Impact of preoperative respiratory distress on outcomes of slide tracheoplasty.

OBJECTIVES: Children with congenital tracheal stenosis born in the developing world face a high risk of mortality due to limited access to proper treatment. Patients who required preoperative respiratory support were suspected to have poor survival after slide tracheoplasty; however, this was not clearly demonstrated in the previous studies. This study aims to investigate the impact of preoperative respiratory conditions on outcomes of slide tracheoplasty. METHODS: From 2016 to 2022, children who underwent slide tracheoplasty were retrospectively reviewed. Patients with respiratory distress requiring emergency operations (group A) were compared with patients in stable condition who were scheduled for surgery (group B). RESULTS: Perioperative results revealed that group A (n = 43) had a longer bypass time (P < 0.001), operation time (P = 0.01), postoperative ventilation time (P < 0.001) and length of intensive care unit stay (P = 0.00125) than group B (n = 60). The early mortality rate was 7.8%, and the actuarial 5-year survival rate was 85.3%. The cumulative incidence test revealed that group A was highly significant for overall mortality [sudistribution (SHR) 4.5; 95% confidence interval (CI) 1.23-16.4; P = 0.023]. Risk factors for overall mortality were prolonged postoperative ventilation time (hazard ratio 3.86; 95% CI 1.20-12.48; P = 0.024), bronchial stenosis (hazard ratio 5.77; 95% CI 1.72-19.31; P = 0.004), and preoperative tracheal mucositis (hazard ratio 5.67; 95% CI 1.51-21.31; P = 0.01). Four patients needed reintervention during a follow-up of 28.4 months (interquartile range 15.3-47.3). CONCLUSIONS: Preoperative respiratory distress negatively affected the outcomes of patients who required slide tracheoplasty. Therefore, early detection of congenital tracheal stenosis and aggressive slide tracheoplasty are crucial and obligatory to enhance long-term survival in this lethal congenital airway disease.

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

Long-Term Outcomes of Modified Cone Reconstruction for Ebstein's Anomaly in Pediatric Patients in a Single Center.

BACKGROUND AND OBJECTIVE: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. METHODS: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. RESULTS: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. CONCLUSIONS: Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.

Valve-sparing root replacement in children with connective tissue disease: Long-term risk of aortic events.

BACKGROUND: Cardiovascular involvement in pediatric patients with connective tissue disease (CTD) is life-threatening, with aortic root dilatation being the most prevalent cardiovascular abnormality. We attempted to determine long-term outcomes of valve-sparing root replacement (VSRR) in this group, including cardiovascular reoperations for aortic aneurysm and dissection. METHODS: We conducted a retrospective analysis of pediatric patients with CTD who received VSRR in a single center from 2002 to 2021. The primary end point was a composite event of all-cause death and cardiovascular reoperations. The median follow-up duration was 8.3 years, with a maximum of 20.7 years. RESULTS: The median age of 24 pediatric patients who had VSRR was 14.4 years. Marfan syndrome and Loeys-Dietz syndrome affected 19 (79.2%) and 5 (20.8%) patients, respectively. There was no early death. The 15-year survival rate was 91.7%. At 10 years after VSRR, the cumulative incidence of reoperation for aortic regurgitation was 15.6%, and for aortic aneurysm or dissection, it was 29.1%. The 10-year rate of freedom from the primary end point was 53.1%. The Cox multivariable analysis revealed younger age at surgery (hazard ratio, 1.279; 95% confidence interval, 1.086-1.505; P = .003) and VSRR before 13 years of age (hazard ratio, 5.005; 95% confidence interval, 1.146-21.850; P = .032) as independent prognostic factors for the primary endpoint. CONCLUSIONS: VSRR for aortic root dilatation in pediatric patients with CTD demonstrated good long-term survival and low reoperation rates for aortic regurgitation. However, several patients developed later aortic aneurysm or dissection, and careful surveillance may be required, particularly in those who received VSRR at younger age.

Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study.

