Endovenous Management of Central and Upper Extremity Veins

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Chapter 18 Endovenous Management of Central and Upper Extremity Veins

In the era of central venous catheters and implantable pacemakers and defibrillators, upper extremity deep venous thrombosis (UEDVT) has become more frequent. Traditionally, upper extremity and central vein obstruction was associated with hypercoagulability, malignancy, and superior vena cava (SVC) syndrome. At present, central vein stenosis and thrombosis, usually from neointimal hyperplasia, are commonly seen in dialysis-dependent patients or in patients who have had placement of an indwelling central venous catheter. It is estimated that approximately 10% of all cases of deep venous thrombosis (DVT) occur in the upper extremity veins. Even though these cases are less likely to result in pulmonary embolism and postthrombotic syndrome than is lower extremity DVT, they are associated with a risk of pulmonary embolism (5.6%), venous gangrene, and disabling arm or neck swelling.

Primary Upper Extremity Deep Venous Thrombosis: Paget Schroetter Syndrome

UEDVT can be classified as primary, when it occurs without an inciting cause, and secondary, when it is related to an underlying catheter or previous catheter. Primary UEDVT, also known as effort thrombosis, usually occurs in healthy young individuals (third decade of life) who present with sudden, severe swelling of the upper extremity that may be associated with cyanosis and arm paresthesias. The condition is usually seen in patients after a repetitive activity and is known by the eponym Paget-Schroetter syndrome after a British and a German physician who simultaneously reported it. These patients usually have a compressive phenomenon at the thoracic outlet. It is traditionally seen at the level of the clavicular head and first rib. The scalenus anterior muscle and tendon, along with the bony structures, may compress the subclavian vein at this level, particularly at some stressed positions of the arm. The process can also be seen with the cervical ribs (Figs. 18-1 and 18-2). The combination of the compression of the costoclavicular portion of the axillosubclavian vein and repetitive stress leads to intimal damage and subsequent fibrotic reactions of the underlying vein. This intimal damage then promotes thrombosis. Patients with effort thrombosis must be identified because if this condition is not treated, it can progress and patients may experience chronic disability (25%-74%).

Fig 18–1 Diagram demonstrating the junction of both brachiocephalic veins into the superior vena cava.

Fig 18–2 A frontal chest radiograph (A) and magnified view (B) depict the presence of a right-sided cervical rib (arrow). This is just one of the possible causes of anatomic compression of the axillosubclavian vein.

Treatment

As opposed to patients with secondary UEDVT, in these patients there is an extrinsic venous compression responsible for the thrombosis. The goal of treatment is to restore venous patency and relieve the compression. When effort thrombosis is suspected in a patient, we usually perform catheter-directed chemical thrombolysis (if the patient is a candidate) followed by limited angioplasty if needed to restore flow. The results of the angioplasty are variable depending on the underlying injury to the vein. The thought is that if the vein can be gently dilated to attempt to maintain patency, the patient can then undergo anticoagulation until decompression surgery. It is important to diagnose the compression during the thrombolysis procedure and understand that the long-term patency of angioplasty will be limited in the setting of external compression. Stents should never be placed in this compressive environment.

There is controversy about whether to operate immediately or several weeks after thrombolysis. Some surgeons will only operate in the setting of persistent symptoms. We typically treat these patients with immediate surgery shortly after thrombolysis to relieve the compression. Several weeks after surgery, the status of the vein can be reassessed with ultrasound and angioplasty can be performed as needed to maintain or reestablish flow.

