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Endovascular stenting of nonmalignant superior vena cava syndrome

M.A. Sheikh, B.B. Fernandez Jr., B.H. Gray, L.M. Graham, Teresa L. Carman, MD


Symptoms of superior vena cava obstruction result from the impedance of blood flow returning from the head and upper extremities. The obstruction may be from either extrinsic compression of the SVC or venous injury with resultant stenosis, with or without associated thrombosis. Most patients with SVC syndrome present with facial and upper extremity swelling with visible, dilated venous collateral vessels along the upper extremities and chest wall. Facial plethora, headache, hoarseness, visual disturbance, or altered mental status may also be present and usually necessitate treatment for the relief of symptoms.

In most cases, the diagnosis of SVC syndrome is synonymous with an advanced malignancy and portends a poor prognosis with a limited life expectancy [[1]]. Reported cases of SVC syndrome unrelated to an active malignancy have become more common because of the increasing use of transvenous cardiac devices such as permanent pacemakers and automatic defibrillators as well as chronic indwelling venous access devices for hemodialysis, pharmacotherapy, and parenteral nutrition [[2-4]]. Several other benign conditions may cause SVC syndrome, including fibrosing, inflammatory, or infectious mediastinal disease; compression from vascular anomalies or aneurysms; and nonmalignant tumors that do not limit survival in most individuals [[5]].

The management of SVC syndrome ranges from medical or supportive care to surgical bypass. SVC syndrome from malignant disease usually presents with a rapid onset necessitating symptomatic relief. In these cases, management strategies may include supportive measures using diuretics and head elevation while palliative therapy with either cytotoxic agents or radiation is implemented [[1][6]]. In many cases, endovascular intervention with angioplasty and stent placement may be employed to obtain prompt relief of the symptoms and an improved quality of life.

SVC obstruction from benign processes usually has an insidious onset allowing for the development of collateral venous channels. Many patients develop a physiologic compensation for the obstruction, and intervention for symptomatic relief may not be required. In the past, when intervention has been indicated, patients with SVC obstruction from benign etiologies have had limited options, typically requiring surgical procedures for venous bypass [[6]].

Since the advent of endovascular therapies, few series documenting the utility of such approaches for benign disease have been published [[2-4][7]]. To gain further insight into the indications, optimal treatment, complications, and outcomes, we combined the experiences from two affiliated institutions and present 19 cases of SVC syndrome from benign etiologies treated with angioplasty and stent placement.

Materials and methods

Using the database from the Interventional Vascular Laboratory at the Cleveland Clinic Foundation (CCF) and the Department of Radiology at Cleveland Clinic Florida (CCFL), we identified cases of SVC syndrome treated using endovascular procedures. Following institutional review board (IRB) approval from both institutions, the medical records were reviewed to determine the underlying etiology of the SVC obstruction. We identified 15 cases of SVC obstruction due to benign conditions (nonmalignant etiologies) treated at CCF and 4 cases treated at CCFL.

The indication for intervention was based on clinical presentation as well as radiographic evaluation. Several interventionalists performed the procedures. Each procedure was individualized and not guided by protocol. For the review, we retrospectively collected demographic information and the data regarding the underlying etiology, treatment, outcomes, and follow-up from the available medical record for each case.


The mean age of the patients treated was 46.4 years (range, 4-79 years). Fifty-eight percent (11/19) were female. The etiology of the SVC obstruction included indwelling intravenous access devices (12 cases), transvenous permanent pacemaker (2 cases), mediastinal disease (4 cases), and following surgical repair of transposition of the great vessels in 1 patient. Five patients had no information available in the medical record following intervention and were therefore considered lost to follow-up. The remaining 14 patients had at least one physician visit following intervention. The mean follow-up was 28.8 months (range, 1-86 months). Three patients have been followed for a period exceeding 6 years. Table I details the treatment and outcomes of the individual patients.

Table I. Details of Patient Characteristics and Outcomes Following Intervention

Balloon angioplasty preceded stent placement in all cases. The choice of stent used for implantation was determined by the performing interventionalist. At the time of the initial procedure, six patients had multiple stents deployed (Table I).

Thrombolytic therapy was administered in seven patients prior to definitive intervention. The choice of thrombolytic agent and the indication and duration of thrombolysis was at the discretion of the performing interventionalist. Six patients received urokinase and one received recombinant tissue plasminogen activator (tPA).

