Aortopathy in repaired tetralogy of Fallot and David procedure.

Abstract

Tetralogy of Fallot (TOF) is a condition that often leads to long-term enlargement of the aortic root in after surgery. The aortic dilation is believed to be caused by histological abnormalities of the aortic media and the hemodynamic characteristics of increased aortic flow, compared to pulmonary flow. Severe cyanosis, severe right ventricular outflow tract (RVOT) obstruction, older age at repair, a larger aortic size at the time of repair, and a history of an aortopulmonary shunt parameters related to long-standing volume overload of the aortic root were the reported risk factors. Right aortic arch, male sex, and the association of chromosome 22q11 deletion were also reported to be risk factors. The enlargement of the aortic root can cause aortic regurgitation (AR), leading to left ventricular dysfunction and an increased risk of aortic dissection, necessitating surgical intervention. The outcomes of aortic valve repair for AR have improved, leading to an increasing trend of choosing this approach, particularly in younger patients who would otherwise require mechanical valve replacement, thereby avoiding the need for anticoagulation therapy. The indications and timing of prophylactic aortic root replacement in adult patients with congenital heart disease have not been described, and the current consensus recommends surgical intervention for an ascending aorta with a diameter of ≥55 mm. In this review article, we focus on valve-sparing root replacement (VSRR) in TOF.