目的 总结分析23例复杂先心病合并畸形冠状动脉横跨右心室流出道重建右室流出道的方法.方法 回顾性分析1999年10月～2011年10月期间合并冠状动脉畸形的法洛四联症(18例)和右室双出口(5例)患者的临床资料,其中6例右冠状动脉起源于左冠状动脉或前降支,13例左前降支起源于右冠状动脉,4例有异常粗大的圆锥动脉,均横跨右室流出道(近肺动脉瓣环处).11例采用畸形冠状动脉上下右心室流出道双切口,5例采用畸形冠状动脉下右心室流出道切口,3例采用心外管道,4例采用肺动脉干与右心室直接吻合术.结果 手术死亡3例,死亡率为13%,无远期死亡,手术死亡原因主要为低心排综合征.随访1～30个月,患者右室-肺动脉压差为(27.2±15.3) mm Hg(1 mm Hg=0.133 kPa).结论 应仔细探查畸形冠状动脉的走行;根据右心室流出道狭窄程度和畸形冠状动脉走行特点选择手术方式.%Objective To analyze 23 cases of right ventricular outflow tract reconstruction method in patients with complex congenital heart disease and anomalous coronary artery(ACA) crossing the right ventricular outflow tract (RVOT). Methods The clinical data of 18 cases of TOF and 5 cases of DORV with ACA crossing the RVOT in our hospital from October 1999 to October 2011 were analyzed retrospectively, including 6 cases of the right coronary artery originating from the left coronary artery or left anterior descending coronary artery, 13 cases of left anterior descending artery originated from right coronary artery, 4 cases of abnormal thick tapered artery, all across the right ventricular outflow tract (near the pulmonary valve annulus). A double ventriculotomy superior and inferior to ACA were used in 11 cases, one ventriculotomy inferior to ACA were used in 5 cases, extra cardiac conduits were used in 3 cases, trunk of pulmonary were anastomosis with RVOT in 4 cases. Results There were 3 cases deaths, the mortality was 13%, no late deaths, mainly due to surgery died from low cardiac output syndrome. Follow-up was extended from 1 to 30 months, their right ventricle-pulmonary artery gradient were (27.2±15.3) mm Hg(l mm Hg=0.133 kPa). Conclusion The surgeon should be careful in inspection of distribution of ACA during operation; surgical procedure should be selected according to the extent of RVOT obstruction and distribution of the ACA.
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