Bicuspid aortic valve
A bicuspid aortic valve (BAV) refers to a spectrum of deformed aortic valves with two functional leaflets or cusps which are often unequal in size.
They are most often congenital while an acquired bicuspid valve occurs when there is fibrous fusion between the right and left cusps of a pre-existing trileaflet aortic valve.
A congenitial biscuspid aortic valve is considered to be one of the most common causes of isolated aortic stenosis 4. It is considered a major cause of aortic valve disease in young adults.
The estimated incidence of a congenital bicuspid valve in the general population is thought to be ~ 2% . They may be commoner in males.
BAV refers to a spectrum of deformed aortic valves with two functional leaflets or cusps which are often unequal in size. Only two cusps, commissures and sinuses are seen in the "pure" BAV subtype. However, usually there are three cusps associated with underdevelopment of a commissure and fusion of two adjacent cusps to form a raphe in the more common form of BAV7.
- autosomal dominant polycystic kidney disease
- coarctation of the aorta : up to 50 - 85 % of coarcations are thought to associated with bicuspid aortic valves 2
The usefulness of plain film in the detection of a bicuspid valve is considered to be rather poor. Occasionally the presence of a single calcified raphe at the expected site of the bicuspid valve may suggest a biscuspid valve 4.
While echocardiography is the standard diagnostic procedure for the evaluation of patients with valvular disease, differentiation of bicuspid valve from other types of calcific aortic stenosis can sometimes be difficult 4-5.
At the time of initial writing an echogram for the detection of a bicuspid aortic valve is though to carry 6
- sensitivity : 76 %
- specificity : 60 %
- positive predictive value : 68 %
- negative predictive value : 95 %
At the time of writing, CT has much higher reported detection rate for bicuspid valves and include 6
- sensitivity : 94 %
- specificity : 100 %
- positive predictive value : 100 %
- nagative predictive value : 97 %
Characteristic “fish-mouth” shape of the open valve in systole is noted on ECG gated cardiac CT7.
Recognised complications include
- 1. Hope MD, Hope TA, Meadows AK et-al. Bicuspid aortic valve: four-dimensional MR evaluation of ascending aortic systolic flow patterns. Radiology. 2010;255 (1): 53-61. doi:10.1148/radiol.09091437 - Pubmed citation
- 2. Seibert JJ, James CA. Pediatric Radiology Casebase. George Thieme Verlag. (1998) ISBN:0865776970. Read it at Google Books - Find it at Amazon
- 3. Ko SM, Song MG, Hwang HK. Bicuspid aortic valve: spectrum of imaging findings at cardiac MDCT and cardiovascular MRI. AJR Am J Roentgenol. 2012;198 (1): 89-97. doi:10.2214/AJR.10.6084 - Pubmed citation
- 4. Spindola-franco H, Fish BG, Dachman A et-al. Recognition of bicuspid aortic valve by plain film calcification. AJR Am J Roentgenol. 1982;139 (5): 867-72. AJR Am J Roentgenol (abstract) - Pubmed citation
- 5. Girdauskas E, Borger MA, Kuntze T et-al. Aortopathy in bicuspid aortic valve disease: is it really congenital? Radiology. 2010;256 (3): 1015-6. doi:10.1148/radiol.101046 - Pubmed citation
- 6. Tanaka R, Yoshioka K, Niinuma H et-al. Diagnostic value of cardiac CT in the evaluation of bicuspid aortic stenosis: comparison with echocardiography and operative findings. AJR Am J Roentgenol. 2010;195 (4): 895-9. doi:10.2214/AJR.09.3164 - Pubmed citation
- 7. Agarwal PP, Wells SA, Kolias TJ. AJR teaching file: aortic valve abnormality in a woman with progressive shortness of breath. AJR Am J Roentgenol. 2010;195 (6): S70-2. doi:10.2214/AJR.09.7172 - Pubmed citation
- 6. Chen JJ, Manning MA, Frazier AA et-al. CT angiography of the cardiac valves: normal, diseased, and postoperative appearances. Radiographics. 29 (5): 1393-412. doi:10.1148/rg.295095002 - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Bicuspid aortic valve (BAV)||✗|
|Congenitial biscuspid aortic valve||✗|
|Bileaflet aortic valve||✗|
|Bi-leaflet aortic valve||✗|