**4. Management**

#### **4.1. Medical**

Medical therapies are to try and alleviate symptoms and slow progression. It is generally felt that blood pressure should be aggressively controlled to try and slow the progression of aortopathy [51].

High blood pressure should be aggressively treated in patients with BAV disease. In Mar‐ fan-associated aortopathy, treatment with beta-blockers to slow the rate of progression is the standard of care at many centers, although debate exists about their effectiveness [50,101]. The ACC/AHA guidelines for the management of adult congenital heart disease and guide‐ lines for the management of patients with valvular heart disease suggest that it is reasonable to use beta-blockers in this population [Class IIa recommendation] [53]. There are emerging data in animal models and in 1 small study in humans supporting the use of angiotensin II receptor blockers to decreased aortic root dilation in Marfan syndrome [50]. Whether these agents will have a role in BAV aortopathy has not yet been demonstrated. Also long-term vasodilator therapy in BAV disease with aortic regurgitation is only recommended if there is concomitant systemic hypertension [47]. The relationship between risk factors for athero‐ sclerosis and the development and progression of degenerative aortic valve disease has been well studied [99]. However, the role of treatment with cholesterol-lowering agents is unre‐ solved. The use of lipid lowering agents specifically in young patients with BAV has not been studied, and the current ACC/AHA guidelines for the management of patients with valvular heart disease do not endorse the use of statins to slow the degenerative process in this population [51]. Concomitant conditions and risk factors should be treated as in the nor‐ mal population.

being performed for valve indications. In addition, suggest that changes in root size more

The Bicuspid Aortic Valve http://dx.doi.org/10.5772/52567 349

Recently published Guidelines [53] for the diagnosis and management of patients with

**1.** First-degree relatives of patients with a BAV, premature onset of thoracic aortic disease with minimal risk factors, and/or a familial form of thoracic aortic aneurysm and dissec‐ tion should be evaluated for the presence of a BAV and asymptomatic thoracic aortic

**2.** All patients with a BAV should have both the aortic root and ascending thoracic aorta

**3.** Should undergo elective operation at smaller diameters (4.0 to 5.0 cm)to avoid acute

**4.** Patients with a growth rate of more than 0.5 cm/year in an aorta that is less than 5.5 cm

**5.** Patients undergoing aortic valve repair or replacement and who have an ascending aor‐ ta or aortic root of greater than 4.5 cm should be considered for concomitant repair of

**6.** Elective aortic replacement is reasonable for patients with BAV when the ratio of maxi‐ mal ascending or aortic root area (πr2) in cm2 divided by the patient's height in meters

In regard to valve surgery, there is controversy regarding the use of the Ross procedure and the use of valve repairs in this population. Abnormalities of the media are seen in both the aorta and the pulmonary artery in BAV disease. Intrinsic abnormalities in the wall of the pulmonary artery [neoaorta] may contribute to progressive neoaortic root dilation and/or aortic regurgitation when the pulmonary root is placed in the systemic position [101].

When to surgically treat asymptomatic patients with BAV remains controversial. The risk of sudden death in asymptomatic adult patients with severe aortic stenosis is reported to be less than 1% per year, however, current practice guidelines recommended aortic valve re‐ placement in patients with reduced left ventricular systolic function (EF< 50%) without oth‐

For high-risk patients to undergo conventional novel methods including aortic balloon valvu‐ lotomy or transfemoral valve implantation may be helpful. A patient considered inoperable should be treated orally with angiotensin converting enzyme (ACE) inhibitors, diuretics, and digitalis. In patients with depressed LV associated with pulmonary congestion and atrial fibril‐ lation, diuretics and digitalis may be used with the understanding that in some cases intensive hemodynamic monitoring is needed. Patients with aortic root dilatation > 4.0cm who are not

candidate for surgical treatment should be given β-adrenergic blocking agents [51].

than 0.5 cm/year are an indication for root replacement [53].

disease. (Level of Evidence: C)

dissection or rupture. ()Level of Evidence: C)

exceeds 10 (CLASS IIa, Level of Evidence: C).

er explanation even when they are asymptomatic [51].

CLASS I

thoracic aortic disease recommendations for BAV are summarized below:

evaluated for evidence of aortic dilatation (Level of Evidence: B)

in diameter should be considered for operation. (Level of Evidence: C)

the aortic root or replacement of the ascending aorta. (Level of Evidence: C)

#### **4.2. Surgical**

Indications for valve surgery in patients with BAV are similar to those with tricuspid aortic valve disease or degenerative aortic valve disease [100].

The 2006 AHA/ACC guidelines also suggest concomitant replacement of the ascending aor‐ ta if it is greater than 45 mm in diameter [51]. Estimated 15-year freedom from complications was 86% in patients with an aortic diameter less than 40 mm, dropping down to 81% in those with diameter 40–44 and 43% in patients with a diameter 45 mm or greater.

