**7.3 AS and AI with mild-moderate root phenotype**

For both BAV aortic stenosis and aortic insufficiency valve phenotype and mildmoderate root phenotype with ascending aneurysm and moderate dilatation (SOV 40–45 mm), the fate of the retained sinus segment and the effect of valvular pathology on post-operative sinus growth had been undefined. It has been proposed that the sinus segment in BAV aortopathies is at risk for future aortic events. Therefore, it has been advocated, by some, for removal of all aortic segments in patients with aortopathies despite moderate dilation [4]. A recent swing in the pendulum has occurred advocating for retention of the sinus of Valsalva for moderate root aneurysms [56, 57]. This change results from studies reporting a slower growth rate for the sinus segment and a less aggressive aortic event rate for the preserved moderately dilated aortic root [4, 56–58]. Peters et al. found the sinus segment growth rate was only between 0.27 mm and 0.5 mm per year requiring an average of 29.1 years for the sinus segment to become aneurysmal after AVRSCAAR Please do not use an abbreviation without first defining it [58]. For patients with either BAV aortic stenosis and aortic insufficiency with ascending aneurysm and moderate dilatation (40–45 mm) AVRSCAAR can be performed as a therapeutic option with good long-term results (Consort Diagram **Figure 1**) [4, 56–58].

#### **7.4 Aortic stenosis**

Aortic valve stenosis occurs in approximately 50% of adult patients with BAV valve phenotype that requires aortic valve replacement [12, 59]. Progression to critical aortic stenosis pathophysiology resulting in therapeutic intervention in BAV patients often occurs at a younger age than patients with trileaflet aortic valves.

#### **7.5 Aortic stenosis valve phenotype**

For patients with aortic valve phenotype, but no aortic aneurysm phenotype, aortic valve replacement with a prosthetic aortic valve is the therapeutic option (**Figure 1**). Additional trials are needed to delineate the optimal anatomy, sizing, and implantation techniques for TAVR [60, 61].

The Ross procedure (pulmonary autograft to replace the aortic valve and homograft to replace pulmonic valve) can also be considered as an option to replace the stenotic aortic valve. In patients with the appropriate pulmonary and aortic annular anatomy, good long-term durability has been noted [62].

#### **7.6 Aortic stenosis with root phenotype**

In patients with BAV aortic stenosis phenotype an unrepairable valve and aortic root phenotype with a sinus of Valsalva ≥45 mm, a mechanical or bioBentall procedure is a therapeutic option (**Figure 1**). This involves replacing the aortic valve and ascending/root aorta as a composite and reimplanting the coronary arteries to the tubular graft. This can be either mechanical or bioprosthesis (BioBentall).

For patients with BAV aortic stenosis phenotype an ascending aortic aneurysm and moderate sinus of Valsalva (SOV) dilation (40–45 mm), aortic valve replacement and supracoronary ascending aortic replacement (AVRSCAAR) can be performed thereby preserving the root. Studies have shown that the aortic root remains stable over long-term [4, 56].

**Figure 1** delineates the surgical procedure for AS phenotype and the associated aortic phenotypes.
