A critical review of the American College of Cardiology/American Heart Association practice guidelines on bicuspid aortic valve with dilated ascending aorta.

The 2006 practice guidelines from the American College of Cardiology and the American Heart Association recommend prophylactic aortic replacement for even an asymptomatic patient with a bicuspid aortic valve (BAV) when the aortic dimensions exceed arbitrary ranges based on Marfan syndrome, without comparing risk estimates of aortic dissection with operative risks. In the International Registry of Acute Aortic Dissection, which includes >1,000 autopsied subjects, the average age is 63 years; BAVs are found in only 3%, compared with histories of hypertension in 72%. The risk for valve-sparing aortic replacement is 4% and that for late mortality is 10%, on the basis of 5 publications. The aortic dimensions are from guidelines for Marfan syndrome, with a proved genetic weakness of connective tissue, whereas no culprit genes have been demonstrated in BAV. Although cystic medial necrosis is seen in dilated aortas associated with Marfan syndrome and BAV, it is also seen in dilated aortas with other causes. There is no convincing proof that cystic medial necrosis causes dissection or is simply an effect of dilatation. BAV is not associated with dilatation of the pulmonary arteries, in contrast to Marfan syndrome. Hemodynamic explanations for dilatation of the ascending aorta have been largely ignored because of a belief that it requires severe aortic stenosis or regurgitation. In conclusion, vascular dilatation without a genetic weakness is caused by coarse periodic vibrations from even trivial valve disorders, demonstrated experimentally. There is a natural history of progressive deterioration of the BAV, including the valve left in a valve-sparing aortic replacement, that makes the operation ill advised, as opposed to valve replacement with aortic reinforcement.