[Anesthetic management of tetralogy of Fallot with absent pulmonary valve--a case report].

Tetralogy of Fallot is characterized by a VSD, overriding of the aorta, right ventricular hypertrophy, and pulmonary stenosis. Absence of the pulmonary valve occurs in 2.6-6% of patients with tetralogy of Fallot. Operative procedures to relieve respiratory symptoms have been described with an mortality of 35-100%. Respiratory compromise may be severe in these infants secondary to bronchial compression by the aneurysmally dilated pulmonary arteries and present a different hemodynamic profile than those with classic tetralogy of Fallot. An understanding of the anatomic and physiologic principles and their anesthetic implications will lead to improved management of these extremely ill infants. We reported the anesthetic considerations and management of a case of tetralogy of Fallot with absent pulmonary valve of an infant who experienced severe respiratory distress on the supine position.