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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.
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