ABSTRACT: In patients presenting with deep vein thrombosis (DVT), management includes addition of anticoagulation and may also include placement of an inferior vena cava (IVC) filter to prevent pulmonary arterial embolization. Due to the hypercoagulable state, if not adequately anticoagulated and monitored, the development of a de novo, or progression of an old thrombus distal to the IVC filter, cannot be prevented; the presence of IVC filter further increases the possibility of thrombus formation and extension. We present a case of recurrent DVT, with a thrombus extending from the filter placed at the level of the renal vein, up to the right atrium, giving a “balloon-on-string” appearance.

VASCULAR DISEASE MANAGEMENT 2016;13(12):E281-E284

Key words: anticoagulation, deep vein thrombosis, vascular intervention, thrombosis, interventional cardiology

__________________________________________________________

Recurrent venous thrombotic events with or without presence of a transient predisposing factor(s) suggest presence of thrombophilia. Thrombophilia is a group of disorders, generally inherited, characterized by defects in the normal coagulation pathways, such as antithrombin deficiency, protein C, and protein S deficiency,¹ but it may also be an acquired defect, as observed in autoimmune disorders like systemic lupus erythematosus and primary antiphospholipid antibody syndrome. It might also include more common inherited thrombophilias, such as factor V Leiden mutation and prothrombin G20210A mutation. In patients presenting with deep vein thrombosis (DVT), management primarily includes anticoagulation. However, where anticoagulation – including oral anticoagulant (OAC); parenteral agent like low molecular weight, unfractionated heparin; or pentasaccharides like fondaparinux – is contraindicated or if side effects of anticoagulation occur, the option is to place an inferior vena cava (IVC) filter to prevent pulmonary embolization.² Even after IVC implantation, anticoagulants may be restarted at a later date once the initial contraindication is no longer an issue. In fact, beginning long-term anticoagulation once the window of opportunity opens later is of paramount importance in prevention of thrombotic episodes, especially in thrombotic disorders. Thrombophilia with IVC filter placement demands more aggressive anticoagulation. Due to the hypercoagulable state, if not adequately anticoagulated and monitored, the development of a de novo thrombus or progression of an old thrombus distal to the IVC filter cannot be prevented. The presence of an IVC filter further increases the possibility of thrombus formation and extension. One of the important and devastating complications of IVC filter, though very rare, is total thrombotic occlusion of the filter. In the present report, we are presenting a case of recurrent DVT, with a thrombus extending from the filter placed at the level of renal vein, up to the right atrium, giving a “balloon-on-string” appearance.

Case Presentation

A 25-year-old female presented to the emergency department with shortness of breath and palpitations. She was referred with a possible diagnosis of right atrial myxoma, diagnosed on transthoracic echocardiography. Her past medical records revealed a history of DVT – chronologically, an isolated incident not associated with any precipitating risk factor initially and subsequently, 2 incidents associated with pregnancy. Her family history indicated DVT in her mother and maternal aunt. Laboratory investigations, including clinical biochemistry and hematology, were within normal limits. Echocardiography revealed a large “ball-valve like” thrombus across the tricuspid valve with mild right atrial and right ventricular dilatation and mild pulmonary arterial hypertension. Computed tomographic (CT) pulmonary angiogram revealed thrombus in bilateral lower lobe branches. Whole-abdomen CT with contrast was performed and showed linear eccentric filling defect in the IVC, extending from the IVC filter into the right atrium. A multidetector computed tomography (MDCT) venography confirmed the presence of thrombi with a “balloon-on-string” appearance (Figure 1). Thrombolysis was considered risky due to the potential for catastrophic release of thrombus from the IVC filter into the pulmonary artery, leading to pulmonary embolism because of the long and thin “string-like” attachment. Thrombosuction also carries the risk of breaking loose the “balloon clot” into the pulmonary arterial system. Thrombolysis with recombinant tissue plasminogen activators has the potential of dislodging the thrombus (balloon component) with catastrophic consequences. Hence, surgical treatment was considered the ideal option and the removal of the thrombus was done under deep hypothermic total circulatory arrest (DHCA). The postoperative period was uneventful, echocardiogram showed no evidence of thrombus, and the patient was discharged. 

