Abstract

Objectives: Having collected a large series of congenital mitral stenosis we studied the operative results and long-term outcome of our evolving mitral valve (MV) repair techniques performed to correct this lesion. Methods: Between 1986 and 2012, 137 infants and children (mean age 4.1 ± 5.0 (range 1 month to 16.8 years) underwent surgical correction of congenital mitral stenosis (CMS). In 48 patients, CMS is involved in Shone's anomaly. CMS was defined according to Ruckman and Van Praagh's classification. Type I, typical congenital MS, was seen in 56 patients. Hypoplastic MV (Type II), d was seen in 15 patients. TSupravalvar mitral ring (Type III) was seen in 48 patients. VParachute MV (Type IV) was seen in 10 patients, described as having the usual two leaflets and commissures, but all chordae merged into one major papillary muscle. T Results: MV repair was performed using commissurotomy, division of chordae tendinae, papillary muscle splitting and fenestration, and resection of mitral ring, applied according to the presenting morphology in patients with either previously corrected or concomitant correction of the left-sided obstructive lesions. Postoperative echocardiography showed absence of MV stenosis and immediate improvement of symptoms, except in a 3-month-old infant who died 18 days postoperatively due to myocardial failure. During the 24-year follow-up, 23 patients underwent repeat MV repair and 1 underwent MV replacement after failed attempts at repair. Mean duration of follow-up was 17.5 ± 1.5 years (range 6.4–24.2 years). Freedom from reoperation was 97.6 ± 2.4%, 89.3 ± 5.1%, 77.1 ± 7.2%, 72.0 ± 8.3% and 52.8 ± 11.8%, at 30 days, 1, 5, 10 and 15 years postoperatively, respectively Cumulative survival rate was 97.6 ± 2.4%, 92.3 ± 4.3%, 83.8 ± 6.1%, 75.7 ± 7.8% and 70.3 ± 8.9%, at 30 days, 1, 5, 10 and 15 years postoperatively, respectively Mortality unrelated to valve repair accounted for 9 (20%) deaths. Age at operation, severity of mitral abnormalities and concomitant left ventricular outflow tract lesions proved to be independent risk factors for reoperation and mortality (p < 0.05). Conclusions: Long-term functional outcome of mitral valve repair in children with CMS is satisfactory. Repeat MV repair and/or replacement may be deemed necessary during the course of follow-up.

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