Background- Scimitar Syndrome is a rare complex anomaly where one or all right sided pulmonary veins drains to the IVC, hepatic veins or azygous vein. It is rare with an incidence of 0.5% of all congenital heart disease. Despite various surgical procedures, the reported surgical outcome are very disappointing. We reviewed seven cases of Scimitar syndrome, operated at Prince Charles hospital between 1994 to 2007.

Materials and Methods- Between Dec-1994 to June-2007, seven patients having a scimitar syndrome underwent corrective surgery at our hospital. There were 3 females and 4 males. The youngest was 3 yrs old and the oldest was 74 yrs old. Their follow up ranges from 1yr to 14 yrs. All patients underwent baffle correction of scimitar vein.

Results- All patients undergoing baffle correction of a scimitar vein to the LA were extubated the day following the operation. Immediate post operative period was satisfactory. There was no thirty day mortality. One adult patient had a non coronary cusp aortic leaflet perforation and underwent aortic valve replacement three weeks after surgery. Two patients developed baffle obstruction over a two year period at subsequent follow up. Of the remaining five patients, four are well.One elderly patient died eight years post operatively from a non cardiac cause.


Conclusion- Though rare and technically demanding, correction of scimitar syndrome at all ages is feasible with minimal risk to survival. However disappointing functional results remain in a significant number.