Vol.3 - No.3

Absent Right Superior Vena Cava withAtrial Septal Defect and Unroofed coronarysinus

By : Molavipoor, maleki, alizadeh, Hosseinikhah

Keywords : -

The absence of the right superior vena cava (SVC) is usually associated with the presence
of a left SVC. Here we'll present a case with the absence of the right SVC and a
persistent left SVC found in association with an ostium secundum atrial septal defect
and unroofed coronary sinus

Objectives

Cardiac surgery for congenital heart defects
frequently reveals the presence of
other associated anomalies. The presence
of left superior vena cava (SVC) occurs in
approximately 0.3% to 0.5% of the general
population (1, 2). We are reporting the
case of a congenital heart disease (CHD)
with atrial septal defect (ASD) that underwent
central venous catheterization
(CVC) through right-sided neck vein
and surgical closure of the septal defect
uneventfully. The patient was diagnosed
during the surgery and subsequently in the
postoperative chest radiograph to have an
absent RSVC, which was confirmed by
echocardiography.

Conclusion

In conclusion, this rare venous malformation should be
considered in the differential diagnosis of a dilated coronary
sinus and should be diagnosed preoperatively to avoid
unexpected problems at operation. Both clinicians and
sonographers should be alerted to the possible presence of
this combined venous anomaly. Transthoracic echocardiography
is an important diagnostic tool for accurate diagnosis
of this rare congenital thoracic venous malformation.

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