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Aortic regurgitation (AR) may be caused by primary valve disease, aortic root disease or their combination (Table 257-1).

TABLE 257-1Major Causes of Aortic Valve Disease

Primary Valve Disease

Rheumatic disease results in thickening, deformity, and shortening of the individual aortic valve cusps, changes that prevent their proper opening during systole and closure during diastole. A rheumatic origin is much less common in patients with isolated AR who do not have associated rheumatic mitral valve disease. Patients with congenital bicuspid aortic valve (BAV) disease may develop predominant AR, and ~20% of these patients will require aortic valve surgery between 10 and 40 years of age. Congenital fenestrations of the aortic valve occasionally produce mild AR. Membranous subaortic stenosis results in a high velocity systolic jet that often leads to thickening and scarring of the aortic valve leaflets and secondary AR. Prolapse of an aortic cusp, resulting in progressive chronic AR, occurs in ~15% of patients with ventricular septal defect (Chap. 264), but may also occur as an isolated phenomenon or as a consequence of myxomatous degeneration sometimes associated with mitral and/or tricuspid valve involvement.

AR may result from infective endocarditis (IE), which can develop on a valve previously affected by rheumatic disease, a congenitally deformed valve, or on a normal aortic valve, and may lead to perforation or erosion of one or more leaflets. The aortic valve leaflets may become scarred and retracted during the course of syphilis or ankylosing spondylitis and contribute further to the AR that derives primarily from the associated root disease. Although traumatic rupture or avulsion of an aortic cusp is an uncommon cause of acute AR, it represents the most frequent serious lesion in patients surviving nonpenetrating cardiac injuries. The coexistence of hemodynamically significant AS with AR usually excludes all the rarer forms of AR because it occurs almost exclusively in patients with rheumatic or congenital AR. In patients with AR due to primary valvular disease, dilation of the aortic annulus may occur secondarily and lead to worsening regurgitation.

Primary Aortic Root Disease

AR also may be due entirely to marked aortic annular dilation, i.e., aortic root disease, without primary involvement of the valve leaflets; widening of the aortic annulus and lack of diastolic coaptation of the aortic leaflets are responsible for the AR (Chap. 274). Medial degeneration of the ascending aorta, which may or may not be associated with other manifestations of Marfan syndrome; idiopathic dilation of the aorta; annuloaortic ectasia; osteogenesis imperfecta; and severe, chronic hypertension ...

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