-related quality of life in children with postoperative delirium after surgical repair of ventricular septal defect: short- and mid-term follow-up

BackgroundTo investigate health-related quality of life (HRQOL) in children with postoperative delirium (POD) after surgical repair of ventricular septal defects (VSDs).MethodsA total of 109 patients were enrolled and assigned to the POD group (n = 47) and the non-POD group (n = 62). HRQOL was assessed by the PedsQLTM 3.0 Cardiac Module at discharge, and at the three- and six-month postoperative follow.ResultsSignificant differences were detected in age, operation time, CPB time, mechanical ventilation duration, and length of ICU stay between the two groups, whereas sex and the VSD size did not significantly differ between groups. In terms of “cardiac heart problems and treatment” and “treatment-II”, the HRQOL scores of the non-POD group were significantly better than those of the POD group. In terms of “perceived physical appearance” and “treatment anxiety”, the POD group had significantly higher scores than the non-POD group at discharge and at the three-month postoperative follow-up. In terms of “cognitive problems” and “communication”, the occurrence of POD still affected HRQOL at three months postoperatively, but the effect was significantly reduced at six months postoperatively. In terms of “total scores”, both groups scored increasingly higher over time. The non-POD group had higher scores at discharge and three months postoperatively than the POD group, but no significant difference persisted at six months postoperatively.ConclusionDuring the follow-up period, the HRQOL of the children with POD after surgical repair of VSD was inferior to that of the children without POD at discharge and three months postoperatively. However, the HRQOL did not differ between the two groups at six months postoperatively.

Fate of aortic regurgitation after isolated repair of ventricular septal defect with concomitant aortic regurgitation in children

ObjectivesThe aim of the study was to evaluate the course of aortic valve regurgitation in patients with preoperative aortic valve regurgitation and ventricular septal defect who underwent repair of the ventricular septal defect without aortic valve repair. MethodsA total of 37 consecutive patients with a ventricular septal defect and aortic regurgitation who underwent surgery between April 2007 and March 2016 were included in the study. Demographic, echocardiographic, operative, and clinical data were reviewed. Early and late mortality and morbidity were analyzed. Aortic regurgitation grade, left ventricular function, and dimensions were compared between the preoperative transesophageal echocardiography and postoperative transthoracic echocardiogram at last follow-up. Multivariate logistic regression analysis was performed to determine factors associated with improvement of aortic valve function. ResultsThere was no early or late mortality. No reoperations or reinterventions were required. A total of 17 patients had mild or greater aortic regurgitation preoperatively. Only 5 patients had mild or greater aortic regurgitation at follow-up of 4.3 years (0.5-10.1). Twenty-eight (76%) of the 37 patients showed an improvement in their aortic regurgitation grade. Left ventricular end-systolic and end-diastolic diameter z-scores were significantly lower at follow-up (P = .007 and P = .001, respectively). Multivariable logistic regression identified low preoperative left ventricular ejection fraction as the only predictor of nonimprovement of aortic regurgitation (95% confidence interval, 0.732-0.999, P = .002). ConclusionsRepair of a ventricular septal defect with accompanying aortic regurgitation can be performed with excellent results without surgical intervention on the aortic valve. Accompanying aortic regurgitation, especially trivial to mild, at the time of ventricular septal defect repair improves in the majority of cases. Low preoperative left ventricular ejection fraction is predictive of nonimprovement of aortic regurgitation grade.

A comparison of the changes in serum lactate between surgical repair and transthoracic device closure of ventricular septal defects in pediatric patients.

The purpose of this study was to compare the changes in serum lactate between surgical repair and transthoracic device closure of ventricular septal defects (VSDs) in pediatric patients. This study was a retrospective analysis, and 314 pediatric patients with simple VSD from October 2019 to October 2021 were selected. The patients were divided into the S group (surgical repair) and the D group (transthoracic device closure). The serum lactate value at ICU admission and 6 h after operation, as well as the highest serum lactate value were collected, and the 6-h serum lactate clearance rate was calculated. Through propensity score matching, 43 pairs of cases were successfully matched. Compared with the S group, the D group had a shorter operation duration, ventilation duration, and ICU duration, as well as a lower drainage volume and total hospitalization cost. There was no significant difference between the two groups in the initial and highest serum lactate values after VSD closure, while the 6-h serum lactate value in the D group was significantly lower than that in the S group, and the 6-h serum lactate clearance rate in the D group was five times faster than that in the S group. In addition, the 6-h serum lactate clearance rate in the S group was mainly related to the operation time, CPB time, and ventilation time, while the 6-h serum lactate clearance rate in the D group was only related to the operation time. The initial and highest serum lactate levels were not significantly different between surgical repair and transthoracic device closure of VSD, but the 6-h serum lactate clearance rate of device closure was five times faster than that of surgical repair.

