Double aortic arch (DAA) with subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus (RDA) was not reported before delivery, only in adults with anatomy course findings. We present a case of fetal DAA with subaortic LBCV and RDA using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC).

Abnormalities of the left brachiocephalic vein (LBCVA) are rare and poorly studied prenatally. An association with congenital heart defects (CHD), extracardiac and genetic abnormalities was described. The aim of our study was to estimate the rate and summarize the available evidence concerning prenatal diagnosis, associated anomalies, and outcomes of these anomalies. A systematic literature review was carried out selecting studies reporting on prenatal diagnosis of LBCVA, including unpublished cases from our experience. Frequencies were pooled from cohort studies to calculate prenatal incidence. Pooled proportions were obtained from all the studies including rates of associated CHD, extracardiac or genetic abnormalities and neonatal outcomes. The search resulted in the selection of 16 studies with 311 cases of LBCVA, with an incidence of 0.4% from six cohort studies. CHD occurred in 235/311 (75.6%) fetuses: 23 (7.4%) were major in cases of double, retroesophageal or subaortic course and 212 (68.2%) were minor in cases of absence (always associated with a persistent left superior vena cava) or intrathymic course. Data on other associated outcomes were scarce showing rare extracardiac anomalies (3.5%), rare genetic abnormalities (RASopathies and microdeletions associated with the retroesophageal course), and neonatal outcomes favorable in most cases, particularly in intrathymic forms.

To evaluate the morphology and associated cardiovascular abnormalities in patients with an anomalous subaortic course of brachiocephalic vein on multidetector computed tomography (CT) angiography. A retrospective study was performed at a tertiary referral institute to identify patients with subaortic brachiocephalic vein on multidetector CT (MDCT) angiography using dual source CT scanner between January 2014 and July 2021. The morphology of the subaortic brachiocephalic vein along with the cardiovascular anatomy and associated anomalies were evaluated. Out of 4349 patients who had undergone MDCT angiography for evaluation of congenital heart diseases, we identified 126 (2.9%) patients with subaortic brachiocephalic vein. The subaortic brachiocephalic vein was left-sided in 125 patients while a right-sided subaortic brachiocephalic vein was identified in a patient with left isomerism. Common cardiovascular associations included tetralogy of Fallot (109/126; 88.1%), double outlet right ventricle (8/126; 6.3%) and common arterial trunk (5/126; 3.9%). The presence of a right aortic arch was seen in 78/126 (62%) patients. Some degree of right ventricular outflow obstruction was present in 119/126 (94.4%) patients; pulmonary stenosis was seen in 78 (62%) patients while pulmonary atresia was seen in 41 (32.5%) patients. A subaortic brachiocephalic vein can coexist with various complex congenital heart diseases, most commonly tetralogy of Fallot and commonly associated with right aortic arch and pulmonary stenosis/atresia. It is important to identify this anomalous course of brachiocephalic vein before performing surgical procedures or venous catheterization to avoid potential complications.

Cardiac implantable electronic device (CIED) implantation procedures with transvenous lead placement afford an opportunity to observe vascular anatomic variations. The course of CIED implantation depends largely on morphometric and topographic characteristics of the relevant brachiocephalic vein (BCV), which is the left BCV in the case of lead insertion via the left clavipectoral triangle. This study aims to present left BCV anomalies arising from abnormal systemic vein embryogenesis and encountered during CIED implantation. Venograms obtained during CIED implantation procedures and illustrating left BCV topography/morphometry were analysed retrospectively for two types of anomalies: anomalies of the left BCV itself (data from the period 2014-2018) and a combination of left BCV variations with a persistent left superior vena cava (PLSVC); since the latter instances are rare, the analysed period was longer (2003-2018). Analysis of data from the first, 5-year-long, period included data from a group of 1812 patients and revealed 5 (0.3%) cases of developmental left-BCV anomalies (3 double left BCV and 2 cases of a single subaortic left BCV). The 16-year-long analysed period included 6110 CIED implantation procedures, which showed 12 (0.2%) cases of PLSVC including 4 (33%) cases of left BCV agenesis. The analysed venograms rarely showed isolated left-BCV aberrations (0.3%), with the combination of left-BCV agenesis and PLSVC being much more common (33%). The morphometry and/or topography of aberrant left-BCV may result in difficulties during cardiac lead insertion.

The purpose of this study was to report fetal cases of subaortic and retroesophageal anomalous courses of the left brachiocephalic vein (LBCV) evaluated by fetal cardiac magnetic resonance imaging (MRI). A retrospective review of 7282 fetal cardiac MRI from June 2006 to March 2017, nine cases of anomalous courses of the LBCV were correctly diagnosed by fetal cardiac MRI, one case of abnormal subaortic left brachiocephalic vein (ASLBV) missed by fetal MRI was identified postnatally during further imaging of the TOF. The diagnosis was confirmed postnatally by cardiac CT/MRI. An ASLBV was found in 8 cases, a retroesophageal LBCV was found in 2 additional cases with right aortic arch and aberrant left subclavian artery. 3 of 8 ASLBV cases were with a right aortic arch, 4 ASLBV cases had additional cardiovascular anomalies with one case isolated. 7 of 8 ASLBV and 2 retroesophageal LBCV were correctly diagnosed by fetal cardiac MRI; however fetal cardiac MRI missed 2 cases of associated pulmonary atresia (PA). Prenatal echocardiography (echo) correctly diagnosed five ASLBV and one retroesophageal LBCV as well as associated intracardiac anomalies. Fetal cardiac MRI can be a useful adjunct in the identification of subaortic and retroesophageal anomalous courses of the LBCV prenatally.