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The transposition of the great arteries (TGA) associated with a complete atrioventricular septal defect is a rare and serious congenital cardiac anomaly. In this report, we describe the successful biventricular repair of a TGA with a complete atrioventricular septal defect in an infant. Due to the low body weight of the patient and a complex coronary pattern anomaly, an arterial switch operation was executed, with the Mee procedure and pulmonary arterial banding as initial palliative measures when the infant was 22 days old and weighed 2.5 kg. Subsequently, atrioventricular septal defect repair using the modified one-patch method was performed when the patient was 1.3 years old and weighed 8.8 kg. Remarkably, the postoperative course of the patient demonstrated no notable incidents. To our knowledge, this is the first time a two-stage strategy was applied to repair these complex defects, presenting a promising approach for managing similar cases in future medical practice. [ABSTRACT FROM AUTHOR]

Pulmonary artery (PA) stenosis is a common complication after the arterial switch operation (ASO) for transposition of the great arteries (TGA). Four-dimensional flow (4D flow) CMR provides the ability to quantify flow within an entire volume instead of a single plane. The aim of this study was to compare PA maximum velocities and stroke volumes between 4D flow CMR, two-dimensional phase-contrast (2D PCMR) and echocardiography. A prospective study including TGA patients after ASO was performed between December 2018 and October 2020. All patients underwent echocardiography and CMR, including 2D PCMR and 4D flow CMR. Maximum velocities and stroke volumes were measured in the main, right, and left PA (MPA, LPA, and RPA, respectively). A total of 39 patients aged 20 ± 8 years were included. Maximum velocities in the MPA, LPA, and RPA measured by 4D flow CMR were significantly higher compared to 2D PCMR (p < 0.001 for all). PA assessment by echocardiography was not possible in the majority of patients. 4D flow CMR maximum velocity measurements were consistently higher than those by 2D PCMR with a mean difference of 65 cm/s for the MPA, and 77 cm/s for both the RPA and LPA. Stroke volumes showed good agreement between 4D flow CMR and 2D PCMR. Maximum velocities in the PAs after ASO for TGA are consistently lower by 2D PCMR, while echocardiography only allows for PA assessment in a minority of cases. Stroke volumes showed good agreement between 4D flow CMR and 2D PCMR. [ABSTRACT FROM AUTHOR]

Complete transposition of the great arteries (TGA) is the most common cause of cyanosis in the first week of life. Prenatal diagnosis helps with counseling, planning delivery, and postnatal management for resource‐rich health services. In a resource‐limited setting, postnatal diagnosis is the norm. This work examines cases of complete TGA in one center in Cambodia without prenatal testing. Twenty‐four cases were studied over 5 years. Complex TGA was frequently seen. The majority had normal coronary anatomy and arterial switch operation (ASO) was performed in most cases with a favorable outcome. Complete transposition of the great arteries is the most common cyanotic congenital heart disease in the first week of life. Typically, the left ventricle is connected to the posteriorly placed pulmonary artery whereas the right ventricle gives rise to the anteriorly placed aorta. [ABSTRACT FROM AUTHOR]

Background This case report details the application of left bundle branch pacing in a patient with congenitally corrected transposition of the great arteries (cc-TGA), a rare congenital heart defect characterized by anatomical complexities that pose unique challenges in the management of device-related complications and heart failure. The patient's history is notable for complex anatomical considerations, cardiovascular implantable electronic device (CIED) infection, and heart failure. Case summary The patient underwent a series of interventions, including treatment for pocket-site infections, abandonment of epicardial leads, and an unsuccessful attempt at trans-catheter leadless pacemaker implantation. Given the patient's complex anatomy and prior CIED infection, traditional pacing methods were deemed unsuitable, leading to the selection of left bundle branch pacing. The lead implantation was guided using 3D electro-anatomical mapping to ensure synchronous physiologic pacing in a patient with heart failure. Discussion The case underscores the heightened risks faced by cc-TGA patients, with a focus on systemic right ventricular dysfunction and pacing-induced ventricular dysfunction. In these patients, ventricular synchrony is critical and can be achieved with biventricular pacing. Physiologic pacing emerges as a promising alternative to cardiac resynchronization therapy (CRT), especially in cases where endovascular CRT is unfeasible. This case demonstrates the utilization of 3D electro-anatomical mapping for achieving successful physiologic pacing in complex congenital heart lesions. At the 12-month follow-up, the patient presented with stable clinical status and a narrow QRS complex. Echocardiography indicated improvement in the right systemic ventricular function. [ABSTRACT FROM AUTHOR]

Leadless pacemakers have demonstrated potential as a transvenous pacing option in Adult Congenital Heart Disease patients. Aveir™ single-chamber (VR) leadless pacemakers have demonstrated safety in patients without congenital heart disease in a dual chamber approach. We present a case of dual-chamber pacing using the Aveir dual-chamber (DR) leadless pacemaker in a patient with repaired dextro-transposition of the great arteries with ventricular septal defect (VSD) surgical closure.A 26-year-old male patient with a history of transposition of the great arteries status post arterial switch and VSD repair neonatally had complicated second degree atrioventricular block and sinus node dysfunction necessitating pacemaker placement. Epicardial single-chamber ventricular pacemaker was placed neonatally, which was switched to dual-chamber pacemaker at age 17 due to malfunction. Recent fracture of pacemaker leads led to implantation of new dual chamber leadless pacemaker.Removal of previous pacemaker leads via mechanical extraction occurred and implantation of Aveir DR leadless pacemaker was performed under anesthesia via right femoral vein access without complication. Follow-up demonstrated Aveir VR threshold of 1.0V@0.2 ms, R-wave of 8.9mV, impedance of 490Ω, and the Aveir AR threshold of 0.75V@0.2 ms, P-wave of 3.7mV, and impedance of 400Ω.This case demonstrates safety and efficacy of dual chamber leadless pacemaker implantation in an ACHD patient.

