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Medical Management Medical Therapy Surgery is the definitive treatment for the cyanotic patient with tetralogy of Fallot

(TOF). The primary role of medical therapy is in preparation for surgery. Most infants have adequate saturations and usually undergo elective repair. In infants with acute cyanotic episodes, placing them in a knee-chest position may prove helpful in addition to administering oxygen and intravenous morphine. Asymptomatic infants need no special medical treatment. Surgical Therapy Factors that increase the risk for early repair of TOF include the following:
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Low birth weight Pulmonary artery atresia Major associated anomalies Multiple previous surgeries Absent pulmonary valve syndrome Young age Old age Severe annular hypoplasia Small pulmonary arteries High peak right ventricular to left ventricular pressure ratio Multiple VSDs Coexisting cardiac anomalies

Palliative procedures

The goals of palliation are to increase pulmonary blood flow independent of ductal patency and to allow pulmonary artery growth and even total correction. Occasionally, an infant with pulmonary atresia or an anomalous LAD coronary artery that crosses the right ventricular outflow tract may not be a surgical candidate for establishing transannular right ventricle to pulmonary artery continuity and may require placement of a conduit. Although artificial conduits can be used, infants with extremely small pulmonary arteries may not tolerate total correction in infancy. These infants may require palliation instead of corrective surgery. Various types of palliative procedures have been developed, but the current procedure of choice is the Blalock-Taussig shunt. The Potts shunts has been abandoned because of a tendency toward increased pulmonary blood flow and increasing difficulty with takedown at the time of corrective surgery. The Waterston shunt is sometimes used, but it also increases pulmonary artery

blood flow. This shunt is more related to pulmonary artery stenosis, which generally requires reconstruction. The Glenn shunt is no longer used because of difficulty in performing a subsequent definitive repair. Given the problems associated with the aforementioned shunts, placement of the modified Blalock-Taussig shunt (using a Gore-Tex graft between the subclavian artery and pulmonary artery) is the procedure of choice. Advantages of the modified BlalockTaussig shunt include (1) preservation of the subclavian artery, (2) suitability for use on either side, (3) good relief of cyanosis, (4) easier control and closure at time of primary repair, (5) excellent patency rate, and (6) decreased incidence of iatrogenic pulmonary/systemic artery trauma. Corrective surgery

Primary correction is the ideal operation for treatment of TOF and is usually performed under CPB. The aims of the surgery are to close the VSD, to resect the area of infundibular stenosis, and to relieve the RVOTO. Before CPB is initiated, previously placed systemic-to-pulmonary artery shunts are isolated and taken down. Patients then undergo CPB. Associated anomalies, such as ASD or patent foramen ovale, are closed.