Progressive aortic root dilatation is a common feature after surgical repair of tetralogy of Fallot. This report describes a successful valve-sparing replacement of the aortic root in a patient with significant dilated aortic root and aortic regurgitation after repair of tetralogy of Fallot.Keywords: aortic operation, aortic root, aortic valve repair, congenital heart disease (CHD), tetralogy of Fallot tricular outflow tract (RVOT) patch) at four years of age. He had limited exercise tolerance and occasional chest pain. Echocardiogram revealed that the aortic valve was tricommissural without stenosis with moderate central AR (Fig. 1A). There was retrograde diastolic flow in the descending aorta. The ascending aorta was mildly dilated (40 mm, Z-score 7.4). There was stenosis at the right ventricular outflow tract. Aortogram revealed dilatation of the aortic annulus and ascending aorta (Fig. 2). Cardiac magnetic resonance imaging (MRI) revealed moderate pulmonic regurgitation and moderate AR with regurgitant fraction of 34%. Ejection fraction of the left ventricle (LV) decreased to 33%. The coronary artery anatomy was normal. At 17 years of age, the patient underwent valve-sparing root replacement as well as placement of a pulmonary homograft in the RVOT. Total cardiopulmonary bypass was established between the distal ascending aorta and both vena cavae. Myocardial protection was achieved using moderate systemic hypothermia (32°C) and antegrade cold blood cardioplegia followed by retrograde cardioplegia, repeated every 20 minutes. Although the aortic annulus was dilated, valve tissue was intact.Valve-sparing aortic root replacement with aortic valve re-implantation was performed. A 30 mm Gelweave Valsalva prosthesis (Terumo Cardiovascular Systems Corp., Ann Arbor, Michigan) was used for the reconstruction. The right ventricular outflow tract was reconstructed with a 26 mm allograft. The aortic cross clamp was taken off, and the cardiopulmonary bypass was safely discontinued. Intraoperative echocardiogram after termination