Repair of congenital mitral valve dysplasia in infants and children: is italways possible?

Objectives: Surgical management of congenital malformation of the mitral va lve (MV) in the pediatric age group remains a therapeutic challenge for the wide spectrum of the morphological abnormalities and the high incidence of associated cardiac anomalies. We reviewed our experience so as to assess w hether MV conservative surgery is always advisable and its results are supe rior to MV replacement. Methods: Thirty-four consecutive children (20 male and 14 female) with a mean age of 5.9 years (range 45 days-18 years) treate d surgically for congenital MV disease between January 1987 and June 1999. Four patients (11.7%) were under 12 months of age, while 21 patients (62%) were younger than 5 years. Twenty-two patients presented with MV incompeten ce (or prevalent incompetence), while 12 presented with stenosis (or preval ent stenosis). Associated cardiac lesions were present in 22 patients (62.8 %). Results: Mitral valve reconstruction was possible in all. There were no operative deaths. Three patients required reoperation for MV restenosis (a re-repair in one and MV replacement with mechanical prosthesis in two) 4 m onths, 27 months and 5.6 years after repair with no operative deaths. There was only one late death for prosthetic valve thrombosis. Follow-up data re veal that the 33 surviving patients are asymptomatic and well 4 months-12 y ears (mean 72 months) after surgery. At 12 years, actuarial survival and fr eedom from reoperation are 96.8 and 85.9%, respectively. Echocardiography p erformed in all of them shows no or mild incompetence or stenosis in 26 (78 %), while residual moderate MV incompetence persists in six. Conclusions: O ur experience indicates that MV reconstructive procedures in infants and ch ildren with congenital MV dysplasia may be effective and reliable with low mortality and low incidence of reoperation rate. Mitral valve repair should always be attempted, especially in infants, despite the frequent severity of MV dysplasia, to avoid the drawbacks of the currently available prosthes es. (C) 2000 Elsevier Science B.V. All rights reserved.