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BackgroundTo date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV.MethodsData on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding.ResultsAmong the 359 patients with an sRV (32.2 (IQR 26.4–38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0–9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV.ConclusionsDespite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.

The arterial switch operation for d-transposition of the great arteries achieves anatomic repair but creates the potential for right ventricular outflow tract obstruction as a result of the LeCompte maneuver. The resultant right ventricular hypertension is generally well tolerated but a select group are referred for cardiac catheterization. The outcomes of these catheterizations have not been well described. The objective of this study was to describe the degree and nature of right ventricular outflow tract obstruction found during cardiac catheterization among patients following the arterial switch operation as well as determine the rate of intervention and assess the acute impact of any catheter intervention undertaken. We conducted a retrospective study of patients after arterial switch operation with the LeCompte maneuver and subsequent right heart catheterization. Descriptive statistics were reported, and paired sample t tests were used for analysis. 544 children had an arterial switch operation, of which 110 children (20%) had a cardiac catheterization procedure after surgery and 11% had a right heart catheterization. Of the right heart catheterizations, 90% had an intervention (balloon and/or stent). In the interventional group, the right ventricle to systemic pressure ratio decreased modestly, from 2/3 to half systemic, after balloon dilation and/or stent placement ( p  < 0.01). No serious complications were observed.

Systemic right ventricle (sRV) dysfunction is frequent in patients with congenitally corrected transposition of great arteries (cc-TGA) and those with dextro-transposition of great arteries (D-TGA) after Mustard/Senning operations. This condition should be identified promptly. We aimed to compare echocardiographic parameters with cardiac magnetic resonce (CMR)-derived parameters in patients with sRV and to evaluate their correlation with clinical variables and exercise capacity. Patients with cc-TGA and D-TGA after Mustard/Senning who underwent standard and advanced (speckle tracking and 3D) echocardiography and CMR (including feature-speckle tracking) were included. Clinical and imaging parameters were collected. Echocardiographic-derived right ventricle end-diastolic area and end-systolic area correlated with 3D echocardiographic-derived right ventricle end-diastolic and end-systolic volume (r=0.6, p=0.006 and r=0.8, p=0.002). 3D ejection fraction (EF) correlated with fractiol area change and tricuspid annular plane systolic excursion (TAPSE) (r=0.8, p=0.001 and r=0.7, p=0.03). sRV global longitudil strain (GLS) correlated with systemic atrial strain (sAS) (r=-0.6, p=0.01). CMR-derived EF correlated with CMR-derived GLS both endocardial and myocardial (r=-0.7, p=0.007 and r=-0.6, p=0.005). sRV areas as assessed by echo correlated with CMR-derived volumes (r=0.9, p=0.0001 for diastole and r=0.8, p=0.0001 for systole). Similarly, a correlation was found between sRV echo-derived GLS and CMR-derived GLS, both endocardial and myocardial (r=0.8, p=0.001 and r=0.7, p=0.01). The only imaging parameter that correlated with peak oxygen consumption was sAS (r=0.55, p=0.04). When comparing cc-TGA and D-TGA, the former showed better GLS-derived values as assessed by CMR (CMR-derived right ventricle endocardial longitudil strain -23.2% versus -17.2%, p=0.002; CMR-derived right ventricle myocardial longitudil strain -21.2% versus -16.7%; p=0.05), bigger systemic atrial area (20.2 cm2/m2 versus 8.4 cm2/m2, p=0.005) and higher TAPSE values (16.2 mm versus 12.2 mm, p=0.04). Echocardiography is valid to screen for sRV dilatation and function and to guide the timing for CMR. The investigation of atrial deformation imaging may help to better understand diastolic function. Patients with cc-TGA show better cardiac function compared to patients after atrial switch. Further investigations are needed to identify imaging parameters linked to exercise capacity.

