Persistent left superior vena cava is a congenital
anomaly which affects 0.3% of healthy population
and 1.3-10% of patients with cardiovascular
disease.[
3] An association between PLSVC and absence
of coronary sinus is an extremely unusual condition,
usually associated with an inter-atrial defect.[
3,
4] More
unusual is the PLSVC, absence of coronary sinus, and
partial atrioventricular septal defect.
Some authors hypothesized that this unusual
anatomy is due to the failed process of embryonic venous system lateralization at the level of the left horn and left
anterior cardinal vein.[5] The left anterior cardinal vein
follows the same development of the right, while the last
part of the left horn remains high on the left and behind
the left atrium instead of becoming the coronary sinus.
Because the left horn does not mitigate, the ostium of
coronary sinus will not form.[5]
Furthermore, clinical complications of this anomaly
are cyanosis and reduced strain tolerance.[2] Therefore,
PLSVC carries more importance in the event of
cardiovascular surgery (absolute contraindication of
retrograde cardioplegia), central venous, and permanent
transvenous pacing lead placement.[6,7] This anomaly
may also cause a misdiagnosis such as right-to-left
shunting or Eisenmenger's syndrome.
Several surgical procedures in the correction
surgery have been reported including ligation of the
left SVC, intra-atrial redirection of flow from the
left SVC to the right atrium, and re-implantation
of the left SVC into the right atrium, pulmonary
artery or SVC.[8] Ligation of the vein obliterates the
intracardiac shunt, however, this procedure is risky,
unless there are large collateral links in the head which
allow non-obstructed head and neck venous return
into the heart. Re-implantation of the persistent left
SVC is preferable, when there is a possibility that an
intra-atrial baffle may obstruct systemic or pulmonary
venous return due to the location of the veins orifices,
in particular.[8-10]
In conclusion, our case had partial endocardial
cushion defect with an absent coronary sinus and
PLSVC without any communication between SVCs.
In our case, thebesian veins were opening on way
of the baffle and there was the absence of intercaval
communication and adequate left atrium volume.
Therefore, we used a tube graft PTFE material, as
we thought that the selected material could be more
resistant to compression from the left atrium pressure.
Declaration of conflicting interests
The authors declared no conflicts of interest with
respect to the authorship and/or publication of this
article.
Funding
The authors received no financial support for the
research and/or authorship of this article.