The use of adjunctive pharmacologic agents following the procedure was determined on an individual case-by-case basis by the interventionalist and the physicians caring for the patients. Anticoagulation was the preferred adjuvant therapy. Warfarin was initiated in 10 patients (10/19; 52%); 8 patients were anticoagulated indefinitely while 2 patients were maintained on warfarin for approximately 1 year. Aspirin or clopidogrel was instituted in 4/19 (21%) patients following intervention. The choice and duration of the antiplatelet therapy was individualized and ranged from 30 days to 6 months.

All patients experienced initial relief from their symptoms of SVC syndrome. Of the 14 patients available for follow-up, primary patency was 79% (11/14). Three patients required repeat intervention to maintain patency. One patient developed high-grade restenosis and required repeat angioplasty and stent placement at 3 months and again at 77 months following the initial procedure. Another patient required repeat angioplasty for recurrent symptoms on three occasions since the initial procedure: at 21, 32, and 67 months. One patient developed restenosis and failed a repeat attempt at angioplasty, eventually requiring surgical bypass for catheter-related SVC stenosis. Mid-term patency either primary or primary-assisted was maintained in 93% (13/14) of the patients.

Complications from the interventional procedures included stent migration at the time of initial expansion with partial right atrial deployment in two patients and penetration of the SVC and pericardial space in one patient. The patient with SVC penetration did not suffer tamponade or cardiovascular compromise and did not require evacuation of pericardial hematoma. One patient with partial right atrial deployment was treated with anticoagulation long-term due to concerns of thrombus formation from the malpositioned stent. The second patient was treated with aspirin alone. Neither of the patients with stent malposition have experienced complications since the time of the intervention. There were no bleeding complications documented during thrombolysis. One patient died 24 months following treatment from a subdural hematoma related to the use of anticoagulation.


In the earliest review of SVC obstruction by McIntire and Sykes [[8]] in 1949, approximately one-third of cases were due to primary intrathoracic tumors, one-third were caused by aortic aneurysms, and the remainder were attributed to granulomatous mediastinal disease and a variety of less common causes. Nonmalignant causes of SVC syndrome include infectious diseases such as tuberculosis, histoplasmosis, or pyogenic infection; fibrosing processes either idiopathic or following radiation therapy; endovascular devices such as transvenous cardiac devices or indwelling catheters; vascular anomalies, aneurysms, and vasculitis; and a number of other rarer etiologies [[5]]. In recent reports, SVC syndrome from either primary or metastatic malignancy accounted for 73-93% of all cases, while benign disease is implicated in approximately 7-27% of cases [[2][9][10]]. Older series in the literature cite mediastinal fibrosis and thrombosis as the most frequent etiologies of SVC syndrome related to benign disease [[9]]. However, the increase in permanent transvenous pacemaker and defibrillation devices as well as chronic indwelling catheter placement for parenteral nutrition, chemotherapy, and hemodialysis may increase the number of cases of benign SVC syndrome. In a more recent series, 12/16 (75%) patients who developed SVC obstruction had an indwelling venous device [[2]]. Our series also supports this contention. Seventy-four percent of the patients treated in this series developed SVC obstruction due to a transvenous indwelling device. SVC anastamosis site stenosis is a rare complication of cardiac transplant that may become increasingly problematic as more transplants are performed.

The management of SVC syndrome depends on both the acuity and etiology. Options for treating SVC obstruction due to malignant disease include medical therapy with diuretics, steroids, and head elevation followed by chemotherapy or radiation therapy depending on the type of tumor. The use of endovascular procedures with angioplasty and stent placement to treat SVC syndrome due to malignant disease is becoming relatively common. In these individuals, therapy is aimed at relieving the symptoms of obstruction and improving the patients' quality of life [[1][6]]. The procedure is generally palliative in nature with patients experiencing relief from the congestive symptoms. The life expectancy in cases of malignant SVC syndrome is usually less than 6 months and results from endovascular therapy have been durable in most cases.

SVC obstruction caused by nonmalignant disease is typically insidious in onset due to the development of venous collaterals. Therapy should still be aimed at the underlying etiology; however, in some cases, medical therapy with anticoagulation may provide relief. When medical therapy and anticoagulation alone are not sufficient to alleviate the symptoms or when there is an underlying stenosis or stricture of the vena cava, relief of the congestive symptoms can be accomplished by relieving the stenosis or compression. Historically, this has been accomplished by surgery; however, our series demonstrates that mid-term patency is acceptable in patients undergoing endovascular stent placement in this setting and this may be a feasible therapeutic option for these patients.

