Zhang G C, Wang Z W, Zhang R F, Zhu H Y, Yi D H
Institute of Cardiovascular Surgery, Department of Cardiovascular Surgery, North General Hospital of China, Shenyang, People's Republic of China.
Ann Thorac Surg. 1997 Oct;64(4):1150-3. doi: 10.1016/s0003-4975(97)00822-9.
BACKGROUND; Patients with tetralogy of Fallot and unilateral absence of pulmonary artery are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpose of this report is to review a 29-year experience in the treatment of those patients.
Between May 1966 and February 1995, 2,511 patients underwent correction of tetralogy of Fallot in our department, 24 of those patients with unilateral absence of pulmonary artery (20 had absence of the left pulmonary artery, 4 had absence of the right pulmonary artery). Valved conduits were used in 9 patients, right ventricular patches were used in 4 patients, and transannular patches with a monocusp that was made of the patient's pericardium were used in 11 patients.
There were two operative deaths; both were in patients with hypoplasia of the left ventricle. All survivors had good early and late results.
A right ventricular patch should be used in patients with tetralogy of Fallot and infundibular stenosis; a transannular patch with a monocusp should be used in patients with tetralogy of Fallot and stenosis of the left or right pulmonary artery's origin as well as the pulmonary trunk. A homograft valved conduit is suitable for patients with anomalous coronary artery or pulmonary atresia.
背景；法洛四联症合并单侧肺动脉缺如的患者属于高危群体，对于此类患者的正确治疗方法尚无共识。本报告旨在回顾对这些患者长达29年的治疗经验。
1966年5月至1995年2月期间，我科共有2511例患者接受了法洛四联症矫治手术，其中24例合并单侧肺动脉缺如（20例为左肺动脉缺如，4例为右肺动脉缺如）。9例患者使用了带瓣管道，4例患者使用了右心室补片，11例患者使用了由患者心包制成的单瓣跨环补片。
有2例手术死亡；均为左心室发育不全患者。所有存活患者的早期和晚期结果均良好。
法洛四联症合并漏斗部狭窄的患者应使用右心室补片；法洛四联症合并左或右肺动脉起始部及肺动脉干狭窄的患者应使用带单瓣的跨环补片。同种带瓣管道适用于合并冠状动脉异常或肺动脉闭锁的患者。