Background: In the present study, the population prevalence and postoperative morbidity and mortality in Down syndrome patients who underwent total correction for congenital heart disease were investigated using data from a large national cohort. Methods: Retrospective administrative data from 2,395,966 participants born between 2008 and 2012 were acquired from the National Investigation of Birth Cohort in Korea. Among Down syndrome patients, 58.3% had congenital heart disease and 32.3% underwent total correction. Propensity score matching (maximum 1:1) and stabilized inverse probability treatment weighting (IPTW) were performed for each group (153 Down syndrome patients and 4482 non-Down syndrome patients). Results: T late mortality rate was significantly higher in the Down syndrome group than in the non-Down syndrome group (8.1% vs. 3.8%). No differences were observed in postoperative heart failure and arrhythmias, but pulmonary hypertension was significantly greater in the Down syndrome group than in the non-Down syndrome group (26.9% vs. 7.0%). The length of hospitalization was longer in the Down syndrome group than in the non-Down syndrome group (14 days vs. 11 days; interquartile range (IQR): 10−25 vs. 6−19; p < 0.0001). After total correction, readmission frequency for any reason was minimally but statistically significantly higher in the Down syndrome group compared to the non-Down syndrome group (5 times vs. 5 times; IQR: 3−8 vs. 4−9; p < 0.0001). However, the number of emergency room visits was minimally but significantly lower in the Down syndrome group compared to the non-Down syndrome group (2 visits vs. 2 visits (IQR): 2−7 vs. 1−4; p = 0.016). Conclusions: Down syndrome patients with congenital heart disease undergoing total correction showed pulmonary hypertension after surgery, longer length of hospitalization, frequent hospitalization after surgery, and a higher rate of late mortality.

Midterm results of arch augmentation with autologous vascular patch in interrupted aortic arch.

OBJECTIVES: Aortic arch reconstruction of interrupted aortic arch remains challenging, and subsequent problems, including arch and airway stenosis, may occur. Thus, we investigated midterm results of an augmentation technique using autologous vascular patch. METHODS: This retrospective study included 24 patients who underwent arch reconstruction with an autologous vascular patch for interrupted aortic arch with biventricular physiology from 2006 to 2018. The median age and body weight at operation were 10 days (range 4-77 days) and 3 kg (range 2.5-5.1 kg), respectively. The reconstructed arch was supplemented in the lesser curvature with an autologous vascular patch that was harvested from main pulmonary artery (n = 19), left subclavian artery (n = 3) or aberrant right subclavian artery (n = 1). One patient used patches from both the main pulmonary and left subclavian artery. RESULTS: There was 1 early death due to right heart failure. All survivors were discharged 15 days (range 9-58 days) after surgery without residual arch stenosis. Late death occurred in 1 patient with Cri-du-chat syndrome and airway stenosis. Two reoperations and 1 intervention for arch stenosis were performed. The 1-, 5- and 10-year survival was 92%. Freedom from reoperation or intervention for arch stenosis was 86% 1, 5 and 10 years after surgery. No occurrence of arch aneurysm formation, left main bronchial stenosis and significant hypertension was found during a median follow-up period of 5.5 years (range 0.3-13.3 years). CONCLUSIONS: Augmenting the lesser curvature with an autologous vascular patch during arch reconstruction resulted in reasonable midterm outcomes.

The effects of rational emotive behavior therapy for depressive symptoms in adults with congenital heart disease.

