What's the first step in treating a bicuspid aortic valve with calcified leaflets and fused LCC (Left Common Coronary) and RCC (Right Common Coronary) arteries?

Management of Bicuspid Aortic Valve with Calcified Leaflets

The first step in treatment is comprehensive transthoracic echocardiography to assess the severity of aortic stenosis and/or regurgitation, followed by imaging of the entire thoracic aorta with either cardiac CT or CMR to evaluate for associated aortopathy. 1

Initial Diagnostic Assessment

Echocardiographic Evaluation

• Perform complete TTE to quantify valve dysfunction severity using peak velocity, mean gradient, and calculated aortic valve area by continuity equation 2
• Measure aortic dimensions at multiple levels: annulus, sinuses of Valsalva, sinotubular junction, and ascending aorta 1
• Document the specific fusion pattern (right-left coronary cusp fusion is most common at 70-80% of cases) as this predicts clinical outcomes 1, 3
• Assess left ventricular response to pressure overload including LVEF, wall thickness, and diastolic function 2

Advanced Aortic Imaging

• Obtain cardiac CT or CMR of the entire thoracic aorta as this is mandatory when BAV is first diagnosed 1
• CMR demonstrates superior accuracy (96%) compared to TTE (73%) for valve morphology assessment 1
• Identify the aortopathy phenotype: ascending (70-75%), root (15-20%), or extended (5-10%) 1

Risk Stratification Based on Calcification

The presence of aortic valve leaflet calcification is a critical prognostic marker with a hazard ratio of 4.72 for cardiovascular events 4. Calcified BAV carries specific risks:

• Increased risk of calcific nodule displacement into coronary ostia during any intervention 2
• Higher risk of annular rupture, root perforation, and aortic dissection 2
• Accelerated progression to severe stenosis requiring intervention 4, 5

Clinical Decision Pathway

If Severe Stenosis is Present (AVA ≤1.0 cm²)

• Symptomatic patients (angina, syncope, heart failure) require aortic valve replacement 2
• Surgical AVR remains the gold standard for BAV with calcification 2
• TAVR in BAV shows higher procedural mortality (3.6% vs 0.9% in tricuspid valves) and increased paravalvular regurgitation (28.4% overall, 17.4% with CT-based sizing) 2
• Type 1 BAV (single raphe) has higher post-TAVR regurgitation rates (34.2% vs 13.3% for type 0) due to bulky calcium preventing adequate stent apposition 2

If Severe Regurgitation is Present

• Assess for flail leaflet, which is specific for severe AR and associated with decreased survival 2
• Evaluate LV dimensions and function as surgical timing depends on LVEF and LV end-systolic dimension 2

If Moderate Dysfunction or Asymptomatic

• Establish serial echocardiographic surveillance every 6-12 months depending on severity 2
• Monitor for progression of calcification and valve dysfunction 4
• Screen first-degree relatives for BAV and thoracic aortic disease 6

Critical Pitfalls to Avoid

• Do not delay aortic imaging: The calcified leaflets indicate advanced disease, and 12.3% of BAV patients develop ascending aorta dimensions >45mm requiring intervention 4
• Do not assume TAVR is equivalent to surgical AVR in calcified BAV: The bulky calcium along the raphe prevents adequate stent apposition and increases complications 2
• Do not overlook coronary ostial assessment: Measure the distance from annulus to coronary ostia, as calcified cusp displacement can cause catastrophic coronary obstruction 2

Regarding the Left Atrial Appendage Finding

The normal LAA velocity of 50 cm/s indicates low stroke risk and does not alter the primary management strategy focused on the aortic valve pathology 2.