Superior vena cava (SVC) syndrome results from stenosis and/or occlusion of the central venous system leading to symptomatology associated with head and neck as well as upper-extremity venous congestion. With the rise in central venous catheter use and increasing cases of malignancy, the incidence of SVC syndrome has steadily increased in recent years. This narrative review explores the clinical presentation of SVC syndrome, alongside its evolving etiologies within modern clinical practice and its pathophysiology. In light of this, advanced imaging modalities and management approaches are discussed based on the extensive institutional experience, detailing the preprocedural imaging techniques, conservative and interventional treatment options, and follow-up imaging protocols.
**WilsonLDDetterbeckFCYahalomJ.Superior vena cava syndrome with malignant causes. N Engl J Med2007; 365: 1862–1869.
2.
**KimHJKimHSChungSH.CT diagnosis of superior vena cava syndrome: Importance of collateral vessels. AJR Am J Roentgenol1993; 161: 539–542.
3.
**RiceTWRodriguezRMLightRW.The superior vena cava syndrome: Clinical characteristics and evolving etiology. Medicine (Baltimore)2006; 85: 37–42.
4.
SiegelRLGiaquintoANJemalA.Cancer statistics, 2024. CA Cancer J Clin2024; 74: 12–49.
5.
*LiMToomaySDraznerMHThibodeauJT.Superior vena cava stenosis presenting with bendopnea. BMJ Case Rep2021; 14: e242418.
6.
WanJFBezjakA.Superior vena cava syndrome. Emerg Med Clin North Am2009; 27: 243–255.
7.
ShahRPBolajiODuhanS, et al. Superior vena cava syndrome: An umbrella review. Cureus2023; 5: e42227.
8.
RizviIZamanSZaidiN, et al. Superior vena cava syndrome caused by Hodgkin’s lymphoma in an adolescent girl. BMJ Case Rep2012; 2012: bcr0120125487.
9.
*FriedmanTQuencerKBKishoreSA, et al. Malignant venous obstruction: Superior vena cava syndrome and beyond. Semin Interv Radiol2017; 34: 398–408.
10.
PicasciaOFioriniVVitaleG, et al. Iatrogenic superior vena cava syndrome with concomitant deep vein thrombosis of azygos and hemiazygos veins. Radiol Case Rep2021; 16: 1895–1898.
11.
RossiABaravelliMCattaneoP, et al. Acute superior vena cava syndrome after insertion of implantable cardioverter defibrillator. J Interv Card Electrophysiol2008; 23: 247–249.
12.
KaulPAdluriKJavangulaKBaigW.Successful management of multiple permanent pacemaker complications – infection, 13 year old silent lead perforation and exteriorisation following failed percutaneous extraction, superior vena cava obstruction, tricuspid valve endocarditis, pulmonary embolism and prosthetic tricuspid valve thrombosis. J Cardiothorac Surg2009; 4: 12.
13.
*LabriolaLSerontBCrottR, et al. Superior vena cava stenosis in haemodialysis patients with a tunnelled cuffed catheter: Prevalence and risk factors. Nephrol Dial Transplant2018; 33: 2227–2233.
14.
HerscoviciRSzyper-KravitzMAltmanA, et al. Superior vena cava syndrome – changing etiology in the third millennium. Lupus2021; 21: 93–96.
15.
SarrSAFallPDMboupMC, et al. Superior vena cava syndrome revealing a Behçet’s disease. Thromb J2015; 13: 7.
16.
SanchezLNHerreroFSFraileJBFabrellasEF.Mediastinal fibrosis and superior vena cava syndrome. Arch Bronconeumol2013; 49: 340–342.
17.
Leci-TahiriLZherca-SaraciniHTahiriAKoshiA.Bilateral internal jugular vein thrombosis due to malignant tumor. J Med Case Rep2018; 12: 42.
18.
*SunMChenXLiH, et al. Clinical analysis of 48 cases of malignant superior vena cava syndrome. World J Surg Oncol2021;19: 185.
NifongTPMcDevittTJ.The effect of catheter to vein ratio on blood flow rates in a simulated model of peripherally inserted central venous catheters. Chest2011; 140: 48–53.
