Late outcome of survivors of intervention for neonatal aortic valve stenosis

Background. This study examined the late outcome after intervention for neonatal aortic valve stenosis. Methods. Seventy-three neonates (59 boys and 14 girls) underwent intervention for critical aortic valve stenosis during the first 30 days of life at two institutions, The Hospital for Sick Children, London, and Duke University Medical Center, Durham, North Carolina. Procedures performed include closed valvotomy (n = 12), open valvotomy with inflow occlusion (n = 14), open valvotomy with cardiopulmonary bypass (n = 33), balloon valvotomy (n = 12), and other procedures (n = 2). The mean age at the first intervention was 8 ± 1 days. Results. The hospital mortality was 52.1%. The mean duration of follow-up for the hospital survivors (n = 35) was 8.3 ± 1.1 years. The actuarial survival for the hospital survivors was 93.3% ± 4.7% at 10 years and 83.9% ± 9.8% at 15 years, whereas event-free survival (reintervention, endocarditis, or early death) was 61.8% ± 9.3% at 5 years, 34.2% ± 10.8% at 10 years, and 27.4% ± 10.6% at 15 years. Three patients have died and 11 patients have required aortic valve replacement during the follow-up period. The age at the initial intervention, the type of initial intervention, and the year of initial intervention were not predictive of early death or need for reintervention. At last follow-up, 26 of the long-term survivors (n = 32) were in functional class I and 6 were in functional class II. Conclusions. Aortic stenosis in the neonatal period is a difficult problem with a high initial mortality. Late survival and functional class are excellent for patients surviving the initial hospitalization, but most require further intervention within 10 years.