What is the management and treatment approach for a patient with a bicuspid aortic valve?

Management of Bicuspid Aortic Valve

Patients with bicuspid aortic valve require lifelong surveillance with initial and serial imaging to monitor for valve dysfunction and aortic dilation, with surgical intervention indicated when aortic diameter exceeds 5.5 cm or when significant valve dysfunction develops. 1

Initial Evaluation

• Initial transthoracic echocardiography (TTE) is mandatory to:

  • Assess valve morphology
  • Measure severity of aortic stenosis (AS) and aortic regurgitation (AR)
  • Evaluate aortic root and ascending aorta dimensions 1

• Advanced imaging with cardiac MRI or CT angiography is indicated when:

  • Echocardiography cannot fully visualize the aortic sinuses, sinotubular junction, or ascending aorta
  • MRI is preferred for younger patients due to absence of radiation exposure 1, 2

• Family screening should be considered as 20-30% of patients with bicuspid aortic valve have affected family members 1

Surveillance Protocol

For Valve Function:

• Asymptomatic patients with normal or mildly dysfunctional valve:

  • Annual clinical evaluation
  • Echocardiography every 1-2 years

• Patients with moderate valve dysfunction (AS or AR):

  • Annual clinical evaluation
  • Annual echocardiography to monitor:
    • For AS: jet velocity, gradient, valve area, LV function
    • For AR: LV dimensions and function 3

For Aortic Dimensions:

• No aortic dilation:

  • Continue with routine echocardiography

• Aortic diameter 4.0-4.5 cm:

  • Serial imaging with consistent modality (MRI or CT preferred)
  • Imaging interval determined by rate of progression and family history 1, 2

• Aortic diameter >4.5 cm:

  • Annual imaging with consistent modality 1, 2

• Rapid progression (≥0.5 cm in 1 year or ≥0.3 cm/year for 2 consecutive years):

  • More frequent imaging (every 6 months)
  • Consider surgical referral 2

Medical Management

• No proven drug therapies have been shown to reduce progression of aortic dilation 1

• For patients with hypertension:

  • Strict blood pressure control with any effective antihypertensive
  • Beta-blockers and ARBs have theoretical advantages but lack proven clinical benefit 1

Surgical Indications

For Aortic Valve:

• Severe symptomatic aortic stenosis
• Severe asymptomatic aortic stenosis with:
  • LV dysfunction (EF <50%)
  • Abnormal exercise test
• Severe aortic regurgitation with:
  • Symptoms
  • LV dysfunction (EF <50%)
  • LV end-diastolic dimension >65 mm

For Aortic Dilation:

• Definite indications for surgery:

  • Aortic diameter >5.5 cm 1, 2
  • Symptomatic aneurysm regardless of size
  • Rapid growth (≥0.5 cm in 1 year or ≥0.3 cm/year for 2 consecutive years) 2

• Consider earlier intervention (aortic diameter 5.0-5.5 cm) if:

  • Family history of aortic dissection
  • Concomitant aortic valve replacement is needed (threshold lowered to 4.5 cm) 1
  • Surgery performed by experienced surgeons in a Multidisciplinary Aortic Team 2

Surgical Options

• Isolated valve dysfunction:

  • Aortic valve repair or replacement based on valve morphology and dysfunction

• Combined valve dysfunction and aortic dilation:

  • Aortic valve replacement plus:
    • Aortic root repair/replacement if diameter >4.5 cm 1
    • Ascending aorta replacement if diameter >4.5 cm 1

• Aortic valve-sparing operations may be feasible in patients with aortic dilation who do not have significant AR or valve calcification 1

Key Pitfalls to Avoid

1. Inconsistent imaging measurements - Ensure measurements are performed using the same technique and at the same location across studies 2

2. Inadequate follow-up - Bicuspid aortic valve is a lifelong condition requiring ongoing surveillance, even after valve replacement 4

3. Missing associated conditions - Screen for coarctation of the aorta and other congenital heart defects 1, 5

4. Overlooking family screening - Consider imaging first-degree relatives, particularly if the patient has aortopathy or family history of valve disease 1

5. Underestimating progression - Even after valve replacement, the aortopathy can continue to progress, requiring continued surveillance 4