{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T08:32:43Z","timestamp":1786350763704,"version":"build-2736575974"},"reference-count":30,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T00:00:00Z","timestamp":1785715200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Persistent left superior vena cava (PLSVC) is the most frequently reported anatomical variant of the thoracic systemic venous system. While its association with congenital heart disease (CHD) is well recognized, its prevalence within CHD populations and its relative occurrence compared with non-CHD cohorts remain incompletely characterized. This study aimed to estimate the pooled prevalence of PLSVC among patients with CHD and to assess its relative frequency compared with that in control populations. A systematic review of PubMed, Scopus, and Web of Science was conducted from database inception to January 2026. Studies reporting the prevalence of PLSVC in CHD cohorts were included. A random-effects meta-analysis was performed, and risk ratios (RRs) were calculated for studies that included both CHD and non-CHD groups. Sixteen studies (n = 37,370) were included. The pooled prevalence of PLSVC in CHD patients was 6.36% (95% CI: 4.70\u20138.24). Significant heterogeneity was identified (I2 = 97.6%). An increased likelihood of PLSVC was observed in CHD populations (RR = 15.34; 95% CI: 5.72\u201341.14). However, this relative risk is based on a highly limited number of comparative studies (n = 4) and exhibits substantial heterogeneity (I2 = 92.47%); thus, it must be interpreted with significant caution. Subgroup analyses did not demonstrate statistically significant differences based on nationality (p = 0.59), study type (p = 0.32) and age group (p = 0.95), while imaging modality was statistically significant (p = 0.01). PLSVC is observed in a notable proportion of patients with CHD, although variability in reported prevalence is substantial and likely influenced by methodological differences across studies. Given its potential implications for catheter-based and surgical procedures, systematic evaluation of thoracic venous anatomy should be considered during the diagnostic and preoperative assessment of patients with CHD.<\/jats:p>","DOI":"10.3390\/life16081281","type":"journal-article","created":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T10:24:07Z","timestamp":1785752647000},"page":"1281","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Prevalence of Persistent Left Superior Vena Cava in Patients with Congenital Heart Disease: A Systematic Review with Meta-Analysis"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-0122-2436","authenticated-orcid":false,"given":"George","family":"Triantafyllou","sequence":"first","affiliation":[{"name":"Department of Anatomy, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-6221-7537","authenticated-orcid":false,"given":"Ioannis","family":"Paschopoulos","sequence":"additional","affiliation":[{"name":"Department of Anatomy, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-4945-7062","authenticated-orcid":false,"given":"Daniel","family":"Gondorf","sequence":"additional","affiliation":[{"name":"Department of Anatomy, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niki","family":"Lama","sequence":"additional","affiliation":[{"name":"Research Unit of Radiology and Medical Imaging, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11528 Athens, Greece"},{"name":"Second Department of Radiology, General University Hospital \u201cAttikon\u201d, National and Kapodistrian University of Athens, 12462 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8079-0599","authenticated-orcid":false,"given":"Evangelos","family":"Oikonomou","sequence":"additional","affiliation":[{"name":"Third Department of Cardiology, Sotiria Chest Disease Hospital, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan Jose","family":"Valenzuela-Fuenzalida","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Andr\u00e9s Bello University, Santiago 8370146, Chile"},{"name":"Department of Chemical and Biological Sciences, Faculty of Health Sciences, Bernardo O\u2019Higgins University, Santiago 8370993, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2599-8474","authenticated-orcid":false,"given":"Juan","family":"Sanchis-Gimeno","sequence":"additional","affiliation":[{"name":"GIAVAL Research Group, Department of Anatomy and Human Embryology, Faculty of Medicine, University of Valencia, 46001 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8130-6460","authenticated-orcid":false,"given":"Jose E.","family":"Le\u00f3n-Rojas","sequence":"additional","affiliation":[{"name":"Grupo de Investigaci\u00f3n Bienestar, Salud y Sociedad, Escuela de