The arterial switch operation (ASO) is the standard surgical treatment for transposition of the great arteries (TGA). Postoperative complications such as branch pulmonary artery (PA) stenosis are commonly observed. This study aimed to investigate the possible potential anatomical mechanisms contributing to isolated branch PA stenosis using cardiac computed tomography (CCT).

A retrospective, single-center study was conducted on pediatric patients under 18 years of age diagnosed with TGA, with or without ventricular septal defect (VSD), who underwent ASO and cardiac CCT between January 2004 and October 2022. Baseline characteristics, echocardiographic data, and CCT findings were compared between patients with and without isolated branch PA stenosis. Special attention was given to the angle between the PA bifurcation and the ascending aorta (AAO).

Among 30 patients enrolled, 46.67% (14/30) had isolated branch PA stenosis, while 53.33% (16/30) had no stenosis. Baseline and echocardiographic variables showed no significant differences between groups, except for the PA bifurcation angle relative to the AAO. Patients with isolated right PA (RPA) stenosis demonstrated significantly greater leftward angulation (median angle: 25° [IQR: 24, 30]) compared to the no-stenosis group (median angle: -2° [IQR: -8, 6.5]). Similarly, isolated left PA (LPA) stenosis was associated with greater rightward angulation (median angle: -22° [IQR: -32, -20]) compared to the no-stenosis group (P = 0.042).

Alterations in the PA bifurcation angle relative to the AAO may contribute to the development of isolated branch PA stenosis following ASO. Preoperative planning to optimize the PA bifurcation angle to within ±20 degrees may help reduce the risk of postoperative stenosis. A larger study incorporating advanced cardiac imaging into routine ASO follow-up is warranted.

Left ventricular function after arterial switch operation for d-transposition of the great arteries is notoriously compromised because of abnormal coronary artery anatomy or altered loading conditions. We sought to longitudinally investigate the performance of the left ventricle in a cohort of d-transposition of the great artery patients after arterial switch operation, by using advanced echocardiographic deformation imaging and grouping patients according to pre- and post-surgery variables, labelled as risk factors.

Longitudinal single-centre study involving 53 d-transposition of the great artery patients (81.1% male) after arterial switch operation, the latter being performed as unique surgical procedure in 39 patients (76.5%). Median follow-up was 59 months [23.5-72].

Selected patients were split into two groups according to risk factors. Fifteen patients (30.6%) were grouped into high-risk class (<3 risk factors). Echocardiographic variables such as tricuspid annular plane systolic excursion, ejection fraction, and global longitudinal strain were compared between the two groups. Only global longitudinal strain reached statistical significance (-17.56 ± 2.26 versus -19.82 ± 1.97 %; p < 0.001). To discriminate high- versus low-risk patients, a receiver operating characteristic (ROC) curve identified a global longitudinal strain cut-off value of -17.75% (sensitivity 57.1%, specificity 97%, AUC 80%).

Several neonatal and post-surgical variables might conditionate long-term follow-up of d-transposition of the great artery patients after arterial switch operation, and global longitudinal strain best conveys the overall risk profile of these patients.

The Toronto Adult Congenital Heart Disease (ACHD) Program at the University Health Network, University of Toronto, began in 1959. It traces its origins to a Paul Wood protégé, Dr. John Evans, and to a long-standing and supportive relationship with Hospital for Sick Children (SickKids), located just across the street. Over the decades, the program has grown to become a major center for training and research in ACHD and one of the largest clinical programs for ACHD care globally. This paper recounts the 65-year history of the program, including some of its key individuals, challenges, milestones, innovations, discoveries, and future aspirations.

To determine the influence of coronary anatomy on long-term outcomes of the arterial switch operation (ASO).

We retrospectively reviewed patients with transposition of the great arteries or Taussig-Bing anomaly who underwent ASO at our institution between 1992 and 2022. The primary endpoint was freedom from a composite of death, transplantation, and coronary reintervention.

A total of 632 patients (median age, 5.0 days; interquartile range [IQR], 4.0-7.0 days) underwent ASO. Coronary anatomy included the following categories: usual (n = 411; 65%), circumflex (Cx) from sinus 2 (n = 89; 14%), inverted (n = 55; 9%), single sinus (n = 46; 7%), and intramural (n = 31; 5%). Overall operative mortality was 3% (n = 16) and highest in patients with intramural cardiac anatomy (n = 3; 10%), although it dropped to 0% in this group in the most recent decade. The median duration of follow-up was 14.5 years (IQR, 6.0-20.3 years). Twenty-year freedom from the primary endpoint was 95 ± 1% for usual anatomy, 99 ± 1% for Cx from sinus 2, 90 ± 4% for inverted, 91 ± 4% for single sinus, and 80 ± 9% for intramural (P < .001). Intramurals had the highest 20-year incidence of coronary reintervention (11 ± 8%). Cox modeling identified intraoperative coronary revision (hazard ratio [HR], 20.1; 95% confidence interval [CI], 9.4-53.9; P < .001), Taussig-Bing anomaly (HR, 4.9; 95% CI, 2.2-10.9; P < .001), and an intramural coronary artery (HR, 2.9; 95% CI, 1.0-8.2; P = .04) to be risk factors for the composite endpoint.

Rare coronary artery variants-particularly intramural-are associated with increased mortality and coronary reinterventions after ASO. A low threshold for unroofing intramurals is likely associated with declining mortality and improved outcomes. Additional investigations are needed to determine the long-term fate of the coronary arteries after ASO.

Complete transposition of the great arteries is a common life-threatening complex cyanotic congenital heart disease in infants, resulting in the operation usually performed about one week after birth. However, little is known about the surgical strategy and experience of transposition of the great arteries with an intact ventricular septum in older patients. Herein, we present an abandoned 7-year-old boy with severe cyanosis with clubbed fingers and toes and then diagnosed with transposition of the great arteries with an intact ventricular septum, atrial septal defect, patent ductus arteriosus, and pulmonary hypertension. The patient underwent a two-staged procedure: an aortopulmonary shunt and pulmonary artery banding were performed at the first stage, followed by the Switch operation, defect repair, and patent ductus arteriosus ligation, all of which were successfully performed. The patient was discharged on the 15th day after the operation, and the arterial oxygen saturation returned to normal level (99%). The illustrative report highlights the essence of raising awareness and developing accurate treatment strategy of transposition of the great arteries, especially in remote rural areas of eastern countries, where the level of health care and services is relatively underdeveloped.

