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Bicuspid Aortic Valve Disease

para>Prior to pregnancy, women with known BAV should have the entire aorta imaged. If the aortic diameter is seen to be >4.5 cm, the patient should be advised not to become pregnant (4)[C].

Follow-Up Tests & Special Considerations

  • Transesophageal echocardiogram should be performed if the valve leaflets are not well seen on surface echo to confirm the diagnosis (3)[C].
  • MRI or CT angiograph should be performed if proper visualization of the aorta cannot be achieved with echocardiography alone (3)[C].

Diagnostic Procedures/Other

No procedures are necessary for diagnosis beyond the testing previously mentioned.

Test Interpretation

Not applicable

TREATMENT

GENERAL MEASURES

  • Patients found to have aortic diameters >4.5 cm associated with their BAV should be counseled to refrain from contact and high-intensity sports (5)[C].
  • Patients with BAV are NOT required to undergo routine dental prophylaxis following diagnosis of BAV (2)[A].

MEDICATION

First Line

Any antihypertensive medication is considered first-line treatment. Blood pressure should be tightly controlled at <140/90 mm Hg. No study has shown benefit of β-blockers or ACEI/ARBs over other antihypertensive medications in reducing the rate of aortic dilation associated with BAV (3)[C].

Second Line

Statin use is not routinely recommended at this time specifically for the diagnosis of BAV. There is no contraindication to starting statin therapy in someone with BAV who otherwise qualifies for therapy (3)[C].

ISSUES FOR REFERRAL

  • Any patient with a diastolic murmur should be referred for echocardiogram.
  • Patients should follow up with their cardiologist every 1 to 3 years depending on current size and state of their aortic valve and aorta (3)[C].

ADDITIONAL THERAPIES

No additional therapies are required.

SURGERY/OTHER PROCEDURES

There is no need for surgical procedures of an isolated, normally functioning BAV. The need for surgical revision depends on the presence of aortic regurgitation and/or stenosis and follows the standard guidelines for surgery as would a patient with a trileaflet valve (3)[C].

COMPLEMENTARY & ALTERNATIVE MEDICINE

None

INPATIENT CONSIDERATIONS

Admission Criteria/Initial Stabilization

A patient does not need to be admitted upon the diagnosis of a normally functioning BAV.

IV Fluids

There is no preference for any IV fluid over any other in the resuscitation of someone with BAV.

Nursing

Not applicable

Discharge Criteria

Not applicable

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

AHA/ACC recommends that first-degree relatives be screened for BAV and aortopathy with an echocardiogram (3).

Patient Monitoring

  • Patients are followed with serial echocardiography, MRI, or CT angiography to follow the size of the aorta and the degree of aortic valve stenosis/regurgitation (3)[C].
  • If aortic diameter is <4 cm, minimal aortic valve lesions are present, and there has been little to no change on previous imaging studies, consider 2- to 3-year follow-up (3)[C].
  • If aortic diameter is between 4 and 4.5 cm, valvular lesions are minimal, rate of change of the diameter is minimal, and there is no family history of aortic dissection, consider 2-year follow-up (3)[C].
  • If aortic diameter is >4.5 cm, rate of change of the aortic diameter is rapid, there are significant valvular lesions, or there is a family history of aortic dissection, consider annual follow-up (3)[C].

DIET

A healthy cardiovascular diet consists of large amounts of fruits and vegetables and small amount of red meats and sweets. The diet should include nuts and fish as well. An example would be the DASH diet (6).

PATIENT EDUCATION

  • Patients do not need routine dental prophylaxis for uncomplicated BAV (7).
  • Exercise does not appear to increase the rate of change of the size of the aorta in those with BAV (5).
  • Due to increased absolute risk of aortic dissection in those with aortic dilation, consider limiting moderate- to high-intensity activities and contact sports if aortic root diameter is known to be >40 mm (5).

PROGNOSIS

Patients with BAV have not been seen to have a decreased life expectancy, although they have been shown to be at increased risk of cardiac events to include aortic aneurysm, aortic stenosis, and aortic regurgitation (1).

COMPLICATIONS

  • Common complications of BAV include:Early development of aortic valve stenosis, 50s to 70s versus 70s to 90s in patients with trileaflet valvesAortic valve regurgitationAortic dilation
  • Rare complications of BAV include:Aortic aneurysmAortic dissectionEndocarditisSudden death

REFERENCES

11 Siu SC, Silversides C. Bicuspid aortic valve disease. J Am Coll Cradiol. 2010;55(25):2789-2800.22 Mordi I, Tzemos N. Bicuspid aortic valve disease: a comprehensive review. Cardiol Res Pract. 2012;2012:196037.33 Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014;63(22):2438-2488.44 Verma S, Siu SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med. 2014;370(20):1920-1929.55 Iskandar A, Thompson PD. Diseases of the aorta in elite athletes. Clin Sports Med. 2015;34(3):461-472.66 Eckel RH, Jakicic JM, Ard JD, et al. 2013 AHA/ACC guideline on lifestyle management to reduce cardiovascular risk: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014;63(25, Pt B):2960-2984.77 Walter W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116(15):1736-1754.

SEE ALSO

Aortic Valvular Stenosis; Aortic Regurgitation, Pediatric; Aortic Dissection

CODES

ICD10

Q23.1 Congenital insufficiency of aortic valve

ICD9

746.4 Congenital insufficiency of aortic valve

SNOMED

bicuspid aortic valve (disorder)

CLINICAL PEARLS

  • Patients with BAV do not need dental prophylaxis.
  • First-degree family members should receive screening for BAV via echocardiogram, CT angiography, or MRI.
  • Patients with BAV should not be restricted from exercise unless they have known aortic dilation.
  • Although BAV puts patients at risk for increased cardiac morbidity, it has not been shown to significantly affect mortality.