What is the most likely diagnosis and acute management for a patient presenting with elevated jugular venous pressure, arm edema without leg edema, and pleural effusion?

Superior Vena Cava Syndrome (SVC Syndrome)

The combination of elevated JVP, arm edema without leg edema, and pleural effusions is pathognomonic for superior vena cava (SVC) obstruction, most commonly caused by malignancy (particularly lung cancer or lymphoma) or thrombosis from central venous catheters. 1, 2

Clinical Presentation and Pathophysiology

This distinctive pattern occurs because SVC obstruction causes venous hypertension specifically in the upper body drainage territory:

• Elevated jugular venous pressure reflects impaired venous drainage from the head and upper extremities back to the right atrium 3, 4
• Arm edema without leg involvement is the hallmark finding - the lower extremities drain via the inferior vena cava and remain unaffected 1
• Pleural effusions develop from increased hydrostatic pressure in the upper thoracic venous system 1
• Facial swelling, neck vein distension, and upper chest wall venous collaterals may also be present 2

Differential Diagnosis Priority

While elevated JVP typically suggests heart failure, the absence of leg edema essentially excludes biventricular heart failure as the primary diagnosis 5, 6. In heart failure, elevated right atrial pressure causes symmetric bilateral lower extremity edema due to gravity-dependent fluid accumulation 5, 6. The selective upper body distribution points to anatomic venous obstruction rather than cardiac dysfunction.

Internal jugular vein thrombosis can present similarly but typically causes unilateral neck/arm swelling rather than bilateral findings 7, 2. Bilateral upper extremity involvement strongly suggests SVC-level obstruction 1, 2.

Acute Diagnostic Workup

Immediate contrast-enhanced CT of the chest with venography is the diagnostic test of choice 2:

• Identifies the location and extent of SVC obstruction
• Reveals underlying malignancy (mediastinal mass, lymphadenopathy) in 60-85% of cases 2
• Detects thrombus versus extrinsic compression 1, 2

Duplex ultrasound of neck veins can provide rapid bedside confirmation of venous obstruction 2, though CT remains definitive for treatment planning.

Acute Management

Immediate Stabilization

• Elevate head of bed to 45-90 degrees to reduce venous pressure and improve cerebral drainage 2
• Supplemental oxygen for respiratory distress from pleural effusions 5
• Avoid central venous access in upper extremities or internal jugular veins 2

Definitive Treatment Based on Etiology

For malignant SVC syndrome (most common):

• Urgent radiation therapy for radiosensitive tumors (small cell lung cancer, lymphoma) provides relief within 72 hours 2
• Endovascular stenting provides immediate mechanical relief and is first-line for severe symptoms or non-radiosensitive tumors 2
• Chemotherapy for chemosensitive malignancies 2

For thrombotic SVC syndrome:

• Systemic anticoagulation with therapeutic-dose low molecular weight heparin or unfractionated heparin 7, 2
• Catheter-directed thrombolysis or mechanical thrombectomy for acute symptomatic thrombosis 2
• Remove offending central venous catheter if present 2
• Continue anticoagulation for minimum 3-6 months 7, 2

Critical Pitfalls to Avoid

• Do not assume heart failure based solely on elevated JVP and pleural effusions - the distribution of edema is the key discriminating feature 5, 6, 3
• Do not delay imaging if SVC syndrome is suspected - rapid progression can cause laryngeal edema and airway compromise 2
• Do not attempt central line placement in the upper body venous system - use femoral access if needed 2
• Do not give aggressive diuretics - these patients have regional venous obstruction, not volume overload, and diuresis may worsen hemodynamics 5