A Comprehensive Approach To The Management Of Patients With Hypoplastic Left Heart Syndrome (HLHS) And HLHS-Related Malformations: An Analysis Of 96 Patients (2015-2023) With 97.3% Norwood Survival
Mark Bleiweis, MD, Giles Peek, MD, Jeffrey P. Jacobs, MD.
University of Florida, Gainesville, FL, USA.
Objective(s): We report our comprehensive approach to the management of 96 patients with HLHS and HLHS-related malformations with ductal-dependent systemic circulation.
Methods: We reviewed our entire current single center experience with 96 neonates and infants with HLHS and HLHS-related malformations (2015-2023). 76% of patients (73/96) were standard risk and underwent initial Norwood (Stage 1) Operation. 10.4% of patients (10/96) were high-risk patients with risk factors other than major cardiac risk factors (such as severe necrotizing enterocolitis or Turner Syndrome=[45,XO]) and underwent initial Hybrid Stage 1 palliation. 13.5% of patients (13/96) were high-risk patients with major cardiac risk factors (coronary fistulas with ventricular-dependent coronary circulation or severe atrioventricular valvar regurgitation). Ten were bridged-to-transplantation with initial combined Hybrid Stage 1 palliation and VAD insertion (HYBRID+VAD). (Three were bridged-to-transplantation with prostaglandin.)
Results: Overall 1-year mortality=8/96=8.3% (Table). Operative Mortality for Norwood Operation=2/73=2.7%. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD=0/10. Of 10 HYBRID+VAD patients, 7/10=70% underwent successful cardiac transplantation and are alive today and 3/10=30% died on VAD awaiting transplantation. Median length of VAD support=136 days (mean=135.8, range=56-226).
Conclusions: A comprehensive approach to the management of patients with HLHS is associated with Operative Mortality after Norwood of 2/73=2.7% and a one-year mortality of 8/96=8.3%. A subset of 10/96=10.4% were stabilized with HYBRID+VAD while awaiting transplantation. 
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