Reduced Length of Stay after Implementation of a Clinical Pathway following Repair of Ventricular Septal Defect

There is variation in care and hospital length of stay following surgical repair of ventricular septal defects. The use of clinical pathways in a variety of pediatric care settings have been shown to reduce practice variability and overall length of stay without increasing the rate of adverse events.

Impella Support for Surgical Ventricular Septal Defect Repair.

Acute postinfarct ventricular septal defect (VSD) is associated with high mortality due to a combination of cardiogenic shock and a complex repair in recently infarcted fragile myocardium. 1 Although the Impella heart pump is established as support for cardiogenic shock, it is relatively contraindicated in postinfarct VSD because of potential right-to-left shunt or stroke due to VSD tissue-related embolus. On autopsy, early repair is technically difficult due to tissue friability and as a result, 38% of surgically repaired patients have evidence of recurrent interventricular septal rupture. 2 Delayed surgical repair (>7 days) is associated with superior survival-54% after 7 days versus 18% prior-but hemodynamic instability may prevent delay. 3 Case reports have shown successful early left ventricular unloading with Impella patients with acute postinfarct VSD before surgical repair. 4,5 We discuss our algorithm for pre-repair Impella support in which we stratify pre-repair support based on the Qp/Qs ratio. For VSD with Qp/Qs >2.5, we use a preoperative Impella heart pump and have not demonstrated reversal in the left-to-right shunt on echocardiography and/or stroke. Our findings are consistent with theoretical models of unloading as demonstrated by shifts in pressure-volume loops. 6.

Routine Detachment of the Anterior and Septal Tricuspid Leaflets Simplifies VSD Closure and Improves the Outcomes.

Background and Objectives: The closure of perimembranous ventricular septal defects (VSDs) is one of the most common surgeries performed in infancy. The technique of detachment of the anterior and septal leaflets of the tricuspid valve (TV) with subsequent leaflet augmentation is frequently used for isolated as well as non-isolated VSD closure. In this study, we compared the incidence of tricuspid regurgitation (TR) in patients who underwent a VSD repair with and without detachment of the TV in the short- and long-term follow-up. Materials and Methods: A retrospective study that included 140 patients who underwent perimembranous VSD closure at our center from 2011-2016, where 102 of these patients underwent the procedure with detachment of the TV, was performed. The follow-up data were obtained from postoperative echocardiography performed in the follow-up visits. A total of 62 patients underwent follow-up at our center, where the follow-up time ranged from 1 to 9 years, with a mean of 71 ± 2.47 months. Results: Regarding patients who underwent a VSD repair with a detachment of the TV, 98.1% of the patients had none to mild TR, compared to 94.7% in patients without intraoperative TV detachment at the time of discharge. There were no reported cases of moderate to severe TR, atrioventricular blocks, aortic insufficiency, or deaths. A total of 98.1% of patients who underwent follow-up at our center with a TV detachment had none to mild TR compared to 94.7% in the group without TV detachment. Conclusion: TV detachment with leaflet augmentation for VSD closure is safe and effective and does not increase the incidence of TR in the short- and long-term follow-up.

Becoming a Teenager after Early Surgical Ventricular Septal Defect (VSD) Repair: Longitudinal Biopsychological Data on Mental Health and Maternal Involvement

Beside somatic strains of congenital heart diseases (CHD), affected children often show developmental impairments in the long term. Ventricular septal defect (VSD) is the most common congenital heart defect and early surgical repair is associated with positive somatic outcomes. However, psychological adjustment is of lifelong relevance. We investigated 24 children with a surgically-corrected isolated VSD and their mothers from primary school (6-9 years) to adolescence (10-14 years) and compared them to controls. Both times, mothers reported child internalizing/externalizing problems, mothers and children rated child quality of life, and children performed neurodevelopmental tests. Adolescents also rated internalizing/externalizing problems themselves, and their hair cortisol levels were analyzed. Maternal anxiety and proactive parenting behavior were considered as moderators. Results revealed no group differences in child neurodevelopment (language, cognition), externalizing problems, and cortisol levels at any time. In reports from mothers, internalizing problems (depression, anxiety) were elevated in children with a VSD at both times-when mothers reported anxiety symptoms themselves. In adolescent reports, VSD patients' quality of life was increased and internalizing problems were decreased-proactive parenting behavior went along with decreased symptoms in VSD-affected adolescents and with increased symptoms in controls. The findings pronounce the crucial role of parenting behavior and the influence of maternal anxieties on child mental health after surgical VSD repair and might highlight the need for parent-centered interventions.