Infants with dextro-transposition of the great arteries (d-TGA) are at high risk for hemodynamic compromise in the immediate postnatal period due to dependence on intracardiac mixing for oxygen delivery. This period of profound hypoxemia may have long-term implications, as previous studies demonstrated patients with d-TGA are at increased risk for neurocognitive delays despite effective surgical correction in the neonatal period. Balloon atrial septostomy (BAS) is an established intervention that improves intracardiac mixing and perioperative hemodynamics. This retrospective study aimed to quantify the time from birth to BAS and compare short-term outcomes for patients with prenatal and postnatal diagnoses of d-TGA. We identified 68 newborns born with d-TGA who were admitted to our facility between 2013 and 2022 and required BAS within 48 h after birth. Halfway through this study, our cardiac interventional team began traveling to a nearby delivery center where a bedside BAS could be performed prior to transferring the patient. We divided the patients into 3 groups-postnatal diagnosis (n = 27), prenatal diagnosis with rapid transport (n = 24), and prenatal diagnosis with interventional team performing a BAS at the delivery hospital (n = 17). The time from birth to BAS was significantly shorter for patients in the interventional program group (1.1 h) compared to the rapid transport (4.5 h) and postnatal diagnosis groups (9.3 h, p value < 0.01). The interventional program group also had lower lactate levels and less acidotic pH compared to the other groups. There was no significant difference in lowest oxygen saturation level, pre-surgical neurologic complication rate, time to surgery, or hospital length of stay. The interventional program proved to be a safe and effective model, as there were no procedural complications and the time to BAS decreased. Long-term follow-up is needed to determine if abating this initial period of hemodynamic instability will lead to improved neurodevelopmental outcomes.

Patients with d-transposition of the great arteries (d-TGA) who have undergone an arterial switch operation (ASO) can develop right ventricular outflow tract (RVOT) dysfunction with pulmonary regurgitation (PR) or stenosis. In these patients, treatment may include transcatheter pulmonary valve replacement (TPVR). Coronary compression is a contraindication occurring in 5% of typical TPVR cases. After ASO, there are various anatomical considerations that can confound TPVR, including potential coronary artery compression. Our goal is to understand feasibility of TPVR in patients following ASO.

This was a retrospective multicenter cohort study of patients with RVOT dysfunction after ASO who underwent cardiac catheterization with intention to perform TPVR from 2008 to 2020.

Across nine centers, 33 patients met inclusion criteria. TPVR was successful in 22 patients (66%), 19 receiving a Melody valve and 3 a SAPIEN valve. RVOT stenosis in isolation or with PR dictated need for TPVR in nearly all patients. One serious adverse event occurred with valve embolization. After TPVR, the RVOT peak gradient decreased from 43 to 9 mm Hg (p < 0.001); PR was trivial/none in all but one patient, in whom it was mild. Coronary compression prohibiting TPVR occurred in eight patients (24%) and two patients (6%) had severe aortic regurgitation from aortic root deformation precluding TPVR. Seven patients underwent RVOT reintervention a median of 5.3 years post-TPVR.

TPVR in patients with d-TGA after ASO is feasible, but in this cohort, coronary compression or aortic root distortion precluded TPVR in one-third of patients. The rate of RVOT reintervention after TPVR was higher in this cohort of ASO patients than in prior studies.

Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5-7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly's development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations. Correction of the defect during infancy is the preferred treatment for D-TGA. Balloon atrial septostomy (BAS) is necessary prior to the operation. The recommended surgical correction methods include arterial switch operation (ASO) and atrial switch operation (AtrSR), as well as the Rastelli and Nikaidoh procedures. The most common postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency and neopulmonic stenosis, right ventricular (RV) outflow tract obstruction (RVOTO), left ventricular (LV) dysfunction, arrhythmias, and heart failure. Early diagnosis and treatment of D-TGA is paramount to the prognosis of the patient. Improved surgical techniques have made it possible for patients with D-TGA to survive into adulthood.

Background: We sought to evaluate the outcomes in patients who underwent the arterial switch operation (ASO) over a 20-year period at a single institution. Methods: The current study is a retrospective review of 180 consecutive patients who underwent the ASO for biventricular surgical correction of dextro-transposition of the great arteries (d-TGA) between 2002 and 2022. Results: Among 180 patients, 121 had TGA-intact ventricular septum, 47 had TGA-ventricular septal defect and 12 had Taussig-Bing Anomaly (TBA). The median follow-up time was 6.7 years (interquartile range: 3.9-8.7 years). There were five early (2.8%) and one late (0.6%) mortality. Survival was 96.6% at one year and beyond. Reoperations were performed in 31 patients (17%). Taussig Bing Anomaly was found to increase the risk of reoperation by 17 times (P < .0001). A total of 37 (21%) patients underwent 53 reinterventions (14 surgical procedures, 39 catheter interventions) specifically addressing pulmonary artery (PA) stenosis. Freedom from PA reintervention was 97%, 87%, 70%, and 55% at 1, 5, 10, and 15 years, respectively. By bivariable analysis, TBA (P = .003, odds ratio [OR]: 6.4, 95% confidence interval [CI]: 1.9-21.7), mild PA stenosis at discharge (P ≤ .001, OR: 6.1, 95% CI: 2.7-13.6), and moderate or severe PA stenosis at discharge (P ≤ .001, OR: 12.7, 95% CI: 5-32.2) were identified as predictors of reintervention on PA. In the last follow-up of 174 survivors, 24 patients (14%) had moderate or greater PA stenosis, two (1%) had moderate neoaortic valve regurgitation, and 168 were New York Heart Association status I. Conclusions: Our results demonstrated excellent survival and functional status following the ASO for d-TGA; however, patients remain subject to frequent reinterventions especially on the pulmonary arteries.

Arterial switch operation (ASO) is the standard surgical choice for D-transposition of great arteries (D-TGA). However, the implications of ASO on pulmonaries, coronaries, and aorta have not been adequately investigated. The current study evaluates arterial morphologic changes post-ASO at intermediate-term surveillance.

