CARDIOVASCULAR JOURNAL OF AFRICA • Vol 21, No 3, May/June 2010
166
AFRICA
associated congenital heart disease should be meticulously
searched for.
When PLSVC is present, the ECG often shows an abnormal
P-wave axis and a normal or shortened PR interval. A geometric
change in the LA may be a possible mechanism for the left-axis
deviation of the P wave.
14
On chest X-ray, a crescent-shaped
shadow of the PLSVC can be seen at the aortic knob or left upper
mediastinum. After insertion of a pulmonary artery catheter into
the left subclavian or jugular vein, a control chest X-ray gives the
false appearance that the catheter has passed through the vessel.
The diagnosis can be confirmed by TTE, transoesophageal
echocardiography (TEE), venous angiography, computed tomog-
raphy (CT) or magnetic resonance imaging (MRI).
On two-dimensional B-mode TTE, the characteristic finding
is a dilated CS on parasternal long-axis view. The normal diam-
eter of the CS is smaller than 1 cm and in the case of isolated
PLSVC, severely increased flow can cause a truly giant CS.
15,16
Other causes of dilated CS are: increased RA pressure, an anom-
alous systemic or pulmonary venous connection or a fistulous
connection with the coronary arteries.
17
The next step in the echocardiographic evaluation should be
contrast application with agitated saline. In normal individuals,
agitated saline injection from the left or right antecubital vein
results in opacification of the RA. In isolated PLSVC, as in our
case, contrast given from the left or right arm opacifies the CS.
When PLSVC is associated with an unroofed CS, contrast injec-
tion from either arm results in opacification of the LA. If RSVC
accompanies the PLSVC, contrast given from the left arm first
appears in the CS, whereas contrast given from the right arm first
appears in the RA (Table 1).
On TEE, the anomalous PLSVC and absence of RSVC can
be well visualised. In mid-oesophageal views, the PLSVC can be
seen near to the left atrial appendage and left upper pulmonary
vein. In the bicaval view, the absence of RSVC can be demon-
strated. Other techniques (venous angiography, CT, MRI) direct-
ly visualise the venous anatomy and confirm the diagnosis.
In the absence of an RSVC, central venous access should be
made from the femoral vein in patients with PLSVC. During
right-sided open-heart surgical procedures, a PLSVC has to be
drained by inserting a separate cannula into it. If the PLSVC
drains into the LA and creates a large right-to-left shunt, surgical
correction should be made. Again, central venous access via the
femoral vein is a safer choice in this variation. When implanting
permanent pacemakers, the left subclavian vein is preferred, as
lead manipulation is easier. There is an acute angle between the
CS ostium and the tricuspid valve, therefore the lead should be
looped in the RA in order to enter the right ventricle.
18
Hand-
shaped stylets and active fixation leads are also helpful to over-
come technical difficulties.
19
Finally, a wide spectrum of clinicians (radiologists, sonog-
raphers, intervenists, intensivists, anaesthesiologists, cardiotho-
racic surgeons) should be aware of PLSVC and its variations in
order to avoid possible complications.
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TABLE 1. ECHOCARDIOGRAPHIC DIAGNOSIS
OF PLSVCAND ITS POSSIBLEVARIATIONS
WITH CONTRASTAPPLICATION
Normal
PLSVC with-
out RSVC*
PLSVC with
RSVC
PLSVC with
unroofed CS
Contrast from
the left arm
RA CS
→
RA CS
→
RA LA
Contrast from
the right arm
RA CS
→
RA RA
LA
PLSVC: persistent left superior vena cava, RSVC: right superior vena
cava, RA: right atrium, LA: left atrium, CS: coronary sinus.
* isolated PLSVC.
