Elevated Jugular Venous Pressure
By C. Andrew Kistler, MD, PharmD, RPh
54 year old man presents to the emergency department with progressive right-sided facial swelling.
He first noticed it approximately two months ago however more people in his family were commenting on it recently, therefore he decided to go to the emergency department. He denies dysphagia, new or unusual foods, confusion, photophobia, pleuritic chest pain, dysarthria, drooling, cough, head trauma, hemoptysis, fevers, chills, or rash.
Relevant Medical History
The patient has a 40 pack-year smoking history and has been diagnosed with lung cancer. He drinks 1 to 2 beers per weekend, and denies IVDA. He denies sick contacts, recent travel, or new medications.
His family history is significant only for diabetes in his grandparents. He is up-to-date with all of his vaccines. He denies any unusual environmental exposure and works as an accountant. He has no known food or drug allergies.
Assessment and Relevant Test Results
On exam, his vital signs are within normal limits. He does not appear toxic and is AAOx3. Facial swelling is prominent on the right side of his face and without facial droop or ptosis. He does not have any lymphadenopathy or stridor. His cranial nerve exam is intact bilaterally.
Jugular venous pressure (JVP) is approximately 9 cm at 45 degrees (Slide 2).
His heart exam is within normal limits and no murmurs are auscultated in the chest or abdomen. His lungs are clear to auscultation. His right upper extremity has minimal non-pitting edema when compared to his left upper extremity however temperature and pulses are symmetrical.
He has prominent veins that are visible on his bilateral anterior chest wall. He has no lower extremity edema. On labs, his kidney function is within normal limits. The rest of his labs are pending.
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This patient has a significant smoking history and presents with evidence of venous obstruction causing elevated JVP, dilated veins on his chest wall along with right upper extremity and facial swelling. His presentation is concerning for superior vena cava (SVC) syndrome.
The SVC is a relatively large vein that carries up to 1/3 of the body’s blood supply to the heart (from the head, upper extremities and thorax). If this blood vessel becomes obstructed, it causes backup of venous return to the heart as evidenced by facial edema (can be right-sided or generalized), dilated collateral veins in the neck (as evidenced by elevated jugular venous pressure) extremities, and chest wall. Therefore patients with SVC syndrome typically present with these findings along with dyspnea and cough.
SVC syndrome has both benign (~35%) and malignant (~65%) etiologies.1 Benign etiologies include DVT (e.g., from a central catheter), infections, and aortic aneurysms. The most common malignancies causing SVC syndrome include non-small cell lung cancer (~50%), small-cell lung cancer (~22%), and lymphoma (12%).1
The imaging study of choice is CT of the chest with IV contrast.1,2 The patient’s kidney function is within normal limits and has no known drug allergies. IV contrast is needed in order to better visualize the SVC, therefore a non-contrast CT of the chest would not be as helpful. In addition to confirming the SVC lesion, this CT of the chest with IV contrast may also further elucidate any lung masses seen on a chest X-ray or suspected based on exam.
TTE may provide information about the patient’s ejection fraction and overall cardiac function, however it will not help make the formal diagnosis of SVC syndrome. The patient’s elevated JVP is from his SVC syndrome; his lungs are clear and there is no lower extremity edema to suggest heart failure. In addition, his presentation and exam are not consistent with an aortic aneurysm (in which case a TTE may provide some pertinent information).
MRI/MRA neck is not the correct imaging choice for this patient as it will not completely identify the SVC obstruction. The patient’s presentation is not consistent with a CVA or TIA in which case MRI/MRA of the neck may be useful. MRI of the chest can be considered in patients who cannot tolerate the IV contrast administered during the preferred CT.
CXR is a reasonable initial imaging study, however it will not necessarily confirm the diagnosis of SVC syndrome as indicated by the question stem. The CXR would be helpful to initially identify any suspicious lung mass or to evaluate for a widened mediastinum. Pleural effusions may also be identified on CXR, which is seen in more than 50% of patients with SVC syndrome.1
Non-contrast CT of the head would be more helpful if you thought the patient was having an acute intracranial hemorrhage or significant cerebral edema. Based on the patient’s history and physical exam, neither one of these scenarios is likely.
Patients with SVC syndrome are treated based on the underlying cause. In malignant etiologies, the treatment plan is multifactorial and based on the type and stage of cancer along, patient’s prognosis and level of acuity on presentation.3 The overall prognosis in patients with SVC syndrome is poor with a median survival rate of 6 months.1
Treatment options include radiation therapy, venous stenting, steroids, chemotherapy and anticoagulation (if there is clot present). Patients that require more emergent therapies include those with respiratory compromise or cerebral edema.
- Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007;356(18):1862-1869.
- Eren S, Karaman A, Okur A, et al. The superior vena cava syndrome caused by malignant disease. Imaging with multi-detector row CT. Eur J Radiol 2006;59(1):93-103.
- Kvale PA, Selecky PA, Prakash UB, et al. Palliative care in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Chest. 2007;132(3 Suppl):368S-403S.