Blood Clots, Embolisms, and Death?
I’ll treat it with painkillers, and if it gets bad I’ll go to the doctor then.
—TOS patient
Don’t do this!
As you recall, Paget-Schroetter syndrome or effort thrombosis, is a complication of venous thoracic outlet syndrome that got out of control!
This is a condition where the compression of the vein in your shoulder gets so bad that a blood clot forms that blocks the blood flow out of your arm. This can get really scary, really fast.
If the clot breaks away from the vein, it can travel through the circulation all the way to your lung (pulmonary embolism) and cause what is called a lung infarction. This can lead to temporary or permanent difficulty breathing, or in some cases death.
Most doctors tell the nurse to get you to the operating room right away.
Not so fast... Read on...
Paget-Schroetter Syndrome or Effort Thrombosis
Effort thrombosis, or Paget-Schroetter syndrome, is an outcome of venous thoracic outlet syndrome that got much worse! It is when the compressed vein in your chest under your collarbone (axillary- subclavian vein) has a blood clot (thrombosis). This is usually associated with strenuous and repetitive activity of the upper extremities.
This is not thoracic outlet syndrome or a type of it. It is a complication of venous thoracic outlet syndrome. Venous thoracic outlet syndrome is caused by vein compression with or without a blood clot. Paget-Schroetter syndrome or effort thrombosis is caused by subclavian vein compression with a blood clot.
So if you have effort thrombosis, aka Paget-Schroetter syndrome, you have effort thrombosis (Paget- Schroetter syndrome) caused by thoracic outlet syndrome (1) (2).
If the blood clot (thrombus) releases to fl ow through the vein it is called an embolism. This embolism travels through the veins, to the heart. Then it is pumped out of the heart into the lung where it lodges in the more narrow branch(s) of the artery. This causes a blockage of blood fl ow causing the lung and the lung tissue in this area to die.
Incidence
Paget-Schroetter syndrome is often regarded as the most common vascular problem in athletes (4). Although more prevalent in male athletes, it is now increasingly affecting young women as they become seriously involved in athletics. Not surprisingly, effort thrombosis is more common in young and otherwise healthy men between the ages of 15–30. It preferentially involves the dominant arm (5).
Signs and Symptoms
The primary symptom is arm swelling, frequently accompanied by cyanosis, pain, and occasionally paresthesia (6). Cyanosis is a blue discoloration of the skin and mucous membranes resulting from inadequate oxygenation of the blood.
Symptoms can be excruciating deep pain the chest, shoulder, and entire upper extremity, accompanied by a feeling of heaviness that occurs especially after activity. The patient will present with cyanotic discoloration and distended collateral veins, potentially accompanied by edematous increases in the volume of the extremity (the arm is swollen) (7).
Collateral veins form when the subclavian vein is narrowed or blocked. They form and widen to allow the blood to flow around the blocked subclavian vein. It would be similar to what happens when the main highway is blocked, so you get off the highway and take side streets. The side streets are the collateral veins.
Swelling and arm discomfort are the most frequent presenting problems. Other symptoms include heaviness, redness of arm, cyanosis, and dilated, visible veins across the shoulder and upper arm (Urschel’s sign) (1). Subclavian vein effort thrombosis can present itself as a rapid swelling of the entire arm, often with a blue discoloration, heaviness, and pain (8).
The symptoms of subclavian vein effort thrombosis are as follows.
- A heaviness after activity
- A deep pain in the chest
- A cyanotic (blue) discoloration in the arms
- A redness discoloration in the arms
- Some distended and dilated collateral veins—Urschel’s sign
- Some visible edema and swelling
- Discomfort
Signs You Might Have Paget-Schroetter Syndrome
- Arm swelling is usually substantial
- Fatigue
- Tightness
- Heaviness
- Pain in the arm, especially with use or overhead positioning Many who get this have enlarged veins in the upper arm, around the shoulders, or in the upper front of the chest wall.
Some people feel something simple, like they strained a muscle (9).
Cause
Effort thrombosis usually follows sporting activities, such as wrestling, playing ball, gymnastics, and swimming, which involve vigorous and sustained shoulder and arm movements (10). A majority of patients report a discrete precipitating event, usually sports-related arm exertion (10). Approximately 60–80 percent of patients diagnosed with Paget-Schroetter syndrome report a history of repetitive or vigorous overhead activity (20). Occasionally, minor, relatively innocuous day-to-day, activities can precipitate effort thrombosis (10) (1).
