Open Atrial Septectomy on Placental Support: A Novel and Logistic Approach for Hypoplastic Left Heart Syndrome With Intact Atrial Septum

In this article, the authors describe a novel strategy for neonates with hypoplastic left heart syndrome (HLHS) and intact atrial septum (IAS). Due to the emergency need for creating unrestrictive atrial communication in these patients, traditional interventions include fetal balloon atrial septostomy or postnatal transcatheter, surgical, or hybrid interventions. However, none of these interventions have been associated with excellent results for HLHS/IAS, resulting in dismal outcomes for these patients.  

 The authors present a case of a neonate with a fetal diagnosis of HLHS/IAS, in which they planned an elective cesarean section at 38 weeks. During the procedure, they performed an open atrial septectomy using a bicaval inflow occlusion technique while the baby was mid-delivery, as a part of the ex utero intrapartum treatment (EXIT) procedure. The procedure was uneventful, and it was followed the next day with Stage I Norwood/Sano palliation. The patient did extremely well, was discharged two weeks later, and continues to do well during his follow-up visits. This approach is both novel and logistic for this challenging group of patients, and the authors hope it will open the door for similar patients in the future.