Psicolog\u00eda y Educaci\u00f3n, Universidad de Las Am\u00e9ricas, Quito 170124, Ecuador"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9904-3558","authenticated-orcid":false,"given":"Charalambos","family":"Vlachopoulos","sequence":"additional","affiliation":[{"name":"First Department of Cardiology, General Hospital of Athens \u201cHippokrateion\u201d, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerasimos","family":"Siasos","sequence":"additional","affiliation":[{"name":"Third Department of Cardiology, Sotiria Chest Disease Hospital, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4831-8005","authenticated-orcid":false,"given":"Maria","family":"Piagkou","sequence":"additional","affiliation":[{"name":"Department of Anatomy, School of Medicine, Faculty of Health Sciences, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2241","DOI":"10.1016\/j.jacc.2011.08.025","article-title":"Birth Prevalence of Congenital Heart Disease Worldwide","volume":"58","author":"Konings","year":"2011","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1093\/ije\/dyz009","article-title":"Global Birth Prevalence of Congenital Heart Defects 1970\u20132017: Updated Systematic Review and Meta-Analysis of 260 Studies","volume":"48","author":"Liu","year":"2019","journal-title":"Int. J. Epidemiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1097\/RTI.0000000000000273","article-title":"Pictorial Review of Surgical Anatomy in Adult Congenital Heart Disease","volume":"32","author":"Muscogiuri","year":"2017","journal-title":"J. Thorac. Imaging"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1002\/uog.15914","article-title":"Systematic Review and Meta-analysis of Persistent Left Superior Vena Cava on Prenatal Ultrasound: Associated Anomalies, Diagnostic Accuracy and Postnatal Outcome","volume":"48","author":"Gustapane","year":"2016","journal-title":"Ultrasound Obstet. Gynecol."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Gondorf, D., Triantafyllou, G., Paschopoulos, I., Arkoudis, N.-A., Prountzos, S., Samolis, A., Tsakotos, G., and Piagkou, M. (2026). Prevalence of Persistent Left Superior Vena Cava in the General Population: A Systematic Review with Meta-Analysis. Anat. Sci. Int.","DOI":"10.1007\/s12565-026-00928-8"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.ijcard.2020.05.017","article-title":"Clinical and Procedural Implications of Congenital Vena Cava Anomalies in Adults: A Systematic Review","volume":"315","author":"Shafi","year":"2020","journal-title":"Int. J. Cardiol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"n71","DOI":"10.1136\/bmj.n71","article-title":"The PRISMA 2020 Statement: An Updated Guideline for Reporting Systematic Reviews","volume":"372","author":"Page","year":"2021","journal-title":"BMJ"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.aanat.2015.12.002","article-title":"Methods of Evidence-Based Anatomy: A Guide to Conducting Systematic Reviews and Meta-Analysis of Anatomical Studies","volume":"205","author":"Henry","year":"2016","journal-title":"Ann. Anat."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1002\/ca.22799","article-title":"Development of the Anatomical Quality Assessment (AQUA) Tool for the Quality Assessment of Anatomical Studies Included in Meta-Analyses and Systematic Reviews","volume":"30","author":"Henry","year":"2017","journal-title":"Clin. Anat."},{"key":"ref_10","first-page":"195","article-title":"A New Improved Graphical and Quantitative Method for Detecting Bias in Meta-Analysis","volume":"16","author":"Barendregt","year":"2018","journal-title":"JBI Evid. Implement."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Papadopoulos, V. (2026). On the Construction and Interpretation of the Doi Plot in Prevalence Meta-Analyses. Anat. Sci. Int.","DOI":"10.1007\/s12565-026-00937-7"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1111\/echo.13447","article-title":"Persistent Left Superior Vena Cava Accompanying Congenital Heart Disease in Children: Experience of a Tertiary Care Center","volume":"34","author":"Ari","year":"2017","journal-title":"Echocardiography"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1111\/j.1651-2227.1980.tb07039.x","article-title":"Persistent Left Superior Vena Cava","volume":"69","author":"Bjerregaard","year":"1980","journal-title":"Acta Paediatr."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1177\/2150135117708706","article-title":"Persistent Left Superior Vena Cava: Incidence and Management in Patients Undergoing Repair of Partial Anomalous Pulmonary Venous Connection","volume":"8","author":"Clarke","year":"2017","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"21679","DOI":"10.3402\/ljm.v8i0.21679","article-title":"High Prevalence Rate of Left Superior Vena Cava Determined by Echocardiography in Patients with Congenital Heart Disease in Saudi Arabia","volume":"8","author":"Eldin","year":"2013","journal-title":"Libyan J. Med."