Superior vena caval thrombosis after a neonatal arterial switch procedure

Superior vena cava (SVC) and inferior vena cava obstructions were once well-documented complications from the Mustard repair for D-transposition of the great arteries (TGA), occurring in 10%-40% patients; however, they are rarely documented with the current, more common arterial switch operation (ASO). Similarly, SVC thrombosis is an uncommon, severe complication following neonatal cardiac surgery. We report a case of persistent SVC thrombosis, SVC syndrome, and chylothorax arising after ASO, refractory to thrombolysis and stent placement. A 6-day-old neonate with prenatally known TGA underwent an arterial switch procedure. Despite an initially unremarkable postoperative course, he developed respiratory difficulty after starting enteral feeding. Soft-tissue swelling was noted in the neck, chest, and upper face. An SVC thrombus was identified with cardiac catheterization. Multiple thrombolytic modalities were attempted. His postoperative course was further complicated by recurrent chylothoraces, respiratory failure, sepsis, anasarca, and renal failure. He was eventually transferred to a larger center for a special lymphatics evaluation, where two lymphovenous anastomoses were unsuccessful. He was sent to his home hospital, where he died from extended-spectrum beta-lactamase Klebsiella sepsis. Early diagnosis of SVC syndrome and prompt thrombolysis may prevent the complications encountered in this patient. More research is needed in neonatal thrombolysis and anticoagulation.