Abstract 13582: Fetal Cardiac Intervention for Hypoplastic Left Heart Syndrome With Intact or Restrictive Atrial Septum: A Report From the International Fetal Cardiac Intervention Registry

Abstract

Background: Fetal cardiac intervention (FCI) for fetuses with hypoplastic left heart syndrome with restrictive or intact atrial septum (HLHS/IAS) has been reported in single-institution series as a modality that may improve outcomes in a high-risk subset of HLHS patients who historically have very high neonatal mortality. Methods: The International Fetal Cardiac Intervention Registry was established in 2010. For this descriptive analysis, the database was queried for fetuses with HLHS/IAS. Maternal-fetal dyads from 2001 through March 2015 were included. Results: Data from 15 institutions were submitted by data harvest. Of 87 cases of HLHS/IAS entered, 46 cases from 8 sites underwent FCI: 27 with atrial septal perforation/ balloon dilation and 19 with atrial septal stent placement. There were 34 procedural successes with a similar success rate between stents and balloons dilations (12/19=63% stent, 22/27=81% balloon dilation, p=0.19). Fetal complications were common (64% of cases), most frequently a pericardial effusion requiring treatment (51%), but no maternal complications. Procedure-related fetal demise occurred in 6 (13%). Seventeen had a clinical success (atrial septum not requiring urgent intervention at delivery), which was more likely in fetuses with a successful stent (75% stent vs 36% balloon dilation, p=0.03). Delivery by scheduled Cesarean section to facilitate immediate post-natal intervention was less common in those who underwent successful FCI compared with those with unsuccessful FCI or no FCI (3/34=8% FCI vs 18/46=39%, p=0.002). There was a trend toward improved survival to discharge for liveborn infants with HLHS/IAS in those who underwent successful FCI compared with those with unsuccessful FCI or no FCI (16/34=47% FCI vs 13/46=28%, p=0.10). Survival to hospital discharge was greater in those with clinical success compared to a procedural success but no clinical success (71% vs 24%, p=0.01). Conclusion: The early multicenter experience of FCI for HLHS/IAS has good procedural success, with atrial septal stent placement more likely to result in clinical success at delivery. There was a trend toward better survival if FCI was successful, and significantly improved survival if FCI resulted in clinical success at delivery.