冠状动脉畸形对复杂先心病右室流出道重建的影响

目的探讨冠状动脉畸形对复杂先心病右室流出道（RVOT）重建的影响。方法自1989年4月至2004年5月治疗18例合并冠状动脉畸形的法洛四联症（14例）和右室双出口（4例）患者。右冠状动脉开口缺如7例,左冠状动脉开口缺如4例,左前降支起自右冠状动脉6例,左右冠状动脉交通支形成蔓状血管丛1例。均在全身麻醉低温体外循环下手术,1例行姑息手术,17例行根治术,RVOT重建的方法：游离畸形冠状动脉从其下方做切口,RVOT心包补片扩大术4例;3例在异常交通支下方平行切口修补室间隔缺损（VSD）,交通支上方纵切口加宽RVOT和肺动脉瓣环;4例行右心房切口修补VSD,RVOT小切口和肺动脉切口心包补片扩大术;3例肺动脉主干与RVOT直接吻合;3例同种带瓣主动脉重建RVOT。结果术后早期死于顽固性低心排血量综合征1例,无严重出血并发症和呼吸道并发症,姑息手术1例,血氧饱和度由术前的68．0％升至82.0％。16例随访10个月～8年,1例术后1年不明原因猝死;超声心动图示轻度残余漏3例,残余梗阻3例。结论复杂先天性心病并冠状动脉畸形RVOT重建时,应根据冠状动脉畸形特点选择手术方式。

The effects of coronary abnormalities on reconstruction of right ventricular outflow tract in patients with complex congenital heart disease

Objective To explore the effects of coronary abnormalities on reconstruction of right ventricular outflow tract in patients with complex congenital heart disease.Methods Eighty patients,including tetralogy of Fallot 14 cases and double outlet right ventricle(DORV) 4 cases with anomalous coronary artery,underwent surgical treatment from April 1989 to May 2004.The coronary diseases included single left or right coronary artery in 11 cases, left anterior decending coronary artery originating from right coronary artery in 6 cases, vascular plexus on right outflow tract in 1 case.One-stage correction was undergone in 17 cases, palliative procedure in 1 case. The reconstruction methods were as follows: mobilizing coronary artery and expanding incision under coronary artery with pericardial patches in 4 cases; repairing ventricular septal defect(VSD)via the incision beneath the coronary artery and expanding right ventricular outlet tract (RVOT) and pulmonary via longitudinal incision over the coronary artery in 3 cases. VSD were repaired via right atrium and minimal incision on RVOT plus incision on pulmonary were made to expand RVOT. Trunk of pulmonary were anostomisis with RVOT in 3 cases. Homograft valved aorta were used in 3 cases.Results One case died of serious low cardiac output syndrom postoperatively. There was no critical complication of hemmoraghe and respiratory tract.Oxygen saturation rose from 68.0%to 82.0% after treated by palliative procedure. Seventeen cases were followed from 10 months to 8 years,1 case suddenly died with no clear cause.Three cases were with residual leak, 3 with residual obstruction.Conclusions Surgical procedure should be selected according to the charicateristics of coronary disease with complex congenital heart disease.