Case Report
Cardiology 2006;105:48–51
DOI: 10.1159/000089247
Superior Vena Cava Anomalies in the
Generation of Angina Pectoris
A Report of Two Cases
Sandeep Hindupur a Francisco J. Lammoglia b
a
Department of Internal Medicine, Washington University School of Medicine, Saint Louis, Mo., and
b
Department of Cardiology, Heartland Health System, Saint Joseph, Mo., USA
Key Words
Superior vena cava
Congenital heart disease

Venous system anomalies
Abstract
Superior vena cava anomalies are rare occurrences
caused by variations in the development of the embry-
onic venous system. Persistent left superior vena cavae
are the most common congenital aberrations in the tho-
racic venous system, with an incidence of 0.3–0.5%, but
their association in the absence of a right superior vena
cava is extremely rare and scarcely reported. We report
two case studies describing persistent left superior vena
cavae found with and without a right superior vena cava
in patients presenting with chest pain. A discussion re-
garding superior vena cava abnormalities as well as the
etiology, associations, and diagnosis of these unusual
entities follows.
Copyright © 2006 S. Karger AG, Basel
Superior vena cava anomalies are relatively rare oc-
currences. These congenital abnormalities result from
variations of the venous system created during the early
embryonic period. Persistent left superior vena cavae
© 2006 S. Karger AG, Basel
0008–6312/06/1051–0048$23.50/0
Fax +41 61 306 12 34
E-Mail karger@karger.ch Accessible online at:
www.karger.com www.karger.com/crd
Received: June 14, 2005
Accepted: August 23, 2005
Published online: October 27, 2005
(PLSVC) are the most frequent congenital abnormality
in the thoracic venous system with an incidence of 0.3–
0.5% in the general population. While its association with
other congenital heart diseases is more frequent, a PLS-
VC without a right superior vena cava (RSVC) in the ab-
sence of dextrocardia is rarely reported. We report two
case studies describing incidentally found PLSVC with
and without RSVC in patients presenting with complaints
of chest pain without other congenital abnormalities.
Case Report
Case Report 1
A 50-year-old white male presented to the emergency depart-
ment with a 4-hour history of chest discomfort. The patient began
experiencing substernal chest pain with radiation to the neck ac-
companied by diaphoresis and dizziness. He denied shortness of
breath, nausea, and vomiting. The pain resolved following sublin-
gual nitroglycerin and all vital signs were normal. The past medical
history was significant for mild coronary artery disease diagnosed
several years prior to presentation, hypertension, hyperlipidemia,
and transient ischemic attacks. He had been subsequently treated
with optimal medical management. An adenosine thallium stress
test performed 1 year prior to admission was negative for any signs
of ischemia. He denied tobacco, alcohol, or illicit drug use. Family
history included coronary artery disease and diabetes mellitus.
Sandeep Hindupur, MD
Washington University School of Medicine, Barnes-Jewish Hospital
4950 Children’s Place, Mailstop: 9021342
Saint Louis, MO 63110 (USA)
Tel. +1 314 362 5060, Fax +1 314 362 6959, E-Mail shindupu@im.wustl.edu
Fig. 1. A left superior vena cava (arrow) adjacent to the transverse
aorta.
Physical examination was unremarkable and an electrocardio-
gram (EKG) illustrated a normal sinus rhythm and rate with no
acute changes when compared with previous EKGs. A chest radio-
graph was significant only for borderline cardiomegaly. A CT scan
of the chest was performed to rule out pulmonary embolism as an
etiology for the patient’s chest pain. While there was no pulmonary
embolism seen, a vascular structure thought to be a left-sided su-
perior vena cava was noted (fig. 1). Initial laboratory data were
normal and an acute myocardial infarction was ruled out with neg-
ative serial cardiac enzymes.
The patient’s chest pain was consistent with angina and, al-
though he had a recent negative adenosine thallium stress test, a
cardiac catheterization was recommended for definitive diagnosis
due to his atherosclerotic coronary disease and risk factors. A left
heart catheterization revealed mild 25% narrowing of the left ante-
rior descending and circumflex arteries. A guidewire was then
placed in the right femoral vein and was advanced through the in-
ferior vena cava, right atrium, and RSVC. The wire was then ma-
nipulated across the left brachiocephalic vein and down through a
left superior vena cava terminating in the coronary sinus (fig. 2).
The cardiac catheterization showed minimal coronary artery dis-
ease with a normal ejection fraction and a PLSVC emptying into
the coronary sinus.
Case Report 2
A 51-year-old white male presented to the emergency depart-
ment with complaints of a 1-day history of intermittent left arm
and neck pain. Seven hours prior to presentation, he experienced
chest pain radiating to the back and shortness of breath. There was
no associated nausea, vomiting, dizziness, or diaphoresis. Upon
Superior Vena Cava Anomalies
Fig. 2. Guidewire (arrow) advanced through the inferior vena cava
and RSVC, across the left brachiocephalic vein, and descending
through the left superior vena cava into the coronary sinus.
arriving, two sublingual nitroglycerin tablets significantly improved
his chest pain. All vital signs were stable. He denied any past med-
ical history, tobacco, alcohol, or illicit drug use, but did report a
strong family history of coronary artery disease.
Physical examination revealed no abnormalities. A chest radio-
graph was unremarkable, while an EKG showed a normal sinus
rhythm and heart rate, poor R wave progression, and non-specific
ST segment changes. All initial laboratory data were within normal
limits. A CT scan of the chest revealed no pulmonary emboli and
an acute myocardial infarction was ruled out.