BACKGROUND: Over 30% of adults with congenital heart disease (CHD) experience depression. OBJECTIVES: To evaluate the effectiveness of Rational Emotive Behavior Therapy (REBT) in reducing depressive symptoms in adults with CHD. METHODS: Forty-two adults with CHD were recruited from an outpatient clinic and randomized to a study group (n = 21), which comprised 8-weekly group-based counseling sessions, or a control group (n = 21), which received usual care. REBT effectiveness was examined using the Hamilton Depression Rating Scale, Beck Depression Inventory, Shorten General Attitude and Belief Scale, and salivary cortisol levels before therapy, after the last session, and at follow-up 4 weeks later. RESULTS: Average participant ages were 30.1 ± 7.58 and 33.3 ± 7.1 years in study and control groups, respectively; 52.4% of participants in each group were female. After REBT, depression (p < 0.001), irrational beliefs (p < 0.001), and salivary cortisol levels (p = 0.006) were significantly lower in the study group than in the control group. Effects of REBT in the study group remained consistent at the 4-week follow-up. CONCLUSION: REBT may be effective in reducing depression in adults with CHD.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Successful Modified Nikaidoh Procedure (Pivot Rotation) in a Patient with Double Outlet Right Ventricle and Pulmonary Atresia: Case Report.

Double outlet right ventricle (DORV) with pulmonary atresia (PA) is an uncommon congenital disease. Because of anatomical and physiological anomalies in the systemic and pulmonary circulation, corrective surgery may be challenging. We present the case of a patient with DORV and PA. This condition was successfully corrected using a modified Nikaidoh procedure, resulting in reduced obstruction of the left ventricular outflow tract.

Impact of a Multidisciplinary Team Approach on Extracorporeal Circulatory Life Support-Bridged Heart Transplantation.

BACKGROUND: The number of heart transplantations (HTx) is increasing annually. Due to advances in medical and surgical support, the outcomes of HTx are also improving. Extracorporeal circulatory life support (ECLS) provides patients with decompensated heart failure a chance to undergo HTx. A medical approach involving collaboration among experienced experts in different fields should improve the outcomes and prognosis of ECLSbridged HTx. METHODS: From December 2003 to December 2018, 1,465 patients received ECLS at Samsung Medical Center. We excluded patients who had not undergone HTx or underwent repeated transplantations. Patients younger than 18 years were excluded. We also excluded patients who received an implantable durable left ventricular assist device before HTx. In total, 91 patients were included in this study. A multidisciplinary team approach began in March 2013 at our hospital. We divided the patients into 2 groups depending on whether they were treated before or after implementation of the team approach. RESULTS: The 30-day mortality rate was significantly higher in the pre-ECLS team group than in the post-ECLS team group (n=5, 18.5% vs. n=2, 3.1%; p=0.023). The 1-year survival rate was better in the post-ECLS team group than in the pre-ECLS team group (n=57, 89.1% vs. n=19, 70.4%; p=0.023). CONCLUSION: We found that implementing a multidisciplinary team approach improved the outcomes of ECLS-bridged HTx. Team-based care should be adapted at HTx centers that perform high-risk HTx.

Surgical Repair of a Sinus of Valsalva Aneurysm: A 22-Year Single-Center Experience.

BACKGROUND: Several reports described the repair of sinus of Valsalva aneurysms (SVAs); however, there is still debate regarding the optimal method of operation. We investigated the determinants of the development of significant aortic regurgitation (AR) and long-term survival after surgical repair. METHODS: Between January 1995 and December 2016, 71 patients (31 females; median age: 33.3 years) underwent surgical SVA repair with (n = 60) or without (n = 11) rupture. Aortic valvuloplasty (AVP) was performed using Trusler's technique in 28 patients (39.4%), and 11 patients (15.5%) underwent aortic valve replacement during the first operation. RESULTS: There was no early mortality, and three deaths occurred during follow-up (median: 65.4 months). Patients with grade II preoperative AR who underwent AVP tended to develop significant postoperative AR, but freedom from significant AR did not differ statistically (p = 0.387). Among patients who underwent AVP, freedom from significant AR did not differ statistically between those with grades I and II and those with grades III and IV (p = 0.460). CONCLUSION: Surgical repair of SVA with or without rupture can be performed safely using the dual approach technique. Concomitant aortic valve repair can be performed without difficulty and should be recommended not only for patients with moderate or severe preoperative AR (grades III and IV) but also for those with minimal or mild preoperative AR (grades I and II), whose aortic valve geometry needs correction.