Four stents have been used most commonly for treating benign and malignant SVC syndrome, the Gianturco Z-stent (Cook, Bloomington, IN), the balloon-expandable Palmaz stent (Cordis, Miami, FL; Johnson and Johnson Interventional System, Warren, NJ), and the self-expanding Wallstent or Symphony (Boston Scientific, Natick, MA). None of the available stents are approved for SVC deployment. All four stents come in diameters sufficient for use within large central venous structures. The Gianturco Z-stent was the first used for SVC stenting. It is a stainless steel wire bent into a Z or zigzag pattern with anchoring hooks to prevent migration. It is self-expanding and placed through an 8 to 16 Fr introducer. The Palmaz stent is a stainless steel balloon-mounted and expanded stent. It has considerable radial force and is more rigid than self-expanding stents. The 8 series Palmaz stent can be expanded from 8 to 18 mm. Foreshortening occurs with more extreme expansion diameters. The Wallstent is self-expanding woven stainless steel mesh. It has significant radial force yet remains flexible. It has to undergo significant shortening to achieve ideal radial diameter, therefore the lesion must be properly dilated prior to deployment [[11][12]]. The Symphony stent is made of nitinol and expands to 14 mm. It is easily delivered via a 7 Fr sheath and has large fenestrations in the design to minimize vessel wall coverage. In our series, the choice of stent was at the discretion of the interventionalist and reflects the changing practice patterns of stent use. We deployed several stent types, all with good technical and clinical results.

Stent placement has been used less frequently for benign SVC obstructive disease than malignant and most of the published literature consists of case reports or small series [[2-4][7]]. Results, however, are encouraging.

Initial technical success is achieved in the vast majority of patients with nonmalignant SVC syndrome. Documented technical success in reported series is 88-100% [[2-4]]. Our 19 cases further support the success of this approach. All patients in our series had reasonable technical results despite SVC penetration in one case and stent migration in two. Clinical resolution of symptoms followed technical success.

When thrombo-occlusive disease contributes to the SVC occlusion, adjuvant thrombolytic therapy may be helpful. Successful thrombolysis allows the underlying stenosis to be delineated prior to definitive treatment. Gray et al. [[13]] published their success and safety using streptokinase and urokinase for thrombolysis in SVC syndrome of both malignant and benign etiologies. They demonstrated successful results when thrombolytic infusion was administered through an indwelling catheter within 5 days of the onset of symptoms. In central venous thrombosis, direct infusion techniques via a catheter embedded in thrombus or through a preexisting indwelling catheter are the most commonly used techniques in treating SVC syndrome. Other authors have demonstrated the utility of thrombolysis prior to angioplasty and stent placement. Rosenblum et al. [[4]] employed thrombolysis in 4/6 cases of SVC syndrome related to chronic indwelling catheter use. In another series, thrombolysis preceded definitive therapy in 4/9 patients [[7]].

Similar to these case series, we used thrombolytic agents, urokinase and recombinant tissue plasminogen activator, prior to angioplasty and stent deployment in 7/19 patients. No patients in our series suffered an adverse event related to the thrombolysis. These results support the use of adjuvant thrombolytic therapy in patients with underlying thrombo-occlusive disease and no contraindications to thrombolytic therapy.

The overall durability and longevity of the endovascular procedure is a concern in patients with benign disease and a normal life expectancy. Long-term success was achieved in the majority of patients with nonmalignant SVC syndrome. Rosenblum et al. [[4]] found no recurrent thromboses or stenoses in four patients followed 5-24 months. One of 12 patients followed 1-36 months required repeat intervention for benign SVC obstruction [[3]]. Bornak et al. [[7]] reported a 67% patency at 12 months. Two patients had recurrent symptoms and were treated with repeat intervention with satisfactory results. Kee et al. [[2]] followed 10 patients for a mean of 17 months without recurrent symptoms. One patient required multiple repeat interventions to maintain patency. These reports suggest that results of endovascular treatment of nonmalignant SVC obstruction are satisfactory at 1-2 years. Our results also support this contention. Our documented follow-up of more than 6 years in three patients is the longest we identified in the literature. This seems to speak to the overall durability of the procedure. Three patients required repeat intervention because of recurrent symptoms. Successful repeat angioplasty or stent placement was obtained in two of three patients. One patient eventually required surgical bypass for the SVC obstruction. The prior stent placement did not contribute to surgical complications at the time of bypass.

