Tetralogy of Fallot with absent pulmonary valve syndrome

Tetralogy of Fallot with absent pulmonary valve syndrome,10.1053/j.pcsu.2004.02.010,Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac

Tetralogy of Fallot with absent pulmonary valve syndrome   (Citations: 9)
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Tetralogy of Fallot with absent pulmonary valve syndrome is a rare variant of tetralogy of Fallot marked by severe pulmonary insufficiency in utero and significant, sometimes massive, dilation of the pulmonary arteries. This syndrome can be associated with airway abnormalities that can cause respiratory compromise. Patients who present with respiratory symptoms early in life continue to present a major clinical challenge associated with relatively high mortality rates, typically in the 20% to 30% range. A variety of surgical techniques have been developed to address absent pulmonary valve syndrome that may improve long-term outcomes.
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    • ...Infants might present with mild respiratory symptoms or with significant respiratory compromise requiring early surgical repair [1]...

    Paul Chenet al. Newborn with tetralogy of Fallot and absent pulmonary valve

    • ...Other options for the management of the dilated pulmonary arteries include surgical resection and replacement with a pulmonary homograft or the anterior translocation of the pulmonary arteries (Lecompte’s maneuver), moving them away from the bronchi [2, 3, 4, 5]. The foramen ovale is left open to allow for a right-to-left shunt to preserve the cardiac output during the early postoperative period...

    Constantinos Chrysostomouet al. Tetralogy of Fallot with Absent Pulmonary Valve

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