Bicuspid aortic valve disease and pulmonary autograft root dilatation after the Ross procedure: a clinicopathologic study

Objective: Bicuspid aortic valve disease has been associated with histologic abnormalities of the aortic root. Recent reports have suggested similar alterations may exist in the pulmonary artery of patients with bicuspid aortic valve. The present study was undertaken to define the histologic condition of the aortic and pulmonary artery root in bicuspid aortic valve disease and the relationship with pulmonary autograft root dilatation after the Ross procedure. Methods: In 17 patients undergoing aortic root replacement with the pulmonary autograft, biopsy specimens of the aortic root and pulmonary artery trunk were collected. Clinical and histologic findings of patients with bicuspid aortic valves were compared with those with tricuspid aortic valves. Results: There were 9 patients (8 male, 1 female) with bicuspid aortic valve (group 1) and 8 (all male) with tricuspid aortic valve (group 2). Mean age was comparable (24.4 ± 9.8 vs 23.6 ± 10.8 years, P = .9). Aortic insufficiency as an indication for operation was more common in group 1 (9/9 vs 5/8, P = .007), whereas preoperative aortic root dilatation was equally prevalent (4/9 vs 1/8, P = .1). Prior aortic valve repair had been performed in 2 patients (1/9 vs 1/8, P = .9). Prevalence of cystic medionecrosis of the aortic wall was similar in the 2 groups (4/9 vs 3/8, P = .6). Cystic medionecrosis of the pulmonary artery trunk was found only in 1 patient with tricuspid aortic valve (0/9 vs 1/8, P = .3). During a mean follow-up of 26.5 ± 12.2 months (32.1 ± 12.7 vs 20.1 ± 7.4 months, P = .04), prevalence of pulmonary autograft root dilatation (greater than 4.0 cm) was equally represented in patients with native bicuspid or tricuspid aortic valve (3/9 vs 2/8, P = .6). Conclusions: Histologic abnormalities of the pulmonary artery root are rare and equally prevalent in young patients with bicuspid and tricuspid aortic valves. On the contrary, root dilatation is relatively common late after autograft root replacement but appears unrelated to bicuspid aortic valve disease or to pre-existing degenerative changes of the pulmonary artery root.