Open Access Review
Article DOI: 10.7759/cureus.42227
Superior Vena Cava Syndrome: An Umbrella
Review
Review began 07/11/2023
Rajendra P. Shah 1 , Olayiwola Bolaji 2 , Sanchit Duhan 3 , Anderson C. Ariaga 1 , Bijeta Keisham 4 , Timir Paul
5 , Wael Aljaroudi 6 , M. Chadi Alraies 7
Review ended 07/19/2023
Published 07/20/2023
© Copyright 2023 1. Department of Internal Medicine, Vassar Brothers Medical Center, Poughkeepsie, USA 2. Department of Internal
Shah et al. This is an open access article Medicine, University of Maryland Capital Regional Medical Center, Largo, USA 3. Department of Internal Medicine,
distributed under the terms of the Creative Sinai Hospital of Baltimore, Baltimore, USA 4. Sinai Center for Thrombosis and Research, Sinai Hospital of Baltimore,
Commons Attribution License CC-BY 4.0., Baltimore, USA 5. Section of Interventional Cardiology, University of Tennessee at Nashville/Ascension Saint Thomas
which permits unrestricted use, distribution,
Hospital, Nashville, USA 6. Department of Cardiology, Augusta University Medical College of Georgia, Augusta, USA 7.
and reproduction in any medium, provided
Department of Cardiology, Detroit Medical Center, Detroit, USA
the original author and source are credited.
Corresponding author: M. Chadi Alraies,
Abstract
Superior vena cava syndrome (SVCS) is a medical emergency that encompasses an array of signs and
symptoms due to obstruction of blood flow through the superior vena cava (SVC). It poses a significant
healthcare burden due to its associated morbidity and mortality. Its impact on the healthcare system
continues to grow due to the increasing incidence of the condition. This incidence trend has been attributed
to the growing use of catheters, pacemakers, and defibrillators, although it is a rare complication of these
devices. The most common cause of SVCS remains malignancies accounting for up to 60% of the cases.
Understanding the pathophysiology of SVCS requires understanding the anatomy, the SVC drains blood
from the right and left brachiocephalic veins, which drain the head and the upper extremities accounting for
about one-third of the venous blood to the heart. The most common presenting symptoms of SVCS are
swelling of the face and hand, chest pain, respiratory symptoms (dyspnea, stridor, cough, hoarseness, and
dysphagia), and neurologic manifestations (headaches, confusion, or visual/auditory disturbances).
Symptoms generally worsen in a supine position. Diagnosis typically requires imaging, and SVCS can be
graded based on classification schemas depending on the severity of symptoms and the location,
understanding, and degree of obstruction. Over the past decades, the management modalities of SVCS have
evolved to meet the increasing burden of the condition. Here, we present an umbrella review providing an
overall assessment of the available information on SVCS, including the various management options, their
indications, and a comparison of the advantages and disadvantages of these modalities.
Categories: Cardiac/Thoracic/Vascular Surgery, Cardiology
Keywords: superior vena cava (svc), cerebral venous thrombosis (cvt), endovascular stent therapy, mediastinitis,
superior vena cava syndrome
Introduction And Background
Superior vena cava syndrome (SVCS) is a clinical condition that comprises a spectrum of signs and
symptoms due to obstruction of blood flow through the superior vena cava (SVC) [1]. The first described case
of SVCS was a patient with a syphilitic aortic aneurysm in 1757. In a review published in 1954, from the 274
well-documented cases reviewed by Schecter, about 40% were due to a syphilitic aneurysm or tuberculous
mediastinitis [2]; this study was significant at the time as it showed that SVCS had other etiologies other
than syphilitic aortic aneurysms. It is estimated that malignant tumors account for 60% of cases, while
iatrogenic causes from thrombosis or stenosis caused by central lines or medical devices account for 30-40%
[3]. This review article provides a concise, evidence-based review of the management of SVCS.
The incidence of SVCS continues to rise due to the increasing use of catheters, pacemakers, and
defibrillators [4]. Rice et al. found that 28% of all SVCS is associated with a device or catheter [5]. While
complications arising from these devices contribute to a significant proportion of SVCS cases, Chee et al.
observed that it is a rare complication affecting only about 0.1-3.3% of all pacemaker patients [4]. Major
thrombophilia and Behcet’s disease are also common causes of spontaneous SVCS. In older adults,
malignancy is the most common cause of SVCS [6-8].
