Superior vena cava syndrome following neonatal cardiac surgery

OBJECTIVE: To review the incidence, diagnosis, and management of superior vena cava syndrome (SVCS) after surgery for congenital heart disease. DESIGN: Retrospective clinical review. All patients were computer registered. Our database includes daily follow-up. SETTING: Pediatric cardiac surgery intensive care unit in a university hospital. PATIENTS: A total of 1853 consecutive pediatric cardiac operations performed in 285 neonates and 1568 older children from 1993 to 1999 are reviewed. MEASUREMENTS AND MAIN RESULTS: The diagnosis of SVCS was suspected clinically: Color changes and swelling of the upper part of the body, confirmed by echo-Doppler, showed no or minimal flow in the superior vena cava at the beginning and collateral flow later on. Nine patients developed SVCS (0.5%). All the study patients were neonates. The prevalence of SVCS in our neonatal patients was 3.15% (nine of 285), with no SVCS in older children. Accompanying complications included chylothorax (five), hydrocephalous (four)-three of whom required ventriculoperitoneal shunt during follow-up. Thrombolytic therapy was used in five patients, and thrombectomy was used in one patient. The ventilation period ranged from 4 to 46 days (mean 20.1 days), and the length of hospital stay ranged from 37 to 120 days (mean 61.3 days). No mortality was observed during follow-up. CONCLUSIONS: SVCS is an uncommon, severe complication following neonatal cardiac surgery. It may cause chylothorax, hydrocephalus, and severe respiratory complications leading to high morbidity. Early diagnosis and thrombolytic therapy may prevent the progression of this syndrome to its subsequent sequels.