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. [Display omitted] [ABSTRACT FROM AUTHOR]

Abstract Background Transposition of the great arteries (TGA) is the most common cyanotic congenital heart defect in neonates but with low prenatal detection rate. This study sought to review the prenatal diagnosis, associated abnormalities, and mid-term postnatal outcomes of fetuses with TGA and investigate the integrated prenatal and postnatal management for TGA neonates. Methods A total of 134 infants prenatally diagnosed with TGA in Guangdong Provincial People’s Hospital, China, from January 2009 to December 2022 were included in the study. The prenatal ultrasound data and neonatal records were reviewed to assess the accuracy of prenatal diagnosis. Univariate and multivariate logistic and Cox analyses were used to identify risk factors associated with prognosis in such individuals. Results The population originated from 40 cities in 10 provinces in China, with integrated antenatal and postnatal management rate reaching 94.0% (126/134) and a high accuracy rate (99.3%) of prenatal primary diagnosis. The median period of follow-up was 1.6 [interquartile range (IQR) 0.1–4.3] years. There were 3 (2.2%) postnatal deaths, 118 (88.1%) patients undergoing arterial switch operation (ASO), 3 (2.2%) undergoing Rastelli operations and 5 (3.7%) doing stage operations. Of 118 patients receiving ASO, the major morbidity occurred in 64 patients (54.2%), and right ventricular outflow tract obstruction (RVOTO) in 31 (26.3%). In the multivariate logistic analysis, gestational ages at birth (OR = 0.953, 95% CI 0.910–0.991; p = 0.025) and cardiopulmonary bypass (CPB) time (OR = 1.010, 95% CI 1.000–1.030; p = 0.038) were identified as independent risk factors associated with major morbidity. In the Cox multivariate analysis, aortic cross-clamping time (HR = 1.030, 95% CI 1.000–1.050; p = 0.017) was identified as independent risk factor associated with RVOTO. Conclusion Earlier gestational ages at birth and longer CPB time are significantly associated with increased morbidity. Integrated prenatal and postnatal management is recommended for patients with prenatal diagnosis of TGA.

Objectives: Transposition of the great arteries (TGA) is a rare congenital heart disease that occurs in 3 in 10,000 newborns and is rapidly fatal (90%) within one year if left untreated. The prognosis of this pathology changed after introducing an early arterial switch operation (ASO), while the left ventricle could still adapt to systemic high-pressure conditions. Appropriate regulation of thyroid hormones positively impacts metabolism, cardiac function, and postoperative recovery. Therefore, regular thyroid hormone monitoring and thyroid function monitoring of TGA patients may help to improve the health status and prognosis of this group of postoperative patients. Methods: In our study, 127 patients who underwent ASO at our pediatric cardiac surgery clinic between 01.01.2014 and 18.09.2021 were retrospectively analyzed and included. Among the patients, 43% (n=54) were females, and 57% (n=73) were males. Results: The coronary arteries were normal in 89.7% (n=114) and abnormal in 10.3% (n=13) of the patients. Twenty-one of the patients exited, and mortality was calculated to be 16.5%. There were no significant differences in mortality or thyroid stimulating hormone (TSH), free thyroxine (T4), or free triiodothyronine (T3) values (P=0.674, P=0.345, P=0.478). In our study, in which we investigated the effect of thyroid hormone levels on prognosis in neonatal patients with TGA with normal free T3, T4, and TSH values, we found that TSH levels were greater in the group with advanced aortic regurgitation and exitus, although the effect of thyroid hormones on postoperative results was not statistically significant. Conclusions: Congenital hypothyroidism is a common disease with cardiac effects. During the neonatal period, this disease may conceal itself. Careful, expert clinical follow-up and clinical trials are crucial to improve outcomes in the surgical treatment of transposition of the great arteries, a complex congenital heart disease. [ABSTRACT FROM AUTHOR]

Background: Transposition of the great arteries (TGA) is the most common cyanotic congenital heart defect in neonates but with low prenatal detection rate. This study sought to review the prenatal diagnosis, associated abnormalities, and mid-term postnatal outcomes of fetuses with TGA and investigate the integrated prenatal and postnatal management for TGA neonates. Methods: A total of 134 infants prenatally diagnosed with TGA in Guangdong Provincial People's Hospital, China, from January 2009 to December 2022 were included in the study. The prenatal ultrasound data and neonatal records were reviewed to assess the accuracy of prenatal diagnosis. Univariate and multivariate logistic and Cox analyses were used to identify risk factors associated with prognosis in such individuals. Results: The population originated from 40 cities in 10 provinces in China, with integrated antenatal and postnatal management rate reaching 94.0% (126/134) and a high accuracy rate (99.3%) of prenatal primary diagnosis. The median period of follow-up was 1.6 [interquartile range (IQR) 0.1–4.3] years. There were 3 (2.2%) postnatal deaths, 118 (88.1%) patients undergoing arterial switch operation (ASO), 3 (2.2%) undergoing Rastelli operations and 5 (3.7%) doing stage operations. Of 118 patients receiving ASO, the major morbidity occurred in 64 patients (54.2%), and right ventricular outflow tract obstruction (RVOTO) in 31 (26.3%). In the multivariate logistic analysis, gestational ages at birth (OR = 0.953, 95% CI 0.910–0.991; p = 0.025) and cardiopulmonary bypass (CPB) time (OR = 1.010, 95% CI 1.000–1.030; p = 0.038) were identified as independent risk factors associated with major morbidity. In the Cox multivariate analysis, aortic cross-clamping time (HR = 1.030, 95% CI 1.000–1.050; p = 0.017) was identified as independent risk factor associated with RVOTO. Conclusion: Earlier gestational ages at birth and longer CPB time are significantly associated with increased morbidity. Integrated prenatal and postnatal management is recommended for patients with prenatal diagnosis of TGA. [ABSTRACT FROM AUTHOR]

