Congenital mitral valve stenosis (CMVS) is a heterogeneous group of lesions that can be isolated or part of left heart obstructive syndrom. Historically, mitral valve surgery has been associated with significant morbidity and mortality. Surgical techniques have recently improved. We review here a single-center experience in mitral valve (MV) surgery for CMVS.

Between 1975 and 2013, 50 pts underwent MV surgery for CMVS. Six (12%) were adults (>18yrs) and 18 were <2yrs (36%). Median age was 3yrs (19d–30yrs, mean 6.3yrs), median weight 13kg (2.7–80kg, mean 19.2kg). In 35 pts, CMVS was part of left heart obstructive syndrome, with 1 or more previous non-MV surgery in 25. Among the 15 pts with “isolated” CMVS, 2 had previous non-MV surgery. Two pts had previous mitral valvuloplasty in another center.

First choice MV surgery was valvuloplasty in 48 pts, prosthetic valve replacement (MVR) in 2. In the valvuloplasty group, 14 pts were reoperated with a median delay of 11mo (mean 22mo, 19d–16.6yrs). Second surgery was MVR in 3, valvuloplasty in 11. Five pts had a 3rd surgery (3 MVR, 3 valvuloplasty), 2 had a 4th surgery (redo MVR). There were 6 deaths (overall mortality 12%): 2 after first surgery (1 valvuloplasty, 1 MVR, respectively 8 and 14days postop), 4 after the second surgery (24days and 3.5mo after valvuloplasty, 30days and 31yrs after MVR). Risk factors for postoperative death were: presence of supravalvular lesions (left superior vena cava in the coronary sinus and/or supramitral ring, P<0.04), surgery for supravalvular lesions (P<0.04), longer cardiac bypass time (P<0.03) and longer ICU stay duration (P<0.01). Risk factors for reoperation were younger age (P=0.008) and low weight (P<0.04) at first surgery.

Valvuloplasty as first-choice surgery for CMVS carries an acceptable initial mortality but a relatively high rate of reoperations (29%). Iterative plasty is possible but mortality is higher (18%).