Superior vena cava syndrome

Last reviewed: June 2019

Last updated: July 2019

Summary

Clinical condition that occurs due to obstruction of the superior vena cava.

Most common etiology is malignancy; however, there has been an increase in benign causes due to more frequent use of intravascular devices.

Although rarely fatal, may sometimes present as life-threatening upper airway obstruction.

High index of suspicion is required to make the diagnosis in many cases.

Treatment and prognosis depend on underlying etiology.

Definition

Superior vena cava (SVC) syndrome is a clinical condition that occurs as a result of obstruction of the SVC, leading to interrupted venous return from the head, thorax, and upper extremities to the right atrium. The increased venous pressure results in edema of the head, neck, and arms, often with cyanosis, plethora, and distended subcutaneous vessels. [1] It can be caused by either intraluminal obstruction of the SVC or extrinsic compression.

History and exam

Key diagnostic factors

history of smoking, pacemaker, or central venous catheter
localized edema of the face and upper extremities
dyspnea
facial plethora
cough
distended neck veins
distended chest veins
hoarseness of voice
lymphadenopathy
blurred vision
stridor
confusion/stupor

Full details

Other diagnostic factors

anorexia
weight loss
hemoptysis
headache
chest pain
mental changes
fever
skin rash
arthralgia
laryngeal edema
cyanosis
papilledema
coma

Full details

Risk factors

smoking
multiple pacemaker leads
central venous catheters/ports
age >50 years
radiation

Full details

Diagnostic investigations

1st investigations to order

chest x-ray
chest CT
chest MRI
ultrasound of upper extremities

Full details

Investigations to consider

venography
biopsy
sputum cytology
thoracentesis
sputum culture
erythrocyte sedimentation rate
C-reactive protein

Full details

Treatment algorithm

ACUTE

acute airway obstruction

ONGOING

malignant etiology

infectious etiology

iatrogenic etiology

Contributors

Authors VIEW ALL 

Kul Aggarwal, MD, MRCP, FACC

Professor of Clinical Medicine

University of Missouri

Columbia

Disclosures

KA declares that he has no competing interests.

Albert K. Chan, MD

Assistant Professor of Clinical Medicine

University of Missouri

Columbia

Disclosures

AKC declares that he has no competing interests.

Acknowledgements

Professor Kul Aggarwal and Dr Albert K Chan would like to gratefully acknowledge Dr Nipun Arora and Lokesh Tejwani, previous contributors to this topic. NA declares that he has no competing interests.

Peer reviewers VIEW ALL 

Debabrata Mukherjee, MD

Gill Foundation Professor of Interventional Cardiology

Director of Cardiac Catheterization Laboratories

Gill Heart Institute

Division of Cardiovascular Medicine

University of Kentucky

Lexington

Disclosures

DM declares that he has no competing interests.

Syed Wamique Yusuf, MD, MRCPI, FACC

Associate Professor

University of Texas MD Anderson Cancer Center

Department of Cardiology

Houston

Disclosures

SWY declares that he has no competing interests.

Andrew Turley, MB ChB

Cardiology Specialist Registrar

The James Cook University Hospital

Middlesbrough

Disclosures

AT declares that he has no competing interests.

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Superior vena cava syndrome

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Investigations to consider

Treatment algorithm

acute airway obstruction

malignant etiology

infectious etiology

iatrogenic etiology

Contributors

Authors VIEW ALL 

Kul Aggarwal, MD, MRCP, FACC

Disclosures

Albert K. Chan, MD

Disclosures

Acknowledgements

Peer reviewers VIEW ALL 

Debabrata Mukherjee, MD

Disclosures

Syed Wamique Yusuf, MD, MRCPI, FACC

Disclosures

Andrew Turley, MB ChB

Disclosures

Differentials

Guidelines

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