Prior to treatment, it is common for patients to have a venous duplex ultrasound demonstrating thrombosis. A computed tomography scan or magnetic resonance image highlighting the venous phase may be helpful in demonstrating the compression and excluding other sources of central occlusion (Fig. 18-3). Ultrasound-guided vascular access is achieved traditionally into the basilic or brachial vein. It is important to establish that the vein being punctured is patent. A 4-Fr or 5-Fr mini-stick microcatheter system is used to perform an initial venogram and document the extent of thrombosis (Fig. 18-4). Once the entry vein is found to be satisfactory, a 5-Fr or 6-Fr vascular sheath is placed. The axillosubclavian thrombosis is crossed with a soft tip guidewire (Bentson wire; Cook Medical, Bloomington, IN) and an angled catheter (Kumpe or multipurpose). The catheter is exchanged for a thrombolysis catheter that is placed across the area of thrombosis (Fig. 18-5). The catheter infusion length is matched to the length of thrombosis, and the patient is treated overnight with thrombolytics. The choice and dose are usually dependent on the operator’s choice, amount of thrombus, and patient risk factors. We usually coadminister peripheral low-dose heparin during the infusion, typically 300 units of heparin per hour (Fig. 18-6). In the setting of an acute decompensation requiring rapid thrombectomy or a patient who may not be a candidate for chemical thrombolysis, mechanical thrombectomy may be performed.

Fig 18–3 A, A coronal reformation of a computed tomography scan during the venous phase demonstrating a focal thrombosis of the left axillosubclavian vein (arrow). B, A sagittal reformation of a computed tomography scan during the venous phase demonstrating a focal thrombosis of the left axillosubclavian vein (arrow). Note location of the vein between the clavicle and the first in both reformations.

Fig 18–4 A frontal left upper extremity venogram demonstrating occlusion with thrombosis of the left axillosubclavian vein in a subtracted (A) and unsubtracted (B) image.

Fig 18–5 Frontal image demonstrating a wire across the occlusion and terminating in the inferior vena cava with a thrombolysis catheter (6-Fr EKOS ultrasound-aided infusion catheter [Bothell, WA]) positioned across the occlusion for overnight chemical thrombolysis.

Fig 18–6 Image demonstrating a 6-Fr sheath in the brachial vein with an EKOS infusion catheter through the sheath. This patient received 0.5 mg/hr of TPA (alteplase; Genentech, San Francisco, CA) infused via the EKOS catheter and 300 units/hr of heparin via the sheath.

After overnight catheter-directed thrombolysis, a venogram is performed to assess the patency of the axillosubclavian vein (Fig. 18-7). Usually, overnight thrombolysis is sufficient to clear the associated thrombus, but in certain patients a second day of thrombolysis may be necessary. As with any thrombolysis procedure, the patient is informed of the potential risk of bleeding and monitored carefully as well as with fibrinogen levels. A venogram is then performed in a provocative position to assess the amount of compression and secure the diagnosis. We usually abduct and externally rotate the arm over the patient’s head. The area of compression on the vein is usually still present after thrombolysis (Figs. 18-8 and 18-9). Angioplasty can then be performed if there is a persistent high-grade stenosis (Fig. 18-10). A low-pressure balloon is used not solely to treat the venous obstruction but also to assess the amount of fibrosis and neointimal damage because a severely damaged vein may require a venous patch at the time of surgical decompression. The surgical decompression may be performed from a transaxillary or supraclavicular approach. A portion of the first rib along with the tendinous attachment of the transected anterior scalene muscle is usually removed (Fig. 18-11).

Fig 18–7 Venogram after overnight thrombolysis demonstrating clearing of thrombus with minimal irregularity at the level of compression.

Fig 18–8 Venogram performed with the patient in a stressed position.

Fig 18–9 Positioning of patient for stressed views. We usually abduct and externally rotate the arm.

Fig 18–10 Dilating the residual narrowing or venous thickening after lysis.

Fig 18–11 Surgical specimen of the first rib with associated anterior scalene tendinous attachment.

(From Abilio Coello, MD, FACS, Miami Vascular Specialists.)

Secondary Primary Upper Extremity Deep Venous Thrombolysis

The frequent use of central and peripheral catheters has increased the incidence of upper extremity venous stenosis and DVT. These patients usually present with pain and/or arm swelling. Depending on the clinical situation, peripheral venous catheters are removed in favor of other access sites. Patient symptoms and extent of thrombosis determine if anticoagulation should be initiated for peripheral upper extremity venous thrombosis. Axillary, subclavian, and jugular thrombosis is usually treated with anticoagulation to improve symptoms and prevent pulmonary embolism. The placement of SVC filters in patients who are intolerant of anticoagulation is controversial, and usually only patients who have a suspected upper extremity thrombus pulmonary embolism or are at high risk of a life-threatening pulmonary embolism are treated with SVC filters. SVC filter placement is a higher risk procedure that should be carefully considered, especially because the incidence of life-threatening pulmonary embolism from an upper extremity source is unknown.