From May 2021 to May 2022, patients with D-TGA who underwent ASO for more than six months were recruited. Preoperative and operative data were collected. Patients were assessed using echocardiography (ECHO) and multislice CT angiography (MSCT) to evaluate pulmonary, coronary, and aortic arterial anatomy.

Twenty patients were included with median age of 11 (10-23.25) days at ASO and 14 (7.25-32.75) months on last follow-up. Neo-aortic regurgitation was detected in 12(60%) and neo-pulmonary regurgitation in 3 (15%). Using ECHO, complete evaluation of pulmonary arteries (PAs) was not achieved in 35% and incomplete coronaries assessment in 40% of cases. No stenosis was detected in coronaries using MSCT, although coronary anomalies were found in 9/20 (45%). Dilated Aortic annulus was detected in 16/20 (80%), dilated aortic root in 18/20 (90%), and dilated sinotubular junction in 70%. Right PA stenosis was diagnosed in 10/20 (50%) and left PA(LPA) stenosis in 7/20 (35%). Although Z-score of PAs did not correlate with aortic data, LPA bending angle was positively correlated to neo-aortic root diameter and Z-score (rho = 0.65,p = 0.016; rho = 0.69,p = 0.01), respectively.

Echocardiography alone is not a conclusive surveillance tool for detecting late post-ASO anatomic changes in D-TGA patients. Cardiac MSCT should be considered for comprehensive evaluation on the intermediate-term follow-up post-ASO to accurately track morphologic abnormalities in the aorta, pulmonary, and coronary arteries.

Embryological misalignment between the aorta and pulmonary trunk gives rise to the congenital anomaly of the heart known as transposition of the great arteries (TGA). TGA is a type of parallel circulation, where the heart pumps oxygenated blood from the left ventricle into the pulmonary trunk. The deoxygenated blood from the right ventricle is circulated into the body as it pumps blood into the aorta. This type of parallel circulation is not compatible with life unless there is communication between oxygenated and deoxygenated blood. The presence of a ventricular septal defect (VSD) or patent ductus arteriosus (PDA) in TGA patients serves as this communication. Cyanosis in the first month of life is the most common presenting feature. We had a five-and-a-half-year-old male child presenting with cyanosis and congestive cardiac failure (CCF), along with infective endocarditis with mitral valve regurgitation, which is an unusual complication of dextro-TGA (d-TGA) with pulmonary stenosis (PS) with VSD.

Evaluating the relation of non-cardiac comorbidity and socio-demographic factors to physical and mental health-related quality of life (QOL) which has been partially found at elevated risk in young adults after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA). In a prospective reassessment study, results of 92 unselected young adults (22.8 ± 2.6 years) having undergone evaluation of QOL (SF-36) were related to non-cardiac comorbidity with special respect to neurologic and psychiatric comorbidity and to socio-demographic parameters. Neurologic (14%) contrary to psychiatric comorbidities (6.5%) were more frequent than in the general population. The educational level was higher, the rate of unemployment was double as high compared to the average German population. Significant inverse relations (p = 0.006 to 0.033) existed between physical health domains (physical functioning and general health perception) and non-cardiac, neurologic, and psychiatric comorbidity, as well as correlations between the latter domains and socio-economic status, educational level, and worse employment status (Spearman 0.22-0.41, p < 0.0001 to 0.036). Mental health domains (vitality, social functioning, psychical health) were significantly inversely related with neurologic and psychiatric comorbidity (p = 0.002 to 0.048) and correlated with higher educational level (Spearman 0.25, p = 0.019). Neurologic and psychiatric comorbidities and socio-demographic parameters are significant risk factors for a reduced QOL concerning physical and mental health in young adults with TGA after ASO. Standardized QOL measurement should be part of routine screening programs to detect subclinical physical, neurodevelopmental, and psychosocial comorbidity.

Aortic dilation and valvar regurgitation can develop in transposition of the great arteries (TGA) after the arterial switch operation (ASO). Variation in aortic root rotational position affects flow dynamics in patients without congenital heart disease. The aim of this study was to assess neo-aortic root (neo-AoR) rotational position and its association with neo-AoR dilation, ascending aorta (AAo) dilation, and neo-aortic valvar regurgitation in TGA following ASO.

Patients with TGA repaired by ASO who underwent cardiac magnetic resonance (CMR) were reviewed. Neo-AoR rotational angle, neo-AoR and AAo dimensions indexed (to height), indexed left ventricular end diastolic volume (LVEDVI), and neo-aortic valvar regurgitant fraction (RF) were obtained from CMR.

Among 36 patients, the median age at CMR was 17.1 years (12.3, 21.9). Neo-AoR rotational angle (range - 52 to + 78°) was clockwise ( ≥ + 15°) in 50%, counterclockwise (<-9°) in 25%, and central (-9 to + 14°) in 25% of patients. A quadratic term for neo-AoR rotational angle, indicating increasing extremes of counterclockwise and clockwise angles, was associated with neo-AoR dilation (R² = 0.132, p = 0.03), AAo dilation (R² = 0.160, p = 0.016), and LVEDVI (R² = 0.20, p = 0.007). These associations remained statistically significant on multivariable analyses. Rotational angle was negatively associated with neo-aortic valvar RF on univariable (p < 0.05) and multivariable analyses (p < 0.02). Rotational angle was associated with smaller bilateral branch pulmonary arteries (p = 0.02).

In patients with TGA after ASO, neo-AoR rotational position likely affects valvar function and hemodynamics, leading to a risk of neo-AoR and AAo dilation, aortic valvar incompetence, increasing left ventricular size, and smaller branch pulmonary arteries.

Evaluating the impact of subjective physical activity status and cardiopulmonary exercise capacity on physical and mental health-related quality of life (QOL) in young adults after neonatal arterial switch operation for transposition of the great arteries.

In a prospective reassessment study, 92 young adults (age 22.8 ​± ​2.6 ​y) underwent standardized evaluation of health-related QOL (SF-36), NYHA status and cardiopulmonary exercise performance (CPET).