Surgical Outcomes for Obstructive Total Anomalous Pulmonary Venous Return of the Non-Infracardiac Type Compared with the Infracardiac Type.

BACKGROUND: Infracardiac obstructive total anomalous pulmonary venous return (TAPVR) has a poor outcome following surgical correction. We compared the surgical outcomes of obstructive TAPVR between non-infracardiac and infracardiac types. METHODS: Among 51 patients who underwent surgical repair for obstructive TAPVR, 23 with infracardiac type and 28 with non-infracardiac type were included in this investigation. The study compared the immediate postoperative courses in the intensive care unit and long-term mortality and pulmonary vein stenosis. The risk factors for long-term survival in obstructive TAPVR also were investigated. RESULTS: The postoperative follow-up period was 79.8 ± 81.5 months. Immediate major operative complications were observed in 22 patients (43.1%); 10 patients (19.6%) died, and eight patients (15.7%) experienced pulmonary vein stenosis during the follow-up period. The Kaplan-Meier curve showed better cumulative survival in patients with infracardiac TAPVR (P = 0.308). The significant factors for survival after surgical repair of obstructive TAPVR did not include anatomical type but instead were postoperative course of ventilator care and lengths of intensive care unit and hospital stays. CONCLUSION: Patients with non-infracardiac TAPVR with obstruction had a longer postoperative course and experienced more complications. Their survival rate was poorer, and postoperative pulmonary vein stenosis was more frequent in those patients compared with infracardiac TAPVR patients. However, a large-scale study is mandatory to gather more data and confirm our findings.

Left Ventricular Assist Device Implantation via Dual Left Thoracotomy in an Adult Patient with Congenitally Corrected Transposition of the Great Arteries.

A 59-year-old man presented for possible durable ventricular assist device (VAD) implantation. He had previously been diagnosed with congenitally corrected transposition of the great arteries, a ventricular septal defect, an atrial septal defect, pulmonary valve stenosis, and aortic valve regurgitation. In the previous 22 years, he had undergone palliative cardiac surgery 3 times. VAD implantation as a bridge to transplantation was planned. Owing to severe adhesions, mesocardia, a left ascending aorta, and moderate aortic regurgitation, we performed VAD implantation and aortic valve closure via a dual left thoracotomy and partial sternotomy.

The progression of an acceptable pulmonary stenosis immediately after total correction of tetralogy of Fallot.

BACKGROUND: A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression. METHODS: Fifty-two patients with acceptable pulmonary stenosis immediately after operation were enrolled. Acceptable pulmonary stenosis was defined as peak pressure gradient between 15 and 45 mmHg by Doppler echocardiography. Latent class linear mixed model was used to differentiate patients with progressed pulmonary stenosis, and the factors related to progression were analysed. RESULTS: Pulmonary stenosis progressed in 14 patients (27%). Between the progression group and no progression group, there were no significant differences in operative age, sex, and the use of the transannular patch technique. However, immediate gradient was higher in the progression group (32.1 mmHg versus 25.7 mmHg, p = 0.009), and the cut-off value was 26.8 mmHg (sensitivity = 65.3%, specificity = 65.8%). Main stenosis at the sub-valve was observed more frequently in the progression group (85.7% versus 52.6%, p = 0.027). Despite no difference in the preoperative pulmonary valve z value, the last follow-up pulmonary valve z value was significantly lower in the progression group (-1.15 versus 0.35, p = 0.002). CONCLUSIONS: Pulmonary stenosis immediately after tetralogy of Fallot total correction might progress in patients with immediate pulmonary stenosis higher than ≥26.8 mmHg and the main site was sub-valve area.