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. 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. Propensity score matching was also used to match the neoaortic sinotubular junction reconstruction patients in Fuwai Hospital with 30 non-neoaortic sinotubular junction reconstruction patients in Beijing Children's Hospital. There were 13 early deaths (7.03%) and five late deaths (3.01%). Nineteen patients (11.45%) developed new aortic valve regurgitation and 28 patients (16.87%) developed aortic root dilation. Late right ventricular outflow tract obstruction occurred in 33 patients (19.88%). Late reintervention occurred in 18 cases (10.84%). Multivariate analysis showed that aorto-pulmonary diameter mismatch, previous pulmonary artery banding, and mild moderate or above new aortic valve regurgitation at discharge were independent risk factors for late new aortic valve regurgitation and aortic root dilation. Low surgical weight was an independent risk factor specific to new aortic valve regurgitation, and bicuspid native pulmonary valve was an independent risk factor specific to aortic root dilation. Older surgical age and aortic root dilation were independent risk factors for late right ventricular outflow tract obstruction. Older surgical age, operation before 2014, late right ventricular outflow tract obstruction, and late aortic root dilation were independent risk factors for late intervention. Propensity score matching showed that new aortic valve regurgitation and aortic root dilation were not followed up in the neoaortic sinotubular junction reconstruction group, while seven cases of aortic root dilation and five cases of new aortic valve regurgitation occurred in the non-neoaortic sinotubular junction reconstruction group, respectively, and the differences were statistically significant (P = 0.003; P = 0.015). The increased incidence of new aortic valve regurgitation, aortic root dilation, and right ventricular outflow tract obstruction as children age is a major concern outcome in the future and may mean more late reintervention. neoaortic sinotubular junction reconstruction technique may reduce the incidence of new aortic valve regurgitation and aortic root dilation, and improve the late prognosis of arterial switch operation. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with aorto-pulmonary diameter mismatch, previous pulmonary artery banding, mild new aortic valve regurgitation at discharge, low surgical weight, and bicuspid native pulmonary valve structures.

Though initially described in 1971 by Van Praagh, transposition of great arteries with posterior aorta is rarely reported in last two decades. Since the arrangement of great arteries appears like normally related great arteries, a careful echocardiographic evaluation is necessary in patients with clinical features of transposition of great arteries. In majority of cases with this anatomy, arterial switch operation can be performed without the need of Le Compte manoeuvre.

Ductal stenting in late presenters with transposition of great arteries with intact ventricular septum retrains the left ventricle before arterial switch operation. However, the experience is limited for its efficacy and safety. This study aims to highlight the efficacy and safety of ductal stenting for retraining the left ventricle. Eight children with transposition of great arteries-intact ventricular septum and regressed left ventricle underwent ductal stenting. Serial echocardiographic measurements of left ventricle shape, mass, volume, free wall thickness, and function were done, and arterial switch operation was performed once the left ventricle was adequately prepared. Post-operative outcome in terms of duration of mechanical ventilation, ICU stay, and improvement in left ventricle function were monitored. The procedure was successful in all patients. Babies were divided into two groups on basis of age at ductal stenting (group 1 age less than 90 days and group 2 age more than 90 days) and were evaluated for the degree of left ventricle retraining as evidenced by echocardiographic parameters and post-operative variables. The left ventricle posterior wall thickness and mass index after ductal stenting increased significantly in both the groups. Postoperatively, one baby of group two expired after seven days due to severe left ventricle dysfunction. Rest babies had an uneventful post-operative ICU stay with no statistical difference in the duration of invasive mechanical ventilation or ICU stay. On six-month follow-up, all surviving babies were doing well with normal left ventricle function. Ductal stenting is a good alternative measure as compared to surgical procedures for left ventricle retraining in transposition of great arteries with regressed left ventricle.

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.

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.

There are no reports on the performance of the arterial switch operation (ASO) in a normal heart with normally related great vessels. The objective of this study was to determine whether the ASO could be performed in a healthy animal model. Cardiopulmonary bypass (CPB) and coronary translocation techniques were used to perform ASO in neonatal piglets or a staged ASO with prior main pulmonary artery (PA) banding. Primary ASO was performed in four neonatal piglets. Coronary translocation was effective with angiograms confirming patency. Piglets could not be weaned from CPB due to right ventricle (RV) dysfunction. To improve RV function for the ASO, nine piglets had PA banding. All survived the procedure. Post-banding RV pressure increased from a mean of 20.3 ± 2.2 mmHg to 36.5 ± 7.3 mmHg ( p  = 0.007). At 58 ± 1 days post-banding, piglets underwent cardiac MRIs revealing RV hypertrophy, and RV pressure overload with mildly reduced RV function. Catheterization confirmed RV systolic pressures of 84.0 ± 6.7 mmHg with LV systolic pressure 83.3 ± 6.7 mmHg ( p  = 0.43). The remaining five PA banded piglets underwent ASO at 51 ± 0 days post-banding. Three of five were weaned from bypass with patent coronary arteries and adequate RV function. We were able to successfully perform an arterial switch with documented patent coronary arteries on standard anatomy great vessels in a healthy animal model. To our knowledge this is the first time this procedure has been successfully performed. The model may have implications for studying the failing systemic RV, and may support a novel approach for management of borderline, pulsatile left ventricles.

A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age). Cardiomegaly; attenuated peripheral vascular markings.Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles. Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection. Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery. Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90º rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging. Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest.