CardioVascular and Interventional Radiology

, Volume 35, Issue 5, pp 1053–1058 | Cite as

Efficacy of Lower-Extremity Venous Thrombolysis in the Setting of Congenital Absence or Atresia of the Inferior Vena Cava

  • Suvranu Ganguli
  • Sanjeeva Kalva
  • Rahmi Oklu
  • T. Gregory Walker
  • Neil Datta
  • Eric F. Grabowski
  • Stephan Wicky
Clinical Investigation



A rare but described risk factor for deep venous thrombosis (DVT), predominately in the young, is congenital agenesis or atresia of the inferior vena cava (IVC). The optimal management for DVT in this subset of patients is unknown. We evaluated the efficacy of pharmacomechanical catheter-directed thrombolysis (PCDT) followed by systemic anticoagulation in the treatment of acute lower-extremity DVT in the setting of congenital IVC agenesis or atresia.

Materials and Methods

Between November of 2005 and May of 2010, six patients (three women [average age 21 years]) were referred to our department with acute lower-extremity DVT and subsequently found to have IVC agenesis or atresia on magnetic resonance imaging. A standardized technique for PCDT (the Angiojet Rheolytic Thrombectomy System followed by the EKOS Microsonic Accelerated Thrombolysis System) was used for all subjects. Successful thrombolysis was followed by systemic heparinization with transition to Coumadin or low molecular-weight heparin and compression stockings. Subjects were followed-up at 1, 3, and then every 6 months after the procedure with clinical assessment and bilateral lower-extremity venous ultrasound.


All PCDT procedures were technically successful. No venous stenting or angioplasty was performed. The average thrombolysis time was 28.6 h (range 12–72). Two patients experienced heparin-induced thrombocytopenia, and one patient developed a self-limited knee hemarthrosis, No patients were lost to follow-up. The average length of follow-up was 25.8 ± 20.2 months (range 3.8–54.8). No incidence of recurrent DVT was identified. There were no manifestations of postthrombotic syndrome.


PCDT followed by systemic anticoagulation and the use of compression stockings appears to be safe and effective in relatively long-term follow-up treatment of patients who present with acute DVT and IVC agenesis or atresia.


Inferior vena cava atresia Pharmacomechanical catheter-directed thrombolysis Lower-extremity deep vein thrombosis Postthrombotic syndrome 


Conflict of interest

The authors declared that they have no conflict of interest.


