Eisenmenger syndrome with aorto-pulmonary window

An aortopulmonary (AP) window is a rare cause of Eisenmenger syndrome and results from an abnormal septation of the truncus arteriosus [1]. Most such defects present with early onset congestive heart failure during infancy and adult survival in surgically uncorrected patients is rare 2, 3.

An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.