Emergency transmediastinal pneumonectomy for scimitar syndrome

World J Pediatr Congenit Heart Surg. 2010 Oct;1(3):389-92. doi: 10.1177/2150135110379620.


Repair of scimitar syndrome presenting in infancy involves either tunneling or reimplantation of the anomalous vein to the left atrium and may be fraught with serious complications such as thrombosis and secondary pulmonary infarction necessitating pneumonectomy. The authors present the case of a severely symptomatic infant with scimitar syndrome, managed initially with closure of an atrial septal defect in the hope of avoiding a repair with considerable risk of scimitar vein thrombosis and pulmonary infarction. Despite initial clinical improvement, subsequent rapid development of spontaneous massive emphysematous degeneration of the right lung necessitated emergency pneumonectomy, which was accomplished via the median sternotomy approach.

Keywords: cardiac anatomy/pathologic anatomy; congenital heart disease; emphysema/bullae.