Background: Left atrial (LA) strain, comprising LA reservoir, conduit and contractile function could add mechanistic information of patients after arterial switch operation (ASO) for transposition of the great arteries (TGA). ASO patients might have abnormal ventriculoarterial coupling, which makes them vulnerable to left ventricle (LV) dysfunction and results in reduced exercise capacity. This explorative study aimed to evaluate the relation between LA strain, atrial size, ventricular function, and exercise data obtained by cardiopulmonary exercise testing (CPET). Methods: In a cohort of 44 patients (71% male, mean age 25 ± 4 years) LA strain was measured using transthoracic speckle-tracking echocardiography. Further assessment involved standard echocardiography, CPET evaluation, and blood sampling. LA strain values were compared to normal values. Correlations were calculated. Regression analysis with all strain variables to the CPET data was performed. Results: LA reservoir, conduit and contractile strain were normal in 30%, 89% and 50% of the patients, respectively. LA reservoir/contractile strain correlated to LV ejection fraction (ρ 0.310/−0.159, respectively) and LA reservoir/conduit strain correlated to the LA volume index (ρ 0.336/−0.357, respectively). None of the individual LA strain parameters were associated with the CPET variables. In multivariate regression analysis, LA contractile strain was significantly associated with the percentage of predicted maximal heart rate (β − 2.555). Conclusions: These data suggest that in TGA patients after ASO repair LA strain is impaired and correlates with LA size and LV function. However, impaired LA strain wasn't associated with the standard CPET parameters. As such, clinical significance needs to be further unravelled. [ABSTRACT FROM AUTHOR]

Ventricular remodeling leads to fibrotic changes in systemic right ventricles (RV). Native T1 mapping provides a quantitative measure in myocardial tissue characterization. The aim of our study was to correlate native T1 values of the systemic RV to function and volumetric data. Native T1 maps were generated with a single breath hold Modified Look-Locker Inversion-recovery pulse (MOLLI) sequence was acquired in the mid-ventricular short axis. Regions of interest (ROI) were drawn in both ventricular free walls, the interventricular septum (IVS), superior insertion point (SIP) and inferior insertion point (IIP) to obtain native T1 values. T1 values were compared to CMR ventricular volumes and function using Spearman correlation. The median age was 36 years (IQR 27–48 years). There were elevated mean native left ventricular (LV) T1 and IIP T1 values at 1122 ± 171 ms and 1117 ± 96 ms, respectively. RV dysfunction was associated with elevated IIP T1 (p = 0.007). Significant moderate negative correlations were seen between RV T1 and LV ejection fraction (LVEF) (r= -0.63, p = 0.01), between RV: IVS T1 ratio and LVEF (r= -0.68, p = 0.006), between LVEF and SIP: IVS T1 ratios (r= -0.54, p = 0.04), and RVEF and IIP T1 (r= -0.59, p = 0.02). Fibrosis measured by native T1 mapping in the systemic RV is most prominent in the LV wall and septal insertion point and correlates with decreased function. T1 values can be used in non-invasive imaging assessment of the RV, but further studies with larger cohorts are needed to assess ability to risk stratify and guide therapy. [ABSTRACT FROM AUTHOR]

Background: The Mustard and Senning operations for dextro-transposition of the great arteries (D-TGA) establish a biventricular physiology with a subaortic right ventricle (sRV). While prolonged QRS has been associated with worse prognosis in these patients, current echocardiographic tools fall short in adequately assessing the (mal)performance and function decline of the sRV during follow-up. The present study is the first to characterize Myocardial Work (MW) indices of the sRV in D-TGA patients after Mustard/Senning repair. Methods and results: All adult D-TGA patients at follow-up in the University Hospital of Leuven between 2018 and 2022 were screened for inclusion. We retrospectively collected the most recent electrocardiogram parameters, 2D echocardiographic images and serum biomarkers’ values. Offline calculations of MW indices were performed. We established correlations between all variables and categorized patients into QRS

Background and aims: The objective of this study was to assess the associations of birth weight with cardiac structure and function in adults with dextro-transposition of the great arteries (D-TGA) who underwent the arterial switch operation (ASO). Methods and results: Thirty-nine ASO patients (age 24.4 ± 3.3 years) were included during routine clinical follow-up from July 2019 to December 2021. All patients underwent cardiopulmonary exercise testing and cardiac magnetic resonance imaging at rest and during exercise. Early-life characteristics, including birth weight, were extracted from electronic medical health records. Linear regression analysis showed that lower birth weight was associated with smaller left ventricular (LV) and right ventricular (RV) end-diastolic volume index (LV: −14.5 mL/m2 [95 % confidence interval, CI: −26.5 to −2.5] per 1-kg decrease in birth weight, p = 0.04; RV: −11.2 mL/m2 [-20.7 to −1.7] per 1-kg decrease in birth weight, p = 0.03). Lower birth weight was associated with greater LV and RV ejection fraction at rest (LV: +8.5 % [+4.4 to +12.5] per 1-kg decrease in birth weight, p

