BACKGROUND: Cardiac manifestations of Marfan syndrome include aortic root 
dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP 
in patients with Marfan syndrome undergoing aortic root replacement.
METHODS AND RESULTS: We retrospectively analyzed data from 166 MFS patients with 
MVP who were enrolled in a prospective multicenter registry of patients who 
underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral 
regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR 
was evaluated by echocardiography preoperatively and ≤ 3 years postoperatively. 
Forty-one patients (25%) underwent composite graft aortic valve replacement, and 
125 patients (75%) underwent aortic valve-sparing procedures; both groups had 
similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) 
underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, 
n=4), including all 15 patients with MR grade >2. Only 1 patient required MV 
reintervention during follow-up (mean clinical follow-up, 31 ± 10 months). 
Echocardiography performed 21 ± 13 months postoperatively revealed MR >2 in only 
3 patients (2%). One early death and 2 late deaths occurred.
CONCLUSIONS: Although the majority of patients with Marfan syndrome who undergo 
elective aortic root replacement have MVP, only 20% have concomitant MV 
procedures. These concomitant procedures do not seem to increase operative risk. 
In patients with MR grade ≤ 2 who do not undergo a concomitant MV procedure, the 
short-term incidence of progressive MR is low; however, more follow-up is needed 
to determine whether patients with MVP and MR grade ≤ 2 would benefit from 
prophylactic MV intervention.