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a Department of Surgery, Divisions of Cardiothoracic Surgery and Interventional Radiology, University of Minnesota, Medical School, Minneapolis, Minnesota
b Department of Radiology, Divisions of Cardiothoracic Surgery and Interventional Radiology, University of Minnesota, Medical School, Minneapolis, Minnesota
Accepted for publication November 17, 2008.
* Address correspondence to Dr Molina, University of Minnesota, Department of Cardiothoracic Surgery, 420 Delaware St, SE, MMC 207, Minneapolis, MN 55455 (Email: molin001{at}umn.edu).
| Abstract |
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Methods: We treated 126 Paget-Schroetter syndrome patients (group I) by implementing an emergency protocol of thrombolysis by catheter-directed infusion, followed by immediate surgery through an anterior subclavian approach entailing (1) decompression of the thoracic inlet and (2) repairing the vein with a vein patch to reestablish its normal caliber. In addition, we treated another selective group of 81 patients (group II) for chronic fibrotic obstruction several months after their original event, but only when the inflow was adequate.
Results: Our acute emergency care resulted in a 100% long-term patency rate in group I, with no sequelae. The patency rate in group II was 100% as well, but in 74% a long vein patch, endovascular stents, or homograft implants were used.
Conclusions: Implementation of an emergency approach to treat Paget-Schroetter syndrome is highly recommended to prevent the delayed sequelae of permanent subclavian vein obliteration and disability. In chronic obstruction, when feasible, we recommend a long saphenous vein patch, followed by endovascular stent implant.
| Part I: The Acute Obstruction |
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Beyond that 2-week window, progressive deterioration of an untreated or improperly treated arm leads to fibrosis of the vein and to extension of the thrombosis into the arm, decreasing significantly the chances for cure and complete recovery as more days pass. By the end of 30 days, the vein usually has already fibrosed, and the edema of the arm becomes chronic. The chances of effective intervention diminish to the point that no surgical intervention can offer any hope of reestablishing the normal venous return flow of the affected arm [11, 12]. This invariably leads to some degree of permanent, irreversible disability of the arm.
The mechanism of thrombosis is now clearly understood [13, 14]. The vein is pinched at the inlet of the thoracic upper aperture between the first rib, the subclavius muscle tendon, and the anterior scalene muscle tendon, all of which form the tunnel through which the vein runs into the chest cavity. At that level, as the vein is pinched (as in a vise), the endothelium is disrupted and the thrombosis begins immediately. With total occlusion of the vein, the clot progresses distally into the axillary vein and the veins of the arm. The patient's preinjury coagulation variables are nearly always normal. Coagulation abnormalities are rare [14]. The syndrome is basically a mechanical trauma to the vein that injures the endothelium, and thrombosis is the normal response of the body to such an insult.
During the past 20-plus years, it has been very clearly established that the treatment of choice is catheter-directed thrombolytic therapy, using any of the currently available thrombolytic agents (Table 1). Nowadays, it is unacceptable to treat these patients only with anticoagulation agents (namely, heparin or warfarin) if no other intervention is planned. The current standard of care mandates thrombolytic therapy first, followed immediately by decompression of the thoracic inlet and reestablishment of the normal subclavian vein caliber.
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These 3 steps have been published by our institution [4, 10, 15] and have proven to be 100% effective.
| Patients and Methods |
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The thrombolytic therapy was implemented with tenecteplase (TNK; Genentech, South San Francisco, CA) at an hourly infusion of 0.05 mg/kg (mean, 0.25 mg/h). Successful lysis of the clot occurred within 24 hours; for some, within 2 hours. During thrombolytic therapy, the patient returned to the interventional radiology suite for repeated venograms to verify total lysis of the clot and demonstrate the exact extent of the obstruction. The details of this technique can be seen in our previous reports [10, 15].