Assessment of fetal ventricular function is mostly subjective, and currently, for the objective assessment left ventricular shortening fraction is obtained. However, this by itself is not very reliable. Hence, more tools that can provide an objective assessment are needed to increase the confidence of functional assessment. Speckle tracking imaging can provide one such tool. In this study we sought to establish the normative value of global longitudinal and circumferential strain for our fetal patients and for two major forms of congenital heart diseases, namely atrioventricular canal defects (AVC) and uncorrected dextro-transposition of the great arteries (dTGA) to act as a benchmark. The study was completed via a single center retrospective analysis on 72 fetal echocardiograms (26 normal, 15 dTGA, and 31 AVC). Tomtec Arena™ echocardiography analysis software was used for analysis. In normal fetuses, mean left ventricular (LV) global longitudinal strain (GLS) was − 22.6% (95% CI -24, -21.1) and mean right ventricular (RV) GLS was − 22.1% (95% CI -23.6, -20.6). In AVC patients LV GLS was-26.6% (95% CI -28,-25.3) and mean RV GLS was − 26.5% (95% CI -27.9,-25.2). In dTGA patients LV GLS was − 22.9% (95% CI of -24.8, -21) and RV GLS was − 21.3% (95% CI was − 23.4, -20.8). There was good intra-rater reliability though poor to fair inter-rater reliability. Notwithstanding its current limitations, strain imaging can provide useful information that can increase confidence of cardiac functional assessment in fetal patients. However, to be reliable across the board, further automation and standardization is required. [ABSTRACT FROM AUTHOR]

Introduction: Transposition of the great arteries (TGA) with aortopulmonary window is a rare type of congenital heart disease with limited experience. We reported a neonate aged 25 days receiving the arterial switch operation and assisted with extracorporeal membrane oxygenation. Conclusion: TGA with aortopulmonary window can be safely correctly with the arterial switch operation. [ABSTRACT FROM AUTHOR]

Cardiac surgery on cardiopulmonary bypass (CPB) during the neonatal period can cause perioperative organ injuries. The primary aim of this study was to determine the incidence and risk factors associated with postoperative mechanical ventilation duration and acute lung injury after the arterial switch operation (ASO). The secondary aim was to examine the utility of the Brixia score for characterizing postoperative acute lung injury (ALI). A retrospective study. A single-center university hospital. A total of 93 neonates with transposition of great arteries with intact ventricular septum (dTGA IVS) underwent ASO. None. From January 2015 to December 2022, 93 neonates with dTGA IVS were included in the study. The cohort had a median age of 4.0 (3.0-5.0) days and a mean weight of 3.3 ± 0.5 kg. About 63% of patients had ≥48 hours of postoperative mechanical ventilation after ASO. Risk factors included prematurity, post-CPB transfusion of salvaged red cells, platelets and cryoprecipitate, and postoperative fluid balance by univariate analysis. The larger transfused platelet volume was associated with the risk of ALI by multivariate analysis. The median baseline Brixia scores were 11.0 (9.0-12.0) and increased significantly in the postoperative day 1 in patients who developed moderate ALI 24 hours after admission to the intensive care unit (15.0 [13.0-16.0] v 12.0 [10.0-14.0], p = 0.046). Arterial switch operation results in a high incidence of ≥48-hour postoperative mechanical ventilation. Blood component transfusion is a potentially modifiable risk factor. The Brixia scores also may be used to characterize postoperative acute lung injury. [ABSTRACT FROM AUTHOR]

Background A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all‐cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model. Methods and Results A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18–30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty‐five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5‐year risk patients (Harrell C index of 0.73 [95% CI, 0.65–0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03–0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function. Conclusions We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.

A 31-year-old woman with transposition of the great arteries status post-Senning operation presents with severe pulmonary venous baffle obstruction. Both standards of care (percutaneous stenting or open repair) were deemed suboptimal and/or high risk. A multidisciplinary, hybrid approach via subxiphoid incision, guided by 3-dimensional modeling, provided a lower risk and minimally invasive intervention.

Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks + 6 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (>85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.

We present a case of anatomic repair of dextro-transposition of the great arteries (d-TGA) with ventricular septal defect (VSD) in a 55-year-old man who presented with acute heart failure. This case highlights the importance of multimodal imaging and multidisciplinary involvement in developing a comprehensive surgical and medical plan for adults with congenital heart disease. We think this is the oldest reported patient undergoing anatomic surgical repair of d-TGA with VSD.

The article discusses the results of the PARACYS-RV trial, which aimed to compare the effectiveness of sacubitril/valsartan (an angiotensin receptor-neprilysin inhibitor) to placebo in adults with congenital systemic right ventricular (sRV) heart failure. The trial was prematurely terminated due to safety concerns, as there was a high rate of worsening heart failure events in patients who received placebo compared to those who received sacubitril/valsartan. Although the trial was inconclusive regarding the efficacy of sacubitril/valsartan, the results showed promising trends in improving exercise duration and reducing levels of N-terminal pro-B-type natriuretic peptide. The article emphasizes the need for further research and suggests future trials should avoid crossovers to placebo and discontinuation of other heart failure medications. [Extracted from the article]

