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ABSTRACT
Persistent left superior vena cava (SVC) without the right SVC is very
rare and occurs in 0.07%–0.13% of patients with congenital heart
defects. An enlarged coronary sinus detected during cardiac examination
in the prenatal period is the first finding that raises suspicion for
this anomaly. In this report, a patient was presented who was referred
with the prediagnosis of cor triatriatum sinister in the antenatal
period and diagnosed with isolated persistent left SVC.
INTRODUCTION
Persistent left SVC is the most common variation of the thoracic venous
system1. It is found in 0.3%–0.5% of the general
population and 4%–8% in those with congenital heart disease
(CHD)2-5. In the embryonic period, the anterior
cardinal veins provide venous drainage of the cephalic region and upper
extremity. Except for a small portion forming the left superior
intercostal vein, the left anterior cardinal vein regresses by the
eighth week of embryogenesis. Failure of this regression results in
persistent left SVC6. In most cases, the right and
left SVC coexist. Persistent left SVC without the right SVC, also called
isolated persistent left SVC, is very rare and occurs in 0.07%–0.13%
of patients with congenital heart defects with visceroatrial situs
solitus. An enlarged coronary sinus detected during cardiac examination
in the prenatal period is the first finding that raises suspicion for
this anomaly7. Diagnosis is made with three vessels
view, which is one of the standard cardiac examination sections, and an
additional vessel to the left of the pulmonary artery and ductus
arteriosus in the tracheal image or indirectly draining into the left or
right atrium. The wideness of the coronary sinus may confuse the
diagnosis with cor triatriatum sinister during fetal cardiac evaluation.
Isolated persistent left SVC is generally considered a benign condition.
When present with cardiac and extracardiac anomalies and chromosomal
anomalies, perinatal and neonatal outcomes are adversely
affected8.
CASE
In the fetal echocardiographic evaluation performed during the 33rd week
of pregnancy in a female patient who was sent from an external center
with the prediagnosis of cor triatriatum sinister, it was observed that
the coronary sinus was wide. In the evaluation of the region where the
coronary sinus ostium is located, it was observed that the coronary
sinus was wide and there was an additional structure to the left of the
pulmonary artery in three vessel view (Video S1 and S2). As a
preliminary diagnosis, persistent left SVC was suspected alongside cor
triatriatum sinister. The diagnosis was confirmed as isolated persistent
left SVC by echocardiography performed in the postnatal period and
contrast echocardiography performed with agitated saline (Video S3 and
S4).
CONCLUSION-DISCUSSION
Persistent left SVC without right SVC (isolated persistent left SVC) is
a very rare venous malformation. During normal fetal development, the
left anterior venous cardinal system regresses and lags the coronary
sinus and Marshall ligament. Failure of the left anterior cardinal vein
to close results in persistent left SVC. Generally, persistent left SVC
is associated with right SVC and drains into the right atrium through a
dilated coronary sinus. When developmental arrest occurs at an earlier
stage, the absence of coronary sinus is seen and the persistent left SVC
drains into the left atrium. This venous malformation, either isolated
or associated with right SVC, does not itself cause hemodynamic
disturbance and is usually diagnosed incidentally. In the study of Ari
et al., after the retrospective evaluation of 2663 patients with CHD,
between 2005 and 2012, persistent left SVC was found in 88 (3.3%)
patients, while isolated persistent left SVC was found in 5 patients
(0.001%) 5.
Contrary to most reported cases of isolated persistent left
SVC,9 the case presented herein was a rare condition
due to the presence of situs solitus and right SVC agenesis and no
associated CHD, and it was diagnosed by differential diagnosis from cor
triatriatum in the fetal period.
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