21.
TigheDAPaulJJHuhtaJC.Superior vena caval syndrome: Color flow Doppler detection of collateral venous channels. J Am Soc Echocardiogr2000; 13: 780–784.
22.
*BirchAUmDLaselleB.Ultrasound detection of superior vena cava thrombus. West J Emerg Med2014; 15: 715–718.
23.
AdiOAhmadAHFongCP, et al. Shock due to superior vena cava obstruction detected with point of care ultrasound. Am J Emerg Med2021; 48: 374.e1–374.e3.
24.
AliNTanAChenkinJ.From compression to diagnosis: Identification of superior vena cava syndrome using point-of-care ultrasound in the emergency department. Int J Emerg Med2024; 17: 40.
25.
FadelBMKazziBMohtyD.Ultrasound imaging of the superior vena cava: A state-of-the-art review. J Am Soc Echocardiogr2023; 36: 447–463.
26.
HammerliMMeyerRA.Doppler evaluation of central venous lines in the superior vena cava. J Pediatr1993; 122: S104–S108.
27.
*KimHCChungJWParkJH, et al. Role of CT venography in the diagnosis and treatment of benign thoracic central venous obstruction. Korean J Radiol2003; 4: 146–152.
28.
*ShethSEbertMDFishmanEK.Superior vena cava obstruction evaluation with MDCT. AJR Am J Roentgenol2010; 194: W336–W346.
29.
SinghVSandeanDP.CT patient safety and care. StatPearls Publishing, 2023.
30.
HarigaiMOkadaTUmeokaS, et al. Non-contrast-enhanced MR venography of the upper limb: A comparative study of acquisitions with fresh blood imaging vs. time-of-flight methods. Clin Imaging2012; 36: 496–501.
31.
WoolenSAShankarPRGagnierJJ, et al. Risk of nephrogenic systemic fibrosis in patients with stage 4 or 5 chronic kidney disease receiving a group II gadolinium-based contrast agent: A systematic review and meta-analysis. JAMA Intern Med2019; 180: 223–230.
32.
SanthoshSGorlaAKRBhattacharyaA, et al. Contrast-enhanced fluorodeoxyglucose positron emission tomography/contrast-enhanced computed tomography in mediastinal T-cell lymphoma with superior vena cava syndrome. Indian J Nucl Med2016; 31: 79–80.
33.
OstlerPJClarkeDPWatkinsonAFGazeMN.Superior vena cava obstruction: A modern management strategy. Clin Oncol1997; 9: 83–89.
34.
*StrakaCYingJKongFM, et al. Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome. Springerplus2016; 5: 229.
35.
ChowRSimeoneCB2ndRimnerA.Management of malignant superior vena cava syndrome. Ann Palliat Med2024; 13: 620–626.
36.
GuhlichMMaagTEDrogeLH, et al. Symptom relief, prognostic factors, and outcome in patients receiving urgent radiation therapy for superior vena cava syndrome: A single-center retrospective analysis of 21 years’ practice. Strahlenther Onkol2022; 198: 1072–1081.
37.
SimoffMJLallyBSladeMG, et al. Symptom management in patients with lung cancer. Chest2013; 143: E455S–E497S.
38.
RowellNPGleesonFV.Steroids, radiotherapy, and chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus. Cochrane Database Syst Rev2001; 4: CD001316.
39.
MoseSStabikCEberleinK, et al. Retrospective analysis of the superior vena cava syndrome in irradiated cancer patients. Anticancer Res2006; 26: 4933–4936.
40.
**AziziAHShafiIZhaoM, et al. Endovascular therapy for superior vena cava syndrome: A systematic review and meta-analysis. EClinicalMedicine2021; 37: 100970.
41.
*FagedetDThonyFTimsitJF, et al. Endovascular treatment of malignant superior vena cava syndrome: Results and predictive factors of clinical efficacy. Cardiovasc Intervent Radiol2011; 36: 140–149.
42.
WatkinsonAFYeowTNFraserC.Endovascular stenting to treat obstruction of the superior vena cava. BMJ2008; 336: 1434–1437.
