What is the recommended echocardiogram (echo) monitoring schedule for a patient with a bicuspid aortic valve (BAV)?

Echocardiographic Surveillance for Bicuspid Aortic Valve

All patients with bicuspid aortic valve require an initial transthoracic echocardiogram to assess aortic root and ascending aorta diameters, followed by serial imaging every 2 years if the aorta is <40 mm, or annually if ≥40 mm. 1

Initial Diagnostic Imaging

Obtain a baseline transthoracic echocardiogram (TTE) immediately upon BAV diagnosis to evaluate:

• Valve morphology and function (stenosis vs regurgitation severity) 1, 2
• Aortic root diameter at multiple levels: sinuses of Valsalva, sinotubular junction, and ascending aorta 1, 2
• Left ventricular size and systolic function 1

Use cardiac MRI or CT when TTE cannot accurately assess aortic root morphology or when aortic diameter exceeds 45 mm. 1, 2 MRI is preferred over CT due to absence of ionizing radiation, particularly important given the need for lifelong serial imaging in these patients. 2

Surveillance Schedule Based on Aortic Diameter

For Aortic Diameter <40 mm:

• Repeat echocardiography approximately every 2 years 1, 2
• This applies regardless of valve function severity 1

For Aortic Diameter ≥40 mm:

• Perform annual echocardiographic measurement 1, 2
• Consider more frequent imaging if progression warrants or clinical symptoms change 1

For Aortic Diameter >45 mm or Growth >3 mm/year:

• Annual imaging is mandatory 2
• Use cardiac MRI or CT for precise measurements at this threshold 2

Important Threshold Adjustments

Consider lower diameter thresholds for patients of small stature. 1 The upper limit of normal is 2.1 cm/m² at the aortic sinuses, and dilatation should be defined relative to body surface area, not absolute measurements. 1

Comprehensive Monitoring Beyond Aortic Diameter

Annual clinical follow-up should assess for:

• Progressive aortic stenosis or regurgitation 1
• Left ventricular dysfunction 1
• New symptoms or change in functional capacity 3
• Arrhythmias via resting ECG and periodic ambulatory monitoring 1

Family Screening

Screen all first-degree relatives with echocardiography to detect undiagnosed BAV, as this is a heritable condition. 1, 2

Critical Clinical Pitfalls

Aortic dilation progresses at approximately 0.5-0.9 mm/year regardless of baseline valve function. 4 This means even patients with normal valve function require surveillance, as 50% of BAV patients develop aortic root involvement. 3

Do not rely solely on valve dysfunction to guide imaging frequency—aortic complications can occur independently of stenosis or regurgitation severity. 1, 4

Women with BAV and ascending aorta >45 mm require counseling about high pregnancy risks and should undergo prophylactic surgery before conception. 1, 2

Patients require lifelong cardiology follow-up even after valve replacement, as progressive aortic enlargement can continue post-operatively. 1, 2