Understanding the pathophysiology of SVCS requires an understanding of the anatomy. The SVC drains
about one-third of the venous blood to the heart. It receives venous blood from the right and left
brachiocephalic veins, which drain the head and the upper extremities. In a computed tomography (CT)
scan, the length of the SVC is 7.1 cm ± 1.4 with a diameter of 2.1 cm ± 0.7 (which varies with volume) [9]. A
cross-sectional diameter of < 1.07 cm2 indicates SVC obstruction or compression [10].
The most common presenting symptoms of SVCS are swelling of the face and hand with distension of
vessels in the subcutaneous tissue, cyanosis or plethora, chest pain, respiratory symptoms due to edema, and
How to cite this article
Shah R P, Bolaji O, Duhan S, et al. (July 20, 2023) Superior Vena Cava Syndrome: An Umbrella Review. Cureus 15(7): e42227. DOI
10.7759/cureus.42227
, swelling of parts of the respiratory tract, including the pharynx and larynx (dyspnea, stridor, cough,
hoarseness, and dysphagia), and neurologic manifestations (headaches, confusion, or visual/auditory
disturbances), enlargement of subcutaneous vessels, and edema of the arms, head, and neck. Cardiac
function compromise can also occur due to mass effect on the heart or impaired venous return due to SVC
obstruction [11]. Symptoms generally worsen in a supine position. Other signs and symptoms specific to the
causative agent may also be observed.
Review
Classification and scoring system for SVCS
The severity of symptoms directly correlates to the understanding and extent of the venous obstruction and
inversely to the development of venous collaterals [11,12]. Four main collateral pathways can develop with
SVCS, with the essential collateral pathway being the azygos-hemiazygos pathway (via the intercostal and
lumbar veins). The other collateral pathways that might be created are the internal thoracic route (via the
epigastric veins and superficial thoracic veins), the lateral thoracic route (via the superficial circumflex and
long saphenous and femoral vein), and the vertebral and paravertebral route (Figure 1). In a very severe and
rare form of SVCS, the hot quadrate sign-pathway can connect the superior epigastric and internal thoracic
veins [11,13,14].
2023 Shah et al. Cureus 15(7): e42227. DOI 10.7759/cureus.42227 2 of 10
Article DOI: 10.7759/cureus.42227
Superior Vena Cava Syndrome: An Umbrella
Review
Review began 07/11/2023
Rajendra P. Shah 1 , Olayiwola Bolaji 2 , Sanchit Duhan 3 , Anderson C. Ariaga 1 , Bijeta Keisham 4 , Timir Paul
5 , Wael Aljaroudi 6 , M. Chadi Alraies 7
Review ended 07/19/2023
Published 07/20/2023
© Copyright 2023 1. Department of Internal Medicine, Vassar Brothers Medical Center, Poughkeepsie, USA 2. Department of Internal
Shah et al. This is an open access article Medicine, University of Maryland Capital Regional Medical Center, Largo, USA 3. Department of Internal Medicine,
distributed under the terms of the Creative Sinai Hospital of Baltimore, Baltimore, USA 4. Sinai Center for Thrombosis and Research, Sinai Hospital of Baltimore,
Commons Attribution License CC-BY 4.0., Baltimore, USA 5. Section of Interventional Cardiology, University of Tennessee at Nashville/Ascension Saint Thomas
which permits unrestricted use, distribution,
Hospital, Nashville, USA 6. Department of Cardiology, Augusta University Medical College of Georgia, Augusta, USA 7.
and reproduction in any medium, provided
Department of Cardiology, Detroit Medical Center, Detroit, USA
the original author and source are credited.
Corresponding author: M. Chadi Alraies,
Abstract
Superior vena cava syndrome (SVCS) is a medical emergency that encompasses an array of signs and
symptoms due to obstruction of blood flow through the superior vena cava (SVC). It poses a significant
healthcare burden due to its associated morbidity and mortality. Its impact on the healthcare system
continues to grow due to the increasing incidence of the condition. This incidence trend has been attributed
to the growing use of catheters, pacemakers, and defibrillators, although it is a rare complication of these
devices. The most common cause of SVCS remains malignancies accounting for up to 60% of the cases.
Understanding the pathophysiology of SVCS requires understanding the anatomy, the SVC drains blood
from the right and left brachiocephalic veins, which drain the head and the upper extremities accounting for
about one-third of the venous blood to the heart. The most common presenting symptoms of SVCS are
swelling of the face and hand, chest pain, respiratory symptoms (dyspnea, stridor, cough, hoarseness, and
dysphagia), and neurologic manifestations (headaches, confusion, or visual/auditory disturbances).
