Bicuspid aortic valve

A bicuspid aortic valve is a heart valve with two cusps; situated between the left ventrical and the aorta.^[1]

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Incidence/prevalence

About 1-2% of the population have bicuspid aortic valves, although the condition is nearly twice as common in males.

Bicuspid aortic valve has been found to be an inheritable condition, with a demonstrated association with Notch 1.^[2] Familial clustering as well as isolated valve defects have been documented. The incidence of bicuspid aortic valve can be as high as 10% in families affected with the valve problem. Other congential heart defects are associated with bicuspid aortic valve at various frequencies.

Presentation

In many cases, the condition will cause no problems.^[3] However, especially in later life, a bicuspid aortic valve may become calcified, which may lead to varying degrees of severity of aortic stenosis and aortic regurgitation, which will manifest as murmurs. If these become severe enough, they may require heart surgery.

Diagnosis

The condition can be associated with a heart murmur, and diagnosis can be confirmed with echocardiography.

Treatment/prognosis

Most patients with bicuspid aortic valve whose valve becomes dysfunctional will need careful follow-up and potentially valve replacement in their third or fourth decade of life.

Patients with bicuspid aortic valve should be followed by cardiologist or cardiac surgeon with specific interest in this valve pathology.

Aorta

Another important fact is the aorta of patients with bicuspid aortic valve is not normal. The aorta of a patient with a bicuspid aortic valve does not have the same histological characteristics of a normal aorta. The tensile strength is reduced. These patients are at a higher risk for aortic dissection and aneurysm formation of the ascending aorta. The size of the proximal aorta should be evaluated carefully during the work-up. The initial diameter of the aorta should be noted and periodic evaluation with CT scan (every year or sooner if there is a change in aortic diameter) should be recommended. Therefore, if the patient needs surgery, the size of the aorta will determine what type of surgery should be offered to the patient. Additionally, patients with bicuspid aortic valve are at higher risk of aortic coarctation, an abnormal narrowing of the thoracic aorta.

References

1. ^ Yale Atlas of Echo- Bicuspid aortic valve. Retrieved on 2007-08-08.
2. ^ Garg V, Muth AN, Ransom JF, et al (2005). "Mutations in NOTCH1 cause aortic valve disease". Nature 437 (7056): 270-4. doi:10.1038/nature03940. PMID 16025100.
3. ^ Ask the Pediatric Cardiologist - Bicuspid Aortic Valve. Retrieved on 2007-08-08.

Cohn LH, Edmunds LH Jr. Cardiac Surgery in the Adult. McGraw-Hill, 2003.