Current Anesthesiology Reports

, Volume 9, Issue 2, pp 116–122 | Cite as

Stage 1 Palliation of Hypoplastic Left Heart Syndrome: What the Pediatric Anesthesiologist Needs to Know

  • Dawn M. SweeneyEmail author
  • Jane Arcadi
Pediatric Anesthesia (J Lerman, Section Editor)
Part of the following topical collections:
  1. Pediatric Anesthesia


Purpose of Review

The purpose of this review is to discuss the features of stage 1 palliation of hypoplastic left heart syndrome (HLHS). Comparison will be made between traditional surgical approaches and hybrid approaches. Anesthetic considerations for infants with these types of palliations will be discussed.

Recent Findings

While historically surgical palliation in the neonatal period or neonatal heart transplant has been the primary means of survival for neonates with HLHS, in the last two decades, more centers have been performing hybrid palliation of this defect. This palliation can be performed with the expectation that a comprehensive second-stage palliation will be done in the next several months, or it can be used as a bridge to transplant. Other uses are also being explored as research continues.


Advances in the care of neonates with HLHS have led to increased survival. As surgical technique and non-surgical care of these infants continue to improve, the pediatric anesthesiologist will need to be familiar with the anatomic and physiologic implications of the various types of palliation.


Hypoplastic left heart syndrome Anesthesia Norwood procedure Sano procedure Hybrid palliation Norwood procedure Sano modification 


Compliance with Ethical Standards

Conflict of Interest

Dawn M. Sweeney and Jane Arcadi declare they have no conflict of interest.

