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How We Would Treat Our Own Hypoplastic Left Heart Syndrome Neonate for Stage 1 Surgery

Published:January 08, 2023DOI:https://doi.org/10.1053/j.jvca.2023.01.003
      ALTHOUGH NO LONGER UNIFORMLY fatal, hypoplastic left heart syndrome (HLHS) continues to significantly challenge providers across pediatric specialties. This is especially true in the neonatal period when patients with HLHS undergo stage 1 palliative surgery. Despite advances in medical and surgical management, in-hospital and 1-year mortality remain high.
      • Ohye RG
      • Sleeper LA
      • Mahony L
      • et al.
      Comparison of shunt types in the Norwood procedure for single-ventricle lesions.
      • Mahle WT
      • Spray TL
      • Wernovsky G
      • et al.
      Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution.
      • Forbess JM
      • Cook N
      • Roth SJ
      • et al.
      Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality.
      • Stasik CN
      • Gelehrter S
      • Goldberg CS
      • et al.
      Current outcomes and risk factors for the Norwood procedure.
      • Hirsch JC
      • Copeland G
      • Donohue JE
      • et al.
      Population-based analysis of survival for hypoplastic left heart syndrome.
      • Tweddell JS
      • Sleeper LA
      • Ohye RG
      • et al.
      Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: Risk factors and their interaction with shunt type.
      • Best KE
      • Miller N
      • Draper E
      • et al.
      The improved prognosis of hypoplastic left heart: A population-based register study of 343 cases in England and Wales.
      • Piber N
      • Ono M
      • Palm J
      • et al.
      Influence of shunt type on survival and right heart function after the Norwood procedure for aortic atresia.
      • Karamlou T
      • Diggs BS
      • Ungerleider RM
      • et al.
      Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period.
      • Azakie T
      • Merklinger SL
      • McCrindle BW
      • et al.
      Evolving strategies and improving outcomes of the modified Norwood procedure: A 10-year single-institution experience.
      • Newburger JW
      • Sleeper LA
      • Gaynor JW
      • et al.
      Transplant-free survival and interventions at 6 Years in the SVR trial.
      At present, there is limited evidence-based society guidance on the perioperative management of neonates with HLHS undergoing stage 1 palliation.
      • Donofrio MT
      • Moon-Grady AJ
      • Hornberger LK
      • et al.
      Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association.
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