Aortic valve repair in patients with Marfan syndrome and ascending aorta aneurysms due to degenerative disease.

Patients with Marfan syndrome may require aortic surgery because of aortic insufficiency, aortic root aneurysm, ascending aortic aneurysm, or acute type A aortic dissection. The aortic valve leaflets are often overstretched and the fibrosa layer is damaged in many patients, particularly in those with all the stigmata of Marfan syndrome. However, in some patients the leaflets are normal or only minimally stretched in spite of aortic insufficiency. In these patients the aortic valve can be satisfactorily repaired. When significant annuloaortic ectasia is present, the reconstructive procedure consists of excising all three aortic sinuses and reimplanting the aortic valve inside a Dacron graft. If the annuloaortic ectasia is mild and the principal problem is loss of the sinotubular junction because of aneurysmal dilatation of the sinuses of Valsalva, aortic valve repair is accomplished by replacing one, two, or all three aortic sinuses with a properly tailored Dacron graft. The first type of aortic valve repair has been performed in 18 patients with one early and one late failure. The other 16 patients remained well from 3 to 50 months. The second type of aortic valve repair has been performed in 15 patients during the past 3 years without any failure and all patients remain well. Therefore, in selected patients with aortic insufficiency due to aortic root and/or ascending aorta aneurysm secondary to degenerative disease, the aortic valve can be repaired with satisfactory results.