法乐四联症伴肺动脉闭锁的外科治疗(附14例报告)

报告外科治疗法乐四联症伴肺动脉闭锁１４例。肺动脉瓣水平膜状闭锁１３例，主肺动脉干闭锁１例。肺动脉发育好，肺血由一粗大的动脉导管供给者９例；肺动脉发育不良、无动脉导管，肺血由体肺侧支供给者１例；肺动脉发育不良，由细的动脉导管和体肺侧支共同供给者４例。姑息性手术５例，一期根治９例，二期根治２例；死亡３例。作者对法乐四联症伴肺动脉闭锁的手术适应证、姑息性手术方法的应用及体肺侧支的外科处理原则进行了探讨。

Surgical Treatment of Tetralogy of Fallot with Pulmonary Atresia

Between 1992 and 1995,14 patients with tetralogy of Fallot with pulmonary atresia (TOF PA) underwent surgical treatment.The atresia was at the valvular level in 13 patients and at the main trunk in 1.The pulmonary arteries were well developed,with the pulmonary blood flow supplied principally by a large patent ductus arteriosus (PDA) in 9 patients;pulmonary arteries were hypoplastic and diminutive without patent ductus arteriosus,with pulmonary blood flow supplied exclusively by aortopulmonary collaterals (APC) in 1 patient.And the blood flow in the hypoplastic pulmonary arteries was supplied by a small patent ductus arteriosus and aorto pulmonary collaterals in 4 cases.Corrective procedures were done in 9 cases and palliative in 5 including transannular pericardial patching in 2,right ventricle to pulmonary artery conduit in 2 and right Blalock Taussig (BT) shunt in 1.Two of the later 5 patients received second stage repair.There were 3 postoperative deaths.The authors emphasize that the pulmonary artery index (PAI) and presence or absence of PDA are two important factors for choosing the type of surgery to be performed.The principles in management of aortopulmonary collaterals and choica of staged operation are discussed.