Peak oxygen uptake was 33.1 ​± ​7.1 ​ml/kg/min corresponding to 84.5 ​± ​15.5% of predicted, according to the borderline subnormal range. 65% were classified as normal or borderline, 35% as impaired. 88% were NYHA class I and 12% class II, 45% exercised regularly. Despite average or even elevated mean scores for the QOL domains, more patients reported significantly poorer results, indicated by increased percentages below -2 SD for physical (5.6%), social (7.8%) and emotional role functioning (8.8%). Significant correlations existed between physical health domains and objective exercise performance (r ​= ​0.23 to 0.34, p ​= ​0.001 to 0.032) as well as NYHA class (r ​= ​0.33 to 0.46, p ​< ​0.001 to 0.002). Mental health domains were also significantly correlated with NYHA class (r ​= ​0.22 to 0.41, p ​≤ ​0.001 to 0.04) and peak oxygen uptake.

Despite good results on average, young adults with TGA after ASO are at elevated risk for a reduced QOL. Objective exercise capacity and NYHA class are significant indicators for self-perceived physical and mental health. Applications of QOL measurement should be part of routine clinical follow up in this patient group.

Coronary artery status in adults long after the arterial switch operation (ASO) is unclear. We conducted a systematic review to provide an overview of coronary complications during adulthood and to evaluate the value of routine coronary imaging in adults after ASO, in light of current guidelines. Articles were screened for the inclusion of adult ASO patients and data on coronary complications and findings of coronary imaging were collected. A total of 993 adults were followed with a median available follow-up of only 2.0 years after reaching adulthood. Myocardial ischemia was suspected in 17/192 patients (8.9%). The number of coronary interventions was four (0.4%), and coronary death was reported in four (0.4%) patients. A lack of ischemia-related symptoms cannot be excluded because innervation studies indicated deficient cardiac innervation after ASO, although data is limited. Anatomical high-risk features found by routine coronary computed tomography (cCT) included stenosis (4%), acute angle (40%), kinking (24%) and inter-arterial course (11%). No coronary complications were reported during pregnancy (n = 45), although, remarkably, four (9%) patients developed heart failure. The 2020 European Society of Cardiology (ESC) guidelines state that routine screening for coronary pathologies is questionable. Based on current findings and in line with the 2018 American ACC/AHA guidelines a baseline assessment of the coronary arteries in all ASO adults seems justifiable. Thereafter, an individualized coronary follow-up strategy is advisable at least until significant duration of follow-up is available.

The objective of this study was to assess our 43-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) by analysing cardiac outcome measures (hospital and late mortality, reoperations and catheter interventions, significant coronary artery obstruction) and to identify risk factors for reoperation and catheter interventions.

A total of 490 patients who underwent ASO for TGA from 1977 to 2020 were included in this retrospective, single-centre study. Data on reoperation and catheter intervention of hospital survivors were estimated by the Kaplan-Meier method and compared using a long-rank test. Risk factors for reoperation and/or catheter intervention were assessed by multivariate Cox regression analysis.

Hospital mortality occurred in 43 patients (8.8%), late death in 12 patients (2.9%) and 43 patients were lost to follow-up. Median follow-up time of 413 hospital survivors was 15.6 (interquartile range 7.0-22.4) years. Reoperations were performed in 83 patients (117 reoperations). Neoaortic valve regurgitation with root dilatation was the second most common indication for reoperation (15/83 patients, 18.1%) after right ventricular outflow tract obstruction (50/83 patients, 60.2%). Risk factors for any reoperation on multivariable analysis were: TGA morphological subtype [TGA with ventricular septal defect: hazard ratio (HR) = 1.99, 95% confidence interval (CI) 1.18-3.36; P = 0.010 and Taussig-Bing: HR = 2.17, 95% CI 1.02-4.64; P = 0.045], aortic arch repair associated with ASO (HR = 3.03, 95% CI 1.62-5.69; P = 0.001) and a non-usual coronary artery anatomy (HR = 2.41, 95% CI 1.45-4.00; P = 0.001). One hundred and one catheter interventions were performed in 54 patients, usually for relief of supravalvular pulmonary stenosis (44/54 patients, 81.5%) or arch obstruction (10/54 patients, 18.5%). Main risk factor for catheter intervention on multivariable analysis was aortic arch repair associated with ASO (HR = 2.95, 95% CI 1.37-6.36; P = 0.006). Significant coronary artery stenosis was relatively uncommon (9/413 patients, 2.2%) but may be underrepresented.

Patients after ASO typically have good long-term clinical outcomes but reoperations and interventions remain necessary in some patients. Neoaortic valve regurgitation with root dilatation is the second most common indication for reoperation after right ventricular outflow tract obstruction and an increasing need for neoaortic valve and root redo surgery in future is to be expected.

Pulmonary artery (PA) anatomy in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) with Lecompte manoeuvre is different compared to healthy subjects, and stenoses of the PA are common. Magnetic resonance imaging (MRI) is an excellent imaging modality to assess PA anatomy in TGA patients. However, disease-specific reference values for PA size are scarce.

To establish disease-specific reference ranges for PA dimensions and for biventricular volumes and mass.

Retrospective.

A total of 69 pediatric patients with TGA after ASO (median age 12.6 years; range 5-17.8 years; 13 females and 56 males).

3.0 T, steady-state free precession (SSFP) and gradient echo cine sequences and four-dimensional time-resolved magnetic resonance angiography with keyhole.

Right and left PA (RPA, LPA) were each measured at three locations during its course around the aorta. Ventricular volumes, mass, and ejection fraction were measured from a stack of short axis cine images.

The lambda-mu-sigma (LMS) method of Cole and Green, univariate and multivariate linear models, and t-test.

Centile graphs and tables for PA dimensions, biventricular volumes, mass, and ejection fraction were created. Univariate linear analysis showed significant associations (P < 0.05) between body surface area (BSA), height, and weight with systolic MPA and RPA diameter. In multivariate linear analysis, only BSA remained a strong predictor for main PA and RPA diameters. For biventricular volumes, the univariate linear model revealed a strong influence of BSA, height, weight, and age (all P < 0.05). On multivariate linear analysis, only body height remained associated.

Uni- and multivariate linear analyses showed a strong association between BSA and PA diameters, as well as between height and biventricular volumes, and therefore, centile tables and graphs are presented accordingly. Our data may improve MR image interpretation and may serve as a reference in future studies.

