Superior Vena Cava Syndrome

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• Facial swelling, headache, and arm edema

• Prominent venous pattern may be seen over the anterior chest wall

• Associated conditions may aid in the diagnosis, such as lung cancer, lymphoma, and indwelling catheter or pacemaker

• Venography is diagnostic

  • – Magnetic resonance imaging, ultrasound, and CT are alternates

GENERAL CONSIDERATIONS

• A relatively rare condition in which the thin-walled superior vena cava is compressed extrinsically by neoplasms, fibrosis due to pulmonary infections or drug toxicity, or aneurysm of the aortic arches; or it may be thrombosed due to extension from tributary vein thrombosis or indwelling catheters

• Causative factors are changing (from lung cancer) with increased use of indwelling catheters

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• Acute or subacute symptoms

• Headache, dizziness, visual disturbances, stupor

• Symptoms accentuated by bending over or lying down

PHYSICAL EXAM FINDINGS

• Swelling and flushing of the neck and face progressing to cyanosis

• Dilated cutaneous veins of the upper chest and neck

• Eventually, brawny edema of the face, neck, and arms develops

• Laryngeal edema leads to respiratory insufficiency

DIFFERENTIAL DIAGNOSIS

• Differential diagnosis of causes of superior vena cava syndrome includes:

  • – Lung cancer

  • – Lymphoma

  • – Indwelling catheters and pacemaker leads

  • – Fibrosing mediastinitis

  • – Thoracic outlet syndrome

  • – Retrosternal goiter

DIAGNOSTIC EVALUATION

IMAGING STUDIES

• Chest x-ray: localizes the site of obstruction

• CT scan or MRI: delineates the anatomy

• Phlebography: outlines the venous pattern and collaterals

DIAGNOSTIC PROCEDURES

• Venous pressure measurements in the neck or arm (greater than in the leg)

TREATMENT

CARDIOLOGY REFERRAL

• If syndrome is believed to be due to pacemaker catheters

HOSPITALIZATION CRITERIA

• Marked symptoms

• Need for corrective procedure such as pacemaker lead change

MEDICATIONS

• Cautious use of diuretics

• Heparin or thrombolysis with thrombosis

THERAPEUTIC PROCEDURES

• Mediastinal irradiation for neoplasm

• Removal of venous catheters or pacemaker leads

• Balloon venoplasty and stent placement

SURGERY

• If mediastinal fibrosis is present, surgical excision may be necessary

• Some catheters need to be removed surgically

MONITORING

• Chest x-ray to assess response to therapy

DIET AND ACTIVITY

• Low-sodium diet until obstruction is resolved

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

• Resolution of the obstruction

• Transfer to comfort care

FOLLOW-UP