CARDIOVASCULAR JOURNAL OF AFRICA • Vol 21, No 3, May/June 2010
164
AFRICA
Case Report
Persistent left superior vena cava with absent right
superior vena cava: a case report and review of the
literature
ÖZGÜL UÇAR, LALE PAŞAOĞLU, HÜLYA ÇİÇEKÇİOĞLU, MURAT VURAL, İBRAHİM KOCAOĞLU,
SİNAN AYDOĞDU
Summary
We report on a rare case of persistent left superior vena cava
(PLSVC) with absent right superior vena cava (RSVC), an
anomaly that is also known as isolated PLSVC. This venous
malformation was identified incidentally in a 30-year-old
woman during thoracic multi-detector computed tomogra-
phy (MDCT), which was performed with the suspicion of
intra-thoracic malignancy.
On thoracic MDCT, the RSVC was absent. A bridging
vein drained the right jugular and right subclavian veins
and joined the left brachiocephalic vein in order to form the
PLSVC, which descended on the left side of the mediastinum
and drained into the right atrium (RA) via a dilated coronary
sinus (CS). The patient was referred to the cardiology depart-
ment for further evaluation. Transthoracic echocardiogra-
phy revealed a dilated CS, and agitated saline injected from
the left or right arms revealed opacification of the CS before
the RA. The patient had no additional cardiac abnormality.
Isolated PLSVC is usually asymptomatic but it can pose
difficulties with central venous access, pacemaker implanta-
tion and cardiothoracic surgery. This condition is also associ-
ated with an increased incidence of congenital heart disease,
arrhythmias and conduction disturbances. A wide spectrum
of clinicians should be aware of this anomaly, its variations
and possible complications.
Keywords:
echocardiography, vascular malformation, superior
vena cava
Submitted 11/8/09, accepted 13/10/09
Cardiovasc J Afr
2010;
21
: 164–166
Persistent left superior vena cava (PLSVC) is the most common
congenital malformation of the thoracic venous system and
it affects about 0.3 to 0.5% of the general population.
1
This
incidence increases 10-fold in patients with cardiac malforma-
tions.
2
PLSVC with an absent right superior vena cava (RSVC),
which is also referred to as isolated PLSVC, is very uncommon,
occurring in 0.07 to 0.13% of patients who have congenital
heart defects with viscero-atrial situs solitus. Nearly half of the
patients with isolated PLSVC have other cardiac malformations,
such as atrial septal defect, endocardial cushion defect or tetralo-
gy of Fallot.
3
In this case report we present a patient with isolated
PLSVC with no other cardiac abnormalities, who was diagnosed
incidentally during thoracic computed tomography.
Case report
A 30-year-old Caucasian woman went on contrast-enhanced
thoracic 16-row multi-detector computed tomography (MDCT)
for the suspicion of intra-thoracic malignancy. MDCT showed
a bridging vein draining the right jugular and right subclavian
veins; it joined the left brachiocephalic vein and formed the
PLSVC, which descended at the left side of the mediastinum
leftward of the pulmonary artery and left atrium (LA) before
draining into the right atrium (RA) via a dilated coronary sinus
(CS) (Figs 1–3). The RSVC was absent and the PLSVC carried
all venous blood from the head, neck and upper extremities.
There was no other pathological finding. The visceral organs
were normally positioned. The patient was referred to the cardi-
ology department for further research for a cardiac abnormality.
On examination, the general appearance of the patient was
good. Her blood pressure was 110/80 mmHg and pulse rate was
85 beats/min. Cardiac auscultation and electrocardiogram (ECG)
were normal. Transthoracic echocardiography (TTE) revealed a
dilated CS of 31
×
19 mm (Fig. 4). There was no evidence of
valvular heart disease and the diameters of the heart chambers
were within normal limits. The left ventricular ejection fraction
was 68% and the estimated peak systolic pulmonary artery pres-
sure was in the normal range.
The inter-atrial and inter-ventricular septum were both intact.
All four pulmonary veins drained into the LA. The thickness of
the pericardium was normal with no effusion. An agitated saline
injection was made from the left antecubital vein. The contrast
first appeared in the CS, then in the RA. The injection was
repeated from the right antecubital vein, which also demonstrat-
ed appearance of the contrast first in the CS and afterwards in
the RA. The diagnosis was confirmed and no additional cardiac
abnormality was detected.
Department of Cardiology, Ankara Numune Education and
Research Hospital, Sihhiye, Ankara, Turkey
ÖZGÜL UÇAR, MD,
HÜLYA ÇİÇEKÇİOĞLU, MD
İBRAHİM KOCAOĞLU, MD
SİNAN AYDOĞDU, MD
Department of Radiology, Ankara Numune Education and
Research Hospital, Sihhiye, Ankara, Turkey
LALE PAŞAOĞLU, MD
MURAT VURAL, MD