Anatomical abnormalities at the thoracic outlet and repetitive trauma to the outer covering of the vein of the subclavian vein are key factors in its starting and getting progressively worse (5). It is believed that the full backward rotation of the arm in a pitching motion imposes undue strain on the subclavian vein, leading to microtrauma of the covering of the vein and activation of the clotting of the blood (5). When the shoulder complex is pulled or dragged down into the thoracic outlet, the costoclavicular space between the ribs and the collarbone become narrower. This can cause a compression of the vein and slow the flow of the blood through the vein. Also, when the space is so narrowed, the blood vessels and nerves are more at risk to be damaged when you move your shoulder and arms.
This can lead to a more constant irritation and trauma to the cell wall of the vein causing inflammation and scar tissue formation. This can cause even more narrowing of the costoclavicular space causing even more compression of the vein (5). It can arise as a consequence of compression and repetitive injury of the subclavian vein between the first rib and the collarbone, in addition to the anterior scalene muscle, subclavius muscle, and costoclavicular ligament (8). Subclavian vein (SCV) effort thrombosis is considered primarily a “mechanical” condition caused by compression and irritation of the vein, and, unlike other forms of clotting or deep vein thrombosis (DVT) in the legs, it is not associated with inactivity, obesity, advanced age, underlying coagulation disorders, surgery, or trauma (8). The blood flow can also be reduced, causing a clot to form in the costoclavicular space or subpectoral space. With repetition during a long period of time, this type of focal venous injury leads to progressive scarring and narrowing of the subclavian vein at the level of the first rib, along with scar tissue formation and contraction around the outside of the vein, in addition to scar tissue and narrowing that happens within the inside wall of the vein (8).
You can get a blood clot that results from stagnant and turbulent blood flow in the narrowed segment of the subclavian vein (8). The movement of this clot upstream in the subclavian vein into the axillary vein can then result in further obstruction of critical collateral veins, resulting in the acute clinical presentation of the (blood clot) effort thrombosis syndrome (8).
What activities or conditions put you at greater risk for Paget-Schroetter syndrome?
Thrombophilia is a condition where your blood has an increased risk to form blood clots (11). It’s not certain if oral contraceptives can put you at risk for blood clots in the subclavian vein (12) (13)
(14) (15) (16) (17), but their role in lower leg blood clots (18) and blood clots in the brain (N ) is well established. Pregnant women have a higher than average risk for blood clots.
Might Be Missed or Misdiagnosed
According to Perlowski and Jaff, vascular issues are commonly overlooked in athletes for three main reasons (19).
- The first reason is that most athletes are often young and healthy.
- The second reason is that their symptoms might be similar to, and therefore mistaken for, a musculoskeletal injury.
- The third reason is that many healthcare practitioners might not adequately perform a vascular examination and are unaware of Paget-Schroetter syndrome as a differential diagnosis.
If you get a clot in your vein and you wait too long, the results might not be so good. It’s better to get treated immediately (20) (21) (22).
A blood clot can release and flow through the circulatory system into the heart, then into the lungs and damage them permanently. This happens in approximately 12 percent of those with Paget-Schroetter syndrome. Overall mortality (death) rate for patients with pulmonary embolism is approximately 15– 50 percent (2) (1) (8).
What part of Paget-Schroetter syndrome scares you into getting your thoracic outlet syndrome treated immediately before it gets worse? If you get Paget-Schroetter syndrome, can I still treat you? Yes, however you MUST first get the clot dissolved.
After the clot is dissolved, your doctor will usually want to surgically remove the scalene muscles and first rib within 24 hours of the clot being dissolved. However, in some cases, the doctor might not opt for the full decompression surgery.
If you decide not to have the surgery and treat the thoracic outlet syndrome with the recommendations in this book, I recommend you read this book through and make sure your doctor is following the recommendations exactly. You can call me if you wish to talk this through.
It is important to understand that if you don’t get treated for thoracic outlet syndrome that the risk of pulmonary embolism with effort thrombosis is real and significant (5).
The possibility that you could get Paget-Schroetter syndrome is fairly rare. So while you should not leave thoracic outlet syndrome go you also should not run to the local vascular surgeon and ask for surgery.
Management
The Former Way
The short-term and long-term goals of treatment for SCV effort thrombosis are fourfold (4).
- To provide prompt relief of acute upper extremity symptoms and prevent pulmonary embolism (obstruction of an artery).
- To reduce the likelihood of recurrent venous thrombosis (blood clotting in the veins) following initial management.
- To diminish or prevent the potential development of upper extremity post-thrombotic
- To return to normal unrestricted use of the upper extremity without the need for chronic anticoagulation and other medications. For many years, patients with effort thrombosis were managed conservatively with limb elevation and anticoagulation (23).
