ABSTRACT
Tetralogy of Fallot(TOF) with absent pulmonary valve syndrome is a rare pathologic condition which is characterized by absence of pulmonary valve, dilatation of the pal-monary artery involving its left and right branches in addition to the basic pathoanatomies of TOF. In some cases,the enlarged left pulmonary artery may cause compression of the left bronchus. Its complexity deseves some special attention during corrective surgary. Betwleen 1985 and 1998, six patients with this syndrome underwent surgical correction under CPB combined with moderate hypothermic in our hospi-tal. The surgical procedures consisted of resection of the muscular bands present in RVOT causing obstruction and partial excision of the anterior wall of the markedly dilated left and right pulmonary arteries in 2 cases and transannular pericardial patching in 4 cases. One patiant with absence of left pulmonary artery died on the first postoperative day. The other 5 survivors all recovered. Regarding the proper time for surgical treatment on this particular group of patients, it is advisable that in sick children with no respiratory symptoms, corrective surgery be deiyed to the age of 3 or 4 years old. However, when the sick children developed severe respiratory symptoms, corrective surgery should be done as early as possible. In this special situation, a valved transannular patch is preferred.