Clinical condition that occurs due to obstruction of the superior vena cava.
Most common aetiology 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 aetiology.
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 oedema of the head, neck, and arms, often with cyanosis, plethora, and distended subcutaneous vessels. It can be caused by either intraluminal obstruction of the SVC or extrinsic compression.
History and exam
Key diagnostic factors
- presence of risk factors
- localised oedema of the face and upper extremities
- facial plethora
- distended neck veins
- distended chest veins
- hoarseness of voice
- blurred vision
Other diagnostic factors
- weight loss
- chest pain
- mental changes
- skin rash
- laryngeal oedema
- multiple pacemaker leads
- central venous catheters/ports
- age >50 years
1st investigations to order
- chest x-ray
- chest CT
- chest MRI
- ultrasound of upper extremities
Investigations to consider
- sputum cytology
- sputum culture
- erythrocyte sedimentation rate
- C-reactive protein
acute airway obstruction
Kul Aggarwal, MD, MRCP, FACC
Professor of Clinical Medicine
University of Missouri
KA declares that he has no competing interests.
Albert K. Chan, MD
Assistant Professor of Clinical Medicine
University of Missouri
AKC declares that he has no competing interests.
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.
Debabrata Mukherjee, MD
Gill Foundation Professor of Interventional Cardiology
Director of Cardiac Catheterization Laboratories
Gill Heart Institute
Division of Cardiovascular Medicine
University of Kentucky
DM declares that he has no competing interests.
Syed Wamique Yusuf, MD, MRCPI, FACC
University of Texas MD Anderson Cancer Center
Department of Cardiology
SWY declares that he has no competing interests.
Andrew Turley, MB ChB
Cardiology Specialist Registrar
The James Cook University Hospital
AT declares that he has no competing interests.
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