摘要
目的研究右冠状动脉异常起源于肺动脉(anomalous origin of right coronary artery from the pulmonary artery,ARCAPA)的临床特点,了解不同手术方式的预后情况。方法回顾性分析9例确诊为ARCAPA患儿(患者)的临床资料,包括临床表现、辅助检查、手术方式及随访情况。结果共计8例患儿(儿童),1例患者(成人);男7例,女2例;患儿中位月龄67.5个月,成人患者年龄69岁。患儿中4例因活动后胸闷气促就诊,3例因体检发现心脏杂音就诊,1例因脑梗死就诊完善心脏检查诊断,成人患者因气促胸闷行冠状动脉造影检查后诊断。心电图(ECG)检查示3例患儿无明显心肌缺血表现,1例部分下壁导联深Q波,4例(均>6岁)有不同程度右胸导联ST段抬高表现,成人患者提示完全性右束支传导阻滞(CRBBB)、右胸导联T波双向;经胸超声心动图(TTE)检查显示成人患者术前左心室舒张末期内径(LVEDD)增大明显伴左心室射血分数(LVEF)下降,房室瓣重度反流;有2例患儿术前左心室舒张末期内径增大,左心室射血分数均正常。所有患儿(患者)均完成手术治疗,成人患者行右冠状动脉(RCA)异位开口修补+冠状动脉旁路移植术,5例患儿行右冠状动脉直接移植至主动脉(AO)根部手术,1例患儿右冠状动脉延长后移植至主动脉根部,2例患儿行右冠状动脉结扎术。成人患者术后使用多巴胺5 d,米力农3 d强心治疗,3 d后转出重症监护室,术后复查经胸超声心动图示房室瓣反流好转,目前随访3年,左心室射血分数正常。患儿术后撤机时间为0.5~1 d,使用强心药多巴胺1~2 d,1~2 d后转出重症监护室。目前中位随访时间为36个月,所有术后患者均心功能正常,无需要再次手术患者,无死亡患者。结论ARCAPA患者早期无明显心功能不全症状,临床表现不典型,超声心动图可能漏诊或误诊,及时手术治疗预后效果好。
Objectives To explore the clinical manifestation,treatment and prognosis of anomalous origin of right coronary artery(RCA)from the pulmonary artery(ARCAPA).Methods The clinical data of 9 patients with ARCAPA were retrospectively analyzed which included symptoms,auxiliary examinations before surgery and pre-and post-operative transthoracic echocardiography(TTE)/electrocardiogram(ECG)results,type of operation,post-operative clinical condition.Results The study included 9 patients(7 male,2 female),1 adult and 8 children.The median age of pediatric patients was 67.5 months,the adult patient aged 69.Among the children,4 cases sought the medical advice for chest tightness and shortness of breath after activity,3 cases sought the medical advice for heart murmur found in physical examination,1 case sought the medical advice for cerebral infarction and finished cardiac examination.The adult patient was diagnosed ARCAPA with coronary angiography due to dyspnea and chest distress.ECG examination showed no obvious myocardial ischemia in 3 children,deep Q wave in partial lower wall leads in 1 child,ST segment elevation in right chest leads in 4 children(all>6 years),complete right bundle branch block(CRBBB)and T wave bidirectional in right chest leads in the adult patient.Preoperative left ventricular end-diastolic diameter(LVEDD)was significantly increased in the adult patient examined by TTE,accompanied by a decrease in left ventricular ejection fraction(LVEF)and severe atrioventricular valve regurgitation.LVEDD increased in 2 children patients and LVEF was normal before surgery.Surgical treatment was completed in all cases,including direct transplantation of RCA to the root of aorta(AO)in 5 children patients,extended transplantation of RCA to the root of AO in 1 case,and ligation of RCA in 2 cases.Repair of ectopic opening of RCA and RCA bypass grafting were performed in the adult patient.The adult patient was treated with dopamine for 5 days and milrinone for 3 days after surgery,and was transferred out of the intensive c
作者
章旭
郭颖
徐欣怡
吴怡蓓
高伟
刘建实
黄美容
李奋
付立军
刘廷亮
ZHANG Xu;GUO Ying;XU Xinyi;WU Yibei;GAO Wei;LIU Jianshi;HUANG Meirong;LI Fen;FU Lijun;LIU Tingliang(Department of Cardiology,Shanghai Children′s Medical Center Affiliated to Shanghai Jiaotong University School of Medicine,Shanghai 200127,China;Department of Cardiac Surgery,Shanghai Deda Hospital,Shanghai 200336,China)
出处
《岭南心血管病杂志》
CAS
2023年第1期50-54,共5页
South China Journal of Cardiovascular Diseases
关键词
右冠状动脉异常起源于肺动脉
先天性心脏病
手术
随访
anomalous origin of right coronary artery from the pulmonary artery
congenital heart disease
surgery
follow-up
