Tetralogy of Fallot with acquired pulmonary atresia and hypoplasia of pulmonary arteries. Report of surgical management in infancy.

A perforated intracardiac prosthesis, and patch infundibuloplasty and annuloplasty were employed for the surgical management of an infant with tetralogy of Fallot, pulmonary atresia, and severe hypoplasia of the pulmonary arteries. This approach provides a means of decompression of the right ventricle in the immediate postoperative period. It allows sufficient systemic cardiac output while increasing pulmonary blood flow to relieve severe hypoxemia and cyanosis. In addition, the high risk of further compromise to the diminutive pulmonary arteries by a palliative shunt is avoided. A physiological basis for the use of this technique is discussed.