18
Bicuspid Aortic Valve and Aortopathy
Table 13. Diagnosis and Intervention in Patients With
Bicuspid Aortic Valve and Aortopathy
Recommendations
COR LOE
Diagnosis
An initial TTE is indicated in patients with a known
bicuspid aortic valve to evaluate valve morpholog y, to
measure the severity of AS and AR, and to assess the shape
and diameter of the aortic sinuses and ascending aorta for
prediction of clinical outcome and to determine timing of
intervention.
I B
Aortic magnetic resonance angiography or computed
tomography (CT) angiography is indicated in patients
with a bicuspid aortic valve when morpholog y of the aortic
sinuses, sinotubular junction, or ascending aorta cannot be
assessed accurately or fully by echocardiography.
I C
Serial evaluation of the size and morpholog y of the aortic
sinuses and ascending aorta by echocardiography, cardiac
magnetic resonance (CMR), or CT angiography is
recommended in patients with a bicuspid aortic valve and
an aortic diameter >4.0 cm, with the examination interval
determined by the degree and rate of progression of aortic
dilation and by family history. In patients with an aortic
diameter >4.5 cm, this evaluation should be performed
annually.
I C
Surgical Intervention
Operative intervention to repair the aortic sinuses or
replace the ascending aorta is indicated in patients with a
bicuspid aortic valve if the diameter of the aortic sinuses or
ascending aorta is >5.5 cm
I B
Operative intervention to repair the aortic sinuses or
replace the ascending aorta is reasonable in patients with
bicuspid aortic valves if the diameter of the aortic sinuses or
ascending aorta is >5.0 cm and a risk factor for dissection is
present (family history of aortic dissection or if the rate of
increase in diameter ≥0.5 cm per year).
IIa C
Replacement of the ascending aorta is reasonable in
patients with a bicuspid aortic valve who are undergoing
aortic valve surgery because of severe AS or AR if the
diameter of the ascending aorta is >4.5 cm.
IIa C