Mid-term outcomes of right subaxillary approach versus median sternotomy incision for ventricular septal defect with patent ductus arteriosus

BackgroundThis study aimed to evaluate and compare two surgical approaches to repair ventricular septal defect (VSD) with patent ductus arteriosus (PDA) and to explore the patients’ health-related quality of life (HRQoL).MethodsWe conducted a retrospective study of all patients who had surgical repair of VSD and PDA between 2013 and 2015 using the right subaxillary approach (group A) or the median sternotomy incision (group B). The outcomes of both techniques were compared. Paediatric QoL Inventory 4.0 scale was applied to assess patients’ HRQoL in the 6th postoperative year. Multiple linear regression analysis was performed to explore factors associated with higher HRQoL scores.ResultsA total of 128 patients were included (group A, n = 70 and group B, n = 58). Patients in group A were older and heavier than patients in group B. In group B, the diameters of VSD and PDA were larger and the pulmonary artery pressures were higher than those in group A (p < 0.001). No mortality occurred on a mean follow-up of 8.3 ± 1.2 years. Patients in group A had higher HRQoL scores than those in group B in terms of emotional and social functioning dimensions. The right subaxillary approach (OR: 3.56; 95% CI 1.65–5.46), higher parents’ education level (OR: 1.62; 95% CI 0.65–2.31), and better family economic status (OR: 1.48; 95% CI 0.79–2.45) were associated with higher HRQoL scores.ConclusionsYounger and smaller patients receiving median sternotomy incisions due to large defects and pulmonary hypertension had lower HRQoL scores. The right subaxillary approach, higher parents’ education level, and better family economic status were associated with higher HRQoL scores.

Late Post-Operative Complete Heart Block after Ventricular Septal Defect Repair Procedure

In congenital cardiac surgery, intracardiac repair procedures like ventricular septal defect (VSD), atrioventricular septal defect and tetralogy of fallot (TOF) are associated with conduction system injury. Fewer cases are reported describing life threatening complication of late post-operative complete heart block. We are reporting 2 patients who presented with late complete heart block after ventricular septal defect repair and managed by permanent pacemaker placement in AFIC Cath lab.

Tricuspid valve detachment for ventricular septal defect closure: A meta-analysis of existing evidence.

Ventricular septal defect (VSD) is one of the most common congenital cardiac defects. However, in some cases, VSD sites are difficult to expose due to obstruction from chordal attachments and leaflets of the tricuspid valve (TV). To systematically review the efficacy and safety of tricuspid valve detachment, (TVD) versus conventional surgical repair (non-TVD) in the treatment of ventricular septal defect (VSD). This article is aimed to compare the many outcomes from existing studies and provide evidence regarding the necessity of performing TVD. We searched the following databases: PubMed via NCBI, the Cochrane Central Register of Controlled Trials (no date restriction), Medline via Ovid (from 1966 to May 2020); Embase via Ovid (no date restriction), and China National Knowledge Infrastructure for studies comparing the efficacy of TVD and other surgical techniques in VSD repair. Cardiopulmonary bypass time, Cross-clamp time; postoperative complications including residual defect, postoperative atrioventricular block, implantation of pacemakers, tricuspid regurgitation; length of stay (LOS), length of intensive care unit (ICU) stay were analyzed. Only nine studies were included after selection, including seven retrospective cohort studies, one respective cohort study, and one prospective observational study, a patient pool of 1404 patients with 374 underwent TVD and 1030 underwent non-TVD procedures, met the inclusion criteria. Meta analysis has drawn to the following conclusions. First, TVD prolongs CPB time (MD = 7.75, 95% confidence interval [CI] = 2.60-12.89, p = .003) and cross-clamp time (MD = 7.77, 95% CI = 4.76-10.78, p < .001) compared with non-TVD techniques in VSD repair surgeries. Second, no significant difference exists in LOS, length of ICU stay, postoperative atrioventricular block, implantation of pacemakers, incidence of ≥mild tricuspid valve regurgitation (TR) postoperatively and at discharge, as well as the incidence of ≥small residual VSD after surgery and during follow-up (all p ＞ .05). Third, application of TVD increases the risk of TR during follow-up (odds ratio [OR] = 2.42, 95% CI = 1.55-3.76, p < .001). VSD closure using TVD technique results in longer CPB and cross-clamp time, and increases risk of TR during follow-up. TVD provides equally viable and safe alternative in treating VSD.

