The Southern Medical Journal (SMJ) is the official, peer-reviewed journal of the Southern Medical Association. It has a multidisciplinary and inter-professional focus that covers a broad range of topics relevant to physicians and other healthcare specialists.

SMJ // Article

Review Article

Persistent Left Superior Vena Cava Beyond Anatomy

Authors: Faizan Ahmed Zakir, MD, Muhammad Bilal Akram, MD, Muhammad Moin ud Din Arshad, MBBS, Kamla Kainat, MBBS, Rimsha Maqsood, MBBS, Farwa Saleem, MBBA, Suha Aslam, , Farhan Aslam, MD

Abstract

Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly, occurring in 0.3% to 0.5% of the general population and in 10% of patients with congenital heart disease. Historically considered benign, PLSVC is increasingly recognized for its clinical significance in the fields of interventional cardiology, electrophysiology, and surgery. The present narrative review combines the available evidence regarding PLSVC, focusing on its embryology, anatomical variants, diagnostic challenges, and tailored management strategies, to address clinical uncertainties and improve procedural outcomes for patients undergoing intervention. The clinician needs to understand the risk for right-to-left shunting of PLSVC, procedural risk during placement of the catheter or device, and its association with arrhythmias and stroke. Common in high-risk patients (eg, in congenital heart disorders, unexplained hypoxia), early detection by advanced imaging and forward procedural planning are key to reducing complications and optimizing patient outcomes.
Posted in: Miscellaneous Cardiovascular Disease (Physical Examination Findings, Murmurs)0

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Miraldi F, di Gioia CRT, Proietti P, et al. Cardinal vein isomerism: an embryological hypothesis to explain a persistent left superior vena cava draining into the roof of the left atrium in the absence of coronary sinus and atrial septal defect. Cardiovasc Pathol 2002;11:149-152.
 
2. Choi E, Hong S, Jeong N. Clinical characteristics of prenatally diagnosed persistent left superior vena cava in low-risk pregnancies. Prenat Diagn 2016;36:444-448.
 
3. Mancera M, Genthe N, Lepa N. Case report of a left-sided superior vena cava causing unique positioning of central line. Clin Pract Cases Emerg Med 2020;4:587-590.
 
4. Coimbra MT, Braga B, Silva A, et al. Accidental diagnosis of isolated persistent left superior vena cava after an elective central venous access procedure for chronic hemodialysis: clinical implications and precautions from a case report. Cureus 2023;15:e44212.
 
5. Akş E, Oğ S, Aşü HF, et al. How does persistent left superior vena cava affect human heart health? Acta Cardiol Sin 2021;37:321-322.
 
6. Azizova A, Onder O, Arslan S, et al. Persistent left superior vena cava: clinical importance and differential diagnoses. Insights Imaging 2020;11:110.
 
7. Verma M, Pandey NN, Ojha V, et al. Developmental anomalies of the superior vena cava and its tributaries: what the radiologist needs to know. Br J Radiol 2021;94:20200856.
 
8. Corbacioglu EA, Yuksel A, Cali H, et al. Prenatal diagnosis of persistent left superior vena cava and its clinical significance. Balkan Med J 2014;33:50-54.
 
9. Raissi D, Christie A, Applegate K. Waardenburg syndrome and left persistent superior vena cava. J Clin Imaging Sci 2018;8:44.
 
10. Sinkovskaya E, Abuhamad A, Horton S, et al. Fetal left brachiocephalic vein in normal and abnormal conditions. Ultrasound Obstet Gynecol 2012;40:542-548.
 
11. Schwier E, Schneider A, Henzler D, et al. Left-sided superior vena cava. Can J Anesth 2023;70:271-272.
 
12. Gozar L, Săsăran MO, Cosma MC, et al. Raghib syndrome and pulmonary arterial hypertension in a pediatric patient: case report and literature review. J Clin Med 2024;13:3623.
 
13. Bisoyi S, Jagannathan U, Dash A, et al. Isolated persistent left superior vena cava: a case report and its clinical implications. Ann Card Anaesth 2017;20:104.
 
14. Dave V, Sesham K, Mehra S, et al. Persistent left superior vena cava: an anatomical variation. Med J Armed Forces India 2022;78:S277-S281.
 
15. Khajali Z, Ali Ramezani M. Report of persistent left superior vena cava associated with Ebstein’ anomaly of tricuspid valve. ARYA Atheroscler J 2021;17:2287.
 
16. Loube DK, Sreekrishnan A, Woo JP, et al. Stroke caused by a paradoxical embolus from a rare congenital anomaly in the adult: persistent left superior vena cava draining into the left upper pulmonary vein. Circ Cardiovasc Imaging 2023;16:e014205.
 
17. Adavidoaei CG, Haba AM, Costache II, et al. Cardiac implantable electronic devices in different anatomical types of persistent left superior vena cava: case series and brief review of the literature. Diagnostics (Basel) 2022;12:2596.
 
18. Sonavane SK, Milner DM, Singh SP, et al. Comprehensive imaging review of the superior vena cava. Radiographics 2015;35:1873-1892.
 
19. Sheikh AS, Mazhar S. Persistent left superior vena cava with absent right superior vena cava: review of the literature and clinical implications. Echocardiography 2014;31:674-679.
 
20. Smolarek D, Jankowska H, Dorniak K, et al. A rare case of isolated persistent left superior vena cava diagnosed by echocardiography. J Cardiothorac Surg 2024;19:175.
 
21. Cárdenas-Marín PA, Zambrano-Franco JA, Reyes-Cardona MJ, et al. Postpartum ischemic stroke due to persistent left superior vena cava to left atrium after DORV repair: a case report and literature review. BMC Cardiovasc Disord 2025;25:463.
 
22. Gonzalez-Juanatey C, Testa A, Vidan J, et al. Persistent left superior vena cava draining into the coronary sinus: report of 10 cases and literature review. Clin Cardiol 2004;27:515-518.
 
23. Takenaka S, Enzan A, Ueno A, et al. Atrial fibrillation ablation in a patient complicated by persistent left superior vena cava and absent right superior vena cava. J Arrhythm 2022;38:256-258.
 
24. Hana D, Wahba D, Schwartzman D, et al. Persistent left superior vena cava draining directly into the left atrium with occluded coronary sinus. JACC Case Rep 2023;8:101731.
 
25. Petrac D, Radeljic V, Pavlovic N, et al. Persistent left superior vena cava in patients undergoing cardiac device implantation: clinical and long-term data. Cardiol Res 2013;4:64-67.
 
26. Al-Muhaya MA, Alnajjar AA, Jelly AE, et al. Anomalous drainage of persistent left superior vena cava to the left atrium: case series and literature review. J Saudi Heart Assoc 2020;32:190-193.
 
27. Duong P, Papaioannou V, Moharam-Elgamal S, et al. Case report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement. Eur Heart J Case Rep. 2022;6:ytac382.
 
28. Altin HF, Altunyuva K, Yilmaz EH, et al. Redirection of persistent left superior vena cava using a turned-in left atrial appendage. Innovations (Phila) 2022;17:247-249.
 
29. Turagam MK, Atoui M, Atkins D, et al. Persistent left superior vena cava as an arrhythmogenic source in atrial fibrillation: results from a multicenter experience. J Interv Card Electrophysiol 2019;54:93-100.