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

ObjectiveTo compare long-term survival, reinterventions and risk factors using strict definitions of neonatal critical and non-critical valvular aortic stenosis (VAS).DesignA nationwide retrospective study using data from patient files, echocardiograms and the Swedish National Population Registry.Setting and patientsAll neonates in Sweden treated for isolated VAS 1994–2018. We applied the following criteria for critical aortic stenosis: valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function (fractional shortening ≤27%). Indication for treatment of non-critical VAS was Doppler mean gradient >50 mm Hg.Main outcome measuresShort-term and long-term survival, aortic valve reinterventions need of valve replacements, risk factors for reintervention and event-free survival.ResultsWe identified 65 patients with critical VAS and 42 with non-critical VAS. The majority of the neonates were managed by surgical valvotomy. Median follow-up time was 13.5 years, with no patients lost to follow-up. There was no 30-day mortality. Long-term transplant-free survival was 91% in the critical stenosis group and 98% in the non-critical stenosis group (p=0.134). Event-free survival was 40% versus 67% (p=0.002) in the respective groups. Median time from the initial treatment to reintervention was 3.6 months versus 3.9 years, respectively (p=0.008).ConclusionsCritical VAS patients had significantly higher need for reintervention during the first year of life, lower event-free survival and lower freedom from aortic valve replacement at age ≥18 years, compared with neonates with non-critical stenosis.