Abstract 12999: Persistent Left Superior Vena Cava Draining Directly Into Left Atrium With Occluded Coronary Sinus: A Rare Incidental Finding

Case presentation: A 43-year-old lady with pacing induced cardiomyopathy presented for pacemaker (PM) revision to biventricular PM. Medical history included complete heart block with dual-chamber PM, supraventricular tachycardia, and ventricular septal defect repair. During the procedure, numerous unsuccessful attempts were made to access the right atrium (RA) using a deflectable catheter through the left subclavian vein. Left upper extremity venography exhibited a persistent left superior vena cava (PLSVC) suspected to be entering RA through the coronary sinus (CS). Navigation of the CS was unsuccessful. As a result, the procedure was aborted, and the patient was admitted for further cardiac imaging evaluation. Transesophageal echocardiography demonstrated an abnormal blood vessel between left atrial appendage and left upper pulmonary vein. Findings confirmed with agitated saline injection into left arm. This rang a concern for unroofed coronary sinus. Surprisingly, cardiac computed tomography angiograms and left upper extremity venogram images revealed a PLSVC draining into left atrium (LA) posteriorly. Normal anatomy of the right-sided superior vena cava (RSVC). Also, a CS atresia and coronary venous drainage to LA inferiorly. Discussion: PLSVC is a rare cardiac anomaly with estimated prevalence of 0.2% - 3% among general population. Commonly PLSVC drains into RA through expanded CS and rarely into LA through unroofed CS. Uniquely like our patient, the PLSVC drains directly into the LA with atretic CS and no interatrial communication. PLSVC is usually asymptomatic and detected incidentally during invasive cardiac procedures. Despite existence of right to left shunt, patient was maintaining normal oxygen saturation even after a treadmill stress test. Crowdedness of the RSVC with previously implanted leads is likely the reason for failed passage of the catheter to RA. Cardiac arrhythmias and paradoxical embolization were reported with PLSVC.

Effects of Bobath and Neurodevelopmental Treatment in A 3.5 Years Old Child with Lacunar Pure Motor Stroke Following Ventricular Septal Defect Repair

Lacunar pure motor stroke happens when an artery leading to the deep section of the brain, which contains the organs like the thalamus or basal ganglia is obstructed. Small and occurring outside of the cortex, lacunar strokes are a subtype of ischemic stroke. Lacunar strokes mostly affect patient’s memory, judgmental skills and language. On the basis of symptoms, lacunar stroke is categorized as pure motor hemiparesis (45% cases), pure sensory stroke (7% cases) and ataxic hemiparesis (17-18% cases). We reported a case of 3.5 years old male patient with pure motor lacunar stroke following ventricular septal defect repair who was effectively treated with bobath therapy and neurodevelopmental therapy as bobath is a generally accepted theory in the rehabilitation of hemiparetic stroke victims worldwide. On examination, the patient had a total pure motor based right sided hemiparesis. The lower extremity movements were more compromised than upper extremity, strength of mainly antigravity or postural muscles was compromised and lower extremity muscles were scoring 1/5 on Manual Muscle Testing (MMT) and scored 1 on Modified Ashworth Scale (MAS). As such there was no spasticity factor in the upper or lower limb muscles but there was weakness (right sided hemiparesis), so the treatment plan was given and thoroughly explained to the patient’s caretaker. There was no major cognitive and sensory deficit, as lacunar stroke has a better prognosis than other types of stroke, so, the recovery was good within 3 to 4 months by NDT and bobath therapy. Bobath therapy mainly improves the motor function with postural balance and stability.

Anomalous connection of the circumflex coronary artery to the pulmonary trunk in a patient with Taussig–Bing anomaly: a case report

Coronary anomalies are present in one-third of all patients with transposition of the great arteries (TGA) and have been associated with increased risk of adverse outcomes after the arterial switch operation. Therefore, knowledge about coronary anatomy remains key. A 5-day-old girl with prenatal diagnosis of Taussig-Bing anomaly (double outlet right ventricle with TGA and large subpulmonary ventricular septal defect) along with aortic arch hypoplasia and coarctation of the aorta underwent the arterial switch operation with closure of the ventricular septal defect and aortic arch repair. On preoperative echocardiography, the right (R) and left coronary artery (LCx) connected both to aortic sinus 1, suggesting 1RLCx coronary anatomy according to the Leiden Convention coronary coding system. However, intraoperative inspection led to a reclassification of the coronary anatomy: the right coronary artery and left anterior descending coronary artery connected to aortic sinus 1 (1RL) as had been observed on echocardiography, but-remarkably-the circumflex coronary artery (Cx) connected to the posterior sinus of the pulmonary trunk. As a consequence, cardioplegia was administered into both the aortic and pulmonary roots, and the circumflex coronary artery could stay in its native position without having to be transferred during the arterial switch operation. Various disruptions during embryological development can lead to unusual coronary anatomy in TGA patients. While anomalous connection of a coronary artery to the pulmonary trunk remains exceedingly rare, care should be taken to identify this pattern when present as failure to do so may result in adverse outcomes.