Symptoms generally worsen in a supine position. Diagnosis typically requires imaging, and SVCS can be
graded based on classification schemas depending on the severity of symptoms and the location,
understanding, and degree of obstruction. Over the past decades, the management modalities of SVCS have
evolved to meet the increasing burden of the condition. Here, we present an umbrella review providing an
overall assessment of the available information on SVCS, including the various management options, their
indications, and a comparison of the advantages and disadvantages of these modalities.
Categories: Cardiac/Thoracic/Vascular Surgery, Cardiology
Keywords: superior vena cava (svc), cerebral venous thrombosis (cvt), endovascular stent therapy, mediastinitis,
superior vena cava syndrome
Introduction And Background
Superior vena cava syndrome (SVCS) is a clinical condition that comprises a spectrum of signs and
symptoms due to obstruction of blood flow through the superior vena cava (SVC) [1]. The first described case
of SVCS was a patient with a syphilitic aortic aneurysm in 1757. In a review published in 1954, from the 274
well-documented cases reviewed by Schecter, about 40% were due to a syphilitic aneurysm or tuberculous
mediastinitis [2]; this study was significant at the time as it showed that SVCS had other etiologies other
than syphilitic aortic aneurysms. It is estimated that malignant tumors account for 60% of cases, while
iatrogenic causes from thrombosis or stenosis caused by central lines or medical devices account for 30-40%
[3]. This review article provides a concise, evidence-based review of the management of SVCS.
The incidence of SVCS continues to rise due to the increasing use of catheters, pacemakers, and
defibrillators [4]. Rice et al. found that 28% of all SVCS is associated with a device or catheter [5]. While
complications arising from these devices contribute to a significant proportion of SVCS cases, Chee et al.
observed that it is a rare complication affecting only about 0.1-3.3% of all pacemaker patients [4]. Major
thrombophilia and Behcet’s disease are also common causes of spontaneous SVCS. In older adults,
malignancy is the most common cause of SVCS [6-8].
Understanding the pathophysiology of SVCS requires an understanding of the anatomy. The SVC drains
about one-third of the venous blood to the heart. It receives venous blood from the right and left
brachiocephalic veins, which drain the head and the upper extremities. In a computed tomography (CT)
scan, the length of the SVC is 7.1 cm ± 1.4 with a diameter of 2.1 cm ± 0.7 (which varies with volume) [9]. A
cross-sectional diameter of < 1.07 cm2 indicates SVC obstruction or compression [10].
The most common presenting symptoms of SVCS are swelling of the face and hand with distension of
vessels in the subcutaneous tissue, cyanosis or plethora, chest pain, respiratory symptoms due to edema, and
How to cite this article
Shah R P, Bolaji O, Duhan S, et al. (July 20, 2023) Superior Vena Cava Syndrome: An Umbrella Review. Cureus 15(7): e42227. DOI
10.7759/cureus.42227
, swelling of parts of the respiratory tract, including the pharynx and larynx (dyspnea, stridor, cough,
hoarseness, and dysphagia), and neurologic manifestations (headaches, confusion, or visual/auditory
disturbances), enlargement of subcutaneous vessels, and edema of the arms, head, and neck. Cardiac
function compromise can also occur due to mass effect on the heart or impaired venous return due to SVC
obstruction [11]. Symptoms generally worsen in a supine position. Other signs and symptoms specific to the
causative agent may also be observed.
Review
Classification and scoring system for SVCS
The severity of symptoms directly correlates to the understanding and extent of the venous obstruction and
inversely to the development of venous collaterals [11,12]. Four main collateral pathways can develop with
SVCS, with the essential collateral pathway being the azygos-hemiazygos pathway (via the intercostal and
lumbar veins). The other collateral pathways that might be created are the internal thoracic route (via the
epigastric veins and superficial thoracic veins), the lateral thoracic route (via the superficial circumflex and
long saphenous and femoral vein), and the vertebral and paravertebral route (Figure 1). In a very severe and
rare form of SVCS, the hot quadrate sign-pathway can connect the superior epigastric and internal thoracic
veins [11,13,14].
2023 Shah et al. Cureus 15(7): e42227. DOI 10.7759/cureus.42227 2 of 10
