Congenital critical aortic stenosis (AS) is frequently accompanied by endocardial fibroelastosis (EFE), impairing diastolic compliance and long-term ventricular performance. An early Ross procedure, with or without concomitant EFE resection, may restore left ventricular (LV) function; however, long-term functional outcomes remain poorly characterized. We evaluated long-term clinical and functional outcomes following neonatal and infant Ross, in patients with and without EFE.