Hypoplastic Left Heart Syndrome With Intact or Restrictive Atrial Septum

Abstract

Infants with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum (HLHS-IAS) represent a high-risk subset. Fetal cardiac intervention (FCI) for fetuses with HLHS-IAS has been reported in single-institution series as a therapy that may improve outcomes. This study uses the International Fetal Cardiac Intervention Registry (IFCIR) to examine fetal and maternal characteristics and pregnancy and neonatal outcome data for FCI in this population. For this descriptive analysis, the IFCIR was queried for fetuses with HLHS-IAS evaluated between 2001 and March 2015 for possible FCI and felt to be candidates at the referral center by a variety of published criteria including foramen ovale (FO) size, shunt flow direction across the FO, and pulmonary venous Doppler flow patterns. It includes those who were determined not to be candidates because of other fetal noncardiac conditions, maternal conditions, or maternal preferences, or because FCI was not performed at the participating institution. Further details on the methodology of the IFCIR registry are found in the initial report.1 Participating IFCIR member sites obtained local institutional review board and ethics board approval or a waiver, as governed …