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Chapter 26 - Stage I Palliation, Hypoplastic Left Heart Syndrome

from Section 5 - Single-Ventricle Physiology

Published online by Cambridge University Press:  09 September 2021

By
Bishr Haydar
Edited by
Laura K. Berenstain  and
James P. Spaeth
Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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Summary

Noncardiac surgery in patients with hypoplastic left heart syndrome (HLHS) and other variants of single-ventricle physiology presents unique risks, especially prior to and after the first stage of surgical palliation. Although there are a number of cardiac defects that may be treated with single-ventricle palliation, there are principles that may be generalized for the perioperative care of these patients. Preoperative assessment of their status and knowledge of their pathophysiology may aid the anesthesiologist in mitigating these risks. Laparoscopy remains controversial in this population, as patients with single-ventricle physiology may be ill equipped to tolerate the physiologic derangements that laparoscopy induces. This chapter presents an overview of single-ventricle physiology including bedside clinical assessment, as well as the expected physiologic changes associated with laparoscopy. It then reviews the published outcomes of laparoscopic versus open Nissen fundoplication. Finally, it reviews specific and devastating perioperative complications that may occur in patients with stage I palliation.

Keywords

adverse eventscongenital heart defectcongenital heart diseasehypoplastic left heart syndromelaparoscopic fundoplicationmodified Blalock–Taussig shuntNissen operationnoncardiac surgeryNorwood procedure stage 1Sano shuntsingle-ventricle physiologythrive failure to
Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
 , pp. 187 - 197
Publisher: Cambridge University Press
Print publication year: 2021

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References

References

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Suggested Reading

Chu, D. I., Tan, J. M., Mattei, P., et al. Outcomes of laparoscopic and open surgery in children with and without congenital heart disease. J Pediatr Surg 2018; 53: 1980–8.CrossRefGoogle ScholarPubMed
Nicolson, S. C., Steven, J. M., Diaz, L. K., et al. Anesthesia for the patient with a single ventricle. In Andropoulos, D. B., Stayer, S. A., Mossad, E. B., et al., eds. Anesthesia for Congenital Heart Disease, 3rd ed. Hoboken, NJ: John Wiley & Sons, 2015; 356–72.Google Scholar
Quintessenza, J., DeSena, H. C., Justice, L., et al. Hypoplastic left heart syndrome. In Ungerleider, R. M., Meliones, J. N., Nelson McMillan, K. et al., eds. Critical Heart Disease in Infants and Children, 3rd ed. Philadelphia: Mosby Elsevier, 2019; 778–95.Google Scholar
Short, J. A., Paris, S. T., Booker, P. D., et al. Arterial to end-tidal carbon dioxide tension difference in children with congenital heart disease. Br J Anaesth 2001; 86: 349–53.CrossRefGoogle ScholarPubMed
Watkins, S., Morrow, S. E., McNew, B. S., et al. Perioperative management of infants undergoing fundoplication and gastrostomy after stage I palliation of hypoplastic left heart syndrome. Pediatr Cardiol 2012; 33: 697–704.CrossRefGoogle Scholar

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