| Bicuspid aortic valve Classification and external resources | |
| ICD-9 | 746.4 |
|---|---|
| OMIM | 109730 |
| DiseasesDB | 1392 |
| eMedicine | ped/2486 |
A bicuspid aortic valve is an aortic valve with only two cusps (instead of three); situated between the left ventricle and aorta. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Mendelian Inheritance in Man project is a Database that catalogues all the known Diseases with a genetic component, and—when possible—links them The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors The left ventricle is one of four chambers (two atria and two ventricles in the Human Heart. [1] The function of the bicuspid aortic valve is identical to that of the other heart valves: it ensures the unidirectional flow of blood.
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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. Notch homolog 1 translocation-associated (Drosophila, also known as NOTCH1, is a human Gene encoding a single-pass transmembrane Receptor. [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. Murmurs are abnormal Heart sounds that are produced as a result of turbulent blood flow which is sufficient to produce audible noise 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. Murmurs are abnormal Heart sounds that are produced as a result of turbulent blood flow which is sufficient to produce audible noise
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. An aneurysm (or aneurism) is a localized blood-filled dilation (balloon-like bulge of a blood vessel caused by disease or weakening of the vessel wall 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
- ^ Yale Atlas of Echo- Bicuspid aortic valve. Retrieved on 2007-08-08. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 1220 - Sweden is defeated by Estonian tribes in the Battle of Lihula.
- ^ Garg V, Muth AN, Ransom JF, et al (2005). "Mutations in NOTCH1 cause aortic valve disease". Nature 437 (7056): 270-4. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 16025100.
- ^ Ask the Pediatric Cardiologist - Bicuspid Aortic Valve. Retrieved on 2007-08-08. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 1220 - Sweden is defeated by Estonian tribes in the Battle of Lihula.
Cohn LH, Edmunds LH Jr. Cardiac Surgery in the Adult. McGraw-Hill, 2003.
External Links
eMedicine - Aortic Valve, Bicuspid
Bicuspid Aortic Foundation Homepage
Cedars Sinai Heart Center - Bicuspid Aortic Disease
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