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Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

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.

In selected patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO), the arterial switch operation (ASO) may be the procedure of choice. This study reviews the clinical outcomes of TGA-VSD-LVOTO patients after ASO and compares mechanisms of LVOTO in this patient group to a historical series of cardiac specimens. This retrospective analysis included all cases with TGA-VSD-LVOTO who underwent ASO between January 1977 and December 2023. Additionally, a series of non-operated cardiac specimens with TGA-VSD-LVOTO was selected and examined for morphological comparison. Eleven patients with TGA-VSD-LVOTO underwent ASO. Eight of them had TGA-VSD, and three had Taussig-Bing anomaly. LVOTO mechanisms were multifactorial, including posteriorly deviated infundibular septum and fibrous tissue masses. Median age at ASO was 0.4 (0.07-1.8) years. Ten patients underwent primary LVOTO relief during ASO; no in-hospital mortality occurred. Two patients died >30 days post-ASO at 3.1 months and 6.0 years. Median follow-up was 19.0 (11.1-26.8) years, all survivors in NYHA class I. The patient without initial LVOTO relief did require reoperation during follow-up for progressive LVOTO at 3.5 months post-ASO. Two patients had moderate residual LVOTO at latest follow-up (gradient 30-50 mmHg). No significant neoaortic valve regurgitation was observed. From the anatomical specimen series, 10 of 33 TGA-VSD-LVOTO specimen were deemed eligible for ASO, revealing similar LVOTO mechanisms as the clinical cases. ASO is feasible in selected patients with TGA-VSD-LVOTO showing good long-term outcomes with preserved neoaortic valve function and no reoperations for LVOTO after initial relief.

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.

Background Pulmonary artery stenosis, neoaortic dilatation, and neoaortic valve insufficiency are among the most frequent complications of the arterial switch operation for repair of dextro-transposition of the great arteries (d-TGA). It remains difficult to predict which patients will require great arterial reintervention. Objective We aimed to characterize hemodynamics within the great arteries using 4D flow MRI in patients with d-TGA after the arterial switch operation. Materials and methods Patients with d-TGA after the arterial switch operation and controls with normal cardiac anatomy who underwent 4D flow MRI between 2012 and 2024 were included in this IRB-approved retrospective cohort study. Controls included patients undergoing MRI for other indications who consented or assented to the addition of a 4D flow sequence, as well as patients who underwent clinically indicated 4D flow MRI and were found to have normal cardiac anatomy and function. Velocity, stasis, kinetic energy, energy loss, wall shear stress, and pulse wave velocity were quantified in the aorta and pulmonary arteries. To compare each parameter between d-TGA patients and controls, unpaired t -tests were used for normally distributed data and Mann–Whitney tests for non-normal data. P  < 0.05 was significant. Results Patients with d-TGA after the arterial switch operation (15.7 years ± 2.4, 2 females) demonstrated significantly higher maximum and mean velocity, maximum and mean kinetic energy, energy loss, and maximum and mean wall shear stress within the pulmonary arteries ( P  < 0.0001 for all parameters) compared with age-matched controls (15.5 years ± 2.4, 14 females). Aortic maximum ( P  = 0.001) and mean ( P  = 0.048) velocity, maximum ( P  = 0.0008) and mean ( P  = 0.003) kinetic energy, energy loss ( P  < 0.0001), maximum wall shear stress in five of six regions (range P  < 0.0001 to P  = 0.002), and mean wall shear stress in three regions (range P  = 0.005 to P  = 0.03) were significantly higher in patients with d-TGA after the arterial switch operation patients compared with age-matched controls. Conclusion Patients with d-TGA after the arterial switch operation demonstrate hemodynamic abnormalities within the great arteries, which may provide insight into the mechanisms underlying postoperative consequences of the arterial switch operation. Graphical Abstract

