A national study of the outcome after treatment of critical aortic stenosis in the neonate

Abstract

Objective: This study describes short-term and long-term outcome after treatment of critical valvular aortic stenosis in neonates in a national cohort, with surgical valvotomy as first choice intervention. Methods: All neonates in Sweden treated for critical aortic stenosis between 1994 and 2016 were included. Patient files were analysed and cross-checked against the Swedish National Population Registry as of December 2017, giving complete survival data. Diagnosis was confirmed by reviewing echo studies. Critical aortic stenosis was defined as valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function. Primary outcome was all-cause mortality and secondary outcomes were reintervention and aortic valve replacement. Results: Sixty-one patients were identified (50 boys, 11 girls). Primary treatment was surgical valvotomy in 52 neonates and balloon valvotomy in 6. Median age at initial treatment was 5 days (0–26), and median follow-up time was 10.8 years (0.14–22.6). There was no 30-day mortality but four late deaths. Freedom from reintervention was 66%, 61%, 54%, 49%, and 46% at 1, 5, 10, 15, and 20 years, respectively. Median time to reintervention was 3.4 months (4 days to 17.3 years). Valve replacement was performed in 23 patients (38%). Conclusions: Surgical valvotomy is a safe and reliable treatment in these critically ill neonates, with no 30-day mortality and long-term survival of 93% in this national study. At 10 years of age, reintervention was performed in 54% and at end of follow-up 38% had had an aortic valve replacement.

Outcomes in neonatal critical and non-critical aortic stenosis: a retrospective cohort study

“…Patients were identified in surgical and catheter registries and data collected from patient files at the two centres, referring hospitals and the Swedish Registry of Congenital Heart Disease. The current cohort of neonates with critical aortic stenosis was in part included in a previous study 13. Survival was cross-checked against the Swedish Population Registry as of October 2019.…”

“…The current cohort of neonates with critical aortic stenosis was in part included in a previous study. 13 Survival was cross-checked against the Swedish Population Registry as of October 2019. Indications for treatment were: (a) critical VAS with ductdependent systemic circulation and/or depressed left ventricular function with fractional shortening (LVFS) ≤27% or (b) non-critical AS with Doppler mean gradient >50 mm Hg.…”

“…Longterm transplant-free survival was 91% in critical VAS patients and 98% in non-critical patients (p=0.134) (figure 2A). Median 13 In the non-critical AS group, a 3-year-old boy was found dead in bed; cause of death could not be determined. In addition, in the group of critical VAS two patients had heart transplants at the age of 1 and 15 years, and two patients were converted to univentricular palliation.…”

“…There is no generally accepted definition of critical VAS. In the literature, the definitions includes: age at intervention, <30 days2 3 or <3 months4–7; anatomic features such as duct dependency8 9; functional features such as depressed left ventricular function10; ongoing pharmacological treatment with prostaglandin or inotrope support11 12; or as a combination of criteria 1 13–15…”

“…Valvotomy, surgical or by catheter intervention, is the first-hand treatment in the neonatal period. Data for comparison of treatment methods derive from single-centre reports2–5 8 10–13 16 17 and a few multicentre studies 9 14 15 18. However, comparison of outcome after neonatal treatment of VAS is complicated by the varying definition of critical VAS.…”