Circulation. 2002;106:900-904
doi: 10.1161/01.CIR.0000027905.26586.E8
doi: 10.1161/01.CIR.0000027905.26586.E8
(Circulation. 2002;106:900.)
© 2002 American Heart Association, Inc.
Clinician Update |
Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve
From the Division of Cardiac Surgery (P.W.M.F., S.V., T.E.D., R.D.W.) and Department of Pathology (R.L.L., J.B.), University of Toronto, Toronto General Hospital, Toronto, Ontario, Canada.
Correspondence to Jagdish Butany, MBBS, MS, FRCPC, Professor, Department of Laboratory Medicine and Pathobiology, University of Toronto, Department of Pathology, Toronto General Hospital, E4-322, 200 Elizabeth St, Toronto, Ontario, Canada M5G 2C4. E-mail jagdish.butany@uhn.on.ca
An extract of the first 250 words of the full text is provided, because this article has no abstract. |
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Introduction |
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You are contacted by a concerned 34-year-old airline pilot with a leaky bicuspid aortic valve recently diagnosed by an echocardiogram that had been requested by his new employer as part of a routine medical assessment. He claims that he is perfectly healthy but is at risk of losing his job over his condition. He is concerned that his disease is hereditary and that his children may also be at risk. The patient, his attorney, and his insurance company have requested a statement from you as to the cause, possible complications, and treatment options associated with a congenital malformation of the aortic valve.
The bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, occurring in 1% to 2% of the population. The majority of BAV patients develop complications requiring treatment. Physicians are often challenged when asked to provide evidence-based advice about BAV disease because the pathogenesis and pathophysiology of this disease are not well understood.
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What Causes Bicuspid Aortic Valves? |
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BAVs are the result of abnormal aortic cusp formation during valvulogenesis. Adjacent cusps fuse to form a single aberrant cusp, larger than its counterpart yet smaller than 2 normal cusps combined. BAVs are likely the result of a complex developmental process, not simply the fusion of 2 normal cusps. In fact, congenital aortic valve malformations may reflect a phenotypic continuum of unicuspid valves (severe form), bicuspid valves (moderate form), tricuspid valves (normal), and the rare quadricuspid forms1 (Figure 1).
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