Bicuspid Aortic Valves


Bicuspid aortic valve disease (BAVD) is a deformity where the aortic valve forms with

only two leaflets instead of three. The etiology of this congenital condition is unknown but is suspected to be due to a connective tissue defect. On average, 2% of the population can present with BAVD with a higher prevalence seen with males. The most common fusion seen in BAVD is between the right coronary and left coronary leaflets.


BAVD is typically present at birth but is often asymptomatic until adulthood (40-60

years old). However, there have been a few cases where the BAVD is severe enough to

cause congestive heart failure during infancy. Patients with this condition must be examined for other syndromes including coarctation of the aorta, Turner’s syndrome, and patent ductus arteriosus.


BAVD on a parasternal long axis view shows a “doming” pattern upon opening of the valve. The presence of a raphe, the underdeveloped valve, can confirm the diagnosis. On a short axis view, the opening of the valve forms a “fish mouth” shape.



BAVD can further lead to aortic stenosis and regurgitation leading to various symptoms

including shortness of breath, dizziness, angina, and syncope. Murmurs heard in these patients are characteristic of aortic stenosis and regurge.  As the disease progresses, the symptoms appear more frequently and during rest. A left sided ejection click heard best in the aortic area is suggestive of a non-stenosed BAV.

Complications of BAVD include heart failure from left ventricular hypertrophy. The

connective tissue disorder can also precipitate an aortic aneurysm and put the patient at

risk for rupture and dissection.


For patients who present with mild stenosis or regurgitation, treatment with calcium

channel blockers, ace inhibitors, or beta blockers can prevent complications from

developing. Diuretics can be added in the presence of heart failure.

Newborns who present with severe symptoms are immediate candidates for aortic valve



Surgical management with aortic valve replacement is indicated when patients are

severely symptomatic, hemodynamically unstable or when medical treatment fails.

Replacement is also done on patients with high risk for aortic rupture or dissection.

Replacement with either biological (animal or human tissue)l or mechanical (man-made) valves are based on patient demographic and lifestyle. Two available options is open heart surgery or minimal incision valve surgery (MIVS). Another procedure is the transcatheter aortic valve replacement (TAVR) which is a less invasive procedure where a catheter is introduced through the femoral artery up to the aortic valve.




Dr. Ben-Zur is a cardiologist and electrophysiologist who treats patients from Burbank, West Hollywoood, Encino and the greater Los Angeles area.

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