  1. 1.
    Shah NL, Shanley CJ, Prince MR et al (1996) Deep venous thrombosis complicating a congenital absence of the inferior vena cava. Surgery 120(5):891–896PubMedCrossRefGoogle Scholar
  2. 2.
    Ruggeri M, Tosetto A, Castaman G et al (2001) Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet 357(9254):441PubMedCrossRefGoogle Scholar
  3. 3.
    Chee YL, Culligan DJ, Watson HG (2001) Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol 114(4):878–880PubMedCrossRefGoogle Scholar
  4. 4.
    Obernosterer A, Aschauer M, Schnedl W et al (2002) Anomalies of the inferior vena cava in patients with iliac venous thrombosis. Ann Intern Med 136(1):37–41PubMedGoogle Scholar
  5. 5.
    Vedantham S (2011) Preventing pediatric postthrombotic syndrome: preparing the way. J Vasc Interv Radiol 22(3):405–407PubMedCrossRefGoogle Scholar
  6. 6.
    Kuhle S, Koloshuk B, Marzinotto V et al (2003) A cross-sectional study evaluating post-thrombotic syndrome in children. Thromb Res 111(4–5):227–233PubMedCrossRefGoogle Scholar
  7. 7.
    Goldenberg NA, Durham JD, Knapp-Clevenger R et al (2007) A thrombolytic regimen for high-risk deep venous thrombosis may substantially decrease the risk of postthrombotic syndrome in children. Blood 110(1):45–53PubMedCrossRefGoogle Scholar
  8. 8.
    Broholm R, Jorgensen M, Just S et al (2011) Acute iliofemoral venous thrombosis in patients with atresia of the inferior vena cava can be treated successfully with catheter-directed thrombolysis. J Vasc Interv Radiol 22(6):801–805PubMedCrossRefGoogle Scholar
  9. 9.
    Goldenberg NA, Knapp-Clevenger R, Manco-Johnson MJ (2004) Elevated plasma factor VIII and d-dimer levels as predictors of poor outcomes of thrombosis in children. N Engl J Med 351(11):1081–1088PubMedCrossRefGoogle Scholar
  10. 10.
    Vedantham S, Grassi CJ, Ferral H et al (2009) Reporting standards for endovascular treatment of lower extremity deep vein thrombosis. J Vasc Interv Radiol 20(Suppl 7):S391–S408PubMedCrossRefGoogle Scholar
  11. 11.
    Eklof B, Rutherford RB, Bergan JJ et al (2004) Revision of the CEAP classification for chronic venous disorders: consensus statement. J Vasc Surg 40(6):1248–1252PubMedCrossRefGoogle Scholar
  12. 12.
    Porter D, Rundback JH, Miller S (2010) Sharp recanalization using a subintimal reentry device, angioplasty, and stent placement for severely symptomatic iliofemoral deep venous thrombosis secondary to congenital aplasia of the inferior vena cava. J Vasc Interv Radiol 21(11):1765–1769PubMedCrossRefGoogle Scholar
  13. 13.
    Robbins MR, Assi Z, Comerota AJ (2005) Endovascular stenting to treat chronic long-segment inferior vena cava occlusion. J Vasc Surg 41(1):136–140PubMedCrossRefGoogle Scholar
  14. 14.
    Razavi MK, Hansch EC, Kee ST et al (2000) Chronically occluded inferior venae cavae: endovascular treatment. Radiology 214(1):133–138PubMedGoogle Scholar
  15. 15.
    Anderson RC, Adams P Jr, Burke B (1961) Anomalous inferior vena cava with azygos continuation (infrahepatic interruption of the inferior vena cava). Report of 15 new cases. J Pediatr 59:370–383PubMedCrossRefGoogle Scholar
  16. 16.
    Chuang VP, Mena CE, Hoskins PA (1974) Congenital anomalies of the inferior vena cava. Review of embryogenesis and presentation of a simplified classification. Br J Radiol 47(556):206–213PubMedCrossRefGoogle Scholar
  17. 17.
    Nordstrom M, Lindblad B, Bergqvist D et al (1992) A prospective study of the incidence of deep-vein thrombosis within a defined urban population. J Intern Med 232(2):155–160PubMedCrossRefGoogle Scholar
  18. 18.
    Anderson FA Jr, Wheeler HB, Goldberg RJ et al (1991) A population-based perspective of the hospital incidence and case-fatality rates of deep vein thrombosis and pulmonary embolism. The Worcester DVT Study. Arch Intern Med 151(5):933–938PubMedCrossRefGoogle Scholar
  19. 19.
    Timmers GJ, Falke TH, Rauwerda JA et al (1999) Deep vein thrombosis as a presenting symptom of congenital interruption of the inferior vena cava. Int J Clin Pract 53(1):75–76PubMedGoogle Scholar
  20. 20.
    Dean SM, Tytle TL (2006) Acute right lower extremity iliofemoral deep venous thrombosis secondary to an anomalous inferior vena cava: a report of two cases. Vasc Med 11(3):165–169PubMedCrossRefGoogle Scholar
  21. 21.
    Tsuji Y, Inoue T, Murakami H et al (2001) Deep vein thrombosis caused by congenial interruption of the inferior vena cava—a case report. Angiology 52(10):721–725PubMedCrossRefGoogle Scholar
  22. 22.
    Yun SS, Kim JI, Kim KH et al (2004) Deep venous thrombosis caused by congenital absence of inferior vena cava, combined with hyperhomocysteinemia. Ann Vasc Surg 18(1):124–129PubMedCrossRefGoogle Scholar
  23. 23.
    Parma M, Belotti D, Marinoni S et al (2003) Congenital absence of the inferior vena cava and genetic coagulation abnormalities: a rare associated risk factor for recurrent idiopathic deep vein thrombosis. Clin Appl Thromb Hemost 9(4):347–348PubMedCrossRefGoogle Scholar
  24. 24.
    Schneider JG, Eynatten MV, Dugi KA et al (2002) Recurrent deep venous thrombosis caused by congenital interruption of the inferior vena cava and heterozygous factor V Leiden mutation. J Intern Med 252(3):276–280PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE) 2011

Authors and Affiliations

  • Suvranu Ganguli
    • 1
  • Sanjeeva Kalva
    • 1
  • Rahmi Oklu
    • 1
  • T. Gregory Walker
    • 1
  • Neil Datta
    • 1
  • Eric F. Grabowski
    • 2
  • Stephan Wicky
    • 1
  1. 1.Division of Vascular Imaging and Intervention, Department of ImagingMassachusetts General Hospital and Harvard Medical SchoolBostonUSA
  2. 2.Division of Hematology and Oncology, Department of PediatricsMassachusetts General Hospital and Harvard Medical SchoolBostonUSA

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