Background To evaluate the relationship between IL-1α -889C/T (rs1800587), IL-1β -511C > T (rs16944), TNFα -308G > A (rs1800629), TNFα -238G > A (rs361525), IL-6 -174G > C (rs1800795), and IL-6 -572G > C (rs1800796) polymorphisms and the susceptibility to transposition of the great arteries (TGA). Methods A prospective analysis was performed on mothers whose newborns were diagnosed as having TGA. For each case of TGA, a mother who gave birth to a healthy neonate in the same period was randomly selected for the control group. The sample size was calculated before planning the study with 80% power and 5% alpha. Results Twenty-seven mothers whose newborn had TGA anomalies (group 1) and 27 mothers whose newborn had no TGA (group 2) were included in the study. There were no significant differences between the groups in terms of maternal age, pregestational body mass index, gestational age at birth and infant sex ( p  > 0.05). The genotype and allele distributions of IL-1α -889C/T (rs1800587), IL-1β -511C > T (rs16944), TNFα -308G > A (rs1800629), TNFα -238G > A (rs361525), IL-6 -174G > C (rs1800795) and IL-6 -572G > C (rs1800796) gene variants were not different between the two groups ( p  > 0.05). Conclusions There was no relation between IL-1α, IL-1β, IL-6, and TNFα promoter gene polymorphisms and TGA occurrence in our study group. Trial registration : This present prospective case–control study was conducted in Baskent University Hospital, Ankara, Turkey, between May 2020 and November 2021. Ethical approval was obtained from the university’s Clinical Research Ethics Commitee (No: KA20/211) in accordance with the Declaration of Helsinki.

Congenitally corrected transposition of the great arteries (ccTGA) is a complex cardiac abnormality that represents less than 1% of all congenital heart defects. It is characterized by a unique pathophysiology involving both atrioventricular and ventriculoarterial discordance and may occur with or without cardiac abnormalities such as ventricular septal defects, pulmonary stenosis, or tricuspid valve anomalies. A man in his 20s presented with a 3-week history of mild dyspnea during strenuous activities. The patient was diagnosed with isolated ccTGA based on electrocardiogram and echocardiogram findings. However, as a long-term complication of ccTGA, the patient exhibited mild tricuspid regurgitation, mild mitral regurgitation, and right ventricular hypertrophy. The dyspnea was explained by the failing systemic right ventricle. Despite his cardiac anomaly, the patient leads a normal lifestyle, with ongoing monitoring to ensure optimal management of his condition. ccTGA is even rarer in the absence of additional cardiac abnormalities, and its diagnosis could be delayed due to being asymptomatic. Patients must avoid risk factors and that could potentially aggravate their condition. Regular surveillance is imperative for the early detection of potential complications.

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.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women’s and Children’s Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28% (8/29), ccTGA with significant VSD in 31% (9/29), ccTGA with pulmonary obstruction in 24% (7/29) and ccTGA with complex anomalies in 17% (5/29). Tricuspid regurgitation (TR) was observed in 17% (5/29) and heart block (HB) in 10% (3/29) prenatally. Six, that is 21% underwent genetic testing of which one was abnormal. Five extra-cardiac anomalies were reported prenatally and postnatally. Pregnancy was discontinued in five, of which two had moderate TR. There were thirty-one liveborn. Coarctation of the aorta was found in five postnatally but not suspected prenatally. In one, pulmonary stenosis was underestimated; otherwise, prenatal morphology was confirmed. Cardiac interventions were performed in 77% (24/31) liveborn with 39% (12/31) undergoing neonatal intervention. Overall, 6/31 liveborn died including all three with prenatal heart block and one with TR. Estimated survival for all liveborn at 1, 5 and 10 years was 87% (95% CI 76–100%), 83% (95% CI 72–98%) and 80% (95% CI 66–96%) respectively. Accurate prenatal diagnosis of ccTGA is critical for counseling. Early outcomes are favorable with 77% of liveborn undergoing surgery. Fetuses with prenatal diagnosis of complex associated abnormalities, HB and TR appear to do less well.

Transposition of the great arteries (TGA) is a cyanotic congenital heart disease characterized by ventriculoarterial discordance and atrioventricular concordance with the great arteries in a parallel relationship. Prenatal diagnosis of TGA has implications for postnatal outcomes, allowing for planned delivery and perinatal management. Three-dimensional virtual or physical models of fetal TGA allow better understanding of fetal cardiac anomalies by parents and interactive discussion among the multidisciplinary team (obstetricians, pediatricians, maternal-fetal specialists, pediatric cardiologists, and cardiovascular surgeons), as well as continuing medical education.