Successful staged repair of a symptomatic neonate of tetralogy of Fallot with severe absent pulmonary valve syndrome

Tetralogy of Fallot with absent pulmonary valve has been classified to a few groups. The most severe one is characterized by symptomatic onset immediately after birth. The others are no or slightly symptomatic at least during their neonatal period. In a severely symptomatic 12-day-old neonate of tetralogy of Fallot with absent pulmonary valve who had required intubation immediately after birth, tight pulmonary banding and left side modified Blalock-Taussig shunt were performed on emergency basis. Consequently, prior massive pulmonary regurgitation was decreased significantly. Forty-five days after this first stage operation, he weaned from respiratory management. At 1-year-old, radical repair based on conotruncal repair, which consisted of patch closure of ventricular septal defect preserving the tricuspid septal leaflet function, resection of anterior wall of enlarged left pulmonary artery, and right ventricular outflow tract reconstruction using autologous tissue and a pericardial patch was performed. Bicuspid pulmonary valve, posterior one of procured autologous pulmonary wall and anterior one of polytetrafluoroethylene (PTFE) respectively, was created to minimize deterioration of the pulmonary insufficiency. Although postoperative cardiac function was kept feasible showing his central venous pressure of 7 mmHg in the main, postoperative general course was eventful especially regarding the respiratory function. The patient was weaned from the prolonged ventilator management 5 months after this radical repair eventually. Generally, to diminish the massive pulmonary regurgitation in early lifetime period could reduce a progressive airway obstruction and minimize pulmonary tissue damage. However, even after the total correction in this case, considerable peripheral segmental pulmonary obstructive lesions were persistent according to the perfusion lung scanning with 99mTc macroaggregated albumin and 99mTechnegas ventilation lung scanning studies. This persistent, supposed to be innate, pulmonary obstructive lesions might prevent ordinal recovery after cardiac radical repair for this most severe subtype of absent pulmonary valve syndrome.