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

Abstract

Hypoplastic left heart syndrome with intact inter-atrial septum continues to be a challenge with high mortality, despite representing only 6% of those with hypoplastic left heart syndrome. No standard management exists for these patients, and centers vary in their approach depending on expertise and available resources. Interventions range from fetal transcatheter balloon atrial septostomy with or without stent placement-to-postnatal transcatheter, surgical, and/or hybrid strategies. In the current report, we present the first successful Ex utero, Intrapartum Treatment (EXIT)-to-open atrial septectomy-to-rapid stage I Norwood palliation in a neonate with hypoplastic left heart syndrome and intact inter-atrial septum. Creation of unrestrictive atrial communication (open atrial septectomy) was performed at 38 weeks gestation with partial delivery of the fetus who was kept under placental support till the septectomy was completed and without the use of cardiopulmonary bypass. 24-h later, stage I Norwood palliation was completed successfully. In conclusion, Open atrial septectomy under placental support (EXIT) is a logistic approach for hypoplastic left heart syndrome with intact atrial septum. It provides a controlled environment for left atrial decompression and can be followed swiftly with stage I palliation. This rapid sequence first stage palliation may have the potential for producing better results in this challenging subgroup of patients.