Tet spells

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Children with Tetralogy of Fallot may develop acute severe cyanosis or hypoxic "tet spells". The mechanism underlying these episodes is not entirely clear, but may be due to spasm of the infundibular septum and the right ventricular outflow tract. Whatever the mechanism, there is an increase in resistance to blood flow to the lungs with increased preferential flow of desaturated blood to the systemic circulation. The child will often squat during a Tet Spell to improve venous return to the right side of the heart. Squating increases the systemic vascular resistance and thereby shunts flow to pulmonary circuit. These spells can be fatal, and can occur in patients who are not cyanotic.