Patients with a central catheter–associated thrombosis are at slightly higher risk of pulmonary embolism. Pulmonary embolism has also been associated with the removal of these catheters in the acute setting of thrombosis. We will usually anticoagulate these patients and remove the tunneled central catheters only if the patient’s symptoms persist or worsen on anticoagulation. Prevention of unnecessary subclavian punctures and catheter placement may limit the amount and extent of subclavian venous stenosis and thrombosis.

Dialysis Access–Related Central Venous Stenosis

Central venous stenosis occurs in a significant number (11% to 40%) of patients on hemodialysis. It is the leading cause of shunt dysfunction and is associated with venous hypertension and arm swelling. The cause of dialysis-associated central vein stenosis is unknown but is likely multifactorial. The neointimal fibrosis responsible for these central stenotic lesions may be associated with prior centrally placed catheters. The damage due to previous subclavian punctures has led the Kidney Disease Outcomes Quality Initiative (DOQI) guidelines to strongly discourage unnecessary subclavian punctures, but the presence of a central catheter, even from the jugular approach, may cause sufficient endothelial damage from the trauma associated with its continual motion within the body. The turbulent and high flow associated with upper extremity hemodialysis access may explain central venous stenosis in patients who have never received a central catheter.

The central venous stenosis not only can be symptomatic with severe arm swelling but also may limit the function of a hemodialysis access. The treatment of central vein stenosis associated with hemodialysis is traditionally angioplasty. Unfortunately, the 1-year patency of these treatments has been reported to be between 10% and 30%, and the use of multiple additional procedures to maintain secondary patency is the rule. The initial use of stents to treat central venous stenosis has been discouraged because of a similar patency rate to that of angioplasty and the possibility of stent compression in the thoracic outlet. In addition, there are no U.S. Food and Drug Administration (FDA)–approved uncovered stents for the venous system. Even though the results from angioplasty are limited, endovascular dilatation of these stenoses is preferred over surgical options because of its availability, noninvasiveness, low risk of morbidity, and ability to repeat as needed. DOQI guidelines recommend stent placement for central lesions that recur within 3 months after angioplasty and demonstrate immediate vessel recoil greater than 50% after angioplasty and vessel perforation. The patency of a stent in central venous stenosis is similar to that of angioplasty, but the ability of retreatment becomes limited (Figs. 18-12 and 18-13). The use of covered self-expandable stents has shown promise, particularly in the setting of peripheral dialysis graft anastomotic stenoses. Their use will likely increase in the central veins; however, the operator needs to be careful in not excluding other draining veins with a central covered stent.

Fig 18–12 A 57-year-old woman with end-stage renal disease and central stenosis at the junction of the left brachiocephalic vein and superior vena cava. The patient has an occluded right upper extremity system.

Fig 18–13 A 57-year-old woman with central stenosis resistant to venoplasty and treated with a 14-mm × 60-mm Smart stent (self-expanding nitinol; Cordis Johnson & Johnson [Bridgewater, NJ]) dilated to 10 mm.

Technically, angioplasty of central venous stenosis associated with hemodialysis is usually performed from the fistula and/or graft. In the setting of vessel occlusion, femoral access may also be needed. A sheath is placed in the access site, and a complete shunt study is performed with venography. The lesion is best treated over a working wire (Amplatz, Rosen, Torque). We usually begin with a low-pressure balloon (10- to 12-atm burst pressure). The sizing of the balloon is essential to prevent rupture. The dilatation of the stenosis is performed using a 3-minute inflation and a insufflator device to control the pressure administered. Care must be taken not to thrombose the access. If the stenosis is not dilated by the low-pressure balloon, a high-pressure balloon is used (Blue Max; Boston Scientific, Natick, MA, or Conquest; Bard, Tempe, AZ). If the stenosis was dilated by the low-pressure balloon inflation but demonstrated recoil, the decision can be made to stent the lesion, attempt a high-pressure balloon, or score the stenosis with a cutting balloon or similar product. It is important not to simply redilate with a larger-diameter balloon because this may risk rupturing the vein.

