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Review
. 2022 Mar 24;11(7):1805.
doi: 10.3390/jcm11071805.

Prenatal Diagnosis and Postnatal Outcomes of Left Brachiocephalic Vein Abnormalities: Systematic Review

Affiliations
Review

Prenatal Diagnosis and Postnatal Outcomes of Left Brachiocephalic Vein Abnormalities: Systematic Review

Gerarda Gaeta et al. J Clin Med. .

Abstract

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 A systematic literature review was carried out selecting studies reporting on prenatal diagnosis of LBCVA, including unpublished cases from our experience.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.

Keywords: congenital heart defect; fetal echocardiography; fetal innominate vein; fetal left brachiocephalic vein; fetal venous system; intrathymic left brachiocephalic vein; retroesophageal left brachiocephalic vein; subaortic left brachiocephalic vein; ultrasound.

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Conflict of interest statement

The authors declare no conflict of interest. Authors declare any personal circumstances or interest that may be perceived as inappropriately influencing the representation or interpretation of the reported research results.

Figures

Figure 1
Figure 1
PRISMA flowchart of study selection.
Figure 2
Figure 2
Number and percentage (%) of LBCVA (left brachiocephalic vein anomalies) and CHD (congenital heart defects) associated. LBCVA = left brachiocephalic vein anomalies; CHD = congenital heart defects; VSD = ventricular septal defect; RAA = right aortic arch; PAtr = pulmonary atresia; DA = ductus arteriosus; ALSA = aberrant left subclavian artery; TOF = tetralogy of Fallot; AVSD = atrioventricular septal defect; TA = truncus arteriosus; AbsPulmv = absent pulmonary valve; TAPVC = total anomalous pulmonary venous connection; ARSA = aberrant right subclavian artery; DV = ductus venosus; LSVC = persistent left superior vena cava; SVC = superior vena cava.
Figure 3
Figure 3
Illustration of the normal left brachiocephalic vein (LBCV) anatomy and LBCV anomalies: (a) normal LBCV; (b) dilated LBCV; (c) intrathymic LBCV; (d) absent LBCV with a persistent left superior vena cava (LSVC); (e,f) double LBCV; (g) subaortic LBCV; (h) retroesophageal LBCV. SVCr = right superior; SVCl = persistent left superior vena cava.
Figure 4
Figure 4
Illustration of transverse sections of the upper mediastinum as seen on an ultrasound scan with the left brachiocephalic vein (LBCV) recognizable: (a) normal LBCV; (b) LBCV dilated; (c) intrathymic LBCV; (d) absent LBCV with LSVC; (e,f) double LBCV; (g) subaortic LBCV; (h) retroesophageal LB. SVCr = right superior vena cava; SVCl = persistent left superior vena cava; L = fetal left side; R = fetal right side.

References

    1. Carvalho J.S., Allan L.D., Chaoui R., Copel J.A., DeVore G.R., Hecher K., Lee W., Munoz H., Paladini D., Tutschek B., et al. ISUOG Practice Guidelines (Updated): Sonographic Screening Examination of the Fetal Heart. Ultrasound Obstet. Gynecol. 2013;41:348–359. doi: 10.1002/uog.12403. - DOI - PubMed
    1. Lee W., Allan L., Carvalho J.S., Chaoui R., Copel J., Devore G., Hecher K., Munoz H., Nelson T., Paladini D., et al. ISUOG Consensus Statement: What Constitutes a Fetal Echocardiogram? Ultrasound Obstet. Gynecol. 2008;32:239–242. doi: 10.1002/uog.6115. - DOI - PubMed
    1. Allen H.D., Driscoll D.J., Shaddy R.E., Feltes T.F. Moss & Adams’ Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. Wolters Kluwer Health; Philadelphia, PA, USA: 2013.
    1. Kahkouee S., Sadr M., Pedarzadeh E., Fardin S., Borhani A., Gholami S., Amjad G. Anomalous Left Brachiocephalic Vein: Important Vascular Anomaly Concomitant with Congenital Anomalies and Heart Diseases. Folia Morphol. 2017;76:51–57. doi: 10.5603/FM.a2016.0031. - DOI - PubMed
    1. Morhy Borges Leal S., Andrade J.L., de Souza M., Mussi Soares A., Penha Tavares G.M., Yussef Afiune J., Coimbra Guerra V., Ferreira Demarchi L.M., Mathias W. Anomalous Subaortic Course of the Left Brachiocephalic (Innominate) Vein: Echocardiographic Diagnosis and Report of an Unusual Association. Cardiol. Young. 2002;12:159–163. doi: 10.1017/S104795110200032X. - DOI - PubMed

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