Operative Procedure
The operation has been previously described [4, 10, 15, 16]. It consists of two components: decompression of the thoracic inlet and reestablishment of the vein patency and caliber. The technique involves an anterior subclavicular approach to the first rib, removal of its anterior portion, and removal of the subclavius tendon and costoclavicular ligament, with transection of the anterior scalene muscle. This is all done from an extrapleural approach by detaching the inferior periosteum of the first rib. Then the subclavian vein is dissected and detached from the posterior aspect of the sternum and the stump of the first rib until the entire vein can be mobilized and brought into the field.
It is of utmost importance to dissect the vein more medially in order to reach normal vein in its innominate portion so that a patch of saphenous vein can be laid across the stenotic portion. Doing so reestablishes the normal diameter of the vein, from the normal axillary vein to the normal innominate vein [4]. This is the crucial part of the operation. If it is not done properly, that is, if the patch is not extended to the normal innominate vein, residual obstruction or occlusion may occur in the immediate postoperative period.
Anticoagulation is begun the evening of the procedure (Table 2) and continued with warfarin and clopidogrel for 8 weeks to keep an international normalized ratio between 2 and 3.
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We leave an extrapleural drain, usually a size 19 Jackson-Pratt connected to bulb suction. After the second or third day, when the amount of drainage for 24 hours does not exceed 30 mL total, we remove the drain and discharge the patient.
| Results (Part I) |
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During the follow-up period, clopidogrel was discontinued in 3 patients because of intolerance, and they remained only on warfarin.
| Part II: The Chronic Obstruction |
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Physicians, therefore, should be fully aware of three situations in particular, that without timely and proper treatment, can result in chronic obstruction of the subclavian as well as the axillary or even the innominate vein [1]. Most patients with chronic obstruction of the subclavian vein initially had Paget-Schroetter syndrome. Either they were never properly diagnosed, or if they were, timely and proper care was not provided [2]. Some patients were treated with thrombolytic therapy, but were then underwent faulty or ineffective surgical decompression of the thoracic inlet, without reestablishment of the proper caliber of or flow through the subclavian vein. A common culprit is transaxillary resection of the first rib, an approach that usually fails [3].
Some patients treated with thrombolytic therapy undergo implantation of endovascular stents, without surgical decompression of the thoracic outlet. The vein invariably occludes again, creating a worse situation than the original one because the stent cannot be removed once implanted [17–20]. Also, if a stent is placed after a transaxillary resection of the first rib, the stent often kinks over the residual rib stump, it fails, and the vein occludes again.
Once fibrosis sets in, the walls of the vein become very thick, until the entire vein, with or without a previously implanted stent, becomes a fibrous cord, totally obliterated, with no lumen. The only hope for such patients is surgical intervention, but that may only be feasible under very specific circumstances.
Of our 81 patients with chronic obstruction (Group II) who were selected for an operation, 49 had a short segment (<2 cm) of obstruction; 32, a long segment (3 to >20 cm). The few patients with a long segment (20 cm) had fibrosis that extended from the subclavian vein to the axillary and brachial vein as well. Of those, 4 had already undergone operations elsewhere. Even though they had initially undergone the correct thrombolytic treatment, their procedure was not performed immediately and did not use the subclavicular approach. Instead, the transaxillary route was used, which failed to reestablish the proper caliber of the vein and required implantation of endovascular stents. This second maneuver did not solve the problem either, because the stent invariably kinked over the residual first rib stump that was left anteriorly. In 2 of those 4 patients, the stent broke at that level. In all 4, the vein occluded again. Months after their operation, they were seen at our institution. Three other patients had additional stents placed before being referred to our institution.
The 49 patients with a short segment of chronic obstruction underwent our standard operation. However, 5 (10%) showed residual stenosis on venograms the day after the procedure and an endovascular stent was implanted.
