Staged biventricular repair for absent aortic valve in a neonate

Congenitally absent aortic valve is an extremely rare and fatal cardiac malformation. We report the case of a neonate with absent aortic valve, an interrupted aortic arch, and a normal-sized left ventricle. At age 9 hours, emergency aortic valve closure and a Norwood procedure were performed to maintain coronary circulation. The patient's postoperative course was complicated because of tracheomalacia and a severely dilated aorta that were treated with reduction aortoplasty at age 4 months. Finally, a staged Yasui procedure was performed at 26 months. To our knowledge, this is the first report of a successful biventricular repair for absent aortic valve.