左冠状动脉异常起源于肺动脉的临床特征及治疗策略

Anomalous origin of the left coronary artery from the pulmonary artery in infants：clinical features and the perioperative treatment strategies

目的 探讨左冠状动脉起源于肺动脉（ALCAPA）患儿的临床特征及治疗策略.方法 对2006年至2013年广东省心血管病研究所收治的25例诊断为ALCAPA并行手术治疗的＜1岁患儿的临床资料进行分析,术后随访超声心动图评估心功能.结果 患儿临床特征以气促、多汗、喂养困难、生长发育落后多见.心电图示异常Q波23例,ST-T改变16例.超声心动图检查示左心室扩大25例,心内膜增厚5例,右冠动脉扩张并存在左右冠状动脉交通血流11例.左心室射血分数（LVEF） （45.5±l3.9）％（25％～77％）,左心室缩短率（LVFS） （22.0±7.3）％（12％ ～ 38％）.21例患儿行高速多层心脏CT检查,提示左冠状动脉开口于肺动脉瓣左后侧窦9例,有后侧窦1例,开口于肺动脉主干二下段5例,开口于肺动脉近分叉处l例,开口位置不确定5例.本组25例患儿中17例在外院首诊后转入我院,除l例在外院行心血管导管检查确诊ALCAPA外,其余16例均被误诊;8例我院首诊的病例中,误诊2例,后经超声心动图复查及心脏CT检查后修正诊断.术前根据患儿的心功能情况予以不同程度的强心、利尿、扩血管治疗.2例结扎左冠状动脉口,23例采用左冠状动脉移植技术重建双冠状动脉系统.对于心功能Ⅳ级合并中度以上二尖瓣反流或心功能Ⅲ级合并重度以上二尖瓣反流者同期行二尖瓣成形术.对术后LVEF＜ 30％者,如果应用药物无法维持血压,乳酸呈进行性升高,则施行体外膜肺氧合（ECMO）辅助.2例患儿行左冠状动脉移植术后因顽固性室颤早期死亡;存活的23例患儿,呼吸机使用时间为7 ～500 h,其中11例＜60 h.平均住院天数（23.4±13.9）d（8～65 d）.中位随访时间为28.5个月（1～91个月）,失访1例,因感染性休克术后3个月院外死亡.随访的22例患儿心功能均较术前改善,14例左心室大小恢复至正常,20例LVEF恢复至正常.无行2次手术者.结论 结合病史、心电图、超声心动图及其他多种影像学诊断方法有利于正确诊断ALCAPA;对于危重患儿采取个体化治疗策略,并对术后低心排患儿积极进行ECMO辅助,可显著提高手术成功率.

Objective To investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA）.Method Data of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed.The patients＇ cardiac function was evaluated with echocardiography during follow-up.Result Most patients presented with symptoms of heart failure,such as tachypnea,diaphoresis,poor feeding,failure to thrive etc.Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients,endocardial hyperplasia in 5 patients,dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients.The left ventricular ejection fraction （LVEF） was （45.5 ± 13.9） % （25%-77%）.The left ventricular fractional shortening （LVFS） was （22.0 ± 7.3）% （12%-38%）.Twenty one patients underwent cardiovascular CT scan.Left coronary artery originated from left posterior sinus in 9 patients,from right posterior sinus in 1 patient,from lower main pulmonary artery in 5 patients,from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital,only 1 case who underwent angiography was diagnosed correctly.Two patients were misdiagnosed in 8 patients first-presented in our hospital.Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan.The preoperative therapies included using inotropic agents,diuretics and vasodilators according to cardiac function.Two patients underwent left coronary artery orifice ligation.Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system.Patients of NYHA Ⅳ with moderate mitral regurgitation （MR） and NYHA Ⅲ with severe MR underwent mitral annuloplasty.If LVEF was less than 30% after weaning from cardiopulmonary bypass,blood pressure could not be maintained with medication,or lactates increased progTessively,extracorporeal membrane oxygenation （ECMO） was demanded.Two patients showed low cardiac output syndrome immediately after surgical procedures,died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries,duration of ventilation was 7-500 hours,11 of them were supported with ventilator for less than 60 hours.The mean length of hospital stay was（23.4 ± 13.9） d （8-65 d）.The follow-up duration ranged from 1-91 months （median 28.5 months）.One case was lost to follow up.The patient died from infection 3 months after discharge.The cardiac functions of the remaining 22 patients were improved.The size of left ventricle of 14 patients recovered to normal.LVEF increased to the normal level in 20 cases.No patient underwent redo procedure.Conclusion The accurate diagnosis can be made based on history,electrocardiogram,echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants.Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.