About superior vena cava syndrome

What is superior vena cava syndrome?

Introduction

The superior vena cava is a large vein located in the upper chest, which collects blood from the head and arms and delivers it back to the right atrium of the heart. If this vein is compressed by outside structures, or if a thrombus or clot develops within it, return blood flow to the heart is impeded. When blood flow to the heart is restricted, the increased pressure in the veins of the face and arms causes edema (fluid buildup) in these areas. This condition is referred to as superior vena cava syndrome.

Because the superior vena cava, like all veins, has a thin wall (there are no muscles in the walls of a vein as compared to the walls of an artery), and because there is little pressure inside the vein, it can be easily compressed by outside structures. The superior vena cava lies next to the upper lobe of the right lung and within the mediastinum [the space that contains the central structures of the chest: the heart, the trachea, the esophagus and the great vessels (aorta, vena cava)]. Abnormalities within any of these structures can cause the compression.

What are the symptoms for superior vena cava syndrome?

One sign of superior vena cava syndrome is Swelling in your face, arms, neck, head, or upper body. It may get worse when you bend forward or lie down. You might start to cough or have trouble breathing.

Less common symptoms include:

  • Trouble swallowing
  • Coughing up blood
  • Fluid buildup in arms
  • Chest pain
  • Bluish skin (from lack of oxygen)
  • Scratchy voice and other vocal changes
  • Horner's syndrome: A smaller pupil, sagging eyelid, and little or no sweat on one side of your face

What are the causes for superior vena cava syndrome?

Cancer is the primary cause of superior vena cava syndrome, most typically:

  • Lung cancer
  • Non-Hodgkin's lymphoma
  • Cancers that spread to your chest

Less common cancers that could cause superior vena cava syndrome include:

  • Hodgkin's lymphoma
  • Primary leiomyosarcomas
  • Plasmacytomas

There are a number of common ways that cancer causes superior vena cava syndrome:

  • A tumor in the chest could push on the superior vena cava vein.
  • A tumor might grow into the vein and block it.
  • Lymph nodes near the vein may start to swell or enlarge because of cancer, and so pinch or push on it.
  • Cancer can cause a blood clot in the superior vena cava.

Noncancerous conditions that can cause superior vena cava syndrome include:

  • Blood clots resulting from problems with a pacemaker or a catheter (a flexible tube that goes into a vein)
  • Mediastinal fibrosis: Scarring in the area between your lungs
  • Blood vessel diseases like aneurysms, vasculitis, or arteriovenous fistulas
  • Infections such as tuberculosis, syphilis, or histoplasmosis
  • Noncancerous tumors including dermoid cysts, teratomas, and thymomas
  • Pericarditis: Inflammation of the sac around your heart

What are the treatments for superior vena cava syndrome?

How is superior vena cava syndrome treated?

Rarely does superior vena cava syndrome constitute a medical emergency. The ABCs of care are always a priority, insuring that the Airway is well maintained, Breathing is adequate and the Circulation (blood pressure and heart rate) is stable. The patient may feel more comfortable in an upright position. Furosemide (Lasix) is a diuretic that will decrease the volume of fluid within the bloodstream. With less pressure pushing against the obstructed superior vena cava, symptoms may be temporarily relieved.

Treatment needs to be directed to the underlying cause.

If the cause is a tumor or cancer, the treatment plan will need to be customized to the patient's condition and desires. Options include radiation with or without chemotherapy. Even with aggressive treatment with radiation, only 10 % of lung cancer patients with this syndrome survived to 30 months. Without any treatment, survival may be less than a month.

Steroids [for example, methylprednisolone (Medrol, Depo-Medrol) or prednisone (Deltasone, Liquid Pred)], may be considered to decrease the swelling of a tumor pressing on the superior vena cava and help relieve symptoms.

If the cause of superior vena cava syndrome is a blood clot, anticoagulation with (warfarin) Coumadin may be indicated. The use of thrombolytic drugs [for example, alteplase (Activase, TPA) or TNK] to break up the clot, or placement of a stent to keep the vein open may also be considered.

Special consideration

In children, superior vena cava syndrome is most often caused bynon-Hodgkin's lymphoma. The compression of the superior vena cava may be associated with compression of the trachea as well. The trachea (windpipe) in children is relatively narrow, flexible, and soft as compared with an adult. Airway obstruction may occur and manifest as:

  • difficulty breathing,
  • stridor (high-pitched, abnormal breathing sounds heard when breathing in), and
  • wheezing.

What are the risk factors for superior vena cava syndrome?

Superior vena cava syndrome (SVC) is a condition that occurs when the vein that carries blood from the head, neck, and upper chest back up to the heart is blocked.

The symptoms of SVC depend on which veins are affected, but they can include chest pain, headaches, swelling around the eyes and face, and difficulty swallowing or speaking.

The risk factors for SVC include:

  • Being over 40 years old
  • Having a history of lung disease or cancer
  • Taking birth control pills
  • Having a history of blood clots in your legs
  • Having a history of heart problems like angina or heart attack
  • Age: The condition is most common in people over 65 years old, but it can also occur in younger adults.
  • Ethnicity: African-Americans have a higher risk of developing this condition than Caucasians.
  • Pregnancy: Superior vena cava syndrome can be caused by pregnancy or delivery, and it may be more severe if you have other medical conditions or take certain medications.
Symptoms
Blurred vision,Headache,Nausea and vomiting,Breathlessness
Conditions
Aortic aneurysms (a bulging in the wall of your aorta),Carotid artery disease (narrowing or blockage of your carotid artery),Kidney disease (nephritis),Lung disease (pulmonary edema),Peripheral arterial disease (hardening of arteries outside of your heart)
Drugs
Therapy

Is there a cure/medications for superior vena cava syndrome?

Superior vena cava syndrome is a condition that results when blood flow to the heart is restricted by the way blood flows through your veins. Usually, this is caused by an obstruction or narrowing in one of the veins that carry blood from your upper body back to your heart, but it can also be caused by an injury or other condition that damages the vein wall.

  • The most common treatment is a surgery called a venoplasty. This procedure involves inserting a catheter into the patient's vein and inflating it to widen the vein. This helps reduce compression of the right side of the heart by the enlarged vein.
  • In addition to this procedure, there are medications that can help reduce symptoms such as high blood pressure, chest pain, shortness of breath and fatigue. These include calcium channel blockers and beta-blockers
  • Though there is no cure for superior vena cava syndrome, there are medications that can help manage your symptoms. These include anticoagulants (blood thinners) to prevent clots from forming, nitrates to open up narrowed blood vessels and improve blood flow, a low-salt diet, avoiding alcohol, tobacco and other products that may raise blood pressure, medicines to lower blood pressure, such as ACE inhibitors and beta-blockers.
Symptoms
Blurred vision,Headache,Nausea and vomiting,Breathlessness
Conditions
Aortic aneurysms (a bulging in the wall of your aorta),Carotid artery disease (narrowing or blockage of your carotid artery),Kidney disease (nephritis),Lung disease (pulmonary edema),Peripheral arterial disease (hardening of arteries outside of your heart)
Drugs
Therapy

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