Systemic fibrinolysis is superior to anticoagulation in achieving vein patency but is associated with higher rates of complications such as intracranial hemorrhage (23). Fibrinolysis is a process that prevents blood clots from getting larger and causing further problems.
Catheter Directed Thrombolysis
Treatment
- Limb elevation
- Local catheter-directed thrombolysis (drugs are used to dissolve blood clots)
- Fibrinolytic agent (clot-dissolving drug)
- Remove the extrinsic compression (thoracic outlet surgery)
- Remove the intrinsic stenosis (cut out part of the vein that is narrowed)
- Vein patch angioplasty (patch that part of the vein)
- Percutaneous angioplasty (mechanically widen narrowed or obstructed arteries)
- Subclavian vein narrowing, requiring balloon angioplasty
The Current Way
Treatment with an antiplatelet (anticlotting) agents, such as aspirin or clopidogrel, is often initiated.
The goal of thrombolysis (clot dissolving) is to clear any fresh or recent clot from the axillary and subclavian veins, along with any blocked smaller veins in the area passing through the thoracic outlet space. This usually results in a marked improvement in the appearance of the subclavian vein and a prompt reduction in symptoms of venous obstruction (24). In most cases, thrombolytic therapy is able to effectively dissolve the clot (25).
It is most effective when given within one week of the onset of symptoms, but might be effective up to one month after symptoms develop (26). Long-term anticoagulation might therefore be needed to reduce the potential for recurrent venous thrombosis (27). Most patients require prolonged infusion of the fibrinolytic agent for catheter-directed thrombolysis; average durations vary 24–48 hours (28).
It is notable that there is still a significant risk of recurrent thrombosis following thrombolysis and anticoagulation, with estimates ranging from 50–70 percent (8).
Sometimes nonsurgical management with anticoagulation alone is inadequate and leaves the person unable to function. In these cases, doctors must remove the clot with a local catheter-directed thrombolysis (clot-buster). Surgical treatment, therefore, should be considered in almost all patients with venous TOS and SCV effort thrombosis as the most definitive management approach (8).
Some professionals think that local, catheter-directed thrombolysis has the therapeutic value of systemic thrombolysis without significant systemic side effects. This treatment is recommended in all patients presenting early (28). Doctors refer to this as injecting clot-busters. Others think that a catheter-directed thrombolysis is associated with several disadvantages, including major systemic hemorrhage (25) (29).
Some professionals recommend a conservative approach to remove the extrinsic compression. Soft tissue therapy of the musculature surrounding the costoclavicular space and the shoulder girdle might help to decrease the compression of the subclavian vein. Postural education and correction of abnormal shoulder biomechanics through scapular stabilization, manipulation, or mobilization of the thoracic spine, costovertebral, sternoclavicular, or acromioclavicular joints might also help to prevent the recurrence or persistent symptoms of (24).
Damaged veins are repaired by either endovascular or open techniques (30). After surgery, vein patch angioplasty or venous bypass might be required to restore normal circulation (24). Current estimates indicate that 40–50 percent of patients will demonstrate a residual subclavian vein stenosis, narrowing of the vein. You might be required to get a balloon angioplasty, even several weeks after the first rib resection. Because these narrowed areas are typically composed of dense scar tissue within and around the wall of the vein, balloon angioplasty might be relatively ineffective in this setting. Although placement of subclavian vein stents might be considered, the long-term effectiveness of stents in this position is questionable. However, balloon angioplasty is often unsuccessful in this setting, because the vein is obstructed by scar tissue and external compression between the clavicle and first rib. However, even when improvement is obtained, it is usually short lived (8).
Surgical thrombectomy, balloon venoplasty, and stenting have practically been abandoned due to the limited success, high procedural morbidity (death), and high rates of stent fracture (25).
Thoracic Outlet Surgery
The paraclavicular approach combines the advantages of the supraclavicular exposure used for neurogenic and arterial forms of TOS with an infraclavicular incision that permits complete resection of the medial first rib and wide exposure of the SCV to permit vascular reconstruction (26).
Residual subclavian vein stenosis after operative thoracic outlet decompression is common in patients with venous thoracic outlet syndrome (31). Once the extrinsic compression has been relieved, damaged veins might be repaired surgically (32). In some cases, the veins can be patched, or a vein bypass to go around the damaged area of the vein might be required to restore normal circulation (32).