Objective: The purpose of this study is to compare the long-term outcomes of 2 different tricuspid surgeries including valvuloplasty and replacement for significant tricuspid regurgitation in patients with systemic right ventricle. Method: This is a retrospective study of 34 patients with dextro-transposition of the great arteries or levo-transposition of the great arteries with biventricular circulation and systemic right ventricle undergoing tricuspid valve surgery between April 1979 and April 2022. Patients were divided into 2 groups based on the procedure: tricuspid valvuloplasty (n = 11) and tricuspid valve replacement (n = 23). These groups were compared in terms of survival, tricuspid valve dysfunction, and tricuspid valve–related reoperation. Results: There was no significant difference between the groups in operative age, body weight, the proportion of dextro-transposition of the great arteries, Ebstein-like tricuspid dysplasia, and preoperative right ventricular volume/function. During the median follow-up of 9.7 years, there was 1 early death (tricuspid valvuloplasty group) and 4 late deaths (3 in tricuspid valvuloplasty group and 1 in tricuspid valve replacement group). There were 7 tricuspid valve dysfunctions, including 6 significant tricuspid regurgitations in the tricuspid valvuloplasty group and 1 prosthetic valve dysfunction in the tricuspid valve replacement group, and 4 tricuspid valve-related reoperations (3 in the tricuspid valvuloplasty group and 1 in the tricuspid valve replacement group) were performed. There were significant differences between the groups in survival (tricuspid valvuloplasty vs tricuspid valve replacement: 72.7 vs 94.7% at 10 years after surgery, P = .0328) and cumulative incidence of tricuspid valve dysfunction at 10 years after tricuspid surgery (tricuspid valvuloplasty vs tricuspid valve replacement: 27.3% vs 0%, P = .0121). Conclusions: Tricuspid valve replacement provided better long-term survival and tricuspid function in patients with systemic right ventricle compared with tricuspid valvuloplasty.

Peng An,1,2,&ast; Lina Song,1,&ast; Ping Song,3,&ast; Junyan Zhang,1,3 Yong Lin,1,3 Guoyan Feng,1 Junjie Liu3 1Department of Radiology, Xiangyang No.1 People’s Hospital, Hubei University of Medicine, Xiangyang, 441000, People’s Republic of China; 2Hubei Provincial Clinical Research Center for Accurate Fetus Malformation Diagnosis, Hubei University of Medicine, Xiangyang, Hubei Province, 441000, People’s Republic of China; 3Department of Obstetrics and Gynecology, Xiangyang No. 1 People’s Hospital, Hubei University of Medicine, Xiangyang, 441000, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Junjie Liu; Guoyan Feng, Xiangyang NO.1 People’s Hospital, No. 15 Jiefang Road, Fancheng District, Xiangyang City, Hubei Province, People’s Republic of China, Email liujj2023@yeah.net; fengguoyan2022@yeah.netObjective: The present study aimed to explore the role of modified vascular anatomical molding (MVAM) in prenatal diagnosis teaching and prognosis prediction of fetal complex congenital heart disease (CCHD).Methods: Step 1, MVAM method was used to cast the micro-blood vessels and trachea of 52 CCHD specimens. Subsequently, 52 MVAMs were analyzed and compared with the prenatal ultrasound to summarize their characteristics, misdiagnosis and MVAM’s teaching role. Step 2, the surgical and follow-up data of 206 CCHD cases were retrospectively analyzed. Cases that evolved into critical illnesses or died within 1– 3 years after surgery (poor prognosis) were classified into the study group (n = 77) and those with good prognosis into the control group (n = 129), which were split into the training set and the test set in the ratio 7:3 based on the time cut-off. In the training set, the prognosis of CCHD was predicted using the MVAM anatomical soft markers (distortion and narrowing of aorta/pulmonary artery, right ventricular infundibulum, etc.) and the decision curve analysis (DCA) performed. The model was validated using the test set, and a nomogram was finally established.Results: It was observed that all 52 CCHD cases were confirmed using MVAM. A total of 91 cardiac malformations were recorded, among which 41 malformations were misdiagnosed, and 29 malformations were missed by the prenatal echocardiography. The MVAM method has a good teaching/feedback effect on prenatal diagnosis. The combined model exhibited a higher predictive performance in the training- and test-set. Its high clinical net benefit was proved by DCA. Additionally, the nomogram established using the combined model received a favorable response in clinical practice.Conclusion: The research results indicated that MVAM improved the prenatal diagnosis teaching and training performance. The combined model established based on MVAM anatomical soft markers can offer a high clinical significance for prognosis prediction of CCHD.Keywords: modified vascular anatomical molding, MVAM, complex cardiovascular malformation, CCHD, combined model, CM, interrupted aortic arch, IAA, coarctation of the aorta, COA, pulmonary valve stenosis, PVS, right aortic arch, RAA, transposition of the great arteries, TGA, right aortic arch with mirror-image branching, RAMI, double-outlet right ventricle, DORV

