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Purpose: To analyze anatomic features and associated malformations in 37 prenatally detected cases of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate the prenatal course, neonatal outcome and mid-term follow-up.Methods: Retrospective analysis of prenatal ultrasound of 37 patients with ccTGA in two tertiary centers between 1999 and 2019. All fetuses received fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports.Results: Isolated ccTGA without associated cardiac anomalies was found in 13.5% (5/37), in all other fetuses additional defects such as VSD (73.0%), pulmonary obstruction (35.1%), tricuspid valve anomalies (18.9%), aortic arch anomalies (13.5%), ventricular hypoplasia (5.4%) or atrioventricular block (5.4%) were present. The rate of extracardiac malformations or chromosomal aberrations was low. There were 91.9% (34/37) live births and postnatal survival rates reached 91.2% in a mean follow-up time of 4.98 years. The prenatal diagnosis of ccTGA was confirmed postnatally in all but one documented live birth and the prenatal counselling regarding the expected treatment after birth (uni- versus biventricular repair) was reassured in the majority of cases. The postnatal intervention rate was high, 64.7% (22/34) received surgery, the intervention-free survival was 36.7%, 35.0% and 25.0% at 1 month, 1 year and 10 years, respectively.Conclusions: ccTGA is a rare heart defect often associated with additional heterogeneous cardiac anomalies that can be diagnosed prenatally. The presented study demonstrates a favorable outcome in most cases but the majority of patients require surgical treatment early in life. [ABSTRACT FROM AUTHOR]

Right ventricular (RV) geometry is altered by septal shift after pulmonary artery banding. This may reduce tricuspid regurgitation (TR) and improve ventricular function in patients with corrected transposition of great arteries and systemic right ventricle. However, banding is risky in sick patients with severe RV failure. There are no predictive models in clinical practice to test this septal shift hypothesis before a risky surgery. A transcatheter model to mimic a pulmonary artery band is presented in corrected transposition of great arteries with failing right ventricle and severe TR. [ABSTRACT FROM AUTHOR]

Congenitally corrected transposition of the great arteries is not uncommonly associated with Ebstein's malformation of its left-sided tricuspid valve. The recognition of this rare association in the fetus is important because its postnatal prognosis is guarded. The present case report illustrates fetal echocardiographic clues to the diagnosis that include mesocardia and apically displaced attachment of the left-sided atrioventricular valve to the ventricular septum at four-chamber view. [ABSTRACT FROM AUTHOR]

Congenitally corrected transposition of the great arteries (ccTGA) is an uncommon cardiac defect characterized by the atria connecting with the anatomically discordant ventricles and the ventricles connecting with discordant and transposed great arteries. Parallel vessels are evident in corrected TGA, but as this sign is also present in complete TGA, a heart anomaly requiring major cardiac surgery in the postnatal period, it is important to differentiate between the entities prenatally. Most cases of ccTGA have associated anomalies but isolated forms or those with a mild associated cardiac anomaly are infrequently detected prenatally. We report on three cases detected between 21 and 25 weeks' gestation on screening ultrasound with associated mild findings. One fetus had an isolated ventricular septal defect (VSD) first detected at 34 weeks. The child developed heart block at 4 years of age. The second case was associated with a small VSD, a tiny pulmonary trunk and a persistent right umbilical vein. After birth, mild pulmonary stenosis was found as an additional cardiac finding at 4 months of age. The third fetus had no additional cardiac anomalies prenatally, but after birth a bicuspid aortic valve was detected. The first case needed pacemaker implantation but the other two children required no cardiac surgery. Two of the cases were referred because abnormal vessel anatomy was detected on screening ultrasound. As prenatal detection of TGA is becoming a more frequent occurrence, this paper aims to present clues aiding in the prenatal diagnosis of atrioventricular and ventriculoarterial discordance, especially in its differentiation from complete transposition. These details are crucial for counseling and perinatal management. [ABSTRACT FROM AUTHOR]

Limited information is available concerning the long-term survival of patients with atrioventricular discordance, ventriculoarterial discordance, and two ventricles (corrected transposition). The long-term follow-up of 107 patients examined at the Mayo Clinic over a 30-year period between 1951 and 1981 was reviewed. Overall survival from the date of Mayo Clinic diagnosis was 70% at five years and 64% at ten years. Associated variables were analyzed for their effect on survival including sex, age at diagnosis, presence of ventricular septal defect, pulmonary stenosis, dextrocardia, left atrioventricular valve insufficiency, and complete heart block. There was no significant difference between those with and without a ventricular septal defect (VSD). Pulmonary stenosis was protective when a VSD was present but was not a significant predictor of long-term survival. The only variable that consistently correlated with decreased survival was left atrioventricular valve insufficiency ( p<0.04 for univariate and stepwise Cox, and p=0.08 for multivariate analysis). A logistic model for survival after open-heart surgery failed to identify any significant variable. We conclude that the presence of left atrioventricular valve insufficiency in association with atrioventricular discordance significantly alters the long-term outcome. Atrioventricular valve replacement should be considered in such patients when insufficiency becomes hemodynamically significant. [ABSTRACT FROM AUTHOR]

This report describes a case of corrected transposition of the great arteries (TGA) in which a classic subaortic membrane resulted in significant obstruction to outflow from the morphologically right ventricle. To our knowledge, discrete subaortic obstruction has not been previously reported with corrected TGA. [ABSTRACT FROM AUTHOR]