Practice Trends and Outcomes of Patients With Turner's Syndrome and Hypoplastic Left Heart Syndrome Across the United States
Darren Turner, Nina Price, Amir Mehdizadeh-Shrifi, Haleh Heydarian, Yahia Hejazi, Awais Ashfaq, David Lehenbauer, Marco Ricci, David L. S. MoralesObjective(s)
Patients with Turner syndrome (TS) and hypoplastic left heart syndrome (HLHS) undergoing single-ventricle palliation represent a challenging cohort. We examined birth admission outcomes in TS/HLHS patients, comparing Norwood and hybrid palliation strategies.
Methods
The pediatric health information system (PHIS) database was queried for neonates with a diagnosis of TS and HLHS. Cohort was divided into Norwood procedure, hybrid, or nonoperative, and outcomes were divided into three time eras (Era 1: 2004-2011, Era 2: 2012-2018, Era 3: 2019-2025).
Results
A total of 157 infants were identified: 27/157 (17%) received no operative intervention, 24/157 (15%) underwent hybrid palliation, and 106/157 (68%) underwent the Norwood procedure only. Overall Stage-1 palliation (S1P) hospital survival was 75% (98/130) for both operative cohorts. Hybrid palliations trended upward over time, while Norwood volumes remained stable. In-hospital survival improved across eras for hybrid palliation (75% vs 87%) and the Norwood procedure (71% vs 89%), although neither reached statistical significance. Of 45 PHIS hospitals performing the Norwood, 38 performed S1P for TS patients; 60% (23/38)used the Norwood exclusively, 8% (3/38) used hybrid exclusively, and 32% (12/38) employed both approaches.
Conclusions
Outcomes appear satisfactory with either the hybrid or Norwood strategy, with current survival rates ≥87%, although hybrid palliation was associated with a higher rate of subsequent transplantation. However, 17% of (27/157) TS/HLHS neonates were not offered surgical intervention, presumably because they were deemed to be at higher risk. Given demonstrably improving outcomes among selected TS patients undergoing either procedure, offering S1P more broadly, including to higher-risk TS/HLHS patients, may be worthwhile.