The importance of hybrid stage I palliation for neonates with critical aortic stenosis and reduced left ventricular function.

Abstract

The optimal management strategy for neonates with congenital aortic stenosis, two balanced ventricles, and duct-dependent systemic circulation (critical aortic stenosis) is still controversial. Thirteen patients with critical aortic stenosis underwent balloon aortic valvotomy (BAV) between 1996 and 2013, at the median age of 1 day old (range 0-28). Since 2010, bilateral pulmonary artery banding with ductal stenting following BAV was conducted for patients with reduced left ventricular (LV) function as a hybrid stage I palliation for the bridge to decision for further treatment. A follow-up was completed on all patients and the median follow-up period was 3.3 years (max 16.0). The overall survival rate at 15 years was 67.1 %. Six of the seven patients with maintained LV function could go on to the definitive Ross or Konno-aortic valve replacement at the median duration of 311 days after initial BAV, without any mortality. Three of four patients with reduced LV function died before 2010 with conventional treatment. With use of a hybrid stage I palliation, one of two patients ultimately underwent Fontan completion at 38 months of age and the other successfully underwent the definitive Ross-Konno operation at 9 months of age after recovery of the LV function. Although a statistically significant improvement has not been observed yet, the application of hybrid stage I palliation following BAV would be a favorable alternative for patients with reduced LV function to avoid a high-risk neonatal Ross or Norwood-type operation, and also to determine further treatment carefully.