Multiple left-sided stenotic lesions: outcomes after mitral valve surgery. Arguments for abandoning the eponym 'Shone syndrome'.

Abstract

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. 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. 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. 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.