Congenital density line along the upper left margin of

Congenital anomalies of the superior vena
cava are generally incidentally discovered during procedures. These include central
venous catheter insertion, pacemaker electrode placement and cardiopulmonary
bypass surgery. Persistent left SVC (PLSVC) is a rare  anomaly found in about 0.3% of healthy
subjects 1 .This condition results from failure
of the left anterior cardinal vein to degenerate during embryological
development. Serious complications such as shock, angina and cardiac arrest
have been described during catheterization of adults with a PLSVC.2
Catheterization of this vessel including hemodialysis catheter placement,
appears to be safe if the PLSVC drainage in right atrium is ensured.3 The
main objective for this case report is to emphasize that any operator who is
inserting a hemodialysis  catheter in the
left jugular or subclavian vein should be aware of the existence of PLSVC.

 
PLSVC has an estimated incidence of
0.3-0.5%.4PLSVC occurs due to the failure of involution
of the left cardinal vein during the foetal life. It is rarely reported as
most of the dialysis catheters are inserted through the right IJV and PLSVC
co-exists with the right SVC in> 80% of patients.5,6 PLSVC should
be considered especially when the CVC via the left subclavian vein or IJV proved to be difficult. It may be associated
with other congenital cardiac anomalies such as atrial septal defect (ASD),
ventricular septal defect (VSD) or atrioventricular septal defect.7In this case, there was no evidence of
cardiac anomalies as evidenced by the TTE. In general, it is normally cause
no symptoms and hemodynamically insignificant 8,9 PLSVC is typically detected
incidentally during procedures such as HD catheter placement.  Ultimately, it can potentially lead to many
serious complications such as systemic embolization, provocation of
arrhythmia, vascular thrombosis, shock , angina and cardiac arrest.2.
Suspicion of LSVC may arise on the
posteroanterior chest radiograph where it may appear as widening of the
aortic shadow, paramediastinal bulging, paramedian stripe or a low density
line along the upper left margin of the heart.10
 It can be diagnosed non-invasively by
echocardiography on conjunction with doppler and/or contrast ultrasonography.
Other diagnostic modalities include Ct scan, MRI.11
Lim et al reported a similar case when they performed the procedure under fluoroscopy.
They initially thought it was an arterial puncture; however, they proceeded
with the insertion of the catheter after confirming a venous puncture. A
digital subtraction angiography revealed the PLSVC, and the patient was
hemodialysed using the catheter for five months without any complications.7
Jang et al 8described another case when they had to insert a left
sided temporary dual lumen catheter for a patient with fistula failure, and
the chest radiograph showed that the catheter tip was on the left
paramediastinal side (similar to our case). They did not remove the catheter
immediately, instead they confirmed the location of the catheter by CT scan
and the diagnosis of PLSVC was made. Stylianou et al 12reported
another case of PLSVC which was incidentally diagnosed after the placement of
the hemodialysis (HD) catheter through the left internal jugular vein. The
chest radiograph showed the abnormal position
of the catheter tip in the left paramediastinal area. They proceeded to CT
scan and transthoracic echocardiogram to confirm the diagnosis. The patient
used the catheter for dialysis for a month before she was able to use the
newly fashioned graft.
 
With the rise of ESRD patients on
hemodialysis in Malaysia, it is essential for the clinicians who insert
central venous catheters (CVC) to be aware of this congenital anomaly in
order to prevent further complications. Moreover, it is vital to exclude
other concomitant cardiac anomalies such as septal defects so as to prevent
systemic embolism following thrombosis of the CVC.
 

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