This common cyanotic lesion may escape detection in the nursery, so it is important for emergency medicine physicians to recognize this lesion, as well as to recognize and treat hypercyanotic spells as discussed earlier. The degree of cyanosis is directly proportional to the severity of the pulmonary stenosis. In fact, cyanosis may be subtle or absent at rest and clinically obvious only when infants are active or crying.
Dynamic obstruction below the pulmonary valve can lead to an acute increase in the right-to-left shunt, producing a hypercyanotic spell or syncope with cyanosis. Prolonged or recurrent syncope due to tetralogy of Fallot can be a life-threatening emergency, so referral after initial stabilization is indicated for further diagnostic evaluation and possible urgent surgical intervention.
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