Figure 1. Diagnostic angiography showing the interruption of the IVC in its hepatic segment (A), and...

Figure 2. Phlebography after treatment with thrombolytic agents revealing the presence of partial re...

Figure 3. Magnetic resonance angiography after treatment revealing the patency of the azygos vein an...

Figure 4. Final image of the angiography of the left common iliac vein with venous deployment of sel...

Figure 5. Control phlebography 10 months after the procedure that confirms the patency of the stent ...

Case report

This is the case of a 45-year-old obese woman admitted due to bilateral iliofemoral deep vein thrombosis (DVT) of a 4-day clinical course with ultrasound evidence of compromise to the common femoral and superficial veins, and bilateral deep veins with spread towards both iliac axes and IVC. Due to the epidemiological context under which the patient is admitted to the hospital, a nasopharyngeal swab is performed that tests positive for COVID-19. The patient’s clinical course is interpreted as an asymptomatic infection with the following lab results at admission: platelet count, 455 000 mcL; CRP, 39.8mg/L; ferritin, 115.4 ng/mL; and D-dimer levels, 1.2 mg/L (reference levels < 0.2 mg/L).

Anticoagulant therapy with low-molecular-weight heparin (LMWH) is started, but disease progresses despite the therapy administered. For that reason, she is referred to our center on day 7 for endovascular resolution.

Phlebography via right anterior jugular venous access is performed (ultrasound guided puncture). A multipurpose catheter is advanced that reveals the presence of an interrupted IVC in its hepatic segment with drainage of suprahepatic veins into the right atrium (figure 1). Both femoral veins are punctured, which reveals the presence of bilateral iliofemoral thrombosis spreading towards the infrarenal segment of the inferior vena cava. Drainage through the azygos-hemiazygos venous system—which seem dilated—is performed.

Catheter-directed thrombolysis (CDT) is performed using the FOUNTAIN® INFUSION SYSTEMS catheter (Merit Medical, United States) from both accesses with the infusion of rtPA in doses of 0.5 mg/h plus sodium heparin in anticoagulation doses for 48 hours total.

After the period of treatment established, the patient showed a clear improvement of her clinical signs and symptoms. A new phlebography is performed that confirms the presence of partial residual thrombosis in right common iliac, external, and bilateral femoral veins with images compatible with severe stenosis of the left common iliac vein. Venous drainage through the azygos system is performed that reveals the presence of a severe obstruction at the origin of this vein. Successful thrombus aspiration is performed using an 8-Fr Judkins Right guide catheter with dilatation of the ostial obstruction of the azygos route using two 6.0 mm x 40 mm and 8.0 mm x 60 mm peripheral balloons (Admiral Xtreme™ PTA balloon catheter, Medtronic, Minneapolis, United States). The obstruction of the common iliac vein is dilated. Angiographic control confirms the proper drainage of both iliofemoral axes through the azygos system towards the superior vena cava (Figure 2).

The patient remains on anticoagulant therapy and a magnetic resonance angiography is performed 1 week later to assess the patency of the target vessels (Figure 3). A new occlusion in the left common iliac vein is confirmed, and an angioplasty of this vessel is performed with self-expanding venous stent implantation (VENOVOTM Venous Stent System Bard (Peripheral Vascular Inc., Tempe, AZ, United States) (Figure 4).

Ten (10) months after treatment the patient did not relapse. New phlebographic control was performed that confirmed the patency of both the target venous system and the stent implanted. (Figure 5)

Discussion:

Blood from the venous system reaches the right atrium through the superior and inferior venae cavae. The azygos-hemiazygos system receives venous blood from both the thorax and the abdomen connecting both systems, which is sometimes a circulation alternative.

From the embryological standpoint these anatomical structures mostly originate at the cardinal venous system that includes the anterior and posterior cardinal veins, the common cardinal vein, and the subcardinal veins. The formation of these anatomical structures is completed during the development, anastomosis or involution of the different segments involved, something that occurs between gestational weeks 4 and 8.

The inferior vena cava can be divided into different segments based on their origin: suprahepatic segment (right vitelline vein), hepatic renal segment (anastomosis of the right vitelline vein, and the subcardinal vein), suprarenal segment (right subcardinal vein), renal segment (right sub-supracardinal anastomosis), and infrarenal segment (right supracardinal vein). Finally, the terminal segment of the IVC and both iliac veins originate at the posterior cardinal vein.(1)

Developmental disorders can cause anatomical anomalies such as:

• Double IVC: persistence of both supracardinal veins. Frequency, 0.2% to 3%. The left component often drains into the left renal vein.
• Left IVC: persistence of left supracardinal vein associated with the regression of its contralateral equivalent. Frequency, 0.2% to 0.5%.
• Interruption of the IVC infrarenal segment with suprarenal preservation: pathological development of the cardinal posterior veins and supracardinal veins.
• Abnormalities of the left renal vein: variation in the persistence or regression of the 2 embryological components of the left renal collar.
• Circumcaval urether: abnormality of the right supracardinal system (0.07%).
• Interruption of the IVC hepatic segment with continuation through the azygos system.