},{"key":"ref_16","first-page":"72","article-title":"Persistent Left Superior Vena Cava the Anatomical and Surgical Importance","volume":"56","author":"Uygur","year":"2007","journal-title":"West Indian Med. J."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1002\/uog.4045","article-title":"Clinical Significance of Persistent Left Superior Vena Cava Diagnosed in Fetal Life","volume":"30","author":"Galindo","year":"2007","journal-title":"Ultrasound Obstet. Gynecol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s00246-013-0743-z","article-title":"Persistent Left Superior Vena Cava in Cardiac Congenital Surgery","volume":"35","author":"Perez","year":"2014","journal-title":"Pediatr. Cardiol."},{"key":"ref_19","first-page":"407","article-title":"Safety and Long-Term Outcomes of Defibrillator Therapy in Patients With Right-Sided Implantable Cardiac Devices in Adults with Congenital Heart Disease","volume":"37","author":"Kawada","year":"2021","journal-title":"Can. J. Cardiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1111\/j.1442-200X.2011.03443.x","article-title":"Persistent Left Superior Vena Cava: Experience of a Tertiary Health-care Center","volume":"53","author":"Kula","year":"2011","journal-title":"Pediatr. Int."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1007\/s00246-017-1778-3","article-title":"Incidence of Persistent Left Superior Vena Cava in the Normal Population and in Patients with Congenital Heart Diseases Detected Using Echocardiography","volume":"39","author":"Nagasawa","year":"2018","journal-title":"Pediatr. Cardiol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2254","DOI":"10.1111\/jocs.14897","article-title":"Incidence of Systemic Venous Anomalies in Tetralogy of Fallot on Multidetector CT Angiography: A Retrospective Single-center Study of 973 Patients","volume":"35","author":"Pandey","year":"2020","journal-title":"J. Card. Surg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"5381","DOI":"10.2147\/IJGM.S466195","article-title":"Epidemiology of Congenital Heart Defects in Perinatal Infants in Guangxi, China","volume":"17","author":"Peng","year":"2024","journal-title":"Int. J. Gen. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1016\/j.amjcard.2013.05.079","article-title":"Prevalence of Persistent Superior Vena Cava and Association with Congenital Heart Anomalies","volume":"112","author":"Perles","year":"2013","journal-title":"Am. J. Cardiol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1016\/j.ijcard.2006.12.020","article-title":"Left Superior Vena Cava in Pediatric Cardiology Associated with Extra-Cardiac Anomalies","volume":"123","author":"Postema","year":"2008","journal-title":"Int. J. Cardiol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1080\/15513815.2018.1546355","article-title":"Cardiac Findings in Fetal and Pediatric Autopsies: A 15-Year Retrospective Review","volume":"38","author":"Taweevisit","year":"2019","journal-title":"Fetal Pediatr. Pathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"e20240446","DOI":"10.21470\/1678-9741-2024-0446","article-title":"Frequency of Persistent Left Superior Vena Cava and Its Impact on Outcomes in Children Undergoing Congenital Heart Surgery","volume":"40","author":"Toprak","year":"2025","journal-title":"Braz. J. Cardiovasc. Surg."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"846","DOI":"10.3348\/jksr.2021.0192","article-title":"Pictorial Essay: Understanding of Persistent Left Superior Vena Cava and Its Differential Diagnosis","volume":"83","author":"Jeong","year":"2022","journal-title":"J. Korean Soc. Radiol."},{"key":"ref_29","first-page":"1","article-title":"Embryology and Congenital Heart Disease","volume":"22","author":"Angelini","year":"1995","journal-title":"Tex. Heart Inst. J."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1007\/978-3-031-44087-8_3","article-title":"Cardiac Development and Animal Models of Congenital Heart Defects","volume":"1441","author":"Kelly","year":"2024","journal-title":"Adv. Exp. Med. Biol."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/16\/8\/1281\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T08:23:55Z","timestamp":1786350235000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/16\/8\/1281"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,3]]},"references-count":30,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2026,8]]}},"alternative-id":["life16081281"],"URL":"https:\/\/doi.org\/10.3390\/life16081281","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,3]]}}}