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Chapter 10 - Tetralogy of Fallot, Pulmonary Atresia, and Aortopulmonary Collaterals

from Section 2 - Right-Sided Obstructive Lesions

Published online by Cambridge University Press:  09 September 2021

By
Mikel Gorbea  and
Lisa Wise-Faberowski
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

Tetralogy of Fallot (TOF) with pulmonary atresia and multiple aortopulmonary collaterals represents an extreme variation of tetralogy physiology. While TOF with pulmonary atresia includes an aorta that "overrides" an unrestrictive ventricular septal defect and right ventricular hypertrophy, instead of pulmonary stenosis there is complete atresia of the pulmonary valve and right ventricular outflow tract.Multiple aortopulmonary collaterals are vessels that arise from the descending thoracic aorta or any of its branches (subclavian, bronchial, celiac, or intercostal), often anastomosing proximal to branch pulmonary arteries. Pulmonary blood flow via these vessels is variable and nonuniform. This chapter discusses the anesthetic considerations and complexities involved in caring for a child with palliated TOF with pulmonary atresia and aortopulmonary collaterals for noncardiac surgery.

Keywords

Alagilles syndromechromosome 22q11 microdeletionDiGeorge syndromemultiple aorto-pulmonary collateralsnoncardiac surgerypulmonary atresia with ventricular septal defecttetralogy of Fallotunifocalization
Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
 , pp. 57 - 63
Publisher: Cambridge University Press
Print publication year: 2021

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References

Suggested Reading

Ho, S. Y., Catani, G., and Seo, J. W. Arterial supply to the lungs in tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis. Cardiol Young 1992; 2: 65–72.Google Scholar
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Jefferson, K., Rees, S., and Somerville, J. Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 1972; 34: 418.CrossRefGoogle Scholar
Mainwaring, R. D., Reddy, V. M., Peng, L., et al. Hemodynamic assessment after complete repair of pulmonary atresia with major aortopulmonary collaterals. Ann Thorac Surg 2013; 95: 1397–402.Google ScholarPubMed
Quinonez, Z. A., Downey, L., Abbasi, R. K., et al. Anesthetic management during surgery for tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. World J Pediatr Congenit Heart Surg 2018; 9: 236–41.Google Scholar
Rossi, R. N., Hislop, A., Anderson, B. H., et al. Systemic-to-pulmonary blood supply in tetralogy of Fallot with pulmonary atresia. Cardiol Young 2002; 12: 373–88.CrossRefGoogle ScholarPubMed
Webb, G., Mulder, B. J., Aboulhosn, J., et al. The care of adults with congenital heart disease across the globe: current assessment and future perspective: a position statement from the International Society for Adult Congenital Heart Disease (ISACHD). Int J Cardiol 2015; 195: 326–33.CrossRefGoogle ScholarPubMed

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