The 32 patients with a long segment of obstruction all required a transsternal extension [10, 21] of the subclavicular incision to expose the proximal innominate vein. Then, we either replaced the venous segment using a pediatric-sized thoracic aortic homograft or, in recent years, used a very long venous patch, followed by implantation of a long endovascular stent. We obtained a venogram the day after the operation in all 32 patients. The interventional radiologist, early postoperatively can pass a guidewire through the operated segment, even if it has reclotted, and can declot and stent the segment to reestablish normal flow. For our patients who underwent thoracic aortic homograft implantation, we obtained a venogram at periodic intervals. If the homograft showed signs of stenosis (Fig 1), which sometimes was very severe, it was treated with dilation and implantation of an endovascular stent.
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2 cm; Fig 3). If the obstruction shown by venogram is short, then the standard operation as in Group I for the acute event should be 100% effective, reestablishing the normal caliber and flow of the subclavian vein. If the operation is done correctly, stents are usually not needed.
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Vein Substitution
Currently, no adequate prosthetic tubular grafts will stay open to replace a vein in the subclavian position. We reported in 1999 [23] the use of small-caliber aortic homografts, obtained from the descending thoracic aorta of deceased pediatric donors. Such grafts are usually 10 to 12 mm in diameter, closely matching the normal caliber of an adult subclavian vein. The disadvantage is that the grafts eventually become calcified and need to be dilated (Fig 1). Stent implantation is commonly needed to keep the grafts open because they also tend to narrow with time, usually during the first or second year after implantation.
The second option is to use a long, upper thigh saphenous vein patch to connect the axillary vein to the innominate vein. Doing so reestablishes the continuity of the channel, through which, on the next day, the radiologist can pass a guidewire and implant a stent of the proper caliber (Fig 4). The flow and the caliber of the vein are thus reestablished. We now prefer this approach, given the homograft problems of calcification, reobstruction, and high cost.
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| Results (Part II) |
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Of the 32 patients with a long segment of chronic obstruction, 14 (subgroup A) were initially treated with implantation of thoracic aortic homografts. Of those 14 grafts, 10 (71%) remain patent beyond 5 years. In the other 4 patients, reobstruction occurred and required balloon dilation and implantation of a stent 7 to 18 months postoperatively (Fig 1). Only 2 patients are beyond 5 years with a patent vein.
The other 18 patients (subgroup B) were treated with our current long vein patch protocol. Reclotting of the vein occurred the day after the procedure in 11 patients, and they underwent implantation of a long endovascular stent. Included were 3 patients who had previously undergone transaxillary resection of the first rib, followed by stent implantation. They all have remained patent from 6 months to 4 years.
The patients who had previously undergone transaxillary resection of the first rib elsewhere underwent our standard substernal, anterior operation. We found all of them had residual anterior first rib stumps (1.5 to 2 inches long). They all also had intact subclavius tendons and intact costoclavicular ligaments that had not been resected during the transaxillary operation. Our anterior approach allowed us to remove the remaining rib stumps, ligaments, and subclavius tendons and to leave the vein completely free and without any kinks. The patency rate for these 18 patients was 95%. Our 1 failure was in a patient with multiple endovascular stents that had been previously implanted elsewhere. The other 7 are patent from 8 months to 3 years and have not required stent placement (Fig 5).
| Comment |
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If the patient is not correctly and promptly treated, the normal use of that arm is permanently lost. Very few patients with chronic obstruction of the subclavian vein after an injury several months previously are candidates for operation (81 in this series). Most cannot be helped surgically and will have from some degree of edema and pain for life.
Many vascular surgeons are applying an operation that is not optimal for this condition. The transaxillary resection of the first rib, introduced by Roos in 1966 [25], was meant to solve compression of the brachial plexus and of the subclavian artery, not of the subclavian vein. The vein occupies the most anterior portion of the upper aperture of the chest. As we have frequently seen, the transaxillary approach is not adequate for relieving compression of the subclavian vein [26, 27]. With the transaxillary approach, the surgeon invariably leaves the costoclavicular ligament and the subclavius tendon intact, as well as a significant rib stump anteriorly where the vein or the stent becomes kinked. The proper steps can only be accomplished with the anterior approach [28]. The transaxillary route should be completely abandoned for treating effort thrombosis patients.
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