4 TECHNICAL EFFICACY STAGE: 2.

Dextro-transposition of the great arteries (D-TGA) has undergone a significant evolution in surgical repair, leading to survivors with vastly different postsurgical anatomy which in turn guides their long-term cardiovascular morbidity and mortality. Atrial switch repair survivors are limited by a right ventricle in the systemic position, arrhythmia and atrial baffles prone to obstruction or leak. Functional assessment of the systemic right ventricle is complex, requiring multimodality imaging to include specialised echocardiography and cross-sectional imaging (MRI and CT). In the current era, most neonates undergo the arterial switch operation with increasing understanding of near-term and long-term outcomes specific to their cardiac anatomy. Long-term observations of the Lecompte manoeuvre or coronary stenoses following transfer continue, with evolving understanding to improve surveillance. Ultimately, an understanding of postsurgical anatomy, specialised imaging techniques and interventional and electrophysiological procedures is essential to comprehensive care of D-TGA survivors.

The arterial switch operation (ASO) has excellent early outcomes in the modern era. We sought to determine the long-term outcomes in patients who underwent an ASO at a single institution.

Patients who underwent an ASO between 1983 and 2015 were identified from the hospital database and retrospectively reviewed using hospital records.

From 1983 to 2015, 844 patients with a biventricular circulation underwent an ASO. There were 28 (3.3%, 28/844) early deaths. Follow-up was available for 94% (729/774) of local patients after hospital discharge. Median follow-up was 15 years (interquartile range, 8-20 years). There were 187 (26%, 187/729) patients with more than 20 years of follow-up and 95 (13%, 95/729) patients with more than 25 years of follow-up. Overall survival was 95% (95% confidence interval [CI], 94%-97%) at 10 and 25 years after the ASO. At 25 years after ASO, freedom from overall reintervention was 77% (95% CI, 73%-81%), freedom from reoperation on the neoaortic root or neoaortic valve was 92% (95% CI, 88%-95%), and freedom from coronary reoperation was 99% (95% CI, 98%-99.7%). Left ventricular (LV) systolic function was normal in 595 of 609 (98%) of patients who had LV function quantified at latest follow-up. Of the 95 patients with more than 25 years of follow-up after ASO, 6 (6.3%) had at least moderate neoaortic valve regurgitation (AR) and 8 (8.4%) had undergone replacement of the neoaortic valve.

Overall, survivors of ASO have excellent late survival and normal LV systolic function into adult life. However, AR and reoperation on the neoaortic valve remains an issue for older patients.

Although arterial switch operations (ASOs) have been performed globally to repair d-transposition of the great arteries (d-TGA) in neonates and infants, few studies have been reported regarding the influence of the hemodynamics of patients with d-TGA who have undergone ASO on the development of neo-aortic valve regurgitation (AR). We aimed to investigate the relationship between the hemodynamics and development of AR after ASO in patients with d-TGA by catheter evaluation. This observational study screened 114 consecutive patients who underwent ASO for d-TGA or Taussig-Bing anomaly and who subsequently underwent catheter evaluations in our institution. We reviewed their records for the past 20 years and collected their first catheterization data post-ASO in early childhood. Thirty-six post-ASO patients who underwent catheter evaluations in both the early surgical and long-term phases were finally analyzed. Patients were divided into the following groups according to the presence of significant AR in the long-term phase: the AR group (n = 9 with AR ≥ grade II by the Sellers classification) and the non-AR group (n = 27 with AR < grade II). In the long-term phase, the diastolic blood pressure was significantly lower and the ascending aortic diameter was significantly larger in the AR group than in the non-AR group (p = 0.004 and p = 0.006, respectively). The systolic blood pressure (SBP) and pulse pressure (PP) were similar in both groups. Meanwhile, in the early surgical phase, SBP and PP were significantly higher in the AR group than in the non-AR group (p = 0.029 and p = 0.002, respectively). The receiver operating characteristic curve for late AR showed that the area under the curve for SBP and PP in the early surgical phase were 0.746 and 0.853, respectively. Even though sensitivity analysis was performed, SBP or PP greater than the cutoff value in the early surgical phase was identified as predictors for late AR. Our results suggested that high SBP or PP in the early surgical phase could influence the development of AR in the long term after ASO.

Senning operation is a surgical treatment for transposition of great arteries that can be complicated by post-procedural atrial or caval baffle leaks. We present a 6-year-old boy with a history of Senning repair for transposition of great arteries, who developed a pulmonary venous baffle leak. Percutaneous baffle leak closure was successfully performed using an Amplatzer septal occluder.

Background Despite remarkable success in the surgical and medical management of congenital heart disease ( CHD ), some survivors still experience cardiovascular complications over the long term. The goal of this study was to evaluate the association between CHD and risk of cardiovascular disease ( CVD ) by conducting a meta-analysis of cohort studies. Methods and Results A systematic literature search of several databases was conducted through April 2018 to identify studies reporting the risk of CVD , stroke, heart failure, and coronary artery heart disease in CHD survivors. The quality of individual studies was assessed using the Newcastle-Ottawa scale. The overall risk estimates were pooled using fixed-effects meta-analysis. Subgroup analyses were performed to explore possible sources of heterogeneity. Nine cohort studies comprising 684 200 participants were included. The overall combined relative risks for people with CHD compared with the controls were 3.12 (95% CI, 3.01-3.24) for CVD , 2.46 (95% CI, 2.30-2.63) for stroke, 5.89 (95% CI, 5.58-6.21) for heart failure, and 1.50 (95% CI, 1.40-1.61) for coronary artery heart disease. Significant heterogeneity was detected across studies regarding these risk estimates. Heterogeneity in the risk estimate of CVD was explained by geographic region, type of study design, sample source, age composition, and controlled confounders. Conclusions This meta-analysis of cohort studies of CHD found an association of increased risk of CVD in later life, although we cannot determine whether this association is confounded by a risk factor profile of CVD among CHD survivors or whether CHD is an independent risk factor.

Technical and medical improvements for congenital cardiac disease in children have contributed to an increasing population of patients who survive into adulthood. These patients may be prone to progression of their native palliated disease or suffer from sequelae of their childhood repair that requires repeat surgical intervention. Surgery for adult congenital cardiac disease poses unique challenges and risks.

