左冠状动脉异常起源于肺动脉的外科治疗

Surgical repair of anomalous origin of the left coronary artery from the pulmonary artery

目的 总结左冠状动脉起源于肺动脉的外科治疗经验.方法 分析36例左冠状动脉异常起源于肺动脉患儿的临床资料,男17例,女19例;中位年龄9.5个月,其中伴重度二尖瓣反流12例,左心室室壁瘤10例,卵圆孔未闭2例,动脉导管未闭1例.33例行手术治疗,其中二尖瓣置换术1例,左冠状动脉结扎术3例,左冠状动脉移植术29例;同期行二尖瓣成形术7例,卵圆孔缝闭术2例,二尖瓣置换术和动脉导管切断缝闭术各1例.结果 33例手术患儿术后早期死亡2例.随访31例,失访1例,1例左冠状动脉移植术后患儿出院后3日猝死,1例左冠状动脉结扎术后2年行冠状动脉旁路移植术,余28例随访1～73个月,心功能良好.3例未手术患儿中1例失访,1例出院后2个月猝死,1例随访5个月心功能良好.结论 超声心动图结合多排螺旋CT检查是诊断左冠状动脉异常起源于肺动脉的有效方法.外科治疗效果良好,首选左冠状动脉移植恢复两个冠状动脉系统供血,术后左心室功能可明显改善.合并重度二尖瓣关闭不全者同期行二尖瓣成形可能有助于早期改善心功能,但远期效果可能不佳;合并左心室室壁瘤远期能改善,不建议同期处理.

Objective To review the experience of anomalous origin of the left coronary artery from the pulmonary artery.Methods 36 patients were admitted,17 males and 19 females,the median age was 9.5 months.12 were associated with severe mitral regurgitation,10 with left ventricular aneurysm,1 with patent ductus arteriosus,2 with patent foramen ovale.33 pa tients underwent surgical repair,of which 3 underwent left coronary artery （LCA） ligation,1 underwent mitral valve replacement,29 underwent reestablishment of a dual coronary system with direct aortic reimplantation of the left coronary artery into the aorta,incidental mitral valvoplasty in 7 patients,mitral valve replacement in 1,patent ductus arteriosus ligation in 1 and patent foramen ovale closure in 2.Results Two patients died in the hospital,one postoperative patient lost follow-up,one underwent LCA reimplantation suddenly died 3 days after discharged,one received coronary artery bypass graft 2 years after LCA ligation,the remainder were asymptomatic during a follow-up of 1 to 73 months.one of the patients who did not underwent surgical repair lost follow-up,when one suddenly died 2 months after discharged,the remained one was asymptomatic during a follow-up of 5 months.Conclusion Echocardiography combined with spiral CT angiography is a good method for ALCAPA diagnosis.Good surgical outcome of ALCAPA can be achieved by reestablishing a dual coronary system with direct aortic reimplantation of the left coronary artery into the aorta,and left ventricular dysfunction could be significantly improved.Incidental mitral valvoplasty may be helpful in early rehabilitation but poor in long-term prognosis.Left ventricular aneurysm could be improved so a incidental resection of left ventricular aneurysm was not suggested.