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Chapter 25 - Truncus Arteriosus

from Section 4 - Complex Mixing Lesions

Published online by Cambridge University Press:  09 September 2021

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

Truncus arteriosus is a rare form of cyanotic congenital heart disease in which the embryologic separation of the arterial trunk remains incomplete. The primary anatomic lesion is a common arterial trunk that supplies the pulmonary, coronary, and systemic circulations. Patients become symptomatic early in infancy because pulmonary vascular resistance falls over the first days to weeks of life, resulting in pulmonary overcirculation. Ideally, truncus arteriosus repair occurs within the first weeks of life. The most common extracardiac congenital malformations associated with truncus arteriosus are those within the heterogeneous spectrum of 22q11 deletion syndrome. Features of 22q11DS can pose multiple challenges to anesthesiologists. Dysmorphic facies, including micrognathia, retrognathia, palatal abnormalities, and laryngotracheomalacia, can impede airway management. This chapter describes the care of a child with repaired truncus arteriosus and 22q11 deletion syndrome for palatal surgery and the perioperative management considerations.

Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
, pp. 178 - 186
Publisher: Cambridge University Press
Print publication year: 2021

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References

References

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Suggested Reading

Buckley, J. R., Amula, V., Sassalos, P., et al. Multicenter analysis of early childhood outcomes after repair of truncus arteriosus. Ann Thorac Surg 2019; 107: 553–59.CrossRefGoogle ScholarPubMed
O’Byrne, M. L., Mercer-Rosa, L., Zhao, H., et al. Morbidity in children and adolescents after surgical correction of truncus arteriosus communis. Am Heart J 2013; 166: 512–18.Google ScholarPubMed
Parikh, R., Eisses, M., Latham, G. J., et al. Perioperative and anesthetic considerations in truncus arteriosus. Semin Cardiothorac Vasc Anesth 2018; 22: 285–93.CrossRefGoogle ScholarPubMed
Yotsui-Tsuchimochi, H., Higa, K., Matsunaga, M., et al. Anesthetic management of a child with chromosome 22q11 deletion syndrome. Pediatr Anesth 2006; 16: 454–7.CrossRefGoogle ScholarPubMed

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