Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity

Abstract

Bicuspid aortic valve (BAV) disease is becoming increasingly respected by clinicians. This enhanced appreciation is based on recognition of how common and virulent this disease really is. This disease is now known to be the most common congenital lesion affecting the human heart (with potential competition in frequency posed by mitral valve prolapse). It is estimated that the BAV lesion alone accounts for more morbidity and mortality than all the other congenital cardiac lesions combined. Many authorities feel that this disease is so virulent that every individual with a BAV will, given enough time, develop aortic stenosis, aortic insufficiency or aortic aneurysm/dissection related to the bicuspid valve disease. This review looks in detail at clinical issues related to BAV disease, including genetics, pathophysiology, diagnosis, management and surgical decision making. The picture emerging from basic and clinical studies is of a defect in collagen metabolism affecting not only the aortic valve, but also the wall of the aorta itself. Timely intervention for bicuspid-related aortic valve disease or aneurysm can preserve both duration of life and quality of life in affected individuals.

Endothelial Notch1 Is Required for Proper Development of the Semilunar Valves and Cardiac Outflow Tract

“…BAVs may result from the fusion of any leaflet, although in humans, right–left fusion is most common 34. The process of leaflet fusion is currently not well understood because leaflet fusion may occur early in cushion development or later during valve remodeling.…”

Management of aortic stenosis in pregnancy

“…(3,8,9) It is especially true for patients with a bicuspid aortic valve as fetal congenital heart disease occurs in ±4% of women with congenital AS. Fetal ultrasound is suggested when the mother has a bicuspid aortic valve.…”

Guilt by association: a paradigm for detection of silent aortic disease

“…According to population-based studies, a BAV is estimated to be present in 1-2% of the population (36)(37)(38). Concomitant bicuspid aortopathy is common as well, although the exact estimates vary greatly from 20% to 84% (Figure 8) (38).…”

“…It is important to remember that BAV-related aortic aneurysms are a major causative factor for aortic dissection, which usually occurs long before a clinically evident aortic stenosis will develop (37). At the same time, some recent studies have shown that the long-term survival for patients with a BAV is not much different from a matched control population (40).…”