Achieving an Optimal Outcome After Stage 1 Palliation for Hypoplastic Left Heart Syndrome and Variants: Frequency, Associated Factors, and Subsequent Outcomes

Abstract

Background We sought to measure frequency of achieving an optimal outcome after stage 1 palliation (S1P) for hypoplastic left heart syndrome and variants, determine factors associated with optimal outcomes, and compare outcomes after stage 2 palliation (S2P) using the National Pediatric Cardiology Quality Improvement Collaborative database (2008–2016). Methods and Results This is a retrospective cohort study with optimal outcome defined a priori as meeting all of the following: (1) discharge after S1P in <19 days (top quartile), (2) no red flag or major event readmissions before S2P, and (3) performing S2P between 90 and 240 days of age. Optimal outcome was achieved in 256 of 2182 patients (11.7%). Frequency varied among centers from 0% to 25%. Factors independently associated with an optimal outcome after S1P were higher gestational age (odds ratio [OR], 1.1 per week [95% CI, 1.0–1.2]; P=0.02); absence of a genetic syndrome (OR, 2.5 [95% CI, 1.2–5]; P=0.02); not requiring a post‐S1P catheterization (OR, 2.7 [95% CI, 1.5–4.8]; P=0.01), intervention (OR, 1.5 [95% CI, 1.1–2]; P=0.006), or a procedure (OR, 4.5 [95% CI, 2.8–7.1]; P<0.001) before discharge; and not having a post‐S1P complication (OR, 2.7 [95% CI, 1.9–3.7]; P<0.001). Those with an optimal outcome after S1P had improved S2P outcomes including shorter length of stay, less ventilator days, shorter bypass time, and fewer postoperative complications. Conclusions Identifying patients at lowest risk for poor outcomes during the home interstage period could shift necessary resources to those at higher risk, alter S2P postoperative expectations, and improve quality of life for families at lower risk.

Keywords

• hypoplastic left heart syndrome
• interstage
• Norwood procedure