The adult congenital heart diseases (ACHD) population is exceeding the pediatric congenital heart diseases (CHD) population and is progressively expanding each year, representing more than 90% of patients with CHD. Of these, about 75% have undergone surgical and/or percutaneous intervention for palliation or correction. Autopsy can be a very challenging procedure in ACHD patients. The approach and protocol to be used may vary depending on whether the pathologists are facing native disease without surgical or percutaneous interventions, but with various degrees of cardiac remodeling, or previously palliated or corrected CHD. Moreover, interventions for the same condition have evolved over the last decades, as has perioperative myocardial preservations and postoperative care, with different long-term sequelae depending on the era in which patients were operated on. Careful clinicopathological correlation is, thus, required to assist the pathologist in performing the autopsy and reaching a diagnosis regarding the cause of death. Due to the heterogeneity of the structural abnormalities, and the wide variety of surgical and interventional procedures, there are no standard methods for dissecting the heart at autopsy. In this paper, we describe the most common types of CHDs that a pathologist could encounter at autopsy, including the various types of surgical and percutaneous procedures and major pathological manifestations. We also propose a practical systematic approach to the autopsy of ACHD patients.

We experienced a 33-year-old patient with D-looped transposition of the great arteries (D-TGA) and a history of Senning operation who was referred to our institute with cardiogenic shock and subsequently underwent urgent paracorporeal ventricular assist device (VAD) implantation, which was a first in Japan, that was eventually converted to a durable VAD. Central venous pressure was maintained relatively high to obtain VAD filling and recover end-organ dysfunction, given the migration of the inflow cannula due to rich trabeculae carneae of the anatomical right ventricle (systemic ventricle in this case).

There are limited data on the long-term development of neoaortic root dilatation (NRD) and neoaortic valve regurgitation (AR) after arterial switch operation (ASO) for transposition of the great arteries during adult life.

We performed a retrospective longitudinal analysis of 152 patients older than 15 years who underwent ASO for transposition of the great arteries and who were followed-up for 4.9±3.3 years in 2 referral centers. Sequential changes in body surface-adjusted aortic root dimensions and progression to moderate/severe AR were determined in patients with 2 or more echocardiographic examinations. Risk factors for dilatation were tested by Cox regression to identify predictors of AR progression.

At baseline, moderate AR was present in 9 patients (5.9%) and severe AR in 4 (2.6%), of whom 3 had required aortic valve surgery. Initially, the median neoaortic root dimension was 20.05±2.4mm/m², which increased significantly to 20.73±2.8mm/m² (P <.001) at the end of follow-up. The mean change over time was 0.14mm/m²/y (95%CI, 0.07-0.2). Progressive AR was observed in 20 patients (13.5%) and 6 patients (4%) required aortic valve surgery. Progressive AR was associated with bicuspid valve, AR at baseline, NRD at baseline, and neoaortic root enlargement. Independent predictors were bicuspid valve (HR, 3.3; 95%CI, 1.1-15.2; P=.037), AR at baseline (HR, 5.9; 95%CI, 1.6-59.2; P=.006) and increase in NRD (HR, 4.1 95%CI, 2-13.5; P=.023).

In adult life, NRD and AR progress over time after ASO. Predictors of progressive AR are bicuspid valve, AR at baseline, and increase in NRD.

The arterial switch operation (ASO) for transposition of the great arteries has excellent survival, but a substantial number of patients suffer from a reduced exercise capacity. The goal of this study was to identify imaging parameters associated with a reduced exercise capacity in patients after ASO.

A retrospective analysis was performed of ASO patients who underwent cardiopulmonary exercise testing (CPET) between 2007 and 2017. Reduced exercise performance was defined as a reduced workload peak (W_peak) with Z-score <-2 or a peak oxygen uptake indexed for weight (VO_2peak/kg) with Z-score <-2. Data on echocardiography and cardiac magnetic resonance performed within 1 year of the CPET were collected for comparison.

A total of 81 ASO patients (age 17±7 years) were included. Reduced exercise performance was found in 22 patients (27%) as expressed by either a reduced W_peak and/or a reduced VO_2peak/kg. Main pulmonary artery gradient and tricuspid regurgitation gradient by echocardiography were found to be associated with reduced W_peak (p=0.031; p=0.020, respectively). The main pulmonary artery gradient and tricuspid regurgitation gradient by echocardiography were found to be associated with reduced VO_2peak/kg (p=0.009; p=0.019, respectively). No left ventricular parameters were found to be associated with abnormal exercise performance.

This study demonstrates that ASO patients frequently experience reduced exercise capacity. Echocardiographic evidence of main pulmonary artery stenosis and increased right ventricular pressure were associated with reduced exercise capacity, and are therefore key to monitor during serial follow-up of ASO patients.

Arterial switch operation (ASO) is today the first-choice surgical treatment for patients with transposition of the great arteries. Long-term outcome data still remain scarce. Moreover, the course of these patients is not uneventful. Therefore, we wanted to evaluate long-term outcome and determine on which variables to focus during follow-up.

Clinical records of 318 patients who underwent ASO between October 1981 and July 2018 were reviewed. Perioperative, post-operative, and interventional data were collected to determine mortality and the need for re-intervention. Descriptive statistics and Kaplan-Meier survival analysis were performed.

Mean follow-up time was 11.1 SD 8.5 years (range 0-35) with a mean age of 12.5 SD 9.0 years (range 0-37) at latest follow-up. In-hospital mortality was 7.5% and overall survival 90.9% for a maximum follow-up time of 35 years. Causes of early mortality were cardiogenic shock, severe pulmonary hypertension, septic shock and multiple organ failure. Causes of late mortality were cardiogenic shock, severe pulmonary hypertension, pacemaker lead fracture and fire death. Re-intervention free survival at 5-year was 91.6%, at 10-year 90.7%, at 20-year 79.2%. For all survivors, the most frequent sequelae after ASO were pulmonary artery stenosis (80.9%), of which 13.5% needed an intervention. The threshold for intervening on lesions at the level of the pulmonary artery bifurcation was higher and the percutaneous re-intervention rate was higher for non-bifurcation lesions.

Despite a relatively high peri-operative mortality, TGA patients have an excellent overall long-term survival. However, a large proportion of patients requires re-interventions, mainly for pulmonary artery stenosis.

