Bicuspid Aortic valve
A Bicupid aortic valve may be genetically determined
First degree relatives may have evidence of an enlarged aortic root approximately 30% percent of the time.
a trileaflet valve
An abnormal aorta is often present
Up to 53% of patients with a bicuspid aortic valve may have an enlarged aortic root. This abnormality may not necessarily be present at the time that the bicuspid valve is discovered making it necessary to periodically re-examine the aortic root in 1st degree relatives.
The natural history of an aneurysm is progressive expansion and for a valve is calcification and stenosis and/or regurgitation. Some patients may be at risk for aortic dissection.
A Patients with aortic valve stenosis or regurgitation may undergo surgical repair or replacement
Patients with aortopathy may need to undergo surgical intervention.
Patients with valve disease and/or aortopathy may be asked to limit their exercise depending on the degree of involvement.
Quit smoking and manage other associated cardiovascular risk factors accordingly.
Patients with aortopathy should have their blood pressure managed aggressively, since doing so may delay further progressive enlargement as time passes. Favoured agents include beta blockers, ACE inhibitors, ARB agents.
Statin agents may be protective [studies more so in AAA patients].
Screen for possible associated aneurysms in patient with a thoracic aneurysm, since these patients may also have aneurysmal disease affecting other vascular beds [brain, lower limbs, AAA].
Evaluate for possible genetic syndromes. Counsel Family members if necessary. If one or more first-degree relatives of a patient with known TAAD are found to have thoracic aortic dilatation, aneurysm, dissection, an abdominal aortic aneurysm, or brain aneurysm, then referral to a geneticist is recommended.