Surgical outcomes of 14 children with scimitar syndrome

Abstract

Objective To summarize the diagnostic and surgical experience of children with scimitar syndrome and to explore the immediate/mid-term outcomes of these operations. Methods Retrospective analysis was performed for 14 clinical records of children with scimitar syndrome from January 2010 to December 2017. There were 7 boys and 7 girls with a mean age of (18.23±28.55 months)(23 days to 9 years). The complications included other cardiac abnormalities (n=10), mesocardia (n=8), right pulmonary hypoplasia (n=7) and collaterall circulation (n=8). Follow-ups were conducted for analyzing the immediate/mid-term outcomes of survival and stenosis of corrected scimitar vein (SV). And postoperative survival curves were plotted. Results SVs were dissected from inferior caval vein (IVC) and anastomosed to left artrium (n=9); SVs (2 SVs) were resected together with partial IVC wall and anastomosed to left artrium (n=1); SVs (2 SVs) were resected respectively and anastomosed side-to-side and then anastomosed to left artrium (n=1); SVs were anastomosed to right atrium and interatrium septum be rebuilt (n=2) and SV was connected to artificial conduit and then connected to left atrium (n=1) owing to that SV was too far to left atrium. In the same term of operation, complicated cardiac abnormality was cured. And 2/14 cases died immediately postoperatively with a mortality rate of 14.29%; 1/12 survivors died of pneumonia and respiratory failure at 14 months postoperatively and 1/3/5-year postoperative cumulative survival rates were 86%, 73% and 73% respectively. One corrected SV of 12 survivors was stenosed during discharge and 3 corrected SVs of 11 survivors became stenotic during follow-ups. Conclusions Scimitar syndrome may be complicated with other cardiac abnormality, mesocardia and pulmonary hypoplasia, etc. Most SVs of scimitar syndrome may be cured by anastomosing SV to left artrium. SV should be anastomosed to right atrium instead of left atrium and interatrium septum. Collateral circulation of scimitar syndrome may be plugged during cardiac interventions and even resected intraoperatively. Key words: Scimitar Syndrome; Congenital heart disease; Cardiac surgical procedures