We tested the hypothesis that left ventricular (LV) myocardial stiffness is altered in patients with transposition of great arteries (TGA) after arterial switch operation (ASO) and explored its associations with myocardial calibrated integrated backscatter (cIB) and LV myocardial deformation. Thirty-one patients and twenty-two age-matched controls were studied. LV myocardial stiffness was assessed by diastolic wall strain (DWS) and stiffness indices including (E/e)/LV end-diastolic dimension, (E/LV global longitudinal early diastolic strain rate)/LV end-diastolic volume, and (E/LV global circumferential early diastolic strain rate)/LV end-diastolic volume, where E and e are early diastolic transmitral and mitral annular velocities, respectively. LV myocardial cIB and longitudinal and circumferential myocardial deformation were determined by conventional and speckle tracking echocardiography. Patients had significantly lower DWS, higher stiffness indices, and greater myocardial cIB than controls (all p < 0.05). The LV longitudinal and circumferential systolic strain and systolic and diastolic strain rates were significantly lower in patients than controls (all p < 0.05). Greater average myocardial cIB was associated with lower DWS (r = - 0.44, p = 0.002). Worse DWS and LV stiffness indices were found to correlate with lower mitral annular systolic velocity, mitral annular late diastolic velocity, and LV longitudinal late diastolic strain rate (all p < 0.05). LV longitudinal and circumferential systolic strain and strain rate were also found to correlate with DWS (all p < 0.05). In conclusion, LV myocardial stiffening occurs in adolescents and young adults with TGA after ASO and is associated with impairment of ventricular systolic and diastolic myocardial deformation and myocardial fibrosis.

Transposition of the great arteries (TGA) may present as a life-threatening neonatal malformation. Although prenatal detection facilitates the perinatal management, the impact on outcome is controversial.

This study reviewed the differences in prenatal diagnosis of TGA from 2009 to 2014 among the 5 geographic areas in Ontario and compared the management, morbidity, and mortality among neonates with a prenatal (prenatal cohort; n = 70) vs a postnatal (postnatal cohort; n = 76) anomaly diagnosis. Cases were identified from prospective databases of the provincial cardiac tertiary centres and the coroner's office.

Prenatal TGA detection rates varied significantly among areas (median: 50%; range: 14% to 72%; P = 0.03). Compared with the postnatal cohort, time from birth to tertiary care admission (1.4 vs 10.4 hours, P < 0.001), prostaglandin therapy (0.1 vs 5.3 hours; P < 0.001), balloon atrial septostomy (5.3 vs 14.9 hours; P <0.001), and arterial switch operation (6 vs 9 days, P = 0.002) was significantly shorter in the prenatal cohort. Although other preoperative variables-including the need of ventilation and mechanical support, morbidity score, and lowest pH and preductal oxygen saturations-were comparable, a prenatal diagnosis was associated with improved 1-year survival (odds ratio: 0.108; 95% confidence interval, 0.013-0.88; P = 0.0184).

Prenatal diagnosis of TGA significantly shortened time intervals from birth to neonatal care and surgery and was associated with improved survival. The prenatal detection rate of TGA in Ontario was low (50% or less) outside of Metropolitan Toronto, suggesting the need for new strategies to further improve intraprovincial detection rates.

Assess the potential additional benefit from pulse oximetry screening in the early detection of critical congenital heart disease in a country with a well-developed antenatal ultrasound screening programme.

Live-born infants, pregnancy terminations and stillbirths from 20 weeks' gestational age, between 2013 and 2015, with critical cardiac defects defined as primary or secondary targets of pulse oximetry screening were identified. Critical defects were those resulting in the death of a fetus or an infant in the first 28 days after birth, or a defect requiring intervention in the first 28 days.

Two hundred and sixty-eight infants and Fetuses were identified. Antenatal detection rates improved from 69% to 77% over the study period. An associated co-morbidity improved antenatal detection rates. Twenty-seven live-born infants were diagnosed after discharge: 15 aortic arch obstruction (AAO); 10 d-loop transposition of the great arteries (d-TGA), and two total anomalous pulmonary venous drainage (TAPVD). Of these, five with AAO, nine with d-TGA and likely both with TAPVD could potentially have been detected with oximetry screening.

The antenatal detection of critical cardiac anomalies continues to improve in New Zealand. Despite high antenatal detection rates for most lesions, universal postnatal oximetry screening has the potential to improve early detection.

Congenital heart disease (CHD) is present in 0.8-0.9% of live births. Prevalence of CHD is constantly increasing during the last decades in line with the treatment options for patients ranging from the surgical as well to the interventional spectrum. Most of the women with underlying CHD reach adulthood due to excellent surgical and interventional treatment options and most of them desire pregnancy. In Western countries, maternal heart disease is the major cause of mortality during pregnancy, thus risk estimation should be therefore individualized depending on the underlying CHD, functional class and treatment options. Pre-pregnancy counselling is mandatory in all women, especially in patients with high risk but even in patients with low risk, a plan for labor and delivery should be outlined in a heart pregnancy team. This review addresses the key element of pre-counselling, planning and successful disease management in patients with CHD during pregnancy.

The incidence of late coronary complications is reported around 8% after arterial switch operation (ASO) for d-transposition of the great arteries, but the affected patients are usually asymptomatic. Exercise stress test (EST) and myocardial perfusion imaging (MPI) are common non-invasive modalities to screen for silent myocardial ischemia, but their diagnostic reliability in children after ASO is unclear. We retrospectively reviewed asymptomatic patients following ASO with EST, MPI, and coronary imaging studies (CIS) and examined the reliability of each test in identifying abnormal coronary lesions responsible for myocardial ischemia. Thirty-seven asymptomatic patients (24 males; ages 12.7 ± 3.7 years) had EST, in which 27 and 33 patients also underwent MPI and CIS, respectively. Exercise capacity was comparable to the age- and sex-matched 37 controls. In seven patients with angiographically proven moderate to severe coronary abnormalities, only two patients had positive EST and/or MPI, and five patients were negative including one patient who later developed exercise-induced cardiac arrest due to severe proximal left coronary artery stenosis. Two patients with positive EST or MPI showed no corresponding coronary abnormalities by CIS. Occurrence of acquired late coronary abnormalities did not correlate with the original coronary anatomy or initial surgical procedures. There is no single reliable method to identify the risk of myocardial ischemia after ASO. Although CIS are regarded as a gold standard, multidisciplinary studies are essential to risk-stratify the potential life-threatening coronary lesions after ASO in children.

