Abstract

Background: Infants w/ high risk hypoplastic left heart syndrome and variants (HR-HLHS) due to low birth weight or gestational age experience worse outcomes. Optimal management remains unknown. We sought to 1. compare primary Norwood to hybrid strategies in HR-HLHS 2. identify predictors of futility. Hypothesis: Primary Norwood offers improved outcomes compared to hybrid strategies. Very low birth weight and other anomalies may represent futility. Methods: We reviewed HR-HLHS from the National Pediatric Cardiology Quality Improvement Collaborative database. Patients with birth weight <2.5kg or gestation <35 weeks, and age ≤ 30 days at admission were included (N=398). Norwood (n=225), hybrid (pulmonary artery band (PAB) + ductal stent, n=76), and PAB w/ prostaglandin (PAB/PGE, n=77) were compared. Transplantation referral (n=1) and comfort care (n=19) occurred. Baseline factors, 1-year survival, and stage 2 completion were reviewed. Results: Table shows baseline HR-HLHS features. Norwood had higher risk adjusted 1-year survival and stage 2 completion than hybrid strategies, including in < 2.1 kg (Figure). On multivariable analysis, hybrid (HR 2.8), genetic abnormality (HR 1.5), and ECMO (HR: 7.2) were significantly associated w/ decreased 1-year survival and stage 2 completion, while higher birth weight (HR: 1.5) was associated w/ increased stage 2 completion. Less than 25% of HR-HLHS w/ birthweight < 2.1 kg and ≥ 1 genetic abnormality was alive with stage 2 at the end of follow-up. Conclusions: HR-HLHS have better outcomes following primary Norwood than hybrid palliation. In patients w/ birth weight < 2.1 kg and genetic abnormalities, hybrid or Norwood may be futile.

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