Although limited data are available, our series and those in the literature suggest that endovascular treatment of nonmalignant SVC syndrome compares favorably with surgical therapy. Surgical intervention for SVC syndrome is accomplished by performing a venous bypass procedure usually requiring a median sternotomy and accompanied by a significant risk for postoperative morbidity. Spiral saphenous vein grafts (SSVGs), expanded polytetraflouroethylene (ePTFE) or Dacron grafts, and allografts have been used for bypass [[14-19]]. In general, these procedures are fairly well tolerated. Thrombosis and recurrent stenosis are the reported long-term complications. Doty et al. [[14]] followed nine patients for up to 15 years after SSVGs. Three patients developed recurrent thrombosis, necessitating intervention in two. Two series from one institution documented primary, primary-assisted, and secondary patency rates following bypass of approximately 60%, 80%, and 85% at 1 year and 53%, 70%, and 75% at 5 years [[15][17]]. In most cases of early rethrombosis, repeat surgical intervention with thrombectomy and graft revision is required. Late bypass graft complications have been treated by surgical thrombectomy [[14][16]], graft revision [[17]], and endovascular procedures [[15][17]].

In most reported series, patients are followed clinically for a recurrence of symptoms. The optimal radiologic follow-up techniques and intervals to identify progression of disease or impending recurrence have not been well studied. Noninvasive studies to assess the mediastinal vessels are lacking. The use of magnetic resonance imaging may be limited due to the presence of the metallic stent. Chest X-ray may be beneficial to document stent migration. However, venography would be required to gain information regarding vessel patency. In some cases, duplex ultrasound may provide information regarding proximal/central venous stenosis; however, if significant collateral vessels are present, this too will likely prove unhelpful. The surveillance techniques and intervals that will optimally detect impending reocclusion require further study.

Adjuvant anticoagulation and antiplatelet agents are commonly used following stent placement; however, firm guidelines for the choice of agent are lacking [[12]]. The use of systemic anticoagulation for several months following thrombolysis is well supported. The desire is to allow the endothelium to heal and prevent early recurrent thrombosis. In addition, patients who experience a venous thromboembolic event, such as pulmonary embolism, at the time of intervention should be anticoagulated for at least 3-6 months following the event. The use of anticoagulation, either therapeutic or prophylactic, in patients with a chronic indwelling device could also be supported since the inciting structure remains in situ. Rosenblum et al. [[4]] used anticoagulation in all patients for 2-3 months following the procedure. We used anticoagulation in 10/19 patients, generally following thrombolysis, with a chronic indwelling device, or in the setting of stent malposition. In all cases, the risks and benefits of anticoagulation must be individualized for the patient. Chronic anticoagulation contributed to the single fatality in this series following a successful intervention.

The ideal choice and duration of adjuvant antiplatelet therapy or anticoagulation following SVC stent placement is unknown. In our series, the choice was at the discretion of the interventionalist. Acute venous thromboembolism treated by thrombolysis, angioplasty, and stent placement is usually followed by systemic anticoagulation. While not necessarily representative of the same underlying pathology or interventional considerations, the use of antiplatelet agents following percutaneous coronary intervention is well supported by the interventional cardiology literature. In most cases, aspirin with or without the addition of clopidogrel is well tolerated and decreases the incidence of recurrent arterial events. Further investigation of the optimal adjuvant antiplatelet or antithrombotic therapy is warranted.

In addition to access site complications typical for vascular procedures, procedural complications from SVC endovascular stent implantation that have been reported in the literature include stent malposition or migration, stent fracture, vascular laceration (SVC and aortic), and pericardial puncture causing hemorrhage with or without tamponade. We had two patients in whom initial stent placement was complicated by migration or malpositioning with a partial right atrial deployment. In both cases, a second stent was placed in the SVC to treat the stenosis as well as to anchor the malpositioned stent into place. Both of these individuals remain on indefinite anticoagulation as an empiric maneuver to avoid any risk of thrombosis due to stent protrusion into the right atrium. One patient incurred a penetration of the SVC and pericardial space without hemorrhage or major morbidity. This complication has been reported by others and has the potential for a tragic outcome [[20][21]].

The use of endovascular therapy with angioplasty and stent placement has a role in SVC syndrome from benign etiologies. More information is needed regarding the long-term patency rates, both primary and primary-assisted. Clinical follow-up and observation are important in these cases. None of the available noninvasive imaging modalities are likely to provide a good cost-effective method for periodic surveillance. The use of anticoagulation may have a role in cases complicated by thrombo-occlusive disease. The use of antiplatelet therapy following intervention is somewhat empiric. Therefore, a number of issues relative to this treatment remain unresolved. Additional study is needed to determine the best method and interval timing for periodic surveillance as well as the optimal adjuvant therapy with regard to the use and duration of anticoagulation and antiplatelet agents.


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Autor: M.A. Sheikh, B.B. Fernandez Jr., B.H. Gray, L.M. Graham, Teresa L. Carman, MD

Fuente: Catheterization and Cardiovascular Interventions. Volume 65, Issue 3, Pages 405-411

Ultima actualizacion: 17 DE JULIO DE 2005

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