An echocardiogram was performed revealing no structural ab-
normalities and a normal ejection fraction. An adenosine thallium
stress test demonstrated no significant changes compatible with
ischemia, but was limited symptomatically by subjective chest
pain. A cardiac catheterization was performed for definitive diag-
nosis. Although the coronary arteries were free of any significant
atherosclerotic disease, a dilated coronary sinus with extension to
the left subclavian vein was evident during the levophase of film-
ing, suggesting a superior vena cava abnormality (fig. 3). A guide-
wire was placed in the right femoral vein and advanced through the
inferior vena cava, right atrium, and into the coronary sinus. The
guidewire was then advanced through a left superior vena cava and
into the left brachiocephalic vein. Contrast angiography revealed a
left superior vena cava and an absent right-sided superior vena cava
(fig. 4). The cardiac catheterization revealed no significant coronary
atherosclerotic disease; however, a congenital absence of an RSVC
and a PLSVC draining into the coronary sinus were noted.
Cardiology 2006;105:48–51 49
Fig. 3. Cardiac catheterization revealing a dilated coronary sinus
receiving venous drainage from bilateral superior vena cava. CS =
Coronary sinus.
Discussion
Superior vena cava anomalies such as PLSVC with or
without RSVC are relatively uncommon. The incidence
of PLSVC in the general population is 0.3–0.5%, making
it the most frequent congenital abnormality in the tho-
racic venous system [1]. This percentage increases to 3–
5% in individuals with congenital heart disease [2]. Some
authors have reported a 20% association with tetralogy of
Fallot and an 8% association with Eisenmenger’s syn-
drome [3]. The presence of a PLSVC without an RSVC
in the absence of dextrocardia is an extremely rare abnor-
mality.
These variations in the systemic venous system occur
during early embryonic development. In the fifth week of
life, three major pairs of veins are identified. Of these, the
bilateral cardinal veins form the main venous system with
the anterior subset draining the head and arms [4]. At the
eighth week of gestation, an anastomosis between the left
and right anterior cardinal veins develops to form the left
brachiocephalic vein [1].
Subsequently, most of the blood from the left cephalic
portion of the embryo is shunted to the right causing the
left anterior cardinal vein to degenerate [1, 3–5]. Some
authors believe this mechanism is due to compression of
50 Cardiology 2006;105:48–51
Fig. 4. Contrast angiography via a catheter cannulated through the
inferior vena cava, right atrium, and coronary sinus, demonstrating
a left superior vena cava and left brachiocephalic vein. The RSVC
is absent. LSVC = Left superior vena cava; LBCV = left brachioce-
phalic vein.
the left-sided vena cava between the left atrium and the
hilum of the left lung [6]. The right anterior cardinal vein
matures into the RSVC. When the left anterior cardinal
vein persists, it develops into a PLSVC and usually drains
into the coronary sinus via the vein of Marshall [1, 3].
This is most often seen in the setting of bilateral symme-
try of left or right visceral heterotaxy. Occasionally, the
coronary sinus is absent causing the PLSVC to empty di-
rectly into the right atrium. However, in rare cases, the
PLSVC may drain into the left atrium causing a small
left-to-right shunt. This specific anomaly may or may not
be associated with an absent coronary sinus [7]. In ex-
tremely rare cases of PLSVC, the right anterior cardinal
vein degenerates leading to an absence of an RSVC.
Dilation of the coronary sinus usually occurs second-
arily to receiving the entire venous return from the vena
cavae. Occasionally, this leads to stretching of the atrio-
ventricular node and bundle of His, thereby causing ar-
rhythmias [1, 5]. P wave abnormalities consistent with
right atrial and ventricular enlargement may be noted on
an EKG. PLSVC is also commonly associated with atrial
septal defects which may lead to paradoxical emboli and
strokes [8]. One recent report suggested that PLSVC and
Hindupur /Lammoglia
coronary sinus dilation may lead to mechanical left ven-
tricular inflow obstruction [2]. Nevertheless, if the PLS-
VC is not associated with additional cardiac defects, it is
most often asymptomatic and not hemodynamically sig-
nificant.
PLSVC is usually detected during the placement of a
catheter via the left internal jugular or subclavian vein,
or when there is a longer than anticipated insertion length
of a pulmonary artery catheter [8]. It may also be sus-
pected when a chest X-ray reveals a widened or extra
shadow around the aortic knob, with a low-density line
along the upper left cardiac margin [3, 5], or when an un-
usually large coronary sinus is observed on echocardiog-
raphy. Other tests used in detecting PLSVC include ra-
dionucleotide angiography, computerized tomography,
magnetic resonance imaging, and venography [1].
If a PLSVC should empty directly into the left atrium,
it is routinely surgically moved to the right side. This can
be performed through a baffle from the vena caval open-
ing to the right atrium or by reimplanting the PLSVC
directly into the right atrium or pulmonary artery [5].
There have also been successful reports of moving the
PLSVC to the right atrial appendage [9].
The previous two cases involve presentations of chest
pain resembling coronary artery disease, but without sig-
nificant coronary obstruction. The importance of assess-
ing various imaging modalities when evaluating patients
presenting with chest pain is further exemplified through
these cases. While the frequency of angina as a presenting
symptom in PLSVC is unknown, some theories regarding
the pathophysiological etiology exist. As stated above, sig-
nificant coronary sinus dilation may lead to obstruction
of the left ventricular inflow tract causing an imbalance
between myocardial blood flow and increased oxygen de-
mand from physical exertion culminating in angina [2].
Similarly, the mitral valve may be partially occluded
causing symptoms. Markedly dilated coronary sinuses
may also increase left-to-right shunting in the presence of
an atrial septal defect or cause rhythm anomalies contrib-
uting to discordance between oxygen supply and demand
[2, 5]. Although rare, the occurrence of systemic venous
anomalies should be included in a complete differential
of typical and atypical angina.

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