Long-term Outcomes Of Children With Hypoplastic Left Heart Syndrome And Intact/restrictive Atrial Septum
Karthik Ramakrishnan, Michelle Santoso, Shyam Sathanandam, Umar Boston, Ronak Naik, Nithya Swminathan, Christopher Knott-Craig.
LeBonheur Children's Hospital, Memphis, TN, USA.
Objectives: To review the outcomes of neonates with hypoplastic left heart syndrome and intact/restrictive interatrial septum (HLHS/IAS). Methods: 17 consecutive neonates with HLHS/IAS between March 2013 and March 2022 were included. Results: The median gestational age of the cohort was 37 weeks (range 34 to 39 weeks). 9 patients had intact atrial septum and 7 had a severely restrictive interatrial communication. 4/16 (25%) had genetic abnormalities. The initial procedure was atrial septostomy with stent placement (n=8), atrial septostomy without stent (n=4), Norwood stage 1 (n=3), bilateral pulmonary artery banding alone (n=1), and bilateral pulmonary artery banding with ductal stent placement (n=1). One patient died after attempted balloon atrial septostomy. The second procedure was performed at a median age of 7 days. These included Norwood operation (n=8), and completion of hybrid palliation (n=5). Those who underwent non-Norwood palliation had high risk features like persistent shock (n= 6), intracranial bleed (n=2), severe lung disease (n=4), or multi-organ dysfunction (n=5). The estimated cumulative 10-year probability of survival was 64%. On univariable analysis, intact atrial septum (p<0.001), birth weight (p=0.03), pre-maturity (p=0.04) and non-Norwood palliation(p=0.03) were the significant predictors of mortality. Conclusions: Non-modifiable risk factors like presence of intact atrial septum, pre-maturity and low birth weight contribute to high mortality in neonates with HLHS/IAS. Neonates who undergo an early Norwood operation have a higher probability of surviving to Fontan. 
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