When rheumatic disease is excluded, a significant portion of adults undergoing surgery for aortic valve disease will have a congenitally malformed valve. During childhood, insertion of a prosthetic valve is suboptimal because of the continuing growth of the child. Fortunate‐ ly, at this stage, the aortic valve is usually not calcified and valvuloplasty can successfully disrupt the commissural fusion and relieve obstruction. Valvuloplasty is the interventional strategy of choice in children and in some young adults with BAV and aortic stenosis. Symptomatic aortic stenosis is an indication for intervention, similar to standard indications for trileaflet valve disease. However, in the pediatric setting, indications include children with peak-topeak gradients >50 mm Hg who develop symptom at rest or with exercise. An additional indication includes asymptomatic children with peak-to-peak gradients >60 mm Hg. In adulthood, aortic valve replacement is the most common intervention for either aort‐ ic valve stenosis or incompetence, and valvuloplasty is rarely performed [50,51,62].

BAV disease involves younger patients and involves both the valves and the ascendan aorta; therefore, surgical decision making is more complicated. Approximately 30% of adults un‐ dergoing aortic valve replacement will also need aortic root surgery[63]. The guideline sug‐ gest that a cutoff of 5.0 cm be used for intervention or 4.5 cm if the surgery is otherwise being performed for valve indications. In addition, suggest that changes in root size more than 0.5 cm/year are an indication for root replacement [53].

Recently published Guidelines [53] for the diagnosis and management of patients with thoracic aortic disease recommendations for BAV are summarized below:

#### CLASS I

High blood pressure should be aggressively treated in patients with BAV disease. In Mar‐ fan-associated aortopathy, treatment with beta-blockers to slow the rate of progression is the standard of care at many centers, although debate exists about their effectiveness [50,101]. The ACC/AHA guidelines for the management of adult congenital heart disease and guide‐ lines for the management of patients with valvular heart disease suggest that it is reasonable to use beta-blockers in this population [Class IIa recommendation] [53]. There are emerging data in animal models and in 1 small study in humans supporting the use of angiotensin II receptor blockers to decreased aortic root dilation in Marfan syndrome [50]. Whether these agents will have a role in BAV aortopathy has not yet been demonstrated. Also long-term vasodilator therapy in BAV disease with aortic regurgitation is only recommended if there is concomitant systemic hypertension [47]. The relationship between risk factors for athero‐ sclerosis and the development and progression of degenerative aortic valve disease has been well studied [99]. However, the role of treatment with cholesterol-lowering agents is unre‐ solved. The use of lipid lowering agents specifically in young patients with BAV has not been studied, and the current ACC/AHA guidelines for the management of patients with valvular heart disease do not endorse the use of statins to slow the degenerative process in this population [51]. Concomitant conditions and risk factors should be treated as in the nor‐

Indications for valve surgery in patients with BAV are similar to those with tricuspid aortic

The 2006 AHA/ACC guidelines also suggest concomitant replacement of the ascending aor‐ ta if it is greater than 45 mm in diameter [51]. Estimated 15-year freedom from complications was 86% in patients with an aortic diameter less than 40 mm, dropping down to 81% in

When rheumatic disease is excluded, a significant portion of adults undergoing surgery for aortic valve disease will have a congenitally malformed valve. During childhood, insertion of a prosthetic valve is suboptimal because of the continuing growth of the child. Fortunate‐ ly, at this stage, the aortic valve is usually not calcified and valvuloplasty can successfully disrupt the commissural fusion and relieve obstruction. Valvuloplasty is the interventional strategy of choice in children and in some young adults with BAV and aortic stenosis. Symptomatic aortic stenosis is an indication for intervention, similar to standard indications for trileaflet valve disease. However, in the pediatric setting, indications include children with peak-topeak gradients >50 mm Hg who develop symptom at rest or with exercise. An additional indication includes asymptomatic children with peak-to-peak gradients >60 mm Hg. In adulthood, aortic valve replacement is the most common intervention for either aort‐

those with diameter 40–44 and 43% in patients with a diameter 45 mm or greater.

ic valve stenosis or incompetence, and valvuloplasty is rarely performed [50,51,62].

BAV disease involves younger patients and involves both the valves and the ascendan aorta; therefore, surgical decision making is more complicated. Approximately 30% of adults un‐ dergoing aortic valve replacement will also need aortic root surgery[63]. The guideline sug‐ gest that a cutoff of 5.0 cm be used for intervention or 4.5 cm if the surgery is otherwise

valve disease or degenerative aortic valve disease [100].

mal population.

348 Calcific Aortic Valve Disease

**4.2. Surgical**


In regard to valve surgery, there is controversy regarding the use of the Ross procedure and the use of valve repairs in this population. Abnormalities of the media are seen in both the aorta and the pulmonary artery in BAV disease. Intrinsic abnormalities in the wall of the pulmonary artery [neoaorta] may contribute to progressive neoaortic root dilation and/or aortic regurgitation when the pulmonary root is placed in the systemic position [101].