ObjectiveThis study aimed to assess the diagnostic value of prenatal echocardiography for identifying transposition of the great arteries (TGA) during pregnancy and evaluating the associated outcomes.MethodsWe conducted a retrospective analysis of 121 prenatally diagnosed patients with TGA at our hospital between January 2012 and September 2022. This analysis included prenatal ultrasound, prenatal screening, clinical management and follow-up procedures.ResultsAmong the 103 fetuses considered in the study, 90 (87.4%) were diagnosed with complete transposition of the great arteries (D-TGA), while 13 (12.6%) exhibited corrected transposition of the great arteries (CC-TGA). Diagnoses were distributed across the trimester, with 8 D-TGA and 2 CC-TGA patients identified in the first trimester, 68 D-TGA patients and 9 CC-TGA patients in the second trimester, and 14 D-TGA and 2 CC-TGA patients referred for diagnosis in the third trimester. Induction of labour was pursued for 76 D-TGA patients (84.4%) and 11 CC-TGA patients (84.6%), and 14 D-TGA patients (15.6%) and 2 CC-TGA patients (15.4%) continued pregnancy until delivery. Among the D-TGA patients, 9 fetuses (10.0%) underwent surgery, two of which were inadvertent fatality, while the remaining seven experienced positive outcomes. Additionally, seven TGA patients received palliative care, leading to four fatalities among D-TGA patients (5.2%), whereas 1 D-TGA patients and 2 CC-TGA patients survived.ConclusionThis study underscores the feasibility of achieving an accurate prenatal diagnosis of TGA during early pregnancy. The utility of prenatal ultrasound in the development of personalized perinatal plans and the application of multidisciplinary treatment during delivery are conducive.

Background: Systemic right ventricular (sRV) physiology occurs in patients with congenitally corrected transposition of the great arteries (ccTGA) and D-TGA post atrial switch repair, and the natural history is of progressive sRV dysfunction. No study has assessed longitudinal changes in sRV remodeling by serial cardiovascular magnetic resonance (CMR). Methods: Patients evaluated at two adult congenital heart disease centers and who underwent ≥2 CMR exams were studied. Indexed sRV end-diastolic volume (sRVEDVi), end-systolic volume (sRVESVi), and ejection fraction (sRVEF) were determined by a core laboratory. Concurrent echocardiograms were assessed for degree of systemic tricuspid regurgitation (sTR). Tricuspid valve events were defined as ≥moderate sTR, or interval tricuspid valve replacement (TVR). Generally, the earliest and most recent studies were compared. A subset of patients were followed with ≥moderate sTR, and then subsequently underwent interval TVR. For these patients, two study time intervals were defined to analyze the impact of each event independently. Results: Sixty-seven patients were studied (33 ± 11 years, 48% [32/67] male, 33% [22/67] ccTGA), with 72 total time intervals studied (median interval 9.0 years [interquartile range 4.6–13.3]). There was a small increase in sRVEDVi over time (ΔsRVEDVi 5.5 ± 15.8 mL/m2, p

Patients with dextro transposition of the great arteries (d-TGA) after atrial switch procedure are at risk to develop heart failure and arrhythmias during long-term follow-up. The present study aims to add knowledge on the fate of subjects after Mustard procedure during long-term follow-up into adulthood. A single center, retrospective chart review analysis was conducted. All subjects who had Mustard-type atrial switch procedure between 1969 and 1994 at our institution were included. A total of 92 subjects were included. Early postoperative death was reported in 2 subjects. Long-term follow-up was available in 49 survivors. Of those, 6 individuals died during further follow-up. Sudden cardiac death was the most prevalent cause for fatal outcome. Mortality during long-term follow-up was associated with the presence of additional cardiovascular malformations (complex d-TGA). Sinus node dysfunction was observed in 65% of the patients and atrial tachyarrhythmias were common in adult survivors (63%). Implantation of a pacemaker or a cardioverter defibrillator was required in 31% and 45% of those surviving into adulthood. Complications were frequently observed during follow-up after either pacemaker or cardioverter defibrillator implantation (43%) with lead failure being the most frequent complication. The aging population of patients after Mustard procedure is facing challenging problems mainly resulting from a failing systemic right ventricle, presence of associated cardiac malformations and the presence of atrial baffles associated with relevant atrial scars. Age, associated cardiac malformations, and atrial tachyarrhythmias seem to play a major role in determining the fate of patients with d-TGA after atrial switch procedures. [ABSTRACT FROM AUTHOR]

Implantation of continuous-flow left ventricular assist device in a narrow lumen is technically challenging to secure an optimal support. We experienced a patient with the transposition of the great arteries after the Senning procedure who was initially implanted with Jarvik 2000®. She presented with worsening heart failure symptoms 2 years after implanting Jarvik 2000®. We assumed that the inflow cannula was stuck in the highly developed trabeculae on the interventricular septum, which disturbed the VAD to maintain an expected support. After converting to the EVAHEART® 2, we successfully obtained an adequate inflow. We consider that the tipless cannula of EVAHEART® 2 is the most suitable when there is no sufficient room to place a conventional inflow cannula in the systemic ventricle. [ABSTRACT FROM AUTHOR]

Aims Patients with systemic right ventricles are at high risk of sudden cardiac death. Arrhythmia is a significant risk factor. Routine Holter monitoring is opportunistic with poor adherence. The aim of this study was to determine if continuous rhythm monitoring with an implantable loop recorder (ILR) could allow early detection of clinically important arrhythmias. Methods and results Implantable loop recorder implantation was offered to patients with atrial switch repair for transposition of the great arteries. Recordings were made with symptoms or, automatically for pauses, significant bradycardia or tachycardia and reviewed by the multi-disciplinary team. Twenty-four out of 36 eligible patients underwent ILR implantation with no complication. Forty-two per cent had preserved ventricular function, 75% were NYHA functional class I, 88% had low sudden cardiac death risk, 33% had previous intra-atrial re-entrant tachycardia (IART), and none had known conduction disease. Eighteen out of 24 (75%) patients made 52 recordings (52% automated) over 39.5 months (1.6–72.5). Thirty-two out of 52 (62%) recordings in 15/24 (63%) of the cohort were clinically significant and included sinus node disease (two patients), atrioventricular block (two patients), IART (seven patients), and IART with sinus node disease or atrioventricular block (four patients). Implantable loop recorder recordings prompted medication change in 11 patients [beta-blockers (n = 9), anti-coagulation (n = 5), and stopping anti-coagulation (n = 1)] and device therapy recommendation in seven patients [five pacemakers (three: atrioventricular block) and two defibrillators]. Two patients declined intervention; one suffered an arrhythmic death. Intra-atrial re-entrant tachycardia and clinically relevant conduction disease were detected in patients irrespective of sudden cardiac death risk. Conclusion Continuous monitoring with an ILR in patients with systemic right ventricle following atrial switch detects clinically relevant arrhythmias that impact decision-making. In this cohort, clinically relevant arrhythmias did not correlate with sudden cardiac death risk. [ABSTRACT FROM AUTHOR]

