Hybrid vs. Norwood: An Analysis of the NPC-QIC Database to Determine Optimal Approach for High-Risk Single Ventricle...

We sought to identify the optimal management strategy of infants with high-risk hypoplastic left heart syndrome and variants (HR-SV) and identify factors associated with futility. Patients with birth weight ≤ 2.5 kg 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 with prostaglandin (PAB/PGE, n = 77) were compared from the National Pediatric Cardiology Quality Improvement Collaborative database. Transplantation referral (n = 1) and comfort care (n = 19) occurred. Baseline factors, 1-year survival, and stage 2 completion were reviewed. Norwood had higher gestational age, birth weight, and younger age at intervention than hybrid and PAB/PGE; while more PAB/PGE and hybrid had chromosomal and other organ abnormalities. Norwood had higher survival (70% vs. 54% hybrid, 55% PAB/PGE, p = 0.03) and stage 2 completion (90% vs. 61% hybrid, 67% PAB/PGE, p < 0.001) than hybrid. In birthweight ≤ 2.12 kg, the survival advantage in Norwood was eliminated (59% for Norwood vs. 56% for hybrid and PAB/PGE, p = 0.61). Hybrid (HR 2.8, p < 0.001), genetic abnormality (HR 1.5, p = 0.03), and post stage 1 ECMO (HR: 7.2, p < 0.001) were associated with decreased survival and stage 2 completion, while higher birth weight (HR: 1.5, p < 0.001) was associated with increased stage 2 completion. Less than 25% of HR-SV with birthweight ≤ 2.12 kg and ≥ 1 genetic abnormality was alive with stage 2 at end of follow-up. HR-SV have better outcomes following Norwood than hybrid. In patients with birth weight ≤ 2.1 kg and genetic abnormalities, both strategies offer low survival.