**Abstract**

Bicuspid aortic valve (BAV) is the most common congenital valvular pathology with an incidence of 1–2% in the general population. It is associated with an ascending aortic aneurysm phenotype in 26–50%, and aortic root (+/− ascending aneurysm) phenotype in up to 20–32% of patients. Bicuspid aortic valve patients present with a spectrum of valvular, ascending, and aortic root aneurysmal pathophysiologies. This variable spectrum has mandated the development of an array of surgical procedures to be able to tailor an individualized approach to BAV syndrome for a typically younger BAV population in which long-term outcomes are especially relevant . This chapter will delineate the current evidence-based surgical therapeutic strategies for patients with a BAV syndrome of aortic valve stenosis or insufficiency phenotype and aortic phenotype pathophysiology and include aortic valve replacement, aortic valve repair, aortic valve and supracoronary ascending aorta replacement (AVRSCAAR), Bentall procedure, and valve-sparing root reimplantation.

**Keywords:** bicuspid aortic valve, aortic valve replacement, aortic valve repair, aortic root replacement, valve sparing root reimplantation, Bentall, supracoronary ascending aorta replacement

#### **1. Introduction**

Bicuspid aortic valve (BAV) is the most common congenital anomaly of the cardiac valves. It occurs in approximately 1–2% of the general population [1–5]. Its incidence is known to be higher in Caucasian males and lower in female or non-Caucasian populations [6]. BAV syndrome is a heterogeneous disease that can manifest with various valvular, ascending aortic, and aortic root pathophysiologies. The bicuspid aortic valve can be functionally normal, or it may be insufficient or stenotic. Asymptomatic BAV patients with no other hemodynamic deficiencies have good longterm survival; however, valvular degeneration, either aortic stenosis or insufficiency, may develop with time and require close surveillance [7]. This heterogeneity of BAV syndrome has mandated the development of varied surgical procedures to address the valvular/aortic root pathophysiologies as delineated in **Figure 1** [4]. Therefore, definitive management of BAV syndrome requires a personalized approach according to patient-specific pathophysiology. Therapeutic management strategies for valvular

**Figure 1.** *Bicuspid aortic valve surgical management according to aortic phenotypes.*

phenotype and aortic phenotype utilizing a specific algorithmic approach for variable aortic valve, aortic root, and ascending aorta pathophysiologies in BAV syndrome can provide good long-term functional and clinical results [4] (**Figure 1**).
