transposition of great arteries

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.

OBJECTIVES: Predicting whether balloon atrial septostomy (BAS) will be necessary after birth for fetuses with d-transposition of the great arteries (d-TGA) remains challenging. We sought to determine whether measurements obtained during fetal maternal hyperoxygenation (MH) testing can improve our ability to predict need for postnatal BAS. METHODS: Forty-one mothers carrying fetuses with d-TGA with either intact ventricular septum or small ventricular septal defect measuring <3mm underwent MH testing between 33-38 weeks gestation...

OBJECTIVES: The aims of this study were to evaluate the 16-year experience with  arterial switch operation at Beijing Children's Hospital and to determine early and late mortality and late morbidity, to explore risk factors for late complications and reintervention, and finally to evaluate whether the neoaortic sinotubular junction reconstruction technique reduces late complications of arterial switch operation. METHODS: The clinical data of 185 patients with transposition of the great arteries who underwent arterial switch operation in Beijing Children's Hospital from January 2006 to January 2022 and 30 patients who underwent modified arterial switch operation with neoaortic sinotubular junction reconstruction technique in Fuwai Hospital during the same period were retrospectively analysed...

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...

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35...

BACKGROUND: Adult congenital heart disease (ACHD) services increasingly encounter heart failure (HF) in the ageing ACHD population. Optimal timing of referral for heart transplant (HTx) evaluation in this heterogeneous population is complex and ill-defined. We aim to outline the characteristics and outcomes of ACHD patients referred for HTx from a large Australian ACHD centre. METHOD: Retrospective review of ACHD patients referred for HTx from a primary ACHD centre (1992-2021)...

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OBJECTIVES: This study aimed to evaluate the short-/mid-term outcome of patients with complex dextro-/levo-transposition of the great arteries (d-/l-TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstructions (LVOTO). METHODS: A single-center, retrospective review of all complex d-TGA (n = 85) and I-TGA (n = 22) patients undergoing different surgeries (Arterial switch operation (ASO)+LVOTO-resection (ASO-R), half-turned truncal switch/Mair (HTTS), Nikaidoh and Rastelli) between 05/1990-09/2022 was performed...

BACKGROUND: In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions. OBJECTIVES: We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention. METHODS: TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention...

To evaluate biventricular mechanics by means of echo-derived myocardial work (MW) analysis in patients with a systemic right ventricle (sRV). Comprehensive echo data were collected in all patients with a sRV who underwent transthoracic echocardiography at our tertiary centre between 2020 and 2021 including sRV function indices, global longitudinal strain (GLS) of right and left ventricle (RV/LV), biventricular MW, and atrial strain in those with congenitally corrected transposition of the great arteries (ccTGA)...

BACKGROUND: Unilateral pulmonary artery (PA) stenosis is common in the transposition of the great arteries (TGA) after arterial switch operation (ASO) but the effects on the right ventricle (RV) remain unclear. AIMS: To assess the effects of unilateral PA stenosis on RV afterload and function in pediatric patients with TGA-ASO. METHODS: In this retrospective study, eight TGA patients with unilateral PA stenosis underwent heart catheterization and cardiac magnetic resonance (CMR) imaging...

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.

BACKGROUND: NODAL signaling plays a critical role in embryonic patterning and heart development in vertebrates. Genetic variants resulting in perturbations of the TGF-β/NODAL signaling pathway have reproducibly been shown to cause laterality defects in humans. To further explore this association and improve genetic diagnosis, the study aims to identify and characterize a broader range of NODAL variants in a large number of individuals with laterality defects. METHODS: We re-analyzed a cohort of 321 proband-only exomes of individuals with clinically diagnosed laterality congenital heart disease (CHD) using family-based, rare variant genomic analyses...

OBJECTIVES: Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge. METHODS: We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation...

BACKGROUND: Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes. In acquired heart disease it has been shown that echocardiographic deformation imaging is of superior prognostic value as compared to conventional parameters as ejection fraction...

Acute stroke and the transposition of great arteries are two distinct medical entities that rarely intersect in clinical practice. Acute stroke, a devastating neurological event, occurs due to a sudden interruption of blood flow to the brain, leading to focal neurological deficits. On the other hand, the transposition of great arteries is a congenital heart defect characterized by a complete reversal of the aorta and pulmonary artery, resulting in abnormal blood circulation. Traditionally, transposition of great arteries is diagnosed in infancy and managed with surgical interventions...

• Echo is an essential tool for monitoring long-term complications of D-TGA with ASO. • Multimodality imaging is complementary to echo in the assessment of complications. • 4D flow CMR can assess supravalvular PS post ASO. • Branch pulmonary arteries after LeCompte maneuver can be challenging to image.

• ccTGA accounts for 0.5%-1.4% of patients with congenital heart disease. • Patients with isolated ccTGA can remain undiagnosed for decades. • Very rarely, patients with ccTGA develop symptomatic AS. • Management of severe AS in patients with ccTGA presents unique challenges. • Transcatheter options are limited due to the unique anatomy.

• Periodic surveillance after ASO for TGA with multimodality imaging is recommended. • New aneurysms may be detected in patients with antecedent KD. • KD should be suspected in patients with CAA even without previously known illness.

En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved...