Background Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to tracheo-esophageal compression. Circumflex retro-esophageal aortic arch is an extreme form of true vascular ring. It remains an uncharted territory to many surgeons. Methods A comprehensive search of peer reviewed journals was completed based on the key words, “Circumflex aorta,” “Circumflex retro-esophageal aorta” and “circumflex arch” using Google scholar, Scholars Portal Journals and PubMed. The reference section for each article found was searched to obtain additional articles. Literature on the circumflex aorta was reviewed starting from the embryogenesis to the latest management strategies. Results Right circumflex aorta is more prevalent compared to left circumflex aorta. It can occur in isolation or in association with other intracardiac lesions. Mainly presents in children, however reported in adults too. The presentation may vary from asymptomatic lesion to acute respiratory distress secondary to airway compression. Computerized tomography (CT) and magnetic resonance imaging (MRI) are important tools in delineating the vascular anatomy. Aortic uncrossing is the definitive procedure. However, the role of concomitant tracheobronchopexy is emerging. Native tissue-to-tissue anastomosis is commonly preferred, but cases of extra-anatomic grafts are reported. Conclusion Circumflex aorta is amenable to complete repair. Preoperative delineation of anatomy is important for successful surgical outcome. Division of the retro-esophageal segment is crucial in relieving the compressive symptoms. In addition, tracheobronchopexy is helpful in addressing residual tracheomalacia but this accounts for a high-risk surgery.

Objective To analyze the incremental benefit of 3D/4D spatiotemporal image correlation (STIC) fetal echocardiography over 2D fetal echocardiography with respect to the accuracy of identification of anatomic details crucial for surgical decision‐making and in predicting surgical approach in fetuses with double‐outlet right ventricle (DORV). Methods This was a retrospective study of fetuses with DORV which had undergone both 2D echocardiography and 3D/4D STIC echocardiography and which underwent surgery postnatally in a tertiary pediatric cardiac center in Kerala between October 2015 and March 2019. All such cases with normal atrial arrangement, concordant atrioventricular connections and balanced ventricles were included. 2D and 3D/4D STIC fetal echocardiographic data were analyzed by two experienced observers blinded to the other dataset. Anatomic variables crucial for surgical decision‐making, i.e. location and routability of the ventricular septal defect, relationship of the great arteries and presence of outflow obstruction, were compared between the two modalities with respect to agreement with postnatal echocardiography. The accuracy of prenatal prediction of the surgical pathway was compared between 2D and 3D/4D modalities with respect to the procedure undertaken. Results Included in the study were 22 fetuses with DORV which had undergone both 2D and 3D/4D imaging as well as postnatal surgery. Accuracy of prenatal interpretation of all four anatomic variables was significantly higher using 3D/4D STIC than using 2D fetal echocardiography (19/22 (86.4%) vs 8/22 (36.4%), P < 0.001). Surgical procedures included single‐stage repair in 14 (63.5%) patients and a multistage approach in eight (36.4%). Prenatal prediction of the surgical pathway was significantly more accurate on 3D/4D STIC than on 2D echocardiography (20/22 (90.9%) vs 12/22 (54.5%), P = 0.021). Prenatal predictive accuracy of single‐stage biventricular repair was significantly better for 3D/4D STIC than for 2D echocardiography (14/14 (100%) vs 8/14 (57.1%), P = 0.04). Conclusion Addition of 3D/4D STIC to conventional 2D fetal echocardiography confers incremental benefit on the accuracy of identification of anatomic details crucial for surgical decision‐making and the prediction of postnatal surgical approach in fetuses with DORV, thereby potentially aiding prenatal counseling. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

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Background Recent advances in fetal ultrasound imaging, especially four‐dimensional (4D) spatio‐temporal image correlation techniques permit detailed evaluation of the fetal venous system and its abnormalities. In this report, we present the clinical presentation, conventional/4D spatio‐temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in southern India. Objectives To report the clinical presentation, conventional/4D spatio‐temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in Southern India. Methods Retrospective study (October 2017 to March 2022). All fetuses referred with abnormal cardiac imaging findings who were diagnosed to have anomalies of systemic venous return in the absence of significant intracardiac defects were included. Imaging techniques like 2D with color and 4D spatio‐temporal image correlation fetal imaging were used to evaluate systemic venous anomalies. Systemic venous anomalies were grouped into cardinal venous anomalies and umbilico‐portosystemic venous anomalies. Results Thirty‐nine fetuses were included; this represented 4.4% of all cardiac anomalies diagnosed during the study period. Cardinal venous anomalies were seen in 29 fetuses (74%); the mean gestation age at diagnosis was 25.5 ± 3.4 weeks. Absent dilated coronary sinus was associated with anomalous drainage of superior caval vein through completely unroofed coronary sinus into the left atrium (n = 3) which resulted in cyanosis after birth in all infants. Among the three cases, one underwent surgery at 6 months of age due to a progressive increase in cyanosis (Spo2 78%) and others are awaiting surgery. A prenatal genetic evaluation was performed in 17 (58.6%). Cardinal venous anomalies were not associated with genetic abnormalities. Live births occurred in all fetuses with cardinal venous anomalies. Umbilico‐portosystemic venous anomalies were seen in 10 (26%). The mean gestation age at diagnosis was 26.5 ± 4.5 weeks. Except in a fetus with the extrahepatic portosystemic shunt (umbilical vein to iliac vein), the genetic evaluation was normal in our cohort. Extracardiac anomalies (n = 3/10; 30%) were seen in both intra (n = 1) and extrahepatic portosystemic shunts (n = 2). Conclusion Anomalies of systemic venous return in the absence of significant intracardiac, extracardiac, and genetic abnormalities carried an overall favorable prognosis in our cohort. Precise characterization of anatomic details using advanced imaging techniques helps in the understanding of this complex three‐dimensional anatomy and in the prognostication of these anomalies.