Abstract Background The coexisting of transposition of the great arteries (TGA) with total anomalous pulmonary venous connection (TAPVC) is one of the rare anomalies. The incidence of coexisting TAPVC and TGA is unknown with very few cases ever reported. Case presentation We reported a case of a 13-month-old female toddler with history of cyanosis. Echocardiography revealed atrioventricular ambiguity with pulmonary atresia, all PVs drain into the innominate vein via vertical vein (VV), ostium secundum atrial septal defect (ASD) and ventricular septal defect (VSD) were observed. The CT scan confirmed co-occurrence of TGA and TAPVC. All four confluence PVs behind the small left atrium (LA), drains into an ascending lateral large VV, coursing to innominate vein without any PV access into LA. The superior vena cava, right atrium and right ventricle (RV) were dilated. The RV is the origin of the aortic root. The aorta continues on the right side, with an arterial connection to the right pulmonary aberrant artery at the level of the aortic arch. Main pulmonary artery originates from LV and appears atretic with only connection to the left pulmonary artery. Large ASD and VSD were identified. Conclusions TGA and TAPVC are a rare combination and should be suspected in mild-cyanotic cases with levocardia with situs solitus. CT angiography is one of the modalities of choice to characterize the vasculature anomalies.

Background: After the arterial switch operation (ASO) for transposition of the great arteries (TGA), neo-aortic dilatation and coronary arterial anomalies, especially an interarterial course and acute coronary artery take-off angle, are commonly found. Long-term follow-up data after ASO is scarce. Aim of this study was to determine the prevalence of neo-aortic dilatation and coronary abnormalities, with special emphasis on acute coronary take-off angle, in adult TGA-ASO patients. Methods: In this retrospective cohort study, all adult TGA-ASO patients with ≥1 CT-angiography (CTA) at the age of ≥16 years were included. Results: Eighty-one patients, 69 % male and median age 21.0 [18.5–22.8] years, were included. At baseline, maximum neo-aortic diameter was 39.2 ± 5.3 mm; 35 (43 %) patients had neo-aortic dilatation (neo-aortic diameter of >40 mm), 22 (27 %) patients had an acute coronary take-off angle (40 mm or a coronary take-off angle of

Introduction: Esophageal atresia (EA) is a common congenital abnormality typically treated through a thoracotomy approach. However, the treatment can be challenging when there are associated congenital cardiovascular anomalies. Herein, we describe the case of a newborn with EA and d-transposition of the great arteries (d-TGA) successfully treated via a two-step median sternotomy approach. Case report: A newborn was born at 37 + 6 weeks gestation (weight of 2660 g) by cesarean section due to congenital heart disease. TGA was diagnosed by fetal echocardiogram. A postnatal contrast-enhanced chest computed tomography (CT) confirmed the diagnosis and additionally detected a type-C EA. The EA repair was done two days after birth via median sternotomy. An arterial switch operation for d-TGA was done twelve days after the EA repair through a repeat median sternotomy. Conclusion: This two-step approach might be suitable for selected patients who have combined congenital cardiovascular malformation and type-C EA.

Background and Aims For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. Methods This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. Results From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P <.001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. Conclusions Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery. [ABSTRACT FROM AUTHOR]

Objectives: Literature on cerebroplacental hemodynamics in fetuses with transposition of the great arteries (TGA) is scarce and provides conflicting results regarding the presence of a brain‐sparing effect. The aims of this study were to examine Doppler parameters in the middle cerebral artery (MCA) and umbilical artery (UA) in a large cohort of fetuses with TGA, and to assess their possible utility in predicting the need for urgent balloon atrial septostomy (BAS) in the neonate. Methods: This was a retrospective observational study of fetuses diagnosed with TGA between 2008 and 2022 and an age‐matched cohort of normal fetuses, conducted in a single tertiary fetal cardiology center. Medical records and echocardiographic examinations were reviewed to collect demographic, sonographic and follow‐up data. Selected Doppler parameters were compared between fetuses with TGA and normal fetuses, as well as between TGA fetuses with and those without an associated ventricular septal defect (VSD), to assess the impact of this congenital heart defect on cerebroplacental circulation. Additionally, Doppler indices in patients with a restrictive foramen ovale (FO) were analyzed to identify potential predictors of the need for urgent BAS. Results: A total of 541 examinations of 159 fetuses with TGA performed between 19 and 40 weeks' gestation and 1300 examinations of 1215 age‐matched normal fetuses were included in the study. MCA pulsatility index (PI) and UA‐PI followed expected trends throughout pregnancy, with slightly higher values observed in TGA fetuses, albeit within the limits for the normal population. Cerebroplacental ratio (CPR) values were similar in normal and TGA fetuses. The presence of a small VSD did not have a clinically significant impact on Doppler parameters. Peak systolic velocity (PSV) in the MCA increased gradually after 35 weeks' gestation, especially in fetuses that did not develop restriction of the FO after birth. MCA‐PSV values below 1.16 multiples of the median measured at 38 weeks or later predicted the need for urgent BAS with 81.4% sensitivity and 52.4% specificity. Conclusions: MCA‐PI, UA‐PI and CPR values in fetuses with TGA usually fall within normal limits throughout pregnancy. The presence of a small VSD does not affect the Doppler parameters significantly. MCA‐PSV increases in TGA fetuses after 35 weeks, and its value measured at the last prenatal examination (ideally after 37 weeks) may serve as an additional predictive factor for the need for urgent BAS. © 2023 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

