Aortic valve repair in neonates with aortic stenosis and reduced left ventricular function.

This study assessed outcomes of neonatal aortic valve (AoV) repair in presumed high-risk patients with depressed left ventricular (LV) function. Retrospective analysis of all neonates who underwent isolated AoV repair for severe aortic stenosis (AS). Patients with moderate or severe LV dysfunction were compared to those with normal or mild LV dysfunction. From 1980-2021, 43 neonates underwent isolated AoV repair for AS. Of these, 16 patients (37.2%) had ≥moderate LV dysfunction. Mean LV ejection fraction (EF) was 32.8±9.1%. Valve morphology was mostly unicuspid (68.75%, 11/16). Median age at surgery was 6.5 days (IQR 1-17.5). An optimal repair result with ≤mild AS or aortic regurgitation was achieved in 75% (12/16). There was no early death. One patient (6.25%) required postoperative extracorporeal membrane oxygenation (ECMO) support for 3 days. LVEF improved after surgery to 56.4±12.6% before discharge (P<0.0001) and normalized in 87.5% (14/16) with a median time of 6.4 days (IQR 3.4-39). Freedom from AoV reoperation was 45.1% (95%CI 17.9-69.3%) and 27.1% (95%CI 6.8-53%) at 5 and 10 years, respectively. Freedom from AoV replacement was 59% (95%CI 27-80.8%) and 31.4% (95%CI 6-62.2%) at 5 and 10 years, respectively. While survival was similar, freedom from AoV reoperation and replacement tended to be lower compared to neonates with preserved LVEF. AoV repair was associated with a low incidence of postoperative ECMO and mortality. LV function normalized after relief of obstruction in most patients before discharge. Late reoperation remained common for those with severely dysplastic valves.