  The latter abnormality is due to the lack of anastomosis of the right vitelline vein and subcardinal vein with the corresponding regression of the right subcardinal vein making blood drain directly into the right supracardinal vein. Its incidence rate in the overall population is 0.6% as an isolated finding. However, this rate increases dramatically when it is found together with other developmental disorders like visceral heterotaxy, mainly the polysplenia syndrome. The hepatic segment is not really absent, but instead drains directly into the right atrium together with suprahepatic veins. Therefore, venous drainage occurs alternatively through the azygos-hemiazygos system being the typical finding a dilated azygos vein present in the CT imaging at the entrance of the SVC and the retrocrural space.(1)(2)(3)(4)(5)

  These venous malformations are often asymptomatic. However, different series already published reveals predisposition towards the development of thromboembolic disease due to venous insufficiency. The interruption of the IVC has been described in nearly 5% of all DVTs without an apparent cause in patients < 30 years.(4)(6)

  The management of deep venous thrombosis has been studied for years trying to establish whether anticoagulation alone or associated with CDT or pharmacoinvasive thrombectomy would have better results minimizing post-thrombotic syndrome (PTS), pulmonary thromboembolism (PTE), and keeping venous patency in the short- and long-term.(7)

  Several randomized clinical trials and meta-analyses have demonstrated that in the presence of iliofemoral thrombosis the combination of thrombolytic and anticoagulant agents reduced the development of PTS since it reduced the thrombotic burden even more with a variable rate of bleeding.(8(9)(10)

  Following in these footsteps, the CAVENT trial randomized 209 patients with iliofemoral DVT occurring within the 21 days after symptom onset to receive anticoagulant therapy alone or in combination with CDT with the infusion of alteplase for a maximum of 96 hours. An ARR of 14.4% of post-thrombotic syndrome was reported at the follow-up for a NNT of 7 patients to prevent a single event of PTS with a low rate of bleeding.

  A meta-analysis conducted by Wang Li et al. that compared CDT to anticoagulation for the management of iliofemoral DVT confirmed an odds ratio of 0.38 (95%CI, 0.26–0.55) for the development of PTS favorable to CDT. Also, patency with this treatment was even higher after 6 months (OR, 4.76; 95%CI, 2.14–10.56).(11)

  Mechanical thrombectomy (MT) associated, or not, with the infusion of lytic agents (pharmaco-mechanical thrombectomy) is performed with different devices which–through a process of suction, rotation, rheolytic thrombectomy, ultrasound or a combination of these—reduce the thrombotic burden.(12) Examples of these are the Angiojet system (Boston Scientific), and EKOS lysis catheter (BTG). The development of this technique is based on the hypothesis that shorter procedural times, lower doses of lytic agents or the nonuse of these agents associated with the thrombectomy methods implemented would reduce even further the thrombotic burden and yield better results in the prevention of PTS with a lower rate of bleeding. To this point, evidence is controversial with great heterogeneity in the studies conducted so far.

  The meta-analysis published by Wang et al. that assessed the results of MT in the management of DVT concluded that this treatment with or without CDT proved to be safe and effective with a satisfactory rate of lysis, a low rate of recurring thrombosis and complications, good long-term results, and a low rate of PTS. On the other hand, a rate of re-thrombosis in the short- and long-term of 11.9% and 10.7%, respectively, was reported which may have to do with endothelial damage. The rate of PTS at the follow-up was 15.1% similar to that of CDT.(13)

  Based on the evidence studied to this date, we can conclude that regardless of the method used we should be aggressive in the management of iliofemoral DVT to prevent PTS and pulmonary embolism, as well as any serious complications associated with the clinical course such as phlegmasia cerulea dolens.

  References

  1. Oliveira JD, Martins I, Oliveira and Martins ;Congenital systemic venous return anomalies to the right atrium review; Insights into Imaging 10:115 (2019). https://doi.org/10.1186/s13244-019-0802-y.
  2. (2) Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of Congenital Anomalies of the Inferior Vena Cava: Cross-sectional Imaging Findings; Radiographics May-Jun 2000;20(3):639-52. doi:10.1148/radiographics.20.3.g00ma09639.
  3. Mandato Y, Pecoraro C, Gagliardi G, Tecame M. Azygos and hemiazygos continuation: An occasional finding in emergency department. Radiology Case Reports 2019;14:1063-8.
  4. Skeik N, Wickstrom KK, Schumacher CK, Sullivan TM. Infrahepatic Inferior Vena Cava Agenesis with Bilateral Renal Vein Thrombosis. Ann Vasc Surg 2013;27:973.e19e973.e23. http://dx.doi.org/10.1016/j.avsg.2012.10.030.
  5. Moss and Adams’. Heart Desease in infant, Children and Adolescent; Cap 36; pág 911-33; 9na edición.
  6. Yen-Lin Chee, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol 2001;Sep;114(4):878-80.doi: 10.1046/j.1365-2141.2001.03025.x.
  7. Qais Radaideh, Neel M Patel, Nicolas W Shammas. Iliac vein compression: epidemiology, diagnosis and treatment. Vasc Health Risk Manag 2019;May 9;15:115-122.doi: 10.2147/VHRM.S203349.
  8. Tone Enden, Ylva Haig, Nils-Einar Kløw, et al., on behalf of the CaVenT Study Group. Long-term outcome after additional catheter-directed thrombolysis versus standard treatment for acute iliofemoral deep vein thrombosis (the CaVenT study): a randomised controlled trial. Lancet 2012;379:31-8.
  9. Engelberger RP, Spirk D, Willenberg T, et al. Ultrasound-Assisted Versus Conventional Catheter-Directed Thrombolysis for Acute Iliofemoral Deep Vein Thrombosis. Circ Cardiovasc Interv 2015;8:e002027. DOI: 10.1161/CIRCINTERVENTIONS.114.002027
  10. Vedantham S, Goldhaber SZ, Julian JA, et al., for the ATTRACT Trial Investigators. Pharmacomechanical Catheter-Directed Thrombolysis for Deep-Vein Thrombosis. N Engl J Med 2017;377:2240-52.
  11. Wang Li, Zhang Chuanlin, Mu Shaoyu, Chao Hsing Yeh, Chen Liqun, Zhang Zeju. Catheter-directed thrombolysis for patients with acute lower extremity deep vein thrombosis: a meta-analysis. Review Article Rev Latino-Am. Enfermagem 2018;26:e2990
  12. Wang W, Sun R, Chen Y, Liu C. Meta-analysis and systematic review of percutaneous mechanical thrombectomy for lower extremity deep vein thrombosis. Elsevier Inc. https://doi.org/10.1016/j.jvsv.2018.08.002.
  13. Engelberger RP, Spirk D, Willenberg T, et al. Ultrasound-Assisted Versus Conventional Catheter-Directed Thrombolysis for Acute Iliofemoral Deep Vein Thrombosis. Circ Cardiovasc Interv 2015 Jan;8(1):e002027.

1. Oliveira JD, Martins I, Oliveira and Martins ;Congenital systemic venous return anomalies to the right atrium review; Insights into Imaging 10:115 (2019). https://doi.org/10.1186/s13244-019-0802-y.

2. Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of Congenital Anomalies of the Inferior Vena Cava: Cross-sectional Imaging Findings; Radiographics May-Jun 2000;20(3):639-52. doi:10.1148/radiographics.20.3.g00ma09639.

3. Mandato Y, Pecoraro C, Gagliardi G, Tecame M. Azygos and hemiazygos continuation: An occasional finding in emergency department. Radiology Case Reports 2019;14:1063-8.

4. Skeik N, Wickstrom KK, Schumacher CK, Sullivan TM. Infrahepatic Inferior Vena Cava Agenesis with Bilateral Renal Vein Thrombosis. Ann Vasc Surg 2013;27:973.e19e973.e23. http://dx.doi.org/10.1016/j.avsg.2012.10.030.

5. Moss and Adams’. Heart Desease in infant, Children and Adolescent; Cap 36; pág 911-33; 9na edición.

6. Yen-Lin Chee, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol 2001;Sep;114(4):878-80.doi: 10.1046/j.1365-2141.2001.03025.x.

   Qais Radaideh, Neel M Patel, Nicolas W Shammas. Iliac vein compression: epidemiology, diagnosis and treatment. Vasc Health Risk Manag 2019;May 9;15:115-122.doi: 10.2147/VHRM.S203349.

7. Tone Enden, Ylva Haig, Nils-Einar Kløw, et al., on behalf of the CaVenT Study Group. Long-term outcome after additional catheter-directed thrombolysis versus standard treatment for acute iliofemoral deep vein thrombosis (the CaVenT study): a randomised controlled trial. Lancet 2012;379:31-8.

8. Engelberger RP, Spirk D, Willenberg T, et al. Ultrasound-Assisted Versus Conventional Catheter-Directed Thrombolysis for Acute Iliofemoral Deep Vein Thrombosis. Circ Cardiovasc Interv 2015;8:e002027. DOI: 10.1161/CIRCINTERVENTIONS.114.002027

9. Vedantham S, Goldhaber SZ, Julian JA, et al., for the ATTRACT Trial Investigators. Pharmacomechanical Catheter-Directed Thrombolysis for Deep-Vein Thrombosis. N Engl J Med 2017;377:2240-52.

10. Wang Li, Zhang Chuanlin, Mu Shaoyu, Chao Hsing Yeh, Chen Liqun, Zhang Zeju. Catheter-directed thrombolysis for patients with acute lower extremity deep vein thrombosis: a meta-analysis. Review Article Rev Latino-Am. Enfermagem 2018;26:e2990

11. Wang W, Sun R, Chen Y, Liu C. Meta-analysis and systematic review of percutaneous mechanical thrombectomy for lower extremity deep vein thrombosis. Elsevier Inc. https://doi.org/10.1016/j.jvsv.2018.08.002.

12. Engelberger RP, Spirk D, Willenberg T, et al. Ultrasound-Assisted Versus Conventional Catheter-Directed Thrombolysis for Acute Iliofemoral Deep Vein Thrombosis. Circ Cardiovasc Interv 2015 Jan;8(1):e002027.

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