43.
*BreaultSDoenzFJouannicAMQanadliSD.Percutaneous endovascular management of chronic superior vena cava syndrome of benign causes: Long-term follow-up. Eur Radiol2016; 27: 97–104.
44.
**SecemskyEAAronowHDKwolekCJ, et al. Intravascular ultrasound use in peripheral arterial and deep venous interventions: Multidisciplinary expert opinion from SCAI/AVF/AVLS/SIR/SVM/SVS. J Soc Cardiovasc Angiogr Interv2024; 3: 101205.
45.
DivakaranSMeissnerMHKohiMP, et al. Utilization of and outcomes associated with intravascular ultrasound during deep venous stent placement among Medicare beneficiaries. J Vasc Interv Radiol2022; 33: 1476–1484.
46.
*TranLMGoCZaghloulM, et al. Intravascular ultrasound evaluation during iliofemoral venous stenting is associated with improved midterm patency outcomes. J Vasc Surg Venous Lymphat Disord2022; 10: 1294–1303.
47.
KishoreSKhajaMSThornburgB, et al. Antithrombotic therapy after venous interventions: AJR Expert Panel Narrative Review. AJR Am J Roentgenol2022; 219: 175–187.
48.
RennerEBarnesGD.Antithrombotic management of venous thromboembolism: JACC Focus Seminar. J Am Coll Cardiol2020; 76: 2142–2154.
49.
NutescuEABurnettAFanikosJ, et al. Erratum to: Pharmacology of anticoagulants used in the treatment of venous thromboembolism. J Thromb Thrombolysis2016; 42: 296–311.
50.
NeglenPRajuS.Intravascular ultrasound scan evaluation of the obstructed vein. J Vasc Surg2020; 34: 694–700.
51.
McColloughCRajiahPS.Milestones in CT: Past, present, and future. Radiology2023; 309: e230803.
52.
Van der BieJvan StratenMBooijR, et al. Photon-counting CT: Review of initial clinical results. Eur J Radiol2023; 163: 110829.
53.
MeloniAFrijiaFPanettaD, et al. Photon-counting computed tomography (PCCT): Technical background and cardio-vascular applications. Diagnostics (Basel)2023; 13: 645.
54.
PengGZhuWZuoZ, et al. Efficacy of modified time of flight magnetic resonance venography in diagnosis of iliac vein compression syndrome. Eur J Radiol2023; 166: 111020.
55.
LimRPHornseyERanatungaD, et al. Upper extremity non-contrast magnetic resonance venography (MRV) compared to contrast enhanced MRV and ultrasound. Clin Imaging2017; 45: 51–57.
56.
*UberoiR.Quality assurance guidelines for superior vena cava stenting in malignant disease. Cardiovasc Intervent Radiol2006; 29: 319–322.
57.
KouryJPBurkeCT.Endovascular management of acute upper extremity deep venous thrombosis and the use of superior vena cava filters. Semin Interv Radiol2011; 28: 3–9.
58.
ParkJWJeongWGLeeJE, et al. Pictorial review of mediastinal masses with an emphasis on magnetic resonance imaging. Korean J Radiol2020; 22: 139–154.
59.
HookerJBHawkinsBMAbu-FadelMS.Endovascular stenting in 2 patients with benign superior vena cava syndrome. Tex Heart Inst J2018; 45: 264–269.
60.
KalraMGloviczkiPAndrewsJC, et al. Open surgical and endovascular treatment of superior vena cava syndrome caused by nonmalignant disease. J Vasc Surg2003; 38: 215–223.
61.
*RizviAZKalraMBjarnasonH, et al. Benign superior vena cava syndrome: Stenting is now the first line of treatment. J Vasc Surg2008; 47: 372–380.
62.
SfyoerasGSAntonopoulosCNMantasG, et al. A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology. Eur J Vasc Endovasc Surg2016; 53: 238–254.
63.
AziziAHShafiIShahN, et al. Superior vena cava syndrome. JACC Cardiovasc Interv2020; 13: 2896–2910.
Supplementary Material
Please find the following supplemental material available below.
For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.
For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.
