Ultrasound Obstet Gynecol 2016; 48: 701–708 Published online 1 November 2016 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/uog.15914 Systematic review and meta-analysis of persistent left superior vena cava on prenatal ultrasound: associated anomalies, diagnostic accuracy and postnatal outcome S. GUSTAPANE*, M. LEOMBRONI*, A. KHALIL†, F. GIACCI*, L. MARRONE*, F. BASCIETTO*, G. RIZZO‡, G. ACHARYA§, M. LIBERATI* and F. D’ANTONIO§ *Department of Obstetrics and Gynaecology, SS. Annunziata Hospital, G. D’Annunzio University of Chieti-Pescara, Chieti, Italy; †Fetal Medicine Unit, Division of Developmental Sciences, St George’s University of London, London, UK; ‡Department of Obstetrics and Gynecology, Universit ` a di Roma, Rome, Italy; § Women’s Health and Perinatology Research Group, Department of Clinical Medicine, Faculty of Health Sciences, UiT – The Arctic University of Norway, Department of Obstetrics and Gynaecology, University Hospital of Northern Norway, Tromsø, Norway KEYWORDS: congenital heart disease; fetal echocardiography; persistent left superior vena cava; prenatal diagnosis ABSTRACT Objectives To quantify the prevalence of chromosomal anomalies in fetuses with persistent left superior vena cava (PLSVC), assess the strength of the association between PLSVC and coarctation of the aorta and ascertain the diagnostic accuracy of antenatal ultrasound in correctly identifying isolated cases of PLSVC. Methods MEDLINE, EMBASE, CINHAL and the Cochrane databases were searched from the year 2000 onwards using combinations of keywords ‘left superior vena cava’ and ‘outcome’. Two authors reviewed all abstracts independently. Quality assessment of the included studies was performed using the Newcastle– Ottawa Scale for cohort studies. The rates of the following outcomes were analyzed: chromosomal abnormalities; associated intracardiac anomalies (ICAs) and extracardiac anomalies (ECAs) diagnosed prenatally; additional ICAs and ECAs detected only at postnatal imaging or clinical evaluation but missed at prenatal imaging; and association of PLSVC and coarctation of the aorta. Meta-analyses of proportions were used to combine data. Results In total, 2708 articles were identified and 13 ( n = 501) were included in the systematic review. Associated ICAs and ECAs were detected at the prenatal ultrasound examination or at a follow-up assessment in 60.7% (95% CI, 44.2–75.9%) and 37.8% (95% CI, 31.0–44.8%) of cases, respectively. Chromosomal anomalies occurred in 12.5% (95% CI, 9.0–16.4%) of cases in the overall population of fetuses with PLSVC and in 7.0% (95% CI, 2.7–13.0%) of isolated cases. Additional ICAs and ECAs were detected only after birth Correspondence to: Dr F. D’Antonio, Women’s Health and Perinatology Research Group, Department of Clinical Medicine, Faculty of Health Sciences, UiT – The Arctic University of Norway, Hansine Hansens veg 18, 9019 Tromsø, Norway (e-mail: francesco.dantonio@uit.no) Accepted: 5 March 2016 and missed at ultrasound in 2.4% (95% CI, 0.5–5.8%) and 6.7% (95% CI, 2.2–13.2%) of cases, respectively. Coarctation of the aorta was associated with isolated PLSVC in 21.3% (95% CI, 13.6–30.3%) of cases. Conclusions PLSVC is commonly associated with ICAs, ECAs and chromosomal anomalies. Fetuses with isolated PLSVC should be followed up throughout pregnancy in order to rule out coarctation of the aorta. As most of the data in this review were derived from high-risk pregnancies, the rate of associated abnormalities is likely to be higher than that in the general population of fetuses with PLSVC, for which more data are needed. Copyright  2016 ISUOG. Published by John Wiley & Sons Ltd. INTRODUCTION Persistent left superior vena cava (PLSVC) is the most common variant of anomalous systemic venous return in adults, with an estimated prevalence of 0.3–0.5% in the general population and of 4–8% in patients with congenital heart disease (CHD) 1 – 4 . Although the origin of PLSVC has not yet been elucidated completely, it is thought to be the result of in-utero failure of the left cardinal vein to develop, resulting in the presence of bilateral SVCs. Occasionally, the right SVC is absent and the venous return from the upper body enters the coronary sinus to the right atrium 5,6 . In the majority of cases, PLSVC has no clinical implications as venous blood continues to return to the right atrium, and thus hemodynamic derangement is not induced. Postnatal series have reported a common associa- tion between PLSVC and intracardiac anomalies (ICAs), Copyright  2016 ISUOG. Published by John Wiley & Sons Ltd. SYSTEMATIC REVIEW