Mid-term outcomes of congenital mitral valve surgery: Shone's syndrome is a risk factor for death and reintervention.

OBJECTIVES

To study the recent trends and outcomes of congenital mitral valve surgery in children.

METHODS

From 2008 to 2014, 84 procedures in 66 consecutive patients (41 procedures in 31 patients with mitral stenosis and 43 procedures in 35 patients with mitral regurgitation) were retrospectively evaluated. The mean age at surgery was 4.3 ± 5.4 years, and 27 patients (41%) were neonates or infants.

RESULTS

Seven (11%) patients died during the follow-up period of 3.2 ± 2.3 years and 5 (71%) were <1 year. Ten mitral valve replacements were performed in 8 patients, including 1 pulmonary valve homograft, 3 Contegra conduits of 12 mm thickness in the intra-annular position and 6 mechanical valves. Shone's syndrome, dysplastic valve, a need for valve replacement and age <1 year were the risk factors for death or reoperation in a univariable analysis, while in a multivariable analysis of all patients, valve replacement and age <1 year remained as risk factors. In a multivariable analysis of 27 patients aged <1 year, mitral valve dysplasia was a significant risk factor for reoperation or death. The 5-year rate of freedom from death or reoperation in neonates or infants was 55% and that in patients aged >1 year was 88% (P = 0.003).

CONCLUSIONS

An age of <1 year, mitral valve dysplasia and a need for mitral valve replacement were associated with a higher incidence of death or reoperation. Primary mitral valve replacement or univentricular strategy may have to be considered for symptomatic neonates with Shone's syndrome.