Staged Treatment for Critically Ill Neonates With Ebstein Anomaly: Cone Repair After Starnes Procedure

Abstract

Following the Starnes procedure, a progressive reduction in the RV cavity occurs, suggesting that the RV was unsuitable for a biventricular repair. For that reason, those patients traditionally followed the single ventricle pathway. In 2019, we performed the initial tricuspid valve (TV) repair cases after Starnes. Since then, we have routinely applied the staged treatment with Cone repair after Starnes achieving 2-ventricle or one-and-a-half ventricle repair in 17 patients. A Glenn procedure associated with pulmonary valve (PV) commissurotomy has been offered as an intermediate stage in patients with associated pulmonary atresia (PAt). Aiming to facilitate the following surgical steps and allow RV growth, we have described some maneuvers for these patients during the Starnes and the Glenn procedure. In this paper, we describe the up-to-date staged repair for critical neonatal Ebstein, emphasizing surgical details of the Starnes procedure, the intermediate interventions, and the Cone repair after Starnes.

Keywords: Ebstein anomaly; pulmonary atresia; tricuspid insufficiency.