Three-Dimensional Printed Model of Partial Anomalous Pulmonary Venous Return with Biatrial Connection.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiac anomaly that can be difficult to detect and often remains undiagnosed. PAPVR is diagnosed using non-invasive imaging techniques such as echocardiography, CT, and MRI. Image data are reviewed on a 2-dimensional (D) monitor, which may not facilitate a good understanding of the complex 3D heart structure. In recent years, 3D printing technology, which allows the creation of physical cardiac models using source image datasets obtained from cardiac CT or MRI, has been increasingly used in the medical field. We report a case involving a 3D-printed model of PAPVR with a biatrial connection. This model demonstrated separate drainages of the right upper and middle pulmonary veins into the lower superior vena cava (SVC) and the junction between the SVC and the right atrium, respectively, with biatrial communication through the right middle pulmonary vein.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease.

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.

Repair of tricuspid valve with pulmonary valve replacement in repaired tetralogy of fallot.

Objectives: We aimed to investigate the outcome of tricuspid valve repair (TVR) performed concomitantly with pulmonary valve replacement in repaired tetralogy of Fallot (TOF) patients. Design: This retrospective study included all patients who underwent pulmonary vale replacement from 2000 to 2016 after TOF correction. TVR patient data were compared to those of patients who underwent pulmonary vale replacement alone. Results: Thirty-eight patients were enrolled. The degree of tricuspid regurgitation was significantly decreased after operation in the TVR group. Tricuspid valve annulus and annuloectasia before operation did not vary between groups (21.1 ± 6.3 and 41.4% in no TVR vs. 21.3 ± 4.8 and 52.6% in TVR). However pre-operative right ventricular volumes were larger in the TVR group. Normal tricuspid valve coaptation (body to body) was observed less frequently in the TVR group than in the other group (52.6% vs. 93.1%, p < .001). Pre-operative tricuspid regurgitation had a linear correlation with right ventricular volume, but not with tricuspid annulus size. Conclusion: Tricuspid annulus diameter decreased significantly regardless of TVR. Abnormal coaptations were observed more in patients group and the degree of pre-operative tricuspid regurgitation was linearly correlated with right ventricular volume rather than tricuspid annulus size.

Ultrasound-guided vs. palpation-guided techniques for radial arterial catheterisation in infants: A randomised controlled trial.

BACKGROUND: The usefulness of ultrasound-guided techniques for radial arterial catheterisation has been well identified; however, its usefulness has not been completely evaluated in infants under 12 months of age, who are generally considered the most difficult group for arterial catheterisation. OBJECTIVE: We evaluated whether ultrasound guidance would improve success rates and reduce the number of attempts at radial arterial catheterisation in infants. DESIGN: A randomised, controlled and patient-blinded study. SETTING: Single-centre trial, study period from June 2016 to February 2017. PATIENTS: Seventy-four infants undergoing elective cardiac surgery. INTERVENTION: Patients were allocated randomly into either ultrasound-guided group (group US) or palpation-guided group (group P) (each n=37) according to the technique applied for radial arterial catheterisation. All arterial catheterisations were performed by one of two experienced anaesthesiologists based on group assignment and were recorded on video. MAIN OUTCOME MEASURES: The primary endpoint was the first-pass success. The number of attempts and total duration of the procedure until successful catheterisation were also analysed. RESULTS: The first-pass success rate was significantly higher in the group US than in the group P (68 vs. 38%, P = 0.019). In addition, fewer attempts were needed for successful catheterisation in the group US than in the group P (median 1 [IQR 1 to 2] vs. 2 [1 to 4], P = 0.023). However, the median [IQR] procedural time (s) until successful catheterisation in the two groups was not significantly different (102 [49 to 394] vs. 218 [73 to 600], P = 0.054). CONCLUSION: The current study demonstrated that the ultrasound-guided technique for radial arterial catheterisation in infants effectively improved first-pass success rate and also reduced the number of attempts required. TRIAL REGISTRATION: ClinicalTrials.gov NCT02795468.

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries.

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient, the right subclavian artery originated from the pulmonary artery, which is why he did not show reversed differential cyanosis. We conclude that the presence of an aortic arch anomaly should be considered in patients with d-TGA who do not present with reversed differential cyanosis. A further imaging work-up, including computed tomography or magnetic resonance imaging, might be helpful.