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

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. 1 Ohye RG Sleeper LA Mahony L et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010; 362: 1980-1992 Crossref PubMed Scopus (708) Google Scholar , 2 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. Circulation. 2000; 102: III136-III141 Crossref PubMed Google Scholar , 3 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. Circulation. 1995; 92: II262-II266 Crossref PubMed Google Scholar , 4 Stasik CN Gelehrter S Goldberg CS et al. Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg. 2006; 131: 412-417 Abstract Full Text Full Text PDF PubMed Scopus (211) Google Scholar , 5 Hirsch JC Copeland G Donohue JE et al. Population-based analysis of survival for hypoplastic left heart syndrome. J Pediatr. 2011; 159: 57-63 Abstract Full Text Full Text PDF PubMed Scopus (36) Google Scholar , 6 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. J Thorac Cardiovasc Surg. 2012; 144: 152-159 Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar , 7 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. Front Pediatr. 2021; 9635776 Crossref Scopus (11) Google Scholar , 8 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. Semin Thorac Cardiovasc Surg. 2022; 34: 1300-1310 Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar , 9 Karamlou T Diggs BS Ungerleider RM et al. Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period. J Thorac Cardiovasc Surg. 2010; 139: 119-126 Abstract Full Text Full Text PDF PubMed Scopus (53) Google Scholar , 10 Azakie T Merklinger SL McCrindle BW et al. Evolving strategies and improving outcomes of the modified Norwood procedure: A 10-year single-institution experience. Ann Thorac Surg. 2001; 72: 1349-1353 Abstract Full Text Full Text PDF PubMed Scopus (207) Google Scholar , 11 Newburger JW Sleeper LA Gaynor JW et al. Transplant-free survival and interventions at 6 Years in the SVR trial. Circulation. 2018; 137: 2246-2253 Crossref PubMed Scopus (137) Google Scholar At present, there is limited evidence-based society guidance on the perioperative management of neonates with HLHS undergoing stage 1 palliation. 12 Donofrio MT Moon-Grady AJ Hornberger LK et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014; 129: 2183-2242 Crossref PubMed Scopus (721) Google Scholar Extracorporeal Membrane Oxygenation for Stage 1 Palliation: Early Bridge to Recovery Rather Than a Late Act of Pure DespairJournal of Cardiothoracic and Vascular AnesthesiaVol. 37Issue 8PreviewWe appreciate the article by Zaleski et al., as it described in detail the challenging management of a patient with hypoplastic left heart syndrome undergoing stage I palliation (S1P), one of the most demanding tasks in cardiac surgery and anesthesiology for congenital heart disease.1 The course of the young patient presented in their article was uneventful. Nonetheless, internationally reported premature mortality is high at up to 14.3%, and remains at 8%-to-11% even in high-volume North American centers, even after establishing specialized "single-ventricle” teams. Full-Text PDF