When to surgically treat asymptomatic patients with BAV remains controversial. The risk of sudden death in asymptomatic adult patients with severe aortic stenosis is reported to be less than 1% per year, however, current practice guidelines recommended aortic valve re‐ placement in patients with reduced left ventricular systolic function (EF< 50%) without oth‐ er explanation even when they are asymptomatic [51].

For high-risk patients to undergo conventional novel methods including aortic balloon valvu‐ lotomy or transfemoral valve implantation may be helpful. A patient considered inoperable should be treated orally with angiotensin converting enzyme (ACE) inhibitors, diuretics, and digitalis. In patients with depressed LV associated with pulmonary congestion and atrial fibril‐ lation, diuretics and digitalis may be used with the understanding that in some cases intensive hemodynamic monitoring is needed. Patients with aortic root dilatation > 4.0cm who are not candidate for surgical treatment should be given β-adrenergic blocking agents [51].

#### **4.3. Pregnancy**

During pregnancy there are changes in hemodynamics as well as changes in the aortic me‐ dia, and therefore, women with BAV and significant aortic stenosis and/or dilated aortic roots are at risk for complications during pregnancy.

aorta. All patients should therefore be regularly reviewed to identify progressive root dilata‐

The Bicuspid Aortic Valve http://dx.doi.org/10.5772/52567 351

Bursa Yüksek İhtisas Education and Research Hospital, Cardiology Department, Bursa, Tur‐

[1] Braverman AC, Güven H, Beardslee MA, et al.The bicuspid aortic valve. Curr Probl

[2] Yener N, Oktar GL, Erer D, et al. Bicuspid aortic valve. Ann Thorac Cardiovasc Surg.

[4] Tadros TM, Klein MD, Shapira OM. Ascending aortic dilatation associated with bi‐ cuspid aortic valve: pathophysiology, molecular biology, and clinical implications

[5] Pachulski RT, Weinberg AL, Chan KL. Aortic aneurysm in patients with functionally normal or minimally stenotic bicuspid aortic valve. Am J Cardiol 1991; 67:781–2.

[6] Demir M. Current Approach to Bicuspid Aortic Valve: Review. Global Advanced Re‐

[7] Hutchins GM, Nazarian IH, Bulkley BH. Association of left dominant coronary arte‐ rial system with congenital bicuspid aortic valve. Am J Cardiol 1978; 42: 57–9.

[8] Higgins CB, Wexler L. Reversal of dominance of the coronary arterial system in iso‐

[9] Presbitero P, Demarie D, Villani M, et al. Long term results [15-30 years] of surgical

[10] Demir M. Acute myocardial infarction in a young patient with bicuspid aortic valve.

[11] Suzuki T, Nagai R, Kurihara H, et al. Stenotic BAVassociated with a ventricular sep‐ tal defect in an adult presenting with congestive heart failure: a rare observation. Eu‐

lated aortic stenosis and bicuspid aortic valve. Circulation 1975; 52: 292–6.

repair of aortic coarctation. Br Heart J 1987; 57: 462–7.

Turk Kardiyol Dern Ars. 2009 Oct;37[7]:490-2.

ropean Heart Journal. 1994;15:402-403.

search Journal of Medicine and Medical Sciences. 2012: 1[5]; 105-107.

[3] Ward C. Clinical significance of the bicuspid aortic valve. Heart 2000; 83: 81–5.

tion [6,94].

**Author details**

Mehmet Demir

**References**

Cardiol 2005;30:470-522.

Circulation. 2009 17;119[6]:880-90.

2002 Oct;8[5]:264-7.

key

In rare instances, women will develop progressive symptoms during pregnancy and require either valvuloplasty or valve surgery. Both interventions can be performed during pregnan‐ cy, but are associated with both maternal and fetal risks and should be performed only when necessary.

Although pregnancy can be successfully completed in most instances, aortic surgery may be required early after pregnancy in some women with severe aortic stenosis. Pregnancy itself seems to accelerate the need for surgery postpartum in women with moderate or severe aortic stenosis, perhaps by affecting the ability of the left ventricle to adapt to the fixed out‐ flow obstruction. It is therefore important that women be counseled about both the risk of pregnancy and the potential for late complications [102,103].

Additionally, guidelines suggest that women with BAV and significant aortopathy (ascend‐ ing aorta diameter >4.5 cm)"should be counseled against the high risk of pregnancy" [43].

#### **4.4. Exercise**

There are little data available to support recommendations regarding exercise in subjects with BAV. In children with congenital severe aortic stenosis, for instance, sudden death can occur during exercise [121]. The Task Force on Exercise in Patients with Heart Disease rec‐ ommends that athletes with severe aortic stenosis or severe aortic incompetence with left ventricular dilation [left ventricular dimensions >65 mm] should not participate in competi‐ tive athletics. Athletes with or without aortic valve disease who have dilated aortic roots (>45 mm) are advised to only participate in low-intensity competitive sports. No restrictions exist for those with BAV with no significant valve dysfunction or aortic root/ascending aorta dilation(>40 mm) [50,104,105].