However, the Society for Vascular Medicine (SVM) asks surgeons to refrain from percutaneous or surgical revascularization of peripheral artery stenosis in patients without claudication, which is pain, discomfort, or tiredness that happens when they move or have critical limb ischemia (a sudden lack of blood flow to a limb). SVM says that no evidence exists to support improving circulation to prevent progression of disease. There is no proven preventive benefit, only symptomatic benefit. With proper surgical care, acute limb ischemia is a treatable condition. If it is not caught in time, it can result in disease, amputation, and/or death. One might require an amputation from toxins that buildup from the cells dying, because they could not get oxygen due to the blockage of blood flow to them.
Surgery
A lot of doctors are reactive when they have a patient with Paget-Schroetter syndrome. This is why they cut out the scalene muscles, pectoralis minor muscle, and first rib. Then they follow that with a nonsurgical procedure, percutaneous angioplasty, used to treat the narrowed arteries.
Serious problems can result from untreated Paget-Schroetter syndrome, including blood clots that form and release into the lungs.
After Surgery
Postoperative care for patients undergoing surgical interventions includes ample use of pain medications, muscle relaxants, and anti-inflammatory agents. Therapeutic anticoagulation (heparin/ warfarin), with or without adjunctive antiplatelet therapy (aspirin or clopidogrel), is initiated several days after the operation and then discontinued at 12 weeks (8).
The expected postoperative hospital stay is five to six days, with a drain removed six to seven days after the operation (8).
Inpatient physical therapy is started the day after the operation to maintain range of motion, with postoperative rehabilitation then overseen by a physical therapist with expertise in the management of TOS. No restrictions are placed on upper extremity activity 12 weeks after surgery (8). Full recovery is typically complete within three months of the operation, and a return to previous levels of functional activity can usually be expected (8).
Sometimes a clot releases into the lung after surgery, and this could be a big problem. Doctors call it a pulmonary embolism. The key signs and symptoms of a pulmonary embolism include dyspnea, chest pain, syncope, low-grade fever, and racing heart rate (33–34). It only happens with 5.6 percent of patients, which is rare for Paget-Schroetter syndrome. However, a pulmonary embolism is a serious complication that might result in a fatal outcome, reinforcing the importance of a proper diagnosis (33–34).
Is it possible to just dissolve the clot and prevent another clot from forming without thoracic outlet surgery?
The role of thoracic outlet decompression in the treatment of primary axillary-subclavian vein thrombosis remains controversial. (35)
There was a group of doctors from Stanford University School of Medicine, led by Dr. Lee, who did not elect to do immediate surgery after they dissolved the blood clot in the subclavian artery. They put the patients on anticoagulants for three months and waited to see if the clot returned. (35)
After a month, they determined if the patient needed surgery. The indications for surgery were simple:
- Persistence or recurrence of symptoms of increased blood pressure in the veins due to obstruction or narrowing.
- Positional obstruction or blocking of the collateral veins with blood clots in the axillary- subclavian vein noted in the venography.
- Evidence of a blood clot or a pulmonary embolus.
They had 22 patients in the study.
Thirteen had surgery after the blood clot was dissolved. Of the 13 who had surgery, 11 of these patients returned to their same physical activity as before the surgery (35).
Nine patients did not have surgery after the blood clot was dissolved. Of these nine who did not have surgery, eight of these patients returned to their same physical activity as before the surgery.
What is important to note is that not all patients who have a blood clot in the subclavian vein will require surgery. However, these doctors selected the ones most likely to recover without surgery.
The other important fact is that these doctors only treated the patients with anticoagulants. They did not get any physical therapy. I’m sure the results would have been even better if the nine patients treated without surgery got treated with my human spring, nonsurgical approach to thoracic outlet decompression (35).
What part of Paget-Schroetter syndrome scares you into getting your thoracic outlet syndrome treated immediately before it gets worse? If you get Paget-Schroetter syndrome, can I still treat you?
Yes, however you MUST first get the clot dissolved. After the clot is dissolved, your doctor will want to surgically decompress the thoracic outlet. However, the thoracic outlet can be decompressed with treatment outlined in this book. In some cases, it might be a tough case where you need some extra help.
Feel free to contact me. It would be an honor to help you regain your health!
Summary
If you would like a second opinion on your case with the focus on the results of your diagnostic tests, go to www.thoracicoutletsyndrome.org, or www.drstoxen.com to register for an online consultation, then submit a scan of your MRI report and other medical data. We will give you a second opinion.
You do not want to get surgery for thoracic outlet syndrome unless you contact me fi rst. Call me at 773 735-5200 and lets talk this over.