A 5-week-old male child, weighing 4 kg diagnosed with d-transposition of the great arteries with multiple muscular ventricular septal defects, Yacoub's type-A coronary arterial pattern, successfully underwent arterial switch operation with Dacron patch closure of ventricular septal defects under moderately hypothermic cardiopulmonary bypass and St. Thomas based cold blood cardioplegia under integrated extracorporeal membrane oxygenation. Postoperatively, he required mechanical circulatory assistance for 72 h. At 12 months of follow-up, there was no mitral or tricuspid regurgitation, no neoaortic valve insufficiency with good biventricular function in Ross clinical score of 2. [ABSTRACT FROM AUTHOR]

Patients with d-looped transposition of the great arteries (d-TGA), especially those without an adequate atrial septal defect, can experience severe hypoxemia and hemodynamic compromise in the neonatal period. This can be mitigated by urgent balloon atrial septostomy (BAS). However, some patients with d-TGA are born at centers without this capability. The aim of this retrospective study of d-TGA patients who had urgent or emergent BAS at our institution between 2010 and 2021 was to evaluate time from birth to BAS for infants born at a tertiary care center as compared to those requiring transport from other institutions and to examine correlation between time to BAS and patient outcomes. Our primary outcome was time from birth to BAS. Secondary outcomes included hospital and ICU length of stay, mortality, and evidence of pulmonary or neurologic abnormalities including pulmonary hypertension, abnormal neuroimaging, or seizures. Of 96 patients, 67 (70%) were born at our institution. The median time to BAS was 4 h for patients born at our institution vs. 14.1 h for those born elsewhere (p <.0001). A longer time from birth to BAS was associated with longer ICU (r = 0.21, p = 0.046) and hospital length of stay (r = 0.24, p = 0.02) and increased likelihood of elevated right ventricular pressure on post-operative discharge echocardiogram (p = 0.01). There were no differences in mortality between the groups. Therefore, prenatal planning for patients with known d-TGA should include a delivery plan with access to urgent BAS. [ABSTRACT FROM AUTHOR]

Background Patients with congenitally corrected transposition of the great arteries (ccTGA) are at risk of developing conduction disease and complete atrio-ventricular block and this risk increases after corrective cardiac surgery. However, the optimum pacing modality remains controversial. Case summary Twelve years after a double-switch surgery with ventricular septal defect correction, a 16-year-old ccTGA female was referred with an indication for cardiac resynchronization therapy. In the absence of coronary sinus (CS) or direct access to the conduction system, several therapeutic options were considered. Finally, using a three-dimensional navigation system and customized sheaths, a left bundle branch area pacing (LBBAP) lead was successfully implanted. The implantation resulted in stable pacing parameters and positive haemodynamic changes. At 9-month follow-up, pacing parameters were stable and the patient reported a significant improvement in quality of life. Discussion Cardiac resynchronization therapy in adults with repaired congenital heart disease remains challenging, especially in the absence of CS or direct access to the conduction system. In such a situation, LBBAP appears as an attractive alternative pacing modality. However, pre-operative management is critical to the success of the implantation. [ABSTRACT FROM AUTHOR]

Objective: To report the long-term outcomes of the arterial switch operation for transposition of the great arteries. Methods: The data of 35 patients who underwent an arterial switch operation between April 2006 and May 2021 were retrospectively examined; five had double-outlet right ventricles, three had side-by-side transposition, and 27 had d-transposition. The punch-out technique was the basic method for coronary transplantation; however, if coronary artery kinking after additional peeling was a concern, the trap-door technique was used. In aortic reconstruction, the caliber difference between the neoascending aorta and the native pulmonary artery was fixed using the pulmonary artery flap. In pulmonary reconstruction, the pulmonary artery was bilaterally exposed over the second branch. Results: The median age at operation and follow-up duration were 10.0 (8.0–12.5) days and 72.9 (40.7–139.5) months, respectively. Thirty-four patients survived, and one was lost to follow-up ([coronary transplantation]: right coronary artery, punch-out = 31, trap-door = 2; left coronary artery, punch-out = 28, trap-door = 7; [aortic regurgitation]: no–slight = 27, mild = 6, moderate = 1; [pulmonary regurgitation]: no–slight = 22, mild = 11, moderate = 1). Slight coronary bending occurred in two patients; ischemia was not observed. Catheter stent placement was performed in one of three patients with stenosis at the pulmonary artery bifurcation. Catheter balloon dilation was performed in one patient with supravalvular pulmonary stenosis. There were no cases of coronary, aortic, or pulmonary artery reoperations. The 15-year cumulative reintervention avoidance rate was 91.0%. Conclusions: There were no deaths or reoperations during the long-term follow-up period, and arterial switch operation outcomes at our hospital were satisfactory. [ABSTRACT FROM AUTHOR]