Superior Vena Cava Syndrome

Patients with SVC syndrome present with symptoms that include arm and neck swelling or pain as well as edema, erythema, orthopnea, and paresthesias. The symptoms may worsen with the patient in the recumbent position. SVC syndrome can develop progressively or acutely and is usually related to advanced oncologic disease such as lung cancer or mediastinal disease. However, SVC stenosis and its associated syndrome can also occur as a consequence of benign fibrosing conditions of the mediastinum such as sarcoidosis as well as previous radiation therapy and long-standing hemodialysis and the use of central venous catheters. The underlying condition consists of occlusion or severe stenosis of the central veins, preventing sufficient venous blood flow from returning into the right atrium. The jugular veins on each side of the neck join the subclavian vein to form the brachiocephalic vein in the chest. The right and left brachiocephalic veins then join to form the superior vena cava. The lesions may occur in the SVC or in a number of central extremity veins, creating the equivalent to an SVC lesion. Treatment for SVC syndrome can include chemotherapy or radiation therapy, especially for chemosensitive and radiosensitive masses, but the response may take a few days, which may be unacceptable to patients with severe symptoms.

Even though there are no FDA-approved stents for the venous system, stents are used “off-label” in patients with SVC syndrome with improvement in 70% to 90% of patients’ symptoms. It is important to remember that the goal of treatment in patients with SVC syndrome is palliation. The long-term effects and patency of stents may not be relevant in patients with malignant obstruction or life-threatening symptoms. There is controversy surrounding stenting in the setting of SVC obstruction because a percentage of patients develop collateral pathways and may not become symptomatic. It is important to evaluate every patient individually, assessing symptoms and all available treatment options (Figs. 18-14 through 18-16). It is common for patients on hemodialysis to present with SVC syndrome due to central occlusions and stenosis (Figs. 18-17 through 18-19).

Fig 18–14 A 61-year-old patient with left upper lobe lung cancer presents with left arm and face swelling. Computed tomography scan suggests brachiocephalic compression.

Fig 18–15 Venogram confirms compression and severe stenosis.

Fig 18–16 Venogram after stent placement.

Fig 18–17 A 48-year-old woman with end-stage renal disease presented with a swollen head and face and was found to have obstruction of the proximal superior vena cava (arrow).

Fig 18–18 Venogram demonstrates central outflow via dilated hemiazygous system.

Fig 18–19 After angioplasty and stent placement, there is restored inline flow into the right atrium with prompt symptom improvement (arrow).

Patients with SVC syndrome are initially studied using venography. A complete ultrasound examination including pulsed-wave Doppler and gray-scale imaging may be helpful to establish the number and degree of stenoses or occlusions prior to the venogram. If possible, a cross-sectional study such as a computed tomography angiogram or magnetic resonance angiogram with a venous phase may be obtained to confirm the level of stenosis and occlusion as well as highlight the amount of underlying mass effect or malignancy (Fig. 18-20).

Fig 18–20 A 76-year-old man presented with severe head pain and swelling. Computed tomography scan demonstrates known lung cancer, which is causing narrowing of both brachiocephalic veins. The right brachiocephalic vein appears occluded (arrows).

Although it may not be necessary to treat both innominate veins to achieve a clinical outcome, bilateral central venous stenting may be needed to reestablish optimal flow. The goal is to restore flow into the right atrium (Figs. 18-21 through 18-23). The patency of the jugular veins is important because its intact drainage centrally is usually sufficient to relieve face and head symptoms. At the time of venography, brachial vein access is complemented by femoral and possibly jugular access. In the setting of acute thrombosis, chemical or mechanical thrombolysis may be necessary to uncover the underlying stenosis and minimize embolization. At our institution, we usually attempt to place the central stents first and then extend additional stents peripherally to help anchor the stents in place as needed. Stent migration is a complication and may be limited by the use of proper oversizing of the stents. The SVC is a thin vessel, and aggressive dilatation of stents should be avoided because caval perforation may occur, especially in patients after radiation therapy (Figs. 18-24 through 18-26).