Aortic regurgitation (AR) and subclinical left ventricular (LV) dysfunction expressed by myocardial deformation imaging are common in patients with transposition of the great arteries after the arterial switch operation (ASO). Echocardiographic evaluation is often hampered by reduced acoustic window settings. Cardiovascular magnetic resonance (CMR) imaging provides a robust alternative as it allows for comprehensive assessment of degree of AR and LV function. The purpose of this study is to validate CMR based 4-dimensional flow quantification (4D flow) for degree of AR and feature tracking strain measurements for LV deformation assessment in ASO patients.

A total of 81 ASO patients (median 20.6 years, IQR 13.5-28.4) underwent CMR for 4D and 2D flow analysis. CMR global longitudinal strain (GLS) feature tracking was compared to echocardiographic (echo) speckle tracking. Agreements between and within tests were expressed as intra-class correlation coefficients (ICC).

Eleven ASO patients (13.6%) showed AR > 5% by 4D flow, with good correlation to 2D flow assessment (ICC = 0.85). 4D flow stroke volume of the aortic valve demonstrated good agreement to 2D stroke volume over the mitral valve (internal validation, ICC = 0.85) and multi-slice planimetric LV stroke volume (external validation, ICC = 0.95). 2D flow stroke volume showed slightly less, though still good agreement with 4D flow (ICC = 0.78) and planimetric LV stroke volume (ICC = 0.87). GLS by CMR was normal (- 18.8 ± 4.4%) and demonstrated good agreement with GLS and segmental analysis by echocardiographic speckle tracking (GLS = - 17.3 ± 3.1%, ICC of 0.80).

Aortic 4D flow and CMR feature tracking GLS analysis demonstrate good to excellent agreement with 2D flow assessment and echocardiographic speckle tracking, respectively, and can therefore reliably be used for an integrated and comprehensive CMR analysis of aortic valve competence and LV deformation analysis in ASO patients.

Accurate coronary translocation is very important for a successful arterial switch operation (ASO) for transposition of the great arteries (TGA) and to provide good long-term outcomes. We have previously reported the "bay window" technique as a useful option for coronary translocation with excellent midterm results. However, the long-term results of this technique and the morphological changes in the coronary channel have not yet been reported.

Between September 2001 and December 2012, 33 patients with TGA underwent coronary translocation using the bay window technique concomitantly with ASO. The diagnoses were TGA with intact ventricular septum in 21, TGA with ventricular septal defect in 7, and Taussig-Bing anomaly in 5. The median age of the patients at operation was 12 days, and their median body weight was 2.93 kg. Coronary artery patterns were as follows: Shaher and Puddu's type 1 in 23, single coronary artery in 4, intramural type in 1, and others in 5.

The median follow-up period was 4.5 years. There were no operative deaths, but there was one late death because of sepsis 13 months after ASO. Coronary artery evaluation with angiography, computed tomography, or myocardial scintigraphy was performed in 22 patients. There was no abnormal coronary morphology or perfusion. Coronary channel dilatation was not observed. Three patients underwent reoperation for pulmonary stenosis.

The bay window technique provides excellent long-term results in ASO for TGA. Three-dimensional computed tomography revealed no aneurysmal changes in the bay window channel at nine-year follow-up examination.

To establish the impact that timing of diagnosis and place of birth have on neonatal outcomes in those with readily treatable critical congenital heart disease.

This was a population-based study with a complete national cohort of live-born infants with transposition of the great arteries and aortic arch obstruction in New Zealand between 2006 and 2014. Timing of diagnosis, place of birth, survival to surgery, in-hospital events, and neonatal mortality were reviewed. Live births with a gestation of ≥35 weeks and without associated major extracardiac anomalies were included for analysis.

A total of 166 live-born infants with transposition of the great arteries and 87 with aortic arch obstruction were included. Antenatal detection increased from 32% in the first 3 years to 47% in the last 3 years (P = .05). During the same period, neonatal mortality decreased from 9% to 1% (P = .02). No deaths occurred after surgical intervention. An antenatal diagnosis was associated with decreased mortality (1/97 [1%] vs 11/156 [7%]; P = .03) and birth outside the surgical center was associated with increased risk of mortality (11/147 [7%] vs 1/106 [1%]; P = .02). Those with an antenatal diagnosis required fewer hours of mechanical ventilation (P = .02) and had shorter durations of hospital stay (P = .05) compared with those diagnosed >48 hours after birth.

The mortality risk for transposition of the great arteries and critical aortic arch obstruction is greatest before cardiac surgery. Improved antenatal detection allowing delivery at a surgical center is associated with reduced mortality.

Arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA) has gradually replaced the atrial switch operation and has become the standard operation. To date, the outcomes of pregnant women with d-TGA after this new operation have not been investigated. In this study, we investigated the impact of ASO on pregnant outcomes and mid-term prognosis in women with d-TGA and compared with the atrial switch operation through the literature review.

There were 20 pregnancies in 10 women with d-TGA after ASO and 6 resulted in abortion. Among 14 successful pregnancies in 10 women, 11 pregnancies achieved the term delivery and 3 pregnancies, including 1 twin pregnancy, resulted in preterm labor. Maternal cardiovascular events occurred in 4 (heart failure and arrhythmias in 3 and arrhythmia in 1), and all were controllable with medications. Risk factors for the peripartum cardiac events were older age at ASO and delivery, and higher concentration of brain natriuretic peptide (BNP) at first trimester (p<0.05). In 7-60 month-follow-up after delivery, no case showed deterioration of functional class and systemic ventricular function. According to the literature review, women after ASO demonstrated a better prognosis than those after the atrial switch operation.

The majority of women with d-TGA after ASO tolerated pregnancy and delivery well. The older age at ASO, an elderly pregnancy, and higher BNP levels at the first trimester were possibly risk factors of peripartum cardiovascular events among the group. The literature reviews and this study may indicate the advantage of systemic left ventricle compared with systemic right ventricle in long-term outcomes after delivery.

Knowledge of coronary artery nomenclature in transposition of the great arteries is essential, given the increasing population of adults with congenital heart disease and greater utilization of imaging in these patients. This article reviews appropriate terminology for describing coronary artery anatomy, commonly encountered coronary artery patterns, and postoperative coronary complications in the setting of transposition of the great arteries.