Patients with tetralogy of Fallot with absent pulmonary valve syndrome have dilated pulmonary arteries which can cause airway obstruction or produce a capacitance chamber that hinders antegrade pulmonary blood flow. Previous repair techniques incompletely remove abnormal tissue and can distort the pulmonary arteries. Our technique both resects all of the dilated, abnormal pulmonary arterial tissue and creates a near normal pulmonary outflow tract.

The ventricular septal defect is closed via a right ventriculotomy. Abnormal pulmonary artery tissue is removed by resecting the main and branch pulmonary arteries out to the lobar branches. The posterior walls of the remaining pulmonary artery cuffs are then anastomosed posteriorly. RV-PA continuity is restored with a pulmonary homograft.

With this technique, virtually all abnormal pulmonary artery tissue is resected. In addition the patient is left with a functional homograft valve creating a near normal pulmonary outflow tract.

This technique is particularly suitable for neonates since, after removal of abnormal tissue, the branch pulmonary arteries can be approximated due to the short distance between them. The technique is reproducible, simple and creates the most normal pulmonary outflow tract possible in this difficult group of patients.