Hybrid Versus Percutaneous Left Atrial Decompression in Infants With Hypoplastic Left Heart Variants and an Intact or Highly Restrictive Atrial Septum: A Multicenter PICES Study

BACKGROUND: Neonates with hypoplastic left heart syndrome variants with an intact or highly restrictive atrial septum (HLH-IAS) require immediate postnatal intervention to survive. Emergent left atrial decompression (LAD) via a percutaneous or hybrid approach is standard, but the comparative effectiveness and outcomes of these approaches remain underexplored. METHODS: A multicenter retrospective analysis of all neonates with HLH-IAS who underwent LAD in the first 36 hours of life from January 2009 to March 2020 at 14 North American congenital cardiac programs was conducted. Patient and procedural characteristics and patient outcomes were compared between hybrid and percutaneous LAD. RESULTS: Among 128 patients with HLH-IAS, 105 (80%) underwent percutaneous LAD and 23 (17%) hybrid LAD. No significant differences were observed in preprocedural characteristics such as birth weight (3.1 +/- 0.6 versus 3.2 +/- 0.6 kg; P=0.453), presence of a baseline interatrial communication (66% versus 61%; P=0.659), interatrial communication gradient (14.4 +/- 6.9 versus 12.7 +/- 4.6 mm Hg; P=0.456), or lowest pH (7.13 +/- 0.14 versus 7.09 +/- 0.15; P=0.193). Hybrid LAD resulted in a shorter time from birth to procedure (120 versus 52 minutes; P=0.005) and to atrial septal defect creation (55 versus 29 minutes; P=0.002). All hybrid LAD were technically successful, although 11 (10%) percutaneous LAD were unsuccessful with 7 requiring conversion to surgical septectomy. There was a trend toward fewer serious adverse events with hybrid LAD (36% versus 17%; P=0.141). Most patients (67%) who underwent hybrid LAD had simultaneous pulmonary artery band placement and 21% of patients with percutaneous LAD required reoperation for pulmonary artery band. Long-term survival rates were comparably low between groups, with a 30% transplant-free survival at a median 8-month follow-up. CONCLUSIONS: In patients with HLH-IAS, hybrid LAD allows for faster atrial septal defect creation, has a higher technical success rate, and potentially fewer procedural serious adverse events compared with percutaneous LAD, alongside facilitating simultaneous pulmonary artery band. Our findings underscore the poor prognosis for patients with HLH-IAS despite intervention, highlighting the need for better treatment strategies.