Multiple left-sided stenotic lesions: Outcomes after mitral valve surgery. Arguments for abandoning the eponym ‘Shone syndrome’

Abstract

OBJECTIVES: Management of patients with left ventricular inflow and outflow stenotic lesions can be challenging. Our purpose was to characterize such patients and review the long-term outcomes of those requiring mitral valve (MV) surgery. METHODS: We performed a retrospective study of 40 patients with subaortic, aortic and/or arch stenotic lesion(s) who underwent MV surgery between 1985 and 2016. RESULTS: Associated left-sided stenotic lesions included aortic valve stenosis in 20 patients (50%), subaortic stenosis in 19 (47.5%) patients, coarctation in 23 (57.5%) patients and hypoplastic aortic arch in 16 (40%) patients. Nineteen patients (47.5%) had a supravalvular mitral ring and 15 (37.5%) patients had a parachute MV. The overall mortality rate was 32.5% (13 patients) with a mean follow-up of 16.3 ± 1.8 years. Being <6 months of age at the time of MV surgery (P = 0.02) and having had previous neonatal aortic valve and/or arch surgery (P = 0.01) were associated with death. The incidence of reoperation (95% confidence interval) at 1, 5, 10 and 15 years was 38% (23–53%), 54% (38–70%), 68% (53–84%) and 85% (72–98%), respectively. CONCLUSIONS: Results after MV surgery for children with associated left-sided stenotic lesions are closely age-related. The need for mitral intervention shortly after the initial aortic valve and/or arch intervention was a predictor of dismal outcomes