Human and Animal Rights and Informed Consent

Neither Dr. Arcadi nor Dr. Sweeney performed any animal or human subject studies in this article.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Norwood WI, Kirklin JK, Sanders SP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol. 1980;45(1):87–91.CrossRefGoogle Scholar
  2. 2.
    Bailey LL, Nehlsen-Cannarella SL, Concepcion W, Jolley WB. Baboon-to-human cardiac xenotransplantation in a neonate. JAMA. 1985;254(23):3321–9.CrossRefGoogle Scholar
  3. 3.
    Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. 1983;308(1):23–6.CrossRefGoogle Scholar
  4. 4.
    Sano S, Ishino K, Kawada M, Arai S, Kasahara S, Asai T, et al. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126(2):504–9.CrossRefGoogle Scholar
  5. 5.
    Gibbs JL, Wren C, Watterson KG, Hunter S, Hamilton JR. Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome. Br Heart J. 1993;69(6):551–5.CrossRefGoogle Scholar
  6. 6.
    •• Haller C, Caldarone CA. The evolution of therapeutic strategies: niche apportionment for hybrid palliation. Ann Thorac Surg. 2018;106(6):1873–80. Comprehensive yet very readable article outlining the potential, uses, and variations of the hybrid procedure. CrossRefGoogle Scholar
  7. 7.
    Baba K, Honjo O, Chaturvedi R, Lee KJ, Van Arsdell G, Caldarone CA, et al. “Reverse Blalock-Taussig shunt”: application in single ventricle hybrid palliation. J Thorac Cardiovasc Surg. 2013;146(2):352–7. Epub 2012 Dec 8.CrossRefGoogle Scholar
  8. 8.
    Caldarone CA, Benson LN, Holtby H, Van Arsdell GS. Main pulmonary artery to innominate artery shunt during hybrid palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2005;130(4):e1–2.CrossRefGoogle Scholar
  9. 9.
    Briceno-Medina M, Kumar TKS, Sathanandam S, Boston U, Perez M, Allen J, et al. Femoral vein homograft as Sano shunt results in improved pulmonary artery growth after Norwood operation. Cardiol Young. 2018;28(1):118–25. Scholar
  10. 10.
    Nwankwo UT, Morell EM, Trucco SM, Morell VO, Kreutzer J. Hybrid strategy for neonates with ductal-dependent systemic circulation at high risk for Norwood. Ann Thorac Surg. 2018;106(2):595–601. Scholar
  11. 11.
    Brescia AA, Jureidini S, Danon S, Armbrecht E, Fiore AC, Huddleston CB. Hybrid versus Norwood procedure for hypoplastic left heart syndrome: contemporary series from a single center. J Thorac Cardiovasc Surg. 2014;147(6):1777–82. Scholar
  12. 12.
    •• Lloyd DF, Rutherford MA, Simpson JM, Razavi R. The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus. Cardiol Young. 2017;27(2):217–23. This is the only article I have seen looking at fetal neurodevelopment in hypoplastic left heart syndrome. There is a good discussion of abnormalities of cerebral blood flow in fetuses with HLHS. CrossRefGoogle Scholar
  13. 13.
    • Diaz LK, Gaynor JW, Koh SJ, Ittenbach RF, Gerdes M, Bernbaum JC, et al. Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2016;152(2):482–9. This is the only paper that looks at cumulative exposure to volatile anesthetics in HLHS specifically. While there are no definitive answers on the subject, anesthesiologists need to remain vigilant in the care of these infants who may be at risk for poorer neurodevelopmental outcomes. CrossRefGoogle Scholar
  14. 14.
    • Yerebakan C, Valeske K, Elmontaser H, Yörüker U, Mueller M, Thul J, Mann V, Latus H, Villanueva A, Hofmann K, Schranz D, Akintuerk H. Hybrid therapy for hypoplastic left heart syndrome: myth, alternative, or standard? J Thorac Cardiovasc Surg. 2016;151(4):1112–21, 1123.e1-5. This article discusses in detail the re-interventions commonly needed after hybrid palliation. The discussion section provides further insight into this issue.
  15. 15.
    Mosca RS. Hybrid therapy for hypoplastic left heart syndrome: myth, alternative, or standard—neither minotaur nor Midas. J Thorac Cardiovasc Surg. 2016;151(4):1123–5. Epub 2015Oct 30.CrossRefGoogle Scholar
  16. 16.
    •• Ohye RG, Schranz D, D'Udekem Y. Current therapy for hypoplastic left heart syndrome and related single ventricle lesions. Circulation. 2016;134(17):1265–79 Excellent review of therapy for HLHS, including all 3 stages of palliation. CrossRefGoogle Scholar
  17. 17.
    Watkins SC, McNew BS, Donahue BS. Risks of noncardiac operations and other procedures in children with complex congenital heart disease. Ann Thorac Surg. 2013;95:2014–1.CrossRefGoogle Scholar
  18. 18.
    Mittnacht AJC. Near infrared spectroscopy in children at high risk of low perfusion. Curr Opin Anaesthesiol. 2010;23:342–7.CrossRefGoogle Scholar
  19. 19.
    Galantowicz M, Cheatham JP, Phillips A, Cua CL, Hoffman TM, Hill SL, et al. Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg. 2008;85(6):2063–70. Scholar
  20. 20.
    Cheatham SL, Carey H, Chisolm JL, Heathcock JC, Steward D. Early results of neurodevelopment following hybrid stage I for hypoplastic left heart syndrome. Pediatr Cardiol. 2015;36(3):685–91. Scholar
  21. 21.
    Knirsch W, Liamlahi R, Hug MI, Hoop R, von Rhein M, Prêtre R, et al. Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures. Eur J Cardiothorac Surg. 2012;42(1):33–9. Scholar
  22. 22.
    Malik S, Bird TM, Jaquiss RD, Morrow WR, Robbins JM. Comparison of in-hospital and longer-term outcomes of hybrid and Norwood stage 1 palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2015;150(3):474–80.e2. Scholar
  23. 23.
    •• Cao JY, Lee SY, Phan K, Ayer J, Celermajer DS, Winlaw DS. Early outcomes of hypoplastic left heart syndrome infants: meta-analysis of studies comparing the hybrid and Norwood procedures. World J Pediatr Congenit Heart Surg. 2018;9(2):224–33. This is the only meta-analysis specifically comparing the Norwood procedure with the hybrid procedure. CrossRefGoogle Scholar
  24. 24.
    Murphy MO, Bellsham-Revell H, Morgan GJ, Krasemann T, Rosenthal E, Qureshi SA, et al. Hybrid procedure for neonates with hypoplastic left heart syndrome at high- risk for Norwood: midterm outcomes. Ann Thorac Surg. 2015;100(6):2286–90; Discussion 2291-2. Scholar
  25. 25.
    •• TJ W, McCrindle BW, Hickey EJ, Ziemer G, Tchervenkov CI, Jacobs ML, et al. Is a hybrid strategy a lower-risk alternative to stage 1 Norwood operation? J Thorac Cardiovasc Surg. 2017;153(1):163–172.e6. This article compares both conventional Norwood procedures and those with the RV to PA conduit modification to the hybrid procedure. Most papers do not compare all 3 types of palliation. CrossRefGoogle Scholar
  26. 26.
    • Cleveland D, Adam Banks C, Hara H, Carlo WF, Mauchley DC, DKC C. The case for cardiac xenotransplantation in neonates: is now the time to reconsider xenotransplantation for hypoplastic left heart syndrome? Pediatr Cardiol. 2019;40(2):437–44. Interesting article about a possible future of cardiac transplantation in neonates that discusses genetic engineering and xenotransplantation. CrossRefGoogle Scholar
  27. 27.
    Wilder TJ. Redefining successful outcomes in the hybrid era. World J Pediatr Congenit.Google Scholar
  28. 28.
    Naguib AN, Winch P, Schwartz L, Isaacs J, Rodeman R, Cheatham JP, et al. Anesthetic management of the hybrid stage 1 procedure for hypoplastic left heart syndrome (HLHS). Paediatr Anaesth. 2010;20(1):38–46. Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Anesthesiology and Perioperative MedicineUniversity of Rochester Medical CenterRochesterUSA

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