Her first clinical event was an isolated DVT of the right leg, which occurred when she was 14 years old, for which she was managed conservatively with short-course anticoagulants. This was followed by a second episode of left-leg DVT 10 years later during the first trimester of her first pregnancy, which was successfully managed with anticoagulants. Six months subsequent to the successful delivery, she again developed symptoms suggestive of left leg DVT. Though she was advised postpartum anticoagulation for 6 months, the actual duration for which she took anticoagulant is unknown. Evaluation identified her surprisingly to be 10 weeks into her second pregnancy. Doppler ultrasound revealed thrombosis of IVC extending into major veins of lower limbs, involving both common iliac, external iliac, and saphenous veins. She was hospitalized and high prothrombin time protocols were utilized to maintain optimal PT for managing DVT. Medical termination of pregnancy was done to ensure her safety. A Gunther Tulip IVC filter (Cook Medical) was placed at the level of the renal vein, as a prophylaxis against pulmonary embolism. 

Because the thrombus involved the lower part of the IVC to just below the renal vein, classical infrarenal placement was not possible. In view of the previous history of thromboembolism, she was extensively evaluated for thrombophilia during the hospitalized period, although it did not reveal any abnormality. She was discharged with prescription of oral warfarin and advice for regular monitoring of prothrombin time and international normalized ratio (INR). She was also advised against future pregnancy. Subsequent to hospital discharge, her compliance with DVT prophylaxis advice until the most recent clinical event was not traceable, making it difficult to contribute the present episode to noncompliance, lack of prothrombin and INR tracking, or inability of regular anticoagulant use to control DVT.

The occurrence of the first DVT without any association of any risk factor (idiopathic DVT) points to a diagnosis of thrombophilia; moreover, the probability of inherited thrombotic disorder is high in those with positive family history and young age of initial presentation. Importantly, in those with inherited thrombotic disorder, the risk of recurrent DVT without a precipitating cause increases with the duration of follow-up to about 15%, 41%, and 53% at 1, 5, and 10 years.³ Furthermore, testing for specific underlying coagulation abnormality seems not to be cost effective and seems of questionable significance in predicting future DVT events.⁴

The present case describes an unusual presentation of thrombophilia. It stresses the importance of long-term anticoagulant therapy and the need for close monitoring to prevent recurrence. The presence of an IVC filter demands closer follow-up for potential filter thrombosis, which may occur with thrombophilia or with poor compliance to therapy.

Editor’s note: Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no financial relationships or conflicts of interest regarding the content herein.

Manuscript received May 10, 2016; provisional acceptance given July 19, 2016; manuscript accepted September 7, 2016.

Address for correspondence: Vijaya Chandrareddy, MD, Apollo Hospitals, 21 Greams Lane, Off. Greams Road, Chennai, Tamil Nadu 600 006 India. Email: vijayachandrareddy31@gmail.com

References

1. Christiansen SC, Cannegieter SC, Koster T, Vandenbroucke JP, Rosendaal FR. Thrombophilia, clinical factors, and recurrent venous thrombotic events. JAMA. 2005;293(19):2352-2361.
2. Kearon C, Kahn SR, Agnelli G, Goldhaber S, Raskob GE, Comerota AJ. Antithrombotic therapy for venous thromboembolic disease: American College of Chest Physicians evidence-based clinical practice guidelines (8th edition) [published correction appears in Chest. 2008; 134(4):892]. Chest. 2008;133(6 suppl):454S-545S.
3. Prandoni P, Noventa F, Ghirarduzzi A, et al. The risk of recurrent venous thromboembolism after discontinuing anticoagulation in patients with acute proximal deep vein thrombosis or pulmonary embolism. A prospective cohort study in 1,626 patients. Haematologica. 2007;92(2):199-205.
4. Ho WK, Hankey GJ, Quinlan DJ, Eikelboom JW. Risk of recurrent venous thromboembolism in patients with common thrombophilia: a systematic review. Arch Intern Med. 2006;166(7):729-736.