Bicuspid aortic valve
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| Bicuspid aortic valve Classification and external resources | |
 | |
|---|---|
| Bicuspid aortic valve | |
| ICD-9 | 746.4 |
| OMIM | 109730 |
| DiseasesDB | 1392 |
| eMedicine | ped/2486 |
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For an expanded discussion of this topic, please see aortic stenosis
Overview
A bicuspid aortic valve is a heart valve with two cusps; situated between the left ventrical and the aorta.[1]
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.[1] 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.[1] 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 careful 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 [1].
Pathological Findings
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology
 Aortic Stenosis, Bicuspid valve: Gross; excellent image of bicuspid and calcific valve showing a false raphe. |
Aortic Stenosis, Bicuspid valve: Gross; good example of bicuspid valve |
 Aortic Stenosis, Bicuspid valve: Gross; image of bicuspid aortic valve, an excellent example |
 Aortic Stenosis, Bicuspid valve: Gross; close-up image of bicuspid aortic valve. |
 Aortic Stenosis, Bicuspid valve: Gross; close-up image of bicuspid aortic valve. |
 Bicuspid aortic valve |
 Gross natural color opened first portion aortic arch with bicuspid aortic valve shows stenosis and aortic root is dilated |
 Aortic Stenosis Bicuspid: Gross; natural color opened left ventricular outflow tract with calcific masses on valve as well as anterior leaflet mitral valve probably did not cause significant stenosis |
 Bicuspid Aortic Valve with Repaired Aorta Coarctation: Gross natural color opened left ventricular outflow tract with uncomplicated bicuspid aortic valve repaired coarctation barely visible ruptured postoperative young female with ovaries Turner mosaic not ruled out |
 Bicuspid Aortic Stenosis: Gross; fixed tissue |
 Aortic Stenosis, Bicuspid: Gross; fixed tissue view of stenotic valve through ventricular outlet track |
 Aortic Stenosis Bicuspid: Gross; fixed tissue. Bicuspid valve and false raphe classical |
 Bicuspid aortic valve |
 Bicuspid aortic valve |
 Bicuspid aortic valve |
 Left ventricular hypertrophy due to bicuspid aortic valve |
References
See Also
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
