Damus-Kaye-Stansel procedure 5 years after Fontan operation with ligated main pulmonary artery

We report a case of tricuspid atresia with transposed great arteries and rudimentary right ventricle owing to which the patient developed severe subaortic stenosis and restrictive bulboventricular foramen 5 years after her extracardiac Fontan operation. She underwent a successful modified Damus-Kaye-Stansel operation using her native pulmonary valve. Spiral cardiac computed tomography with three-dimensional reconstructions was instrumental in pre-operative surgical planning.