Superior Vena Cava Occlusion with Persistent Left Superior Vena Cava: a Rare Case of Right to Left Shunt
Muhammad Zeeshan1, Igor Laskowski1, Sateesh Babu2
1Westchester Medical Center, Valhalla, NY;2Westchester Medical Center, valhalla, NY
Persistent left superior vena cava (PLSVC) is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities. The presence of PLSVC can render access to the right side of heart challenging via the left internal jugular or subclavian approach, which is a common site of access utilized when placing pacemakers, Swan-Ganz catheters or central venous catheters. We present an interesting case of PLSVC causing significant right to left shunt.
56-year-old female with history of end stage renal disease s/p renal transplant presented with persistent shortness of breath and poor exercise tolerance. She was noted to be persistently hypoxic with a PaO2 of 52. Patient had a previous left arm HERO graft placed 10 years ago which was no longer being used. She was also noted to have right arm swelling but normal L arm. Hero graft was non-functioning with tip in right atrium. A transesophageal echo did not show any cardiac anomalies. A CT-venogram was done to rule out pulmonary embolism which showed occluded left innominate vein and SVC but there was a large venous connection from left subclavian to left atrium (vertical vein of Marshall/PLSVC).
An open repair of SVC occlusion was recommended. Patient underwent removal of HERO graft by a left supraclavicular incision. A median sternotomy was performed, open endarterectomy of left innominate vein and SVC with bovine pericardial patch angioplasty was performed. The vertical vein of Marshall (PLSVC) was identified entering the left superior pulmonary vein at the left atrium and was ligated. The patient was extubated 6 hours postop, hypoxia (PaO2) improved, right arm swelling and shortness of breath resolved and patient was discharged home on postoperative day 4 in stable condition.
A PLSVC certainly presents technical difficulties with right heart access via the left internal jugular or subclavian vein, but does not preclude insertion of catheters. In rare cases, SVC occlusion can lead to opening of vestigial vein of Marshall which causes significant right to left shunt and severe hypoxemia. Clinicians should be aware of this anomaly, its variations and possible complications
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