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Brain & Heart                                               Modern imaging and management of bicuspid valves



            of patients with BAV and AR demonstrated a 10-year   in  patients  with  BAV aortopathy using beta-blockers
            event-free survival from major aortic complications  (i.e.,   and/or inhibitors of the renin-angiotensin system (e.g.,
            dissection, rupture, death, or the need for proximal aortic   angiotensin receptor blockers). These recommendations are
            surgery), compared to 93% in those with BAV and AS.    extrapolated from evidence in individuals with connective
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            Furthermore, a meta-analysis revealed that patients with   tissue diseases, such as Marfan syndrome. 67,68,76,77  Beta-
            BAV and AR are 10 times more likely to experience aortic   adrenergic blockers are thought to reduce aortic wall shear
            dissection compared to those with AS. For AR, the ACC/  stress,  whereas  angiotensin-receptor  blockers  have  been
            AHA guidelines recommend surgery for patients with   shown to reduce the rate of aortic growth in patients with
            severe regurgitation who either exhibit symptoms, show   Marfan syndrome. Guidelines from AATS also include
            signs of left ventricular dysfunction (e.g., left ventricular   non-pharmacologic recommendations such as limiting
            ejection fraction <50%), or have substantial left ventricular   salt intake, smoking cessation, and weight management.
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            dilation (e.g., left ventricular end-systolic dimension   Intense isometric exertion and heavy weightlifting should
            >50  mm).  In addition, the American Association for   be avoided in patients with aortopathy. Evidence suggests
                    3
            Thoracic Surgery (AATS) advises considering aortic   that the occurrence of an acute and sudden increase in
            repair in patients with BAV and significant regurgitation   systolic blood pressure to >300 mm Hg increases the risk
            when the aortic diameter reaches 5  cm, particularly in   of serious and life-threatening complications. A uniform
            cases with an aortic root phenotype characterized by   consensus; however, suggests that light weightlifting and
            dilation.  The David procedure, also known as aortic   low-intensity aerobic exercise can benefit patients’ physical
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            valve-sparing surgery, is another option, which involves   and mental well-being. 74
            the reimplantation of the aortic valve into a resected aortic
            root.  This procedure preserves the patient’s native valve   5.2. Surgical management
                73
            and is typically used in patients with aortic root disease but   Surgical approaches for BAV include valve replacement
            without significant valve pathology.               with either bioprosthetic or mechanical valves, the Ross

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            4.3. Aortopathy                                    procedure, the Ozaki procedure, and aortic valve repair.
                                                               AVR is the most common intervention for BAV stenosis
            Guidelines emphasize the importance of individualized   or incompetence in adulthood, and valvuloplasty is
            decision-making for aortic repair in BAV patients.   rarely performed.  Surgical intervention for BAV disease
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            The 2022  ACC/AHA guidelines provide a class  I    typically occurs at a younger age than degenerative TAVs
            recommendation that, in patients with a BAV and a   disease. 18,79  Table 2 outlines the surgical indications based
            diameter of the aortic root, ascending aorta, or both of   on current guidelines. Around 30% of adults requiring
            ≥5.5  cm, surgery is recommended to replace the aortic   AVR also undergo aortic root surgery to mitigate the
            root, ascending aorta, or both. There is a lower threshold   risk of future root dilation. 79,80  AVR is a standard surgical
            of 5  cm in the presence of additional risk factors, such   procedure to  treat  severe  aortic valve  disease, such  as
            as rapid aortic growth (>5  mm/year), family history of   AS or regurgitation, and can be performed with either a
            aortic dissection, or coexisting severe valve pathology   bioprosthetic or mechanical valve. Bioprosthetic valves,
                                       74
            (e.g.,  regurgitation  or  stenosis).   Similarly,  the  2021   made from animal tissue, offer the advantage of a lower
            European Society of Cardiology guidelines recommend   risk of thrombosis than mechanical valves, which require
            surgical intervention at a diameter of 5 cm for high-risk   lifelong anticoagulation.  However, bioprosthetic valves
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            BAV patients, including those with systemic hypertension,   have a shorter lifespan, particularly in younger patients.
            coarctation of the aorta, or a family history of dissection.   The surgical approach for AVR typically involves a median
            For patients undergoing concurrent aortic valve surgery,   sternotomy, providing excellent exposure to the heart and
            the threshold for ascending aorta replacement is further   aorta. In some cases, less invasive techniques are employed,
            reduced to 4.5 cm when performed at experienced centers   such as a hemisternotomy or a mini-thoracotomy, which
            capable of achieving high success rates with valve-sparing   offer smaller incisions and may result in quicker recovery
            or combined procedures. 75
                                                               times and reduced post-operative pain.  The choice of
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            5. Treatment modalities                            surgical approach depends on the patient’s anatomy,
                                                               the surgeon’s experience, and the extent of the disease.
            5.1. Medical management                            Still, each approach aims to provide a successful valve
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            The medical management of patients with BAV aortopathy   replacement with minimal complications.  Bioprosthetic
            emphasizes strict blood pressure control alongside   valves offer the advantage of a lower risk of thrombosis and
            comprehensive cardiovascular risk reduction strategies.   eliminate the need for lifelong anticoagulation, making
            The American AATS recommends treating hypertension   them particularly suitable for older patients. In contrast,


            Volume 